Case Reports

Chest. 2011;140(4_MeetingAbstracts):153A. doi:10.1378/chest.1119460

INTRODUCTION: Iatrogenic venous air embolism (VAE) occurs when atmospheric gas is introduced into the systemic venous system. We report a case of massive fatal iatrogenic venous air embolism due to mechanical injection of air with intravenous contrast during contrast-enhanced computed tomography (CECT). This case involves the inadvertent injection of air instead of contrast from an improperly loaded power injector.

CASE PRESENTATION: A 53-year-old male with no past medical history presented to the Emergency Department (ED) complaining of progressively worsening epigastric pain of three days duration. On physical examination, he had no difficulty breathing; arterial oxygen saturation (SpO2) was 98%, on room air; there was moderate diffuse abdominal tenderness to palpation. A CECT of the chest, abdomen and pelvis was ordered to rule out acute aortic dissection and abdominal aortic aneurysm. Immediately following the completion of the CECT, the patient became acutely dyspneic and tachypneic, and appeared ashen and diaphoretic. Upon returning to the ED, his vital signs were notable for blood pressure 107/70, heart rate 140, respiratory rate 28, SpO2 78% on 15 liter non-rebreather mask. His ABG at that time showed: pH 7.19, PaCO2 52, PaO2 59. A repeat ECG demonstrated a new right bundle branch block and right heart strain pattern. Shortly thereafter, the patient precipitously deteriorated; he suffered bradycardic arrest—necessitating cardiopulmonary resuscitation, intubation, and rapidly escalating vasopressor requirements. The patient continued to decompensate despite all measures, and responded poorly to cardiopulmonary resuscitation; he expired shortly thereafter—prior to the review of the CECT. Review of the chest CECT—done immediately prior to his acute demise—was notable for an abrupt interruption of contrast within the vasculature separating the right subclavian vein and the pulmonary veins. In the vasculature separating these landmarks—including the superior vena cava, right atrium and ventricle, right ventricular outflow tract, and main pulmonary arteries—there was largely an absence of contrast, and more significantly—an extensive of amount air was noted.

DISCUSSION: Venous air embolism (VAE) occurs when atmospheric gas is introduced into the venous circulation. VAEs have been associated with neurological procedures, central venous catheterization, penetrating and blunt chest trauma, high-pressure mechanical ventilation, thoracentesis, hemodialysis, and several other invasive procedures.1 More pertinent to our case, are VAEs resulting from inadvertent injection of air during CECT. A recent study showed small air emboli—measuring up to three air bubbles, less than 1 cm in diameter—and medium air emboli—measuring as more than three air bubbles or bubbles 1-2 cm in diameter—were seen in 11.7% patients undergoing CECT studies. 2 Fortunately, the incidence of VAE presenting with dyspnea, hypoxia, and respiratory failure is exceedingly rare; in fact symptoms are usually mild and non-specific. Most VAEs are introduced in small amounts and dissolve in the venous system. Cases of injection of large amounts of air from improperly loaded power injectors have been reported, but are exceedingly rare. The presence of adverse clinical outcomes largely depends on variables such as the volume of air injected, rate of delivery, and the site of injection. Ultimately, iatrogenic VAEs—such as the one described here—can be best prevented by enforcing strict adherence to proper technique and protocol.

CONCLUSIONS: Venous air embolism (VAE) is a known complication of venous access procedures such as CECT. However, it is an iatrogenic complication that can and must be prevented. This can be best achieved by formally educating radiology technicians and limiting use of contrast media injectors to only those with adequate training. Additionally, clinicians must be quick to recognize this complication, allowing for prompt institution of interventions aimed at diminishing the risk of potentially catastrophic and fatal outcomes.

Reference #1 van Hulst RA, Klein J, Lachmann B. Gas embolism: Pathophysiology and Treatment. Clin Physiol Funct Imaging. 2003;23(5):237-46

Reference #2 R, Schaffler GJ, Rienmueller R, at al. Vascular Air Embolism: Location, Frequency, and Cause on Electron-beam CT Studies of the Chest. Radiology. 1997;202:459-462

DISCLOSURE: The following authors have nothing to disclose: Roman Kleynberg, Berj Demirjian, Nader Kamangar

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Case Reports: Sunday, October 23, 2011

Chest. 2011;140(4_MeetingAbstracts):1A. doi:10.1378/chest.11-07326

INTRODUCTION: Acute hepatic failure is rarely caused by metastatic cancer. We present a case of acute hepatic failure due to infiltrating small cell carcinoma.

CASE PRESENTATION: A 55 year-old female presented with abdominal discomfort and nausea. The patient denied constitutional symptoms, shortness of breath, cough, hemoptysis, or chest pain. Her medical history was significant for Crohn's disease controlled with infliximab and azathioprine, prior small bowel obstructions, and chronic kidney disease. She had smoked daily for forty years and denied alcohol, drugs or other ingestions. She had mild jaundice and cachecia. Her abdomen was mildly distended, tympanitic, but soft and non-tender. Laboratory analysis revealed lactic acidosis, macrocytosis and elevated liver associated enzymes (AST 224, ALT 90, total bilirubin 2.5). Her coagulation parameters were normal. Portable chest x-Ray was unremarkable. Abdominal ultrasound revealed a nodular hepatic border without focal mass. Hepatic and portal venous blood flows were normal. CT-Abdomen showed no focal masses or lymphadenopathy. She subsequently deteriorated with development of hepatic encephalopathy, worsening lactic acidosis, transaminitis, and eventual respiratory failure and hepatorenal syndrome. Of note, her LDH was 8625 and her uric acid was 15. Viral and autoimmune serologies and toxin screens were negative. In anticipation of urgent referral for liver transplantation, liver biopsy was performed to confirm suspected drug induced hepatitis. Instead it revealed diffuse infiltrating small cell carcinoma. Chemotherapy was offered, however, the patients family elected to withdraw care.

DISCUSSION: Acute hepatic failure is diagnosed when severe complications of liver disease develop within four weeks of initial presentation. Most common etiologies are viral hepatitis, drug toxicity, ischemia, or vascular thrombosis. However, some studies suggest up to forty percent of cases are not identified prior to death. Malignancy causing acute hepatic failure is exceedingly rare, but must be considered due to implications on treatment and to avoid inappropriate referral for transplantation. Malignancies reported to cause acute hepatic failure include lymphoma, breast, lung, melanoma, colorectal and pancreatic carcinoma. Small cell carcinoma is more common than non-small cell lung cancers. Infiltrating small cell carcinoma is universally fatal. Onset of jaundice to death averages ten days. The longest reported time of survival with hepatic failure due to small cell carcinoma was six months with chemotherapy. Possible mechanisms include massive cytokine release, damage of bile ducts, compression of sinusoids leading to ischemia, tumor replacement of hepatocytes, and tumor emboli compromising the portal system. Severe elevations in lactate dehydrogenase and uric acid are typical. The ratio of LDH to ALT is significantly elevated when compared to non-malignant hepatic failure. Radiologic evaluation is typically nonspecific. The cerebral edema of fulminate hepatic failure has not been seen in cases of malignancy related hepatic failure. Small cell carcinoma has sub-fulminant liver involvement in 22-29% of cases at time of diagnosis. The usual appearance is macroscopic nodules with possible patchy infiltration. Currently, there are less than twenty reported cases of fulminant hepatic failure due to small cell infiltration. Interestingly, most cases do not show any suspect lesions on chest x-ray. Biopsy usually shows diffuse hepatic parenchymal replacement or sinusoidal infiltration.

CONCLUSIONS: Malignant infiltration of the liver is a rare cause of fulminant hepatic failure but must be considered due to implications for treatment and transplantation. Radiologic studies are nonspecific. Clues include elevated LDH, uric acid, and LDH/ALT ratio. Biopsy must be performed, the earlier the better. While infiltrating small cell carcinoma is universally fatal, survival has been seen with non-Hodgkin’s Lymphoma.

Reference #1 Kaira, Kyoichi. Fulminant hepatic failure resulting from small cell lung cancer and dramatic response to chemotherapy. World J.of gasterentrology 12: 2466-2468, 2006

Reference #2 McGuire, Brendan. Small Cell Carcinoma of the lung manifesting as acute hepatic failure. Mayo clinic Proceedings, 72 133-139, 1997

Reference #3 Rajvanshi, Pankaj. Fulminant heptic failure secondary to neoplastic infiltration of the liver. J. of clinical gasteroenterology 39; 339-341, 2005

DISCLOSURE: The following authors have nothing to disclose: Scott Hagedorn, William Kelly

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Chest. 2011;140(4_MeetingAbstracts):2A. doi:10.1378/chest.1114341

INTRODUCTION: Bronchial carcinoid is a rare tumor found in multiple endocrine neoplasia (MEN-1). The MEN-1 gene has been implicated in the pathogenesis of sporadic lung carcinoids, and bronchial carcinoids carrying the MEN-1 gene are more often the aggressive atypical carcinoid. We report the case of a patient with a solitary pulmonary nodule accompanied by fevers, flushing, and wheezing.

CASE PRESENTATION: A 68-year-old man with a long smoking history presents to pulmonary clinic with complaints of flushing, fevers, wheezing, and dyspepsia. Family history was unremarkable. Computed tomography (CT) of the chest showed a 1.5 centimeter solitary pulmonary nodule located near the airway. Bronchoscopy revealed a trail of blood from the right lower lobe bronchus, and endobronchial biopsies revealed a typical carcinoid tumor. Somatostatin receptor scintigraphy (OctreoScan) showed uptake positivity for the lung lesion. The liver was normal on OctreoScan and triple-phase CT. Due to the carcinoid syndrome and dyspepsia, endoscopy was performed and a gastric carcinoid was resected. Finding multiple neuroendocrine tumors (no liver metastasis) lead us to consider the diagnosis of MEN-1. Magnetic Resonance Imaging (MRI) of the brain then revealed a two centimeter pituitary tumor that upon excision demonstrated a prolactin staining positive adenoma. He was diagnosed with MEN-1. Since the patient was an index case of MEN-1 with atypical features, he underwent MEN-1 gene testing, which was negative. The patient recovered from his adenoma resection and was discharged home. He returned shortly thereafter with polyneuropathy, dementia, and pneumonia. He expired one year later without definitive treatment of his bronchial carcinoid.

DISCUSSION: Carcinoid tumors are neuroendocrine tumors of low malignant potential that can arise from the gut or lungs. While carcinoid tumors in the gut may present with the typical “carcinoid syndrome”, carcinoid tumors from the lung are usually asymptomatic unless they cause airway obstruction. Our finding of a symptomatic lung carcinoid led to the discovery of a gastric carcinoid. The typical pattern of metastatic spread of GI carcinoid to the lung involves metastatic disease in the liver. However, our patient had no evidence for liver involvement. We concluded that our patient demonstrated multifocal carcinoid, which can be seen in MEN-1 syndromes. This was confirmed by the finding of an asymptomatic prolactin-secreting pituitary adenoma. While genetic screening can identify 80% of these patients, 20% may carry as yet undescribed genetic mutations. Our patient also developed a wasting neurological syndrome that despite extensive testing could not be fully characterized but may have been related to his underlying MEN-1.

CONCLUSIONS: 1. In patients who present with carcinoid syndrome and an isolated bronchial carcinoid, a further work-up for a gastrointestinal source is indicated. 2. It is important to consider MEN-1 in the differential diagnosis of a patient with multiple neuroendocrine tumors that are seemingly unrelated (no liver metastasis). Additional imaging of the pituitary and pancreas and screening for hyperparathyroidism may be indicated. 3. Patients with MEN-1 gene mutations usually have more aggressive bronchial carcinoids.

Reference #1 Fink G, Krelbaum T. Pulmonary Carcinoid: Presentation, Diagnosis, and Outcome in 142 Cases in Israel and Review of 640 Cases From the Literature. Chest. 2001;119;1647-1651.

Reference #2 Debelenko L, Brambilla E. Identification of MEN1 gene mutations in sporadic carcinoid tumors of the lung. Human Molecular Genetics. 1997;6(13): 2285-2290.

DISCLOSURE: The following authors have nothing to disclose: David Collins, Gurinder Sidhu, Michael Peterson

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Chest. 2011;140(4_MeetingAbstracts):3A. doi:10.1378/chest.1118575

INTRODUCTION: Intrapulmonary teratomas are exceedingly rare. Fewer than fifty cases have been reported in literature. We describe a case of intra-pulmonary cystic teratoma (IPCT) that presented closely mimicking a malignant pulmonary neoplasm.

CASE PRESENTATION: A 39-year-old Hispanic male immigrant from Mexico presented with two-year history of intermittent cough, recent onset hemoptysis, fullness in his chest and ten-pound weight loss in seven months. He was an ex-smoker who had a recent Mantoux test that was negative. On exam, breath sounds were reduced in right upper chest, along with egophony and dullness to percussion. Chest radiograph showed right upper lobe (RUL) opacity and CT Chest confirmed large intra-pulmonary mass (8.5 X 8.9 cm) with multiple cavitations & evidence of infiltration of adjacent mediastinal and vascular structures. Bronchoscopic and CT guided biopsies were inconclusive. Thoracotomy revealed a large mass in the RUL surrounding the bronchus and extending into the mediastinum, which contained hair and particulate matter. Right upper and middle lobectomy was performed along with mediastinal lymph node dissection. Pathology On gross exam there was a mass of dull gray-tan rubbery tissue with attached hair. Microscopy revealed ectodermal components such as sweat, sebaceous glands and hair follicles along with multiple cystic areas consistent with a diagnosis of IPCT. Multiple sections failed to reveal any dysplasia or metaplasia. There was evidence of organizing pneumonia with dense fibrosis in the surrounding lung tissue and intra alveolar hemorrhage secondary to disruption of the blood vessels around the alveoli with evidence of blood in the airways, which caused the patient to have episodes of hemoptysis.

DISCUSSION: Teratomas are tumors consisting of tissues derived from more than one germ cell line. Typically, cystic teratomas are found in the body’s midline such as gonads and the mediastinum. Intra-pulmonary cystic teratoma is an extremely rare tumor, and only few cases have been reported in literature since its first description by Mohr in 1839(1). This tumor most commonly occurs in females and left upper lobe of the lung(2). Our patient was unique as he was a male with RUL mass. Diagnosis is often missed until thoracotomy and resection is usually curative. As seen in our case, they gradually invade the surrounding vascular tissue or bronchus and may cause life-threatening complications.

CONCLUSIONS: IPCT is a rare benign pulmonary tumor. Clinical presentation is myriad and may closely mimic malignant pulmonary neoplasm and needs to be considered in the differential diagnosis when evaluating a pulmonary mass in the young.

Reference #1 Teratoma of the lung. Collier FC, Dowling EA, Plot D, Schneider H. Archives of pathology. 1959; 68:138-142.

Reference #2 Intrapulmonary teratoma: A case report and review of literature. Desiree E. Morgan at al. J Thorac Imaging 1992; 7(3): 70-77.

DISCLOSURE: The following authors have nothing to disclose: Abhishek Sawant, Narinder Gill, Vijay Balasubramanian

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Chest. 2011;140(4_MeetingAbstracts):4A. doi:10.1378/chest.1119474

INTRODUCTION: Exogenous lipoid pneumonia (ELP) is caused by the inhalation of animal fats, vegetable or mineral oil. It is commonly seen in patients at high risk for aspiration and in those using mineral oil as a laxative. Appearance on chest imaging is variable, but focal consolidation mimicking tumor may be seen. Though classically confined only to the lung field, we present an unusual case of ELP initially misdiagnosed as stage IV lung cancer.

CASE PRESENTATION: A 72 year old female with a history of achalasia was referred to the thoracic oncology clinic for evaluation after a chest CT obtained to evaluate chest pain revealed spiculated masses in the right upper and lower lobes with hilar adenopathy. A dilated esophagus containing particulate food matter was also noted. Subsequent PET-CT showed the pulmonary lesions to be FDG avid with SUVmax between 5.3 and 6.2 with avidity also seen in the hilar nodal stations. A strongly avid (SUVmax=40) lytic lesion of the L5 vertebral body was also identified, concerning for a metastatic pulmonary malignancy. To establish a tissue diagnosis, the right upper and lower lobe masses were biopsied transbronchially using electromagnetic navigational bronchoscopy revealing foamy histiocytes and no evidence of malignancy. The hilar lymph nodes were sampled under endobronchial ultrasound guidance and similarly revealed no malignant tissue. This lipoid pneumonia is likely the result of chronic aspiration secondary to the patient’s profound achalasia. Interestingly, no history of aspiration was given. The patient used gel fish-oil capsules but no other lipid rich medications or foods. A biopsy of the L5 vertebral body was also obtained, revealing a highly cellular infiltrate with a monomorphic population of plasma cells consistent with a plasmacytoma. She has subsequently completed a course of treatment with 3500 cGy radiotherapy and 4 cycles of zoledronic acid and is doing well.

DISCUSSION: The term lipoid pneumonia was initially used by Laughlin in 1925 to describe the presence of lipid laden macrophages in lung tissue. This entity has since been divided into endogenous and exogenous categories, describing the route of lipid entry into the lung. Endogenous lipoid pneumonia is felt to represent a post-obstructive state wherein cholesterol and other lipids are released by necrotic tissue distal to the airway obstruction. Conversely, exogenous lipoid pneumonia (ELP) results from the inhalation, aspiration or other absorption of fats or oils. Presentation is quite variable and aspiration events are often asymptomatic as the lipid rich substances frequently do not trigger a cough reflex. Patients will often have insidious onset of cough and infectious symptoms such as fever. Radigraphic findings are varied, with lesions often FDG avid. Biopsy of the lesions reveals lipid laden macrophages and vacuoles may be seen in advanced lesions. Treatment involves primarily supportive care, though corticosteroid use has been described to blunt the inflammatory cascade in patients with acute respiratory distress.

CONCLUSIONS: Lipoid pneumonia, though rare, can produce imaging findings similar to pulmonary malignancy. History of aspiration, use of mineral oil laxatives, or esophageal motility disorders should increase suspicion for this disease entity. This case illustrates the ease with which a benign process may be mistaken for advanced malignancy on chest imaging and the importance of continued advancement of techniques for safe pulmonary biopsy.

Reference #1 Exogenous lipoid pneumonia: serial chest plain roentgenography and high-resolution computerized tomography findings. Chiang IC, Lin YT, Liu GC, Chiu CC, Tsai MS, Kao EL. Kaohsiung J Med Sci. 2003 Dec;19(12):593-8.

Reference #2 Exogenous lipid pneumonia: a retrospective multicentre study of 44 cases in France. Gondouin A, Manzoni P, Ranfaing E, Brun J, Cadranel J, Sadoun D, Cordier JF, Depierre A, Dalphin JC. Eur Respir J. 1996 Jul;9(7):1463-9

DISCLOSURE: The following authors have nothing to disclose: David Odell, Sidharta Gangadharan, Gaetane Michaud

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Chest. 2011;140(4_MeetingAbstracts):5A. doi:10.1378/chest.1119752

INTRODUCTION: Extra-osseous osteosarcomas (EOOs) are malignant tumors arising in the soft tissue without involving the bone or periosteum. These unusual neoplasms (comprising 1% of all soft tissue sarcomas and 2-4% of all osteosarcomas) have a very poor prognosis. This case highlights two complications of thoracic EOOs: malignant pleural effusion and spontaneous pneumothorax.

CASE PRESENTATION: A 94 year-old Caucasian female was referred by her primary care doctor for evaluation of cough progressing over the past three months now complicated by two weeks of exertional dyspnea. Her past medical history included systemic hypertension, hyperlipidemia and hypothyroidism. Her lab work and EKG were within normal limits. A chest radiograph revealed a large, free-flowing, right-sided pleural effusion: subsequent thoracentesis yielded blood tinged fluid which was exudative by Light’s criteria. A repeat chest radiograph post-thoracentesis showed a lobulated mass obscuring the right hemidiaphragm. Follow-up computerized tomography (CT) images revealed a large (7.9x7.1x4.1 cm), partially-calcified pleural based mass. A CT-guided biopsy of the mass revealed spindle-shaped, fibroblastic cells with islands of osteoid matrix formation consistent with extra-skeletal osteosarcoma. The patient was offered an extensive surgical resection including diaphragmatic take-down and reconstruction. Given the high perioperative mortality and her advanced age, she declined the operation. Two weeks later, the patient presented with acutely worsened dyspnea. Chest radiographs showed recurrence of the large right-sided pleural effusion and a new pneumothorax. A thoracostomy tube was placed resulting in the resolution of her hydro-pneumothorax. Pleural fluid cytology specimens were positive for malignant cells. The patient opted for palliative care measures and succumbed to her illness four months after hospital discharge.

DISCUSSION: EOOs are neoplasms characterized by the production of osteoid or bone, sometimes accompanied by cartilage. EOOs have an aggressive clinical course: 70% recur locally and 80% develop metastatic disease within 2 years of resecting the primary tumor. The most frequently affected sites are the soft tissues of the limbs (67%), especially the thighs and retroperitoneum (17%). , While skeletal osteosarcomas predominantly affect adolescents and young adults, EOOs usually develop after the age of 30. Cases have been reported to have arisen in areas that have received radiation therapy or in areas with previous myositis ossificans. Osteosarcomas are associated with spontaneous pneumothoraces - even in the absence of apparent metastasis to the lungs. However, patients with lung metastases have a high incidence of spontaneous pneumothorax, especially following chemotherapy. These neoplasms vary in their degree of histologic differentiation with widely discrepant amounts of spindle cell sarcoma and foci of malignant osteoid. The limited experience with EOOs to date suggests that the best prospect for cure is early, complete surgical excision combined with chemotherapy and/or radiotherapy. Long-term follow-up is required before the efficacy of this approach can be assessed. Median survival time is estimated at 31 months and reported 5-year survival rates are 13-22%. The presence of spontaneous pneumothorax has been shown to portend a worse prognosis.

CONCLUSIONS: EOOs, while uncommon, should be considered in the differential diagnosis of pleural effusion and spontaneous pneumothorax. Early recognition of EOOs is essential as successful treatment of these tumors requires early, aggressive and multidisciplinary measures.

Reference #1 Sordillo PP, et al. Extraosseous osteogenic sarcoma. A review of 48 cases. Cancer. 1983;51:727-734.

Reference #2 Bane B, et al. Extraskeletal osteosarcoma. A clinicopathologic review of 26 cases. Cancer. 1990;65:2762-2770.

DISCLOSURE: The following authors have nothing to disclose: Andrew De Nazareth, Vijaya Gogineni, Jennifer Olivella, Lee Morrow

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Chest. 2011;140(4_MeetingAbstracts):6A. doi:10.1378/chest.1120041

INTRODUCTION: Lymphangitic carcinomatosis (LC) secondary to squamous cell carcinoma (SCC) of the cervix (SCCC) is exceedingly rare. In prior reports, LC coincided with significant abdominal and thoracic lymphadenopathy. Additionally, sputum cytology, bronchial washings and/or transbroncheal biopsies represented successful diagnostic modalities (3). We report a case of LC secondary to SCCC in a patient immunocompromised due to chemoradiation presenting with diffuse bilateral pulmonary infiltrates and hypoxic respiratory failure. Importantly, there was no thoracic, abdominal, or pelvic lymphadenopathy. In addition, multilobar bronchioalveolar lavage (BAL) and transbronchial biopsies were non-diagnostic. Subsequently, open lung biopsies were consistent with LC.

CASE PRESENTATION: A 38-year-old white female presented with a two-month history of worsening dyspnea associated with productive cough, subjective fever, and bilateral pulmonary infiltrates on chest X-ray. She was diagnosed with stage III locally advanced SCCC sixteen months prior. On presentation, she was in remission after having undergone total hysterectomy, adjuvant platinum-based chemotherapy, and radiotherapy. Her physical examination revealed respiratory distress, oxygen saturation of 88% on room air, and bilateral diffuse inspiratory crackles. CD4 count was 240; HIV serology, p24, and PCR were negative. A CT scan of the chest showed diffuse centrilobular ground glass opacities with interstitial and peribronchial thickening. Importantly, there was no radiographic evidence of thoracic, abdominal, pelvic lymphadenopathy or masses. Cultures, serology, BAL, and transbronchial biopsies were negative for infection and inflammatory disease. In addition, neither transbronchial biopsies nor BAL fluid cytology revealed any malignant cells. Based on the severity of the patient’s symptoms and lack of diagnosis, an open lung biopsy was performed. Unexpectedly, this demonstrated neoplastic sheets of cells with squamous morphology features along the perivascular, peribronchial, and subpleural lymphatic vessels as well as in pulmonary arterioles. Immunohistochemical analysis was strongly positive for p16 (a marker used to differentiate between primary pulmonary and cervical SCC) and cytokeratin (CK)-7, while negative for CK20, p63 and thyroid transcription factor 1, findings consistent with a diagnosis of LC from SCCC (1). Unfortunately, due to extend of pulmonary tumor burden, the patient was not extubatable after the lung biopsy, and care was withdrawn.

DISCUSSION: SCCC spreads most frequently by direct extension to the surrounding tissues. Pulmonary metastasis in form of LC, though rare, indicates advanced metastatic disease with a grave prognosis. Its non-specific clinical manifestations (e.g. cough, dyspnea) often lead to an incorrect diagnosis (e.g. pneumonia, or congestive heart failure). The mechanism of SCCC metastasis in form of LC is poorly understood. The significant involvement of hilar lymph nodes in previously reported cases suggests tumor cell invasion through the lymphatic channels, with proliferation in hilar lymph nodes leading to obstruction of lymphatic flow, and subsequent retrograde spread of tumor cells within the pulmonary lymphatics. However, our case suggests an alternative mechanism, as there was no radiographic evidence of abdominal or thoracic lymph node involvement. This suggests potential other routes of tumor spread, such as hematogenous tumor microembolization to the pulmonary lymphangitic system (2). Our patient’s immunocompromised condition from recent chemoradiation may have facilitated such a process by allowing tumor cells to escape from immunosurveillance (2). A second significant finding of our case is the non-diagnostic result of multilobar BAL and transbronchial biopsies despite the diffuse bilateral lung involvement.

CONCLUSIONS: Our case is the first report of a misleading presentation of LC from SCCC in an immunocompromised host in the absence of thoracic, abdominal or pelvic lymphadenopathy with nondiagnostic BAL and transbronchial biopsies. A high clinical suspicion and aggressive diagnostic strategy are needed for pulmonary abnormalities in patients with a history of SCCC, especially if immunocompromised.

Reference #1 Wang CW. Am J Clin Pathol 2009.

Reference #2 Shin MS. Invest Radiol 1995.

Reference #3 Storck K. Gynecol Oncol 2004.

DISCLOSURE: The following authors have nothing to disclose: Amir Emtiazjoo, Tim Lahm, Francis Sheski

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Chest. 2011;140(4_MeetingAbstracts):7A. doi:10.1378/chest.1118790

INTRODUCTION: Carbon dioxide angiography may be an alternative for IVC filter placement.

CASE PRESENTATION: 60 y/o African American female was diagnosed with pulmonary embolism by CT after one week of progressive shortness of breath. She had negative duplex dopplers of the lower extremities . Anti coagulation was initiated and the patient was discharged home. Upon leaving, she fell and sustained a fracture of the right distal fibula which was casted. She was readmitted for open repair of her fracture and to manage anticoagulation. Her past medical history was significant for diabetes mellitus, hypertension, hyperlipidemia and gout. Her physical exam was notable for a morbidly obese female, resting comfortably. Her temperature was 37.4, BP 160/70, pulse 74, respiratory rate 19, and oxygen saturation 94% on 2L NC. Notable exam findings included Mallampati 4 airway and pedal edema with her right leg in a removable cast with chronic venous stasis change. Laboratory data suggests chronic carbon dioxide retention and renal insufficiency with values of 36 and 1.8 respectively: ABG 7.32/pCO2 80/pO2 75. Multiple surgeries were planned with recurrent need to interrupt anticoagulation. Her ABG was suggestive of OHS and potentially OSA. The recommendations included retrievable IVC filter, nocturnal BiPAP and outpatient sleep study. Interventional radiology placed an IVC filter using carbon dioxide angiography secondary to her renal disease. She did not receive any sedation. Post procedure she was obtunded on 100% NRB with an ABG: 7.14/pCo2 greater than 100, p02 120. She was intubated with a diagnosis of hypercapnic respiratory failure secondary to carbon dioxide angiography.

DISCUSSION: Carbon dioxide has been used since the 1960s for IVC filters. The carbonic anhydrase catalyzes carbon dioxide and water to form carbonic acid which subsequently dissociates into hydrogen ions and bicarbonate. The bicarbonate moves quickly into the plasma where it dissolves. The reverse process occurs to release carbon dioxide into the lungs. Large IVC injection and selective pulmonary artery injections into rats demonstrated no gas in the pulmonary vein suggesting that even massive volumes are eliminated in one pass through the lungs. The advantages to carbon dioxide angiography are that it is non-toxic, invisible, highly compressible, non viscous, buoyant and rapidly absorbed. There is no allergic potential and no renal toxicity thereby it is ideal for complex cases requiring larger amounts of contrast. Additionally, collateral arteries are better demonstrated, arteriovenous shunting can be observed in tumors and AV malformations and minute amounts of bleeding can be visualized. Carbon dioxide use specifically for IVC filter placement is indicated only if contrast contraindicated. More numerous and selective injections are required as the right renal vein as a posterior structure does not readily fill with carbon dioxide and an IVC thrombus may go undetected. Additionally, the use of carbon dioxide in patients with respiratory compromise is debated secondary to the theoretical risk of exacerbating carbon dioxide retention. Therefore, it is recommended to decrease the volume and increase the time interval for injection. Most centers do not have a protocol and follow the dictum “as much as we need.” “Carbon dioxide should not be a problem unless a patient….cannot respond with increased ventilation.”

CONCLUSIONS: In a morbidly obese patient with baseline hypercarbia/hypoventilation the additional carbon dioxide load introduced during carbon dioxide angiography resulted in hypercarbic respiratory failure secondary to the patient’s inability to respond to the increasing carbon dioxide load with increased ventilation. Carbon dioxide as a contrast agent holds promise for complicated procedures and patients who could not otherwise have contrast but there are no sizeable human trials to demonstrate safety. There are no standard protocols at this time although many institutions are developing guidelines to regulate speed and amount of carbon dioxide is administered.

Reference #1 Hawkins,Caridi, Carbon Dioxide (CO2) digital subtraction angiography: 26 year experience at the University of Florida. European Radiology. 1998;8: 391-402

Reference #2 Shaw,Kessel, The current status of the use of carbon dioxide in diagnostic and interventional angiographic procedures. Cardiovascular Interventional Radiology. 2006; 29: 323-331

Reference #3 Hawkins et al, Carbon dioxide in angiography to reduce the risk of contrast-induced nephropathy. Radiol Clin N Am, 2009; 47: 813-825

DISCLOSURE: The following authors have nothing to disclose: Kelly Newton

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Chest. 2011;140(4_MeetingAbstracts):8A. doi:10.1378/chest.1114492

INTRODUCTION: Balloon kyphoplasty is a recently introduced procedure for the treatment of vertebral compression fractures, especially from osteoporosis, tumors and burst fractures. With the increase in frequency of procedures being performed more complications from kyphoplasty are seen. The risk of pulmonary embolism ranges from 3.5% to 23% for osteoporotic fractures. We report a case of an asymptomatic cement pulmonary embolism following a balloon kyphoplasty procedure.

CASE PRESENTATION: An 82 year old woman was admitted for a dorsal spine wound infection and was found to have an abnormal chest x-ray. She has a past medical history of osteoporosis, hypertension, hypercholesterolemia and multiple vertebral compression fractures for which she had undergone a kyphoplasty of L3 and L4 8 weeks prior to admission. She had no respiratory complaints or weight loss. Oxygen saturation was 98% on room air. Her lung exam was clear to auscultation bilaterally. The only significant physical finding was related to her back where she had a foul smelling wound. Chest x-ray revealed multiple tubular filling defects in the right and left pulmonary arteries in comparison to her chest x-ray pre-kyphoplasty which was normal. Subsequently, a CT scan of her chest was performed which showed multiple filling defects with HU of 2600 consistent with pulmonary cement embolism.

DISCUSSION: This case presents a case of post kyphoplasty pulmonary cement embolism. Although most leaks are asymptomatic there are case reports of fatalities. Cement leakage is the most frequent complication after percutaneous kyphoplasty. Due to the risk for radiation exposure, chest x-rays after kyphoplasty are not usually performed. In asymptomatic peripheral cement embolism the recommendation is to follow clinically and not anticoagulate. In cases of central or peripheral symptomatic cement embolism it is recommended to anticoagulate for 6 months.

CONCLUSIONS: Physicians should be aware of the possibility of cement embolism following both kyphoplasty and vertebroplasty. A routine chest x-ray post-procedure may be of value in evaluating the incidence of pulmonary cement embolism. As with our patient, most cases are without respiratory complaints and there is no need to start anticoagulation for asymptomatic cement embolism when diagnosed on chest x-ray. However, the recommendation for treatment of symptomatic cement embolism is anticoagulation. Diagnosis of cement embolism on a routine post-kyphoplasty chest x-ray may initiate appropriate treatment earlier including close clinical follow up for incidental asymptomatic emboli.

Reference #1 Kruger A, Bliemel C, Zettl R, Ruchholtz S. Management of pulmonary cement embolism after percutaneous vertebroplasty and kyphoplasty: a systematic review of the literature. Eur Spine J 2009; 18:1257-1265

Reference #2 Radcliff K et al. Pulmonary cement embolism after kyphoplasty: a case report and review of the literature. The Spine Journal 2010;10 e1-e5

DISCLOSURE: The following authors have nothing to disclose: George Apergis, Mara Lagzdins, Ali Soueidan

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Topics: lung , embolism
Chest. 2011;140(4_MeetingAbstracts):9A. doi:10.1378/chest.1117025

INTRODUCTION: The etiology of bronchiectasis may be idiopathic in up to one half of adults. Consideration of Primary Ciliary Dyskinesia (PCD) may not be considered in adults without dextrocardia. We present a subject with bronchiectasis with PCD and central pair absence, a rare variant of PCD not previously diagnosed at such a late age.

CASE PRESENTATION: A 37-year-old recent immigrant from Pakistan presented to the Chest Clinic with recurrent upper and lower respiratory tract infection which he had for many years, decreased exercise tolerance, weight loss, fatigue, and night sweats. He was a current smoker of 3-4 cigarettes/day . He had chronic hepatitis C and complained of anosmia. He had no allergies, no alcohol or drug use, and denied any family history of similar problems. He has one son with mental retardation. His pulmonary function revealed moderate obstruction with normal total lung capacity and diffusion. Clinical examination revealed a gentleman with wheezing at all visits with a chronic cough. A CT scan revealed bronchiectasis predominant in the right middle lobe and less in both lower lobes. Sputum for AFB smear and culture were negative for TB. HIV infection, cystic fibrosis, and alpha-1 antitrypsin deficiency were ruled out and work-up for collagen vascular diseases was negative including a negative ANA and RF. His total immunoglobulins IgG, IgA, IgM, and IgE levels were normal except for subclass analysis of IgG-4 levels which were slightly low. He has been managed with smoking cessation (he has quit for over 1 year), and has been treated intermittently with different antibiotic regimens with sputum cultures growing out multi-drug resistant Pseudomonas, Aspergillus Fumigatus, and Mycobacterium mucogenicum. He also is on inhaled steroid and bronchodilators to try to minimize his chronic wheezing. In order to try to make a more definitive diagnosis he was referred to ENT and had both maxillary sinus drainage and nasal epithelial biopsy to rule out primary ciliary dyskinesia (PCD). Electron microscopy revealed ciliary cross sections with absence of the central doublet/microtubule pair consistent with PCD with central pair absence(1).

DISCUSSION: PCD is a ciliopathy that is associated with an array of ultrastructural defects of cilia. This genetic disorder is primarily autosomal recessive and is seen in about 1:15-30,000 live births (1,2). Cases of PCD can be grouped into dynein arm defects (78-96%) and central ciliary defects (4-22%). Central ciliary defects, as seen in this case, are not associated with defects in laterality whereas more then 50% of PCD patients have laterality defects and up to 50% have Kartagener’s syndrome (dextrocardia, sinusitis, bronchiectasis). Genetic linkage studies have revealed heterozygous nonsense mutations in radial spoke head protein genes, probably RSPH9 and RSPH4A, causing PCD with the central microtubular defect as seen here (2). Interestingly, ciliary beat frequency can be normal, but beat pattern can be circular instead of the normal forward-backward motion. Our patient was also noted to have heterogeneous ultrastructural abnormalities of some peripheral microtubules, consistent with acquired defects related to recurrent airway damage. It is unknown if IgG-4 subclass deficiency is related to PCD.

CONCLUSIONS: Adults with recurrent sinopulmonary infections and idiopathic bronchiectasis should be considered for evaluation of PCD. The presence of fertility and absence of typical features, like childhood presentation, situs inversus, family history, and infertility do not exclude the possibility of PCD. PCD with central pair abnormality, although rare, is not associated with laterality defects and this is the first reported case of this specific abnormality detected at such a late age. Definitive detection of causes of bronchiectasis will lead to better understanding of disease processes, facilitate patient management, and impact on potential decisions regarding the possibility of lung transplants.

Reference #1 Papon JF, Coste A, et. al. A 20-year experience of electron microscopy in the diagnosis of primary ciliary dyskinesia. Eur Respir J 2010;35:1057-63.

Reference #2 Castleman, VH, Romio L, et al.Mutations in Radial Spoke Head Protein Genes RSPH9 and RSPH4A Cause Primary Ciliary Dyskinesia with Central-Microtubular-Pair Abnormalities. Am Journal of Human Genetics 2009;84(2):197-209.

DISCLOSURE: The following authors have nothing to disclose: Sikander Zulqarnain, Gene Pesola

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Topics: bronchiectasis
Chest. 2011;140(4_MeetingAbstracts):10A. doi:10.1378/chest.1120046

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that typically presents with progressive muscle weakness. Respiratory symptoms due to diaphragmatic weakness generally occur late in the clinical course. We describe a case of ALS with the rare presentation of diaphragmatic paralysis as the initial manifestation.

CASE PRESENTATION: A 69 year old veteran with a past medical history of psoriasis, diabetes mellitus and hypertension was referred to the VA pulmonary clinic with complaints of progressive dyspnea on exertion, hoarseness, and orthopnea. Initial chest radiograph and computed tomography of the chest were notable for subsegmental atelectasis and elevated right hemidiaphragm suspicious for diaphragmatic paralysis. He was subsequently lost to follow-up, but ultimately was admitted to a large community hospital with worsening dyspnea. He was hypoxic, hyperreflexic and was noted to have muscle atrophy in his upper and lower extremities. His chest radiograph showed diminished lung volumes, atelectasis and elevation of both hemidiaphragms. Pulmonary function testing showed severe restrictive disease. Ultrasonography of the diaphragms demonstrated bilateral dysfunction. He returned four months later with continued dyspnea and new-onset muscle fasciculations and bulbar weakness. Electromyography (EMG) was consistent with Amyotrophic Lateral Sclerosis (ALS).

DISCUSSION: ALS is a fatal neurodegenerative disease of unknown etiology characterized by progressive muscular paralysis due to degeneration of the upper and lower motor neurons. It generally affects patients between 40 and 60 years of age and is uniformly fatal with a median survival time of 36-48 months from the time of initial diagnosis. Recent reports indicate that veterans are at increased risk of developing ALS. The typical presenting symptoms include dysphagia, limb muscle weakness, muscle cramping and atrophy, or fasciculations. Muscular paralysis is progressive and ultimately advances to involve the diaphragm. Patients may have a restrictive ventilatory defect and hypercapnic respiratory failure. In most cases death occurs from respiratory complications, and the presence of diaphragmatic dysfunction is associated with a marked reduction in survival. A decrease in FEV1 and FVC of more than 20% in the supine position compared to erect position suggests diaphragmatic weakness in ALS. Use of sniff testing with fluoroscopy can be misleading in the presence of bilateral diaphragmatic paralysis. Nerve conduction studies and EMG are the diagnostic tests of choice for ALS. Patients with ALS complicated by respiratory insufficiency have shown prolonged survival and improved quality of life with early use non-invasive positive pressure ventilation. Tracheostomy, diaphragmatic pacing and invasive ventilation can also be offered as supportive therapy. Our case was unique because the patient presented with respiratory complaints and he later developed bulbar palsy and fasiculations. Respiratory symptoms are an unusual presenting manifestation of ALS, and are present in less than 3% of the cases at the time of initial diagnosis. Clinicians should be wary of respiratory onset ALS in patients with diaphragmatic paralysis and dyspnea

CONCLUSIONS: Although rare, patients with ALS can present with respiratory symptoms. Given the finding of orthopnea and dyspnea with suspected diaphragmatic paralysis, a thorough neurological evaluation may uncover a diagnosis of ALS.

Reference #1 Similowski, T., Attali, V., Bensimon, G., Salachas, F., Mehiri, S., Arnulf, I., Lacomblez, L., Zelter, M., Meininger, V. and Derenne, J.-P. (2000), Diaphragmatic dysfunction and dyspnoea in amyotrophic lateral sclerosis. European Respiratory Journal, 15: 332-337. 10.1034/j.1399-3003.2000.15b19.

Reference #2 Lechtzin N, Wiener CM, Shade DM, et al. Spirometry in the supine position improves the detection of diaphragmatic weaknessin patients with amyotrophic lateral sclerosis. Chest 2002;121:436-442

DISCLOSURE: The following authors have nothing to disclose: Zeeshan Khan, Abdulilah Arafeh, Allen Blaivas, Ahmar Jafary, Timothy Meehan

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Chest. 2011;140(4_MeetingAbstracts):11A. doi:10.1378/chest.1119731

INTRODUCTION: Chemically considered rather inert, silicone has long been used as a component in many implantable medical devices. Injectable liquid silicone use however has been shown to have significant potential morbidity and mortality. We report on a young woman who suffered severe pulmonary complications from receiving silicone injections in her buttocks for cosmetic purposes.

CASE PRESENTATION: 23-year-old woman with no medical history was admitted with worsening shortness of breath and cough with streaky hemoptysis of 3 days duration. The patient reported pleuritic chest pain accompanying episodes of coughing. She denied any sick contacts or recent travel. The patient however did report receiving multiple silicone injections from an unlicensed practitioner in her buttocks, the last one being 2 days prior to symptom onset. On admission, the vital parameters revealed an obese patient with tachycardia and tachypnea. Rest of the physical examination was unrevealing. Laboratory data was significant for neutrophilic leukocytosis along with a microcytic anemia. Chest radiograph revealed bilateral patchy infiltrates. CT chest revealed peripheral areas of air space disease in dependent areas of both lungs. Blood gas analysis confirmed severe hypoxemia. The atypical pattern of air space disease prompted an extensive work up for infectious, non-infectious, and rheumatological etiologies, all of which was unrevealing. Based upon literature review coupled with a history of recent silicone injections, a presumptive diagnosis of silicone embolism syndrome was made. Patient was treated with supportive care, including supplemental oxygen and steroids. Over the next few days, the patients respiratory status improved steadily and on day 7, the patient was discharged home on a tapering dose of steroids and supplemental oxygen. Patient was successfully weaned off the steroids over the ensuing weeks with complete clinical recovery and marked resolution of infiltrates on follow-up imaging studies.

DISCUSSION: Silicone embolism syndrome (SES) is a rare and potentially fatal complication that was first described in populations of transsexual men who received silicone injections for breast augmentation in the 1970s. While legitimate use of liquid silicone injections in plastic surgery community is rare, illegal use in quasi-sterile conditions is rampant. Two distinct systemic patterns of toxicity have been described: pulmonary and neurologic. The precise mechanism of pulmonary toxicity is unclear. A variety of pathological findings including alveolar hemorrhage as well as diffuse alveolar damage have been reported. High-pressure injection, large volume injection and manipulation at the injection site have all been linked to an increased risk of SES. The clinical presentation of the disease is non-specific; dyspnea and hypoxemia are noted in the vast majority of patients. Available literature describes a consistent pattern on chest imaging of bilateral peripherally distributed ground glass opacities and/or consolidation. Treatment is supportive. Even though there is no consensus regarding therapy with corticosteroids, it has been suggested that their early use may be helpful in reversing the clinical course. The reported mortality from SES varies between 24-33%.

CONCLUSIONS: In these times of austerity where cheaper and at times, illegal alternatives to medical care are frequently sought, awareness of this constellation of clinical and radiologic findings is a pre-requisite for timely diagnosis of this uncommon entity.

Reference #1 Parikh et al. Case report and literature review: acute pneumonitis and alveolar hemorrhage after subcutaneous injection of liquid silicone. Ann Clin Lab Sci. 2008 Autumn; 38(4): 380-5.

Reference #2 Bartsich S, Wu JK. Silicon emboli syndrome: a sequela of clandestine liquid silicone injections. A case report and review of the literature. J Plast Reconstr Aesthet Surg. 2010 Jan; 63(1): e1-3.

Reference #3 Restrepo et al. Silicone pulmonary embolism: report of 10 cases and review of the literature. J Comput Assist Tomogr. 2009 Mar-Apr; 33(2): 233-7.

DISCLOSURE: The following authors have nothing to disclose: Tathagat Narula, Amer Raza, Neha Narula, John Amoss

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Chest. 2011;140(4_MeetingAbstracts):12A. doi:10.1378/chest.1119565

INTRODUCTION: Idiopathic Acute Eosinophilic Pneumonia (IAEP) is a rare cause of acute respiratory failure, with unknown pathogenesis. The authors present one of the most severe reported cases of IAEP, in a young smoker, and the first reported successful use of Extracorporeal Membrane Oxygenation (ECMO) for IAEP in an adult patient.

CASE PRESENTATION: A 20 year old previously healthy Caucasian woman, who started smoking tobacco cigarettes two weeks ago, presented with acute onset of fever, dyspnea and productive cough. She was febrile and hypoxemic, with bilateral diffuse ronchi on chest auscultation. She had leukocytosis of 28.5 k/µL, with neutrophilia. Diffuse bilateral alveolar infiltrates were seen on chest radiograph and CT scan. Broad spectrum intravenous antibiotics were initiated for presumed community acquired bacterial pneumonia. She required invasive mechanical ventilation within 6 hours of admission, and her hypoxemia continued to worsen. An arterial blood gas (ABG), on 100% FIO2 and positive end expiratory pressure of 20 cmH2O on mechanical ventilation, revealed a normal pH and PCO2, with a PaO2 of 50 mmHg. Therefore, veno-venous ECMO was initiated, with 100% FIO2, and sweep gas flow of 6 Liters/ minute. A subsequent ABG showed a PaO2 of 70 mmHg. Blood, urine and stool cultures were negative. Bronchoalveolar lavage (BAL) showed marked eosinophilia of 43%, and no pathogenic bacteria, mycobacteria, fungi or viruses. ANA and ANCA were not detected. The patient was diagnosed with IAEP. Intravenous methylprednisolone was initiated at 60 mg every 8 hours, until she was weaned from ECMO and mechanical ventilation 48 hours later, followed by transition to oral prednisone, which was tapered over eight weeks. She had complete resolution of disease when discharged on the tenth day of hospitalization, with no evidence of recurrence 16 weeks later.

DISCUSSION: First described in 1989, roughly 150 cases of IAEP have been reported worldwide. The diagnostic criteria consists of an acute febrile illness with respiratory failure, diffuse bilateral alveolar or interstitial lung infiltrates on chest radiograph and eosinophilia of 25% or greater in BAL fluid, in the absence of infection or alternative cause for eosinophilia 1. IAEP falls under the heterogeneous group of eosinophilic lung diseases and should be distinguished from clinically similar entities. In our patient, drug, toxin and animal exposure, an infectious etiology and connective tissue disease were excluded. Allergic bronchopulmonary aspergillosis was unlikely given the lack of history for asthma. Although the exact pathophysiology is unknown, IAEP is thought to be caused by acute hypersensitivity to inhaled allergens. Certain inflammatory mediators, particularly interleukin-5, are elevated and have been shown to signal eosinophilic activation and migration 1. Many reported cases appear to be associated with recent smoking, including our patient. However, no direct pathway has been found between cigarette smoke and interleukin activation. Other implicated toxins include tear gas, gasoline, and cocaine 1. Corticosteroid treatment results in a dramatic improvement in symptoms within 48 hours 1. Long term prognosis is excellent, with no reported recurrence. The challenge, however, lies in timely diagnosis and treatment, since IAEP may resemble acute lung injury or acute infectious pneumonia, as in our patient. This is the only reported case of IAEP in an adult patient, where ECMO was used successfully to manage respiratory failure while further diagnostic testing was underway and the therapeutic effect of corticosteroids was awaited.

CONCLUSIONS: IAEP is a rare disease of unclear pathophysiology. Tobacco smoking may be associated with some cases, including our patient. IAEP can cause severe acute respiratory failure, which may be managed successfully with ECMO, while measures are taken to establish a definitive diagnosis and treatment.

Reference #1 Philit F. et al. Idiopathic Acute Eosinophilic Pneumonia: A Study of 22 Patients. Am J Respir Crit Care Med, 2002; 166:1235-1239

DISCLOSURE: The following authors have nothing to disclose: Maryam Gul, Robert Freed, Nida Rizvi, Margaret Wojnar

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Chest. 2011;140(4_MeetingAbstracts):13A. doi:10.1378/chest.1119257

INTRODUCTION: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a powerful tool for the assessment of mediastinal lymphadenopathy. We present a patient who was initially diagnosed with metastatic carcinoid tumor. On the basis of immunohistochemical staining of mediastinal lymph node biopsy obtained via EBUS-TBNS, the correct diagnosis was made.

CASE PRESENTATION: A 25-year-old woman was referred to our institution for management of metastatic tumor. She had presented to an outside hospital with abdominal pain of two weeks duration. A CT scan of the abdomen revealed an 8 x 9 x 7 cm liver mass. She reported chronic coccygeal pain worsening over several years that she attributed to a presumed coccyx fracture. Physical examination revealed a hard, fixed, tender mass at the base of the coccyx. Percutaneous liver biopsy was reported as consistent with carcinoid tumor, as supported by clusters of bland cells with hyperchromatic nuclei. The results of immunohistochemical stains were equivocal. An octreotide scan was negative, suggesting an alternative diagnosis. A CT scan of the chest was performed at our institution due to complaints of dyspnea and cough, revealing massive mediastinal lymphadenopathy and innumerable small pulmonary nodules suggestive of metastatic disease. The patient was referred for EBUS-TBNA as the constellation of clinical findings was unusual for carcinoid tumor. Lymph node histology demonstrated clusters of large mildly pleomorphic malignant epithelioid cells with focal fibrillary cytoplasmic processes, perivascular pseudorosette formation, mitotic activity, and tumor necrosis. Based on the unusual histology, multiple immunohistochemical stains were performed on the aspirate cell block. The tumor cells were extensively positive for vimentin, GFAP, CD56, and NSE, focally positive for EMA, CD99, AE1/3, and S100, and negative for CAM5.2, synaptophysin, and chromogranin. The histologic features and staining pattern supported a diagnosis of metastatic extraspinal ependymoma or myoepithelial carcinoma rather than a carcinoid tumor. The original liver biopsy was restained at our institution and found to have the same staining pattern as the mediastinal lymph node biopsy. In addition, the mass adjacent to the coccyx was surgically excised and was consistent with an extraspinal ependymoma. The coccygeal mass, liver mass, and mediastinal lymph nodes all demonstrated uniform histologic and immunohistochemical features.

DISCUSSION: To our knowledge, this is the first report of utilizing EBUS-TBNA in the diagnosis of extraspinal ependymoma. Primary extraspinal ependymoma is a rare malignancy with approximately 100 cases reported in the literature. The majority occur in the sacrococcygeal region, often being mistaken for benign pathology such as a pilonidal cyst. Fewer than 20% of cases metastasize, and have been reported in the lungs, pleura, bone, regional lymph nodes, and skin.(1) This case demonstrates that material obtained via EBUS-TBNA from mediastinal lymph nodes is adequate to be analyzed by modern microscopical and immunohistochemical techniques to make specific diagnoses of malignant processes arising in distant locations from the lung.

CONCLUSIONS: Accurate diagnosis of disseminated malignancy requires a correlation of clinical, radiologic, and pathologic information. EBUS-TBNA is a relatively noninvasive yet powerful tool for histologic examination and staining with multiple tumor markers. Careful attention to immunohistochemical staining of such biopsies may assist in the diagnosis of unexpected and uncommon malignancies.

Reference #1 Ma YT, Ramachandra P, Spooner D. Primary subcutaneous sacrococcygeal ependymoma: a case report and review of the literature. Br J Radiol 2006; 79: 445-447.

DISCLOSURE: The following authors have nothing to disclose: Joshua Farkas, Llewellyn Foulke, Anwar Haque, Marc Judson, Mark Napier

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Chest. 2011;140(4_MeetingAbstracts):14A. doi:10.1378/chest.1119914

INTRODUCTION: Primary tracheal tumors are rare, and composed of various benign and malignant pathologies. We present a case of an incidentally discovered tracheal tumor in a male patient that was initially diagnosed as a papilloma. On complete resection of the tumor and subsequent analysis it was identified as a tracheal hamartoma

CASE PRESENTATION: A 65-year old male, former smoker, presented to the out-patient pulmonary office for an evaluation of a pulmonary nodule. He denied any complaints of shortness of breath, wheezing, chronic cough, pedal edema, anorexia or weight loss. He also denied a history of prior lung disease, TB or TB exposure. On a recent CT scan of his chest he was noted to have sub-cm pulmonary nodule and an 8mm polypoidal lesion in his trachea His past medical history was significant for congestive heart failure, diabetes mellitus and benign hypertension. His vital signs and physical examination was unremarkable. After reviewing all the relevant data and images he was scheduled for an out-patient bronchoscopy On inspection bronchoscopy a verrucous lesion was noted in the right lateral wall of the lower mid trachea. Multiple biopsies of the lesion were sent for histopathological analysis The histopathology revealed submucosal fibrosis and chronic inflammation with an inflamed squamous papilloma; however the HPV stains were subsequently negative The patient was rescheduled for a repeat bronchoscopy and complete resection of the lesion. Polyp snares with cautery to 40W was used for cutting the polyp, subsequently APC at 40W and 0.4s pulses was used for further destruction of the tumor. The histopathology revealed abundant hypocellular stroma with myxochondroid appearance and a few mature adipocytes. The overlying respiratory epithelium showed regional squamous metaplasia but no squamous hyperplasia, koilocytosis or squamous atypia to warrant a diagnosis of squamous papilloma. It was diagnosed as a Tracheal Hamartoma.

DISCUSSION: Primary tracheal tumors are rare, with most of them arising in the lower one-half to two-thirds of the trachea. The name hamartoma, first introduced by Albrecht in 1904 is derived from the Greek words for ‘error’ and ‘tumor’. On histological examination all the elements of a normal bronchus are seen with cartilage, muscle and epithelial tissue, together with ciliated epithelium lining the clefts in the tumor. Only 3% of hamartomas are endobronchial and there are only 12 cases reported of tracheal hamartomas. In a review of 128 benign tumors of the bronchus and trachea over a 30 year period, Raymond Hurst included 38 cases of hamartoma, but no cases of tracheal hamartoma. Most cases have been reported in men with only three present in women. Bronchoscopic resection is a safe and effective therapy in the management of airway hamartomas. Cosio reports that in 45 cases of endobronchial hamartoma, 42 had a complete resection through endoscopic laser.

CONCLUSIONS: The tracheal origin of the hamartoma in our patient is an unusual and relatively rare entity. Resection of the tumor is safe and should be considered if the patient has symptoms or if the diagnosis is uncertain.

Reference #1 Tracheal hamartoma. IJ Hurst and KG Nelson; Chest 1977;72;661-662

Reference #2 Endobronchial hamartomas. Cosio BG, Villena V, Echave-Sustaeta J, de Miguel E, Alfaro J, Hernandez L, Sotelo T. Respiratory Department, Hospital 12 de Octubre, Madrid, Spain. Chest 2002;122:202-5.

Reference #3 Tracheal hamartoma detected by abnormal breath sounds. Akira Kunisawa Et al; Journal of Bronchology 2000;7;160-163

DISCLOSURE: The following authors have nothing to disclose: Shailesh Pinto, Maria Del Mar Cirino-Marcano, Jay Dobkin, Changcheng Zhu

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Chest. 2011;140(4_MeetingAbstracts):15A. doi:10.1378/chest.1115215

INTRODUCTION: Fiberoptic bronchoscopy (FB) with transbronchial biopsy (TBBX) is a commonly used tool in assessing the etiology of pulmonary parenchymal infiltrates in immunocompromised patients.

CASE PRESENTATION: A 31 year-old male, non-smoker, underwent orthotopic liver transplant in May of 2010 for nonalcoholic steatohepatitis. Two months later, the patient was diagnosed with a pulmonary embolism, for which warfarin therapy was initiated. Five months post-operatively, the patient was hospitalized for abdominal pain and diarrhea. Cytomegalovirus (CMV) DNA quantitative serology was positive for which intravenous ganciclovir was prescribed. Warfarin was held. Aspirin 81mg daily was continued. Mycophenolate mofetil was discontinued but the patient remained on cyclosporine. Pathology from colonoscopy with biopsy was consistent with CMV colitis. Warfarin remained on hold and intravenous unfractionated heparin (IVUFH) was initiated. The patient also noted a nonproductive cough and dyspnea in association with findings of groundglass opacities in the bilateral lower lung fields on computed tomography (CT) of the chest. A bronchoscopy was scheduled. The IVUFH was held for 8 hours prior to the bronchoscopy. There was no thrombocytopenia or renal dysfunction. Pre-procedure PTT was 1.2 times the upper limit of normal (ULN) and INR was 1.40. FB with bronchoalveolar lavage (BAL) and TBBX of the middle lobe was completed without complication. IVUFH was resumed, without a bolus, 8 hours after completion of the procedure. No bacterial, fungal, or viral organisms were isolated from the BAL. Pathology revealed mild peribronchial chronic inflammation with immunostains negative for CMV. Six days following the procedure, the patient developed right-sided pleuritic chest pain and hemoptysis requiring transfer to the intensive care unit. Warfarin had previously been restarted and INR was 1.70 with PTT of 2.4 times the ULN. The patient was given four units of fresh frozen plasma and both warfarin and IVUFH were discontinued. CT scan of the chest revealed no evidence of a pulmonary embolism but was notable for a rounded consolidation predominantly within the right major fissure with partial extension into the middle lobe measuring 11.7 x 6.9 x 7.3 cm, consistent with a pulmonary hematoma. Repeat bronchoscopy revealed a large clot occluding the lateral segment of the middle lobe bronchus. Twenty-four hours later, the hemoptysis resolved. Anticoagulation for the prior pulmonary embolism was not resumed and a retrievable inferior vena cava (IVC) filter was placed. Four weeks later, anticoagulation was not restarted as the hematoma had not decreased in size. The IVC filter was not retrieved given the presence of a 40% occluding intraluminal thrombus. Two months after the episode of hemoptysis, the patient developed another pulmonary embolism and anticoagulation was restarted without incident. In consultation with hematology, the patient was recommended lifelong anticoagulation. Four months following the episode of hemoptysis, the pulmonary hematoma diminished to half of the original size and at six months it has nearly resolved.

DISCUSSION: Although generally well-tolerated, bleeding is the most commonly recognized complication of FB with TBBX in immunocompromised patients. Bleeding is typically minor (less than 100 mL), clinically insignificant, and occurs within the first 24 hours following the procedure. To our knowledge, this is the first reported case of FB with TBBX causing hemoptysis and pulmonary parenchymal hematoma in a liver transplant patient. This phenomenon has rarely been reported to occur 4 to 30 days following FB with TBBX in lung transplant recipients.

CONCLUSIONS: Physicians must be cognizant of pulmonary hematoma as a rare complication of FB with TBBX which can occur beyond 24 hours of procedure completion and appears to occur more frequently in patients who are immunocompromised.

Reference #1 Jain P, Sandur S, Meli Y, et al. Role of Flexible Bronchoscopy in Immunocompromised Patients With Lung Infiltrates. Chest. 2004; 125:712-722.

DISCLOSURE: The following authors have nothing to disclose: Amanda Godfrey, Daniel Ouellette, Krishna Thavarajah

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Chest. 2011;140(4_MeetingAbstracts):16A. doi:10.1378/chest.1119937

INTRODUCTION: Sarcoidosis is a multisystem disease which commonly involves pulmonary parenchyma and lymph nodes. Air flow obstruction due to sarcoidosis is common and is thought to be due to diffuse endobronchial involvement (1).Focal endoluminal stenosis has been described but is rare(2). We present a case of endobronchial sarcoidosis with endoluminal stenosis and discuss the role of endobronchial interventions in treatment.

CASE PRESENTATION: A 28-yo caucasian woman with diagnoses of asthma, sarcoidosis, and obesity was referred for worsening dyspnea, arthralgias, and facial erythematous rash. Symptoms were unresponsive to high-dose inhaled corticosteroids and bronchodilators. A trial of systemic glucocorticoids improved her rash and arthralgias, but her dyspnea persisted. Serial pulmonary function tests demonstrated worsening of both air flow obstruction and air trapping. A computed tomographic scan of her chest was read as normal. Flexible bronchoscopy revealed web-like obstruction of most segmental bronchi. Endobronchial biopsies of these abnormalities demonstrated non-caseating granulomas, confirming a diagnosis of endobronchial sarcoidosis. Serial interventional bronchoscopies using a combination of electrocautery, balloon bronchoplasty, and endobronchial steroid injection led to marked improvement in both symptoms and airflow parameters.

DISCUSSION: Web-like segmental airway obstruction is a rare but reported presentation of sarcoidosis. In our opinion patients with sarcoid who have air flow obstruction refractory to steroid therapy, at least one bronchoscopy is warranted.

CONCLUSIONS: This case highlights the potential utility of endobronchial interventions in treatment of this rare variant of sarcoid-related endobronchial stenosis.

Reference #1 Harrison BD, Shaylor JM, Stokes TC, Wilkes AR. Airflow limitation in sarcoidosis--a study of pulmonary function in 107 patients with newly diagnosed disease. Respir Med. 1991 Jan;85(1):59-64.

Reference #2 Chambellan A, Turbie P, Nunes H, Brauner M, Battesti JP, Valeyre D. Endoluminal stenosis of proximal bronchi in sarcoidosis: bronchoscopy, function, and evolution. Chest. 2005 Feb;127(2):472-81.

DISCLOSURE: The following authors have nothing to disclose: Nutan Bhaskar, Yousef Shweihat, Thaddeus Bartter

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Chest. 2011;140(4_MeetingAbstracts):17A. doi:10.1378/chest.1120126

INTRODUCTION: Bronchial-associated lymphoid tissue (BALT) lymphoma is a rare entity that usually accounts for 1% of all lymphomas 2. BALT lymphoma comprises two-thirds of all primary lung Non-Hodgkin’s Lymphoma, however, endobronchial lesions involving the trachea are uncommon1.

CASE PRESENTATION: This is a 91 year-old female who was referred to our institution after work-up at an outside hospital for shortness of breath, audible wheezing and hypoxia revealed a persistent soft-tissue density in the distal trachea with 2-dimensional size of 1.5X1.7 cm, with a tissue bulk of around 3.6 cm, extending distally into the right mainstem bronchus and bronchus intermedius. This was initially found on a CT-scan of the chest performed few months prior and was suspected to be a mucus plug and since the patient clinically improved on steroids and bronchodilators, it was not pursued further at that time. At our institution, she underwent a rigid and flexible bronchoscopy, which revealed a distal tracheal tumor with near complete occlusion of the distal trachea and a crescent shape opening towards the left mainstem bronchus. The lesion was found to be broad-based, globular in appearance, arising from the right lateral tracheal wall without any identifiable compromise of the right mainstem bronchus which was visualized after advancing the bronchoscope behind the lesion. The frozen section of the biopsy sent to pathology was suspicious for lymphoma. Mechanical tumor debridement was performed using flexible biopsy forceps as it was difficult to core out the lesion with the rigid barrel and complete airway patency was re-established. Final pathology revealed Low-grade B-cell lymphoma consistent with lymphoplasmacytic type. Immunostains were positive for B-cell antigens CD20 and CD79a. Background T-cells were positive for CD3, CD5 and CD43. Flow cytometry revealed 73% of the lymphocyte population to be B-cell with antigen positivity for CD19, CD20, HLA-DR and CD38. No other sites of lymphoma were identified. The post-operative course was complicated with a right pneumothorax which resolved with chest tube drainage and pneumomediastinum which resolved with conservative management. Her symptoms improved and the patient was discharged home with plans for outpatient oncology evaluation.

DISCUSSION: BALT lymphomas are rare and indolent tumors. The primary lesions involving the trachea are even more infrequent form of the NHL (Non-Hodgkin Lymphoma) of the lungs 1,2. These frequently are low-grade B-cell lymphoma and resemble closely to mucosa-associated lymphoid tissue 1. Chronic antigenic stimulation prompted by recurrent infection or auto-immune process may lead to aggregation of lymphoids evloving into BALT lymphoma 3. These lesions have been conventionally treated with radiation, chemotherapy and surgery with resection and/or reconstruction of the trachea 1.

CONCLUSIONS: Isolated endobronchial lymphoma is a rare entity but should be considered as a differential for endobronchial tumors. In patients, where other treatment modalities cannot be performed due to risks of potential complications, endoscopic tumor debulking might be an alternative option.

Reference #1 Primary tracheal non-Hodgkin's lymphoma. A case report and review of the literature. Fidias P, Wright C, Harris NL, Urba W, Grossbard ML Cancer. 1996 Jun 1;77(11):2332-8

Reference #2 Bronchial-associated lymphoid tissue lymphoma: a clinical study of a rare disease. Ahmed S, Kussick SJ, Siddiqui AK, Bhuiya TA, Khan A, Sarewitz S, Steinberg H, Sison CP, Rai KR. European Journal of Cancer. 2004 Jun;40(9):1320-6

Reference #3 A patient with endobronchial BALT lymphoma successfully treated with radiotherapy. Hashemi SM, Heitbrink MA, Jiwa M, Boersma WG. Respiratory Medicine. 2007 Oct;101(10):2227-9

DISCLOSURE: The following authors have nothing to disclose: Ali Ashraf, Ilan Yavitz, Antonio DeGorordo Arzamendi, Samaan Rafeq, Armin Ernst

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Chest. 2011;140(4_MeetingAbstracts):18A. doi:10.1378/chest.1113420

INTRODUCTION: Pseudallescheria boydii is a fungal organism known to affect immunocompromised patients. We report an unusual case of p. boydii pneumonia in an immunocompetent host with previous history of mycobacterium avium complex (MAC) pulmonary infection. This case highlights the importance of including uncommon fungal pathogens in the differential diagnosis of nodular pulmonary granulomatous diseases.

CASE PRESENTATION: A 62 year old female presented with persistent fever, dyspnea, cough, and worsening pulmonary nodules for two months. She had a history of pulmonary MAC infection diagnosed 2 ½ years earlier. Over two years she had been treated with clarithromycin, ethambutol, and clofazimine (based on susceptibilities). There was no history of steroid or tobacco use. Upon completion of the MAC treatment course, symptoms recurred within two months and included weight loss, fatigue, chills and occasional night sweats. Physical exam was positive for bilateral ronchi. There were no ocular or skin lesions. Laboratory testing was unrevealing. Chest radiograph displayed bilateral pulmonary infiltrates. Chest computed tomogram (CT) was remarkable for diffuse bilateral nodular infiltrates, more prominent in bases, and mediastinal lymphadenopathy. Bronchoscopic and CT guided needle biopsies were negative. An open lung biopsy was performed which was consistent with caseating granulomas; AFB and gram staining were negative. Based on high index of suspicion for recurrent infection, she resumed her previous anti-mycobacterial regimen and empiric antibiotics were started. After two months of treatment, there was lack of clinical improvement with persistent cough and intermittent fever. A repeat chest CT showed worsening of bilateral nodular infiltrates. The patient underwent a repeat bronchoscopy. Biopsy demonstrated again caseating granulomas. The BAL showed many pseudohyphae which were subsequently identified as P. boydii. Treatment with voriconazole (200 mg/day PO every 12 hours) improved her symptoms. She was able to gain weight and follow up imaging studies revealed improvement in the infiltrates.

DISCUSSION: Pseudallesheria boydii and its asexual anamorph, Scedosporium, are ubiquitous filamentous fungi found in soil, water, and sewage. P. boydii pneumonia affecting immunocompetent hosts is rare. It is a frequent pathogen in near drowning victims, especially in areas where P. boydii is endemic. This organism has septated, thin-walled, branching hyphae and an angioinvasive tendency. Clinically, P. boydii infection has an insidious onset and is often fatal in immunocompromised hosts. Central nervous system abscesses, rhinosinusitis, endophtalmitis, pneumonia, and skin lesions (madura foot) may be present. In cases where a specimen is obtained, Gomori methenamine silver stains P. boydii hyphae. The diagnosis of this organism is challenging as clinic findings and tomographic imaging are non specific. Chest CT abnormalities may display pulmonary cavitations or non cavitary masses, tree-in-bud nodules, ground-glass opacities, bronchial thickening, and mediastinal lymphadenopathy. The antifungal treatment varies according to immunologic status. Pseudallescheria boydii is generally sensitive to azoles. Voriconazole is the mainstay of treatment in immunocompetent hosts. Various reports indicate an intrinsic P. boydii resistance to polyenes. Surgical resection of pulmonary and central nervous system mycetomas is warranted.

CONCLUSIONS: This case highlights the importance of a high index of suspicion for superimposed fungal infections in patients who are refractory to medical treatment of bacterial pneumonitis such as MAC. Uncommon fungal pathogens should be considered in the differential diagnosis of nodular pulmonary granulomatous disease. Further diagnostic interventions are warranted when insufficient clinical improvement is observed to prevent treatment failure and adverse outcomes.

Reference #1 Lam SM, Lau AC, Ma MW, et al. Pseudallescheria boydii or Aspergillus fumigatus in a lady with an unresolving lung infiltrate, and a literature review. Respirology 2008;13(3):478-80.

DISCLOSURE: The following authors have nothing to disclose: Gustavo Cumbo-Nacheli, Ashish Maskey, David Holden

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Chest. 2011;140(4_MeetingAbstracts):19A. doi:10.1378/chest.1118551

INTRODUCTION: Lemierre’s disease is a complication of oropharygeal infection leading to septic thrombophlebitis of the internal jugular vein. We present a case of Lemierre’s disease in a patient presenting without classic history or examination findings.

CASE PRESENTATION: A 27 year old Caucasian male presented with headache, fever, chills, nausea, photophobia and night sweats for six days. He reported throbbing frontal headache 9/10 in intensity. He reported intermittent fever to 102°F without diurnal variation. Past medical and surgical histories were unremarkable. He served as an army tank driver who returned from Afghanistan 8 months before presentation and was poorly compliant with malaria prophylaxis. He smokes 10 cigarettes daily and denied alcohol or drug use. Upon admission, he was afebrile, tachycardic with 100% O2 saturation on room air. Physical examination was unremarkable except for photophobia and a palpable spleen tip. Laboratory revealed a normal WBC count with elevated neutrophils and banded cells, and thrombocytopenia (32,000 /mm3). Serum chemistries were unremarkable. Chest X-ray and head CT were negative, and CT of the abdomen and pelvis showed splenomegaly. Thin and thick smears for malaria and lumbar puncture were negative. He was started on broad spectrum antibiotics. After two days, he required transfer to the intensive care unit for respiratory distress where he was intubated. Repeat chest X-ray showed diffuse bilateral infiltrates suggestive of developing ARDS, and admission blood cultures were then positive for gram negative bacteria. Transthoracic echocardiogram did not show evidence of endocarditis. He became hypotensive and an ultrasound was performed to assist placement of a central venous catheter in the right internal jugular vein. Ultrasound showed non-compressible right internal jugular vein suggestive of deep vein thrombosis. CT neck confirmed thrombosis running through the entire length of the right internal jugular vein, consistent with the diagnosis of Lemierre’s disease. Chest CT showed diffuse infiltrates with areas of cavitation, consistent with multiple septic emboli. The gram negative bacteria were identified as Fusobacterium Necrophorum and treatment with piperacillin-tazobactam was continued. He improved clinically, was successfully extubated, and subsequently discharged home on oral amoxcillin-clavulanate for four weeks. Thrombocytopenia had resolved at the time of discharge.

DISCUSSION: Lemierre’s disease, also known as post anginal sepsis, or the "forgotten disease", is a rare complication of acute oropharyngeal infection resulting in septic thrombophlebitis of the internal jugular vein with septicemia and metastatic infections. The incidence varies between 1-14 per million cases per year. It is typically seen in young, immunocompetent adults, with a mean age at diagnosis being 20 years. Typical course has three phases. Phase one is the initial infection, which most commonly involves palatine tonsils. Phase two involves lymphangitic spread to the lateral pharyngeal space including thrombophlebitis of internal jugular vein. Phase three involves seeding of septic thromboemboli to various organs, with pleuropulmonary involvement being the most common. Classic Lemierre’s disease is caused by a mono-infection with fusobacterium species. Physical signs include a tender palpable neck mass with cervical lymphadenopathy. Our patient’s presentation was atypical in that there was neither antecedent throat infection nor typical physical findings of Lemierre’s disease. Diagnosis is established by doppler ultrasound or CT with contrast. Due to increasing beta lactam resistance, empiric therapy should include beta lactamase resistant antibiotics. Typical duration of therapy is 4 -6 weeks. Anticoagulation and surgery remain controversial but may be considered if there is documented evidence of clot extension despite adequate antibiotic therapy.

CONCLUSIONS: Lemierre’s disease should be suspected in a young person with antecedent pharyngitis, septic pulmonary emboli and persistent fevers despite antibiotic therapy.

Reference #1 1. Ruirdan T. Human infection with Fusobacterium necrophorum (Necrobacillosis) with a focus of Lemierre’s syndrome. Clin Microbiol Rev 2007;20:622-59.

DISCLOSURE: The following authors have nothing to disclose: Nishant Gupta, Elise Chambers, Dennis McGraw

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Chest. 2011;140(4_MeetingAbstracts):20A. doi:10.1378/chest.1119918

INTRODUCTION: Varicella-zoster infection is typically a self-limited disease of childhood. Disseminated varicella-zoster occurs more commonly in immunocompromised patients and carries a mortality rate of 80%. We report a case of disseminated varicella-zoster that presented with severe right upper quadrant (RUQ) pain several days before the rash in a patient with chronic lymphocytic leukemia (CLL).

CASE PRESENTATION: A 59-year-old woman presented to another institution with five days of severe RUQ pain not related to food intake. She had history of cholecystectomy and CLL in remission (last chemotherapy six months before admission). Physical exam was pertinent for RUQ tenderness. The initial laboratory workup, including liver enzymes, was unrevealing. Imaging studies including CT abdomen, IV pyelogram, and Endoscopic Retrograde Cholangiopancreatography were normal. A biliary stent was placed despite patent bile ducts. Patient developed thrombocytopenia and a petechial rash over the trunk and extremities. On admission to our institution, small red papules were noticed in the trunk, buttocks and vaginal area. Biopsy showed purpura but no vasculitis. Patient developed elevated transaminases and direct bilirubin. Peripheral smear showed toxic changes in white cells. Autoimmune and viral markers were negative. She required mechanical ventilation for worsening mental status and hypoxemic respiratory failure. Skin lesions became crusty with concomitant active lesions in different areas. Given high suspicion for disseminated varicella-zoster, acyclovir was started. A second skin biopsy showed intranuclear viral inclusions consistent with varicella-zoster infection. Intravenous Immunoglobulin (IVIG) was started given severity of disease, based on evidence from isolated case reports. She completed two weeks of acyclovir and five days of IVIG with dramatic clinical improvement. She was started on high-dose prednisone as thrombocytopenia was felt secondary to an autoimmune process triggered by the viral infection. Her liver enzymes, platelet count, hypoxemia and mental status improved significantly. She was extubated and discharged a few days later.

DISCUSSION: Varicella-zoster infection is typically self-limited, presenting with fever and malaise for one to two days, and followed by a vesicular rash. Patients with profound immunosuppression are at the greatest risk of dissemination. Organs usually involved are the lungs, liver and central nervous system. The case we present was unusual, as it presented with severe RUQ pain and was complicated by pneumonia, hepatitis, thrombocytopenia and central nervous system involvement. There are some reports of diffuse abdominal pain as the presenting symptom for disseminated varicella-zoster in immunosuppressed patients.1 To the best of our knowledge, this is the first report of disseminated varicella presenting with RUQ pain. We believe the cause of the abdominal pain was neuropathic, as the workup including blood and imaging studies was unremarkable. The pulmonary involvement was likely varicella-zoster pneumonitis as chest radiography showed diffuse interstitial infiltrates and respiratory cultures were negative before antimicrobials were started. The liver abnormalities were secondary to varicella-zoster hepatitis as the initial liver enzymes, viral workup and imaging studies were unremarkable. Varicella-zoster is notorious for causing autoimmune hematologic abnormalities2, this patient developed thrombocytopenia that could not be otherwise explained and resolved with treatment. Although we could not document varicella-zoster in the CSF, we strongly believe she had varicella-zoster encephalitis given the disseminated presentation. The atypical appearance of the rash raised other possibilities like leukocytoclastic vasculitis and TTP that were ruled out by our extensive workup.

CONCLUSIONS: Varicella-zoster should be considered in the differential diagnosis of immunocompromised patients presenting with abdominal pain, especially when blood and imaging studies fail to reveal the etiology. The presented case is unique because of its presentation, severity of disease and response to therapy.

Reference #1 Magi E. Severe varicella in an immunocompromised adult presenting with abdominal pain. West J Med 2000;173:376-7.

Reference #2 Ali N. Chickenpox associated thrombocytopenia in adults. J Coll Physicians Surg Pak 2006;16:270-2.

DISCLOSURE: The following authors have nothing to disclose: Angel Coz Yataco, Ayan Sen, Rana Awdish

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Chest. 2011;140(4_MeetingAbstracts):21A. doi:10.1378/chest.1118222

INTRODUCTION: Paragonium westermani is food borne parasite endemic to Southeast Asia. These infections in United States are commonly reported in immigrants. The route of transmission is from ingestion of uncooked crabs, crayfish or wild boar meat.

CASE PRESENTATION: 49 year old Burmese man with no past medical history of asthma was referred to our hospital due to persistent hemoptysis. He had recently emigrated from Thailand to United States. In Thailand his diet was rich in crabs. His Tuberculin skin test came back positive but sputum AFBs were negative. Chest x-ray done at that time showed right upper lobe cavity. Despite on anti-tubercular medications for 4 months he continued to have hemoptysis. He did not have any constitutional symptoms. Computed Tomography of chest showed small cavitary lesion in the right upper lobe with loculated pleural effusion along with ground glass opacity in right lung. He underwent bronchoscopy for evaluation of cavitary lung lesion which was unresponsive to antitubercular therapy. Complete blood count and bronchoalveolar lavage revealed eosinophilia. With suspicion of parasitic infection albendazole was started. On regulare follow ups in the pulmonary clinic, the patient continued to have persistent cough, hemoptysis and eosinophilia even after 7 months of treatment with albendazole. The patient underwent a repeat bronchoscopy .This time the trans bronchial biopsy showed Paragonium westermani eggs and patient was put on praziquental. Follow up Computed Tomography of chest after 2 months of treatment showed decrease in size of the cavitary lesions and infiltrates, with resolution of hemoptysis and peripheral eosinophilia.

DISCUSSION: Pulmonary Paragonimiasis is very rare in United States and has non- specific symptoms due to which there is always a delay in diagnosis. Patients frequently undergo lung biopsies for diagnosis and most of the times histopathology shows oval operculated eggs, with birefringence as seen in our patient. Radiological findings vary from pleural thickening to unilateral or bilateral effusions, and pneumothoraces. Parenchymal changes include consolidation, ground-glass opacities, nodular lesions that may cavitate. Thus the infection may mimic neoplasia, fungal, or mycobacterial infection.

CONCLUSIONS: Diagnosing atypical infections in immigrant population in United States is always a challenge which delays treatment and recovery of the patient. Pulmonary Paragonimiasis is frequently indistinguishable from Pulmonary Tuberculosis, leading to improper and inadequate treatment. High index of suspicion should be kept in mind in this era of globalization.

Reference #1 Lane MA, Barsanti MC, Santos CA, et al. Human paragonimiasis in North America following ingestion of raw crayfish. Clinical Infectious Disease 2009;49:e55-e61

Reference #2 Castilla EA, Jessen R, Sheck DN, et al. Cavitary mass lesion and recurrent pneumothoraces due to Paragonimus kellicotti infection: North American paragonimiasis. Am J Surg Pathol. 2003;27:1157-1160

Reference #3 Mukae H, Taniguchi H, Matsumoto N, et al . Clinicoradiologic features of pleuropulmonary Paragonimus westermani on Kyusyu Island, Japan. Chest 2001;120 :514-20

DISCLOSURE: The following authors have nothing to disclose: Ashima Sahni, Aiyub Patel, Maximiliano Tamae Kakazu

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Chest. 2011;140(4_MeetingAbstracts):22A. doi:10.1378/chest.1118171

INTRODUCTION: Coccidioidomycosis infection is endemic in the Southwestern United States and is usually asymptomatic. Pulmonary coccidioidomycosis classically presents with cough and a flu-like illness. We report an unusual case of a patient who presented with significant dysphagia and weight loss secondary to bulky mediastinal lymphadenopathy caused by coccidioidomycosis.

CASE PRESENTATION: A 60-year-old Caucasian male presented to the emergency department with complaints of progressive dysphagia to solids accompanied by nausea, weight loss and cough with blood-streaked sputum that developed within 3 months. He also reported night sweats, fatigue and dyspnea on exertion. He was a resident of Southern California, a lifelong non-smoker, and had no TB risk factors. He was evaluated with a CT chest which revealed extensive pretracheal, subcarinal and right hilar lymphadenopathy and apparent narrowing of his esophagus. Also present was post-obstructive atelctasis of the superior segment of the right lower lobe with a small hypodense area within the atelectatic segment. Laboratory data were equivocal with positive coccidioidomycosis antibodies and negative complement fixation. Because of the hemoptysis and the suspicion for malignancy, a bronchoscopy was performed which revealed a splayed carina and hyperemic airways but no extrinsic compression or endobronchial lesions. Subcarinal transbronchial needle aspiration and transbronchial biopsies of the right lower lobe were negative for malignancy, granulomas, or organisms. The bronchoalevolar lavage showed spherules and the cultures returned positive for C. immitis. Fluconazole treatment was initiated and the patient was discharged from the hospital shortly thereafter. A follow up CT chest a month and a half later showed slight improvement of the lymphadenopathy, mild increase in the hypodense lesion within the right lower lobe, and new small right pleural effusion. The patient remained symptomatic with persistent dysphagia, cough and dyspnea while on lengthy treatment. Subsequently, the patient was re-admitted to the hospital with acute shortness of breath, fever and pleuritic chest pain. Chest imaging confirmed the presence of a loculated pleural effusion due to rupture of a right lower lower subpleural fluid-filled cavity. The patient clinically deteriorated requiring ICU admission for hypoxemia and increased work of breathing. A tube thoracostomy was performed and the patient's antifungal regimen was broadened to liposomal amphotericin B. The patient improved after one week and was transitioned to oral posiconazole upon discharge.

DISCUSSION: Despite the prevalence of coccidioidomycosis infection in Southern California, its course and presentation are not always predictable. Cocciodioidomycosis is a great imitator of other pulmonary diseases. Our patient's initial presentation with dysphagia, hemoptysis and bulky mediastianal and hilar adenopathy was more consistent with bronchogenic carcinoma or other malignancies such as lymphomas. In this patient, the diagnosis of cocciodioidomycosis infection was not initially apparent. Although the coccidioidal antibodies were positive, it is not uncommon for patients living in endemic areas to have seroconversion without clinically evident disease. The complement fixating antibody is a more sensitive and specific test for the diagnosis of active infection, but was negative in our patient. Malignancy was ruled out by bronchoscopy, however, the diagnosis of coccidioidomycosis infection was confirmed one week later via positive culture for c.immitis. The dysphagia in this patient was caused by the unusually bulky lymphadenpathy that was impinging on the distal esophagus. Other reported cases of dysphagia associated with cocciodioidomycosis infection had been attributed to laryngeal involvement. Another interesting aspect of this case was the patients clinical deterioration while on treatment with fluconazole. In most cases, fluconazole is sufficient to achieve clinical resolution, however, our patient's course was further complicated by the the rupture of a right lower lobe pleural-based fluid-filled coccidioidomycosis cavity, causing a large pleural effusion, necessitating chest tube thoracostomy and initiation of intravenous liposomal amphotericin B.

CONCLUSIONS: Our case exemplifies that pulmonary coccidioidomycosis, while common in certain endemic areas, can present atypically. To our knowledge, this is the first reported case of pulmonary coccidioidomycosis infection presenting with dysphagia due to the mediastinal lymphadenopathy, with initial radiographic findings mimicking malignancy.

Reference #1 Coccidioidomycosis with Endolaryngeal Involvement. Harkishen Singh, D.M.D., M.D.; Charles J. Yast, M.D.; John H. Gladney, M.D.; AMA Arch Otolaryngol. 1956;63(3):244-247.

Reference #2 Unusual presentations of coccidioidomycosis: a case series and review of the literature. Crum-Cianflone NF, Truett AA, Teneza-Mora N, Maves RC, Chun HM, Bavaro MF, Hale BR. Medicine (Baltimore). 2006 Sep;85(5):263-77.

DISCLOSURE: The following authors have nothing to disclose: Keren Fogelfeld, Nader Kamangar

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Chest. 2011;140(4_MeetingAbstracts):23A. doi:10.1378/chest.1119777

INTRODUCTION: Blood in the pleural fluid is often an ominous sign of an underlying life-threatening disease entity, most commonly malignancy, infection, or embolism. Diaphragmatic hernias are also a well documented cause, whether congenital in children or traumatic in adults. Rarely, it can be caused by non-traumatic diaphragmatic hernia in adults. Here we describe a case of a hiatal hernia causing bloody pleural effusion.

CASE PRESENTATION: An 87-year-old Caucasian male with history of chronic obstructive pulmonary disease and hypertension who underwent gastropexy for incarcerated gastric hernia two years prior to this encounter, presented to the emergency department with symptoms of dyspnea, weakness, and confusion. On exam, he was hypotensive with wheezes bilaterally and decreased breath sounds at the right lung base. Bowel sounds were hypoactive. Chest x-ray revealed infiltrates on the right side consistent with pneumonia and possible pleural effusion. He was admitted to the intensive care unit and started on antibiotics. The next morning, chest x-ray revealed significant worsening of his pleural effusion. Computed tomography images of the chest revealed the large pleural effusion as well as multiple loops of dilated small bowel. On thoracentesis, about 1000 ml of serosanguinous pleural effusion was evacuated. Patient was subsequently taken to the operating room as there was a high concern for necrotic bowel in the chest. After attempts at reducing the hernia by laparatomy were unsuccessful, thoracotomy was performed. In addition to bloody effusion, a significant amount of black-colored small bowel was found to be trapped in the chest. Necrotic bowel was resected and pleural effusion was drained. Thereafter, patient had a slow recovery, but no recurrence of pleural effusion.

DISCUSSION: Traumatic diaphragmatic hernia has been associated with bloody pleural effusions but there have been very few cases unrelated to trauma in adults. There has been case reports published of non-traumatic diaphragmatic hernias and late onset Bochdalek hernias causing bloody pleural effusion. The proposed mechanism is that there is extravasation of blood from stasis due to omental strangulation, which likely explains the findings in our patient. Although our patient had no symptoms of obstruction, studies have shown the presence of asymptomatic diaphragmatic hernias, found incidentally on imaging, in up to 0.17% of adult subjects.

CONCLUSIONS: Blood in the pleural fluid may provide an additional clue to consider incarcerated diaphragmatic hernia in the differential diagnosis. Our patient is the first documented case to our knowledge of specifically a hiatal hernia causing bloody pleural effusion without history of trauma.

Reference #1 Murchison WG, Harper WK, Putnam JS. Traumatic diaphragmatic hernia; late presentation as bloody pleural effusion. Chest. 1974 Dec; 66(6):734-6.

Reference #2 Schreier L, Cutler RM, Saigal V. Bloody pleural effusion secondary to infarction of omentum through a non-traumatic diaphragmatic hernia. Chest.1988 Jun; 93(6):1314.

Reference #3 Shimizu T, Hira S, Hirooka S, Yonekura T, Tamai H. Late onset of right Bochdalek's hernia with strangulation of the omentum. Acta Paediatr. 2002; 91(4):483-5

DISCLOSURE: The following authors have nothing to disclose: Muhammad Siddique, Jonaid Aslam, Mohammad Syed, Misbat Chaudry, Joseph Henkle

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Chest. 2011;140(4_MeetingAbstracts):24A. doi:10.1378/chest.1119830

INTRODUCTION: B-cell lymphoma usually presents as a constillation of symptoms such as weight loss, fevers, loss of appetite and fatigue along with physical exam and radiographic evidence lymphadenopathy. We present a case of lymphoma presenting primarily as a pleural effusion without physical exam or radiographic findings of enlarged lymph nodes.

CASE PRESENTATION: The patient is an 83 year old male who came to the emergency room with weakness, increasing shortness of breath and lower extremity swelling. He has a history of metastatic prostate cancer, diabetes, congestive heart failure, deep venous thrombosis, and was recently discharged from the hospital for gram negative sepsis. On examination, he was cachectic with no palpable lymph nodes but with a firm mass felt on the abdominal left upper quadrant and left flank. He had decreased breath sounds over the left thorax with dullness to percussion up to the third intercostal space. A helical CT of the chest to rule out pulmonary embolism was performed which revealed no embolism, but showed a large left pleural effusion, numerable non-enlarged mediastinal lymph nodes and blastic lesions within the cervical spine. Ultrasound of the abdomen showed a normal-sized spleen (10.7x3.2cm). A diagnostic and therapeutic thoracentesis was performed which revealed 6,800 nucleated cells and 14,800 red blood cells. The lactate dehydrogenase (LDH) in pleural fluid is 1411 and glucose of 46 with serum glucose of 105 and LDH of 935. Cytology of the nucleated cells revealed CD10 positive large B-cell lymphoma.

DISCUSSION: In patients with metastatic disease, the possibility of a new primary cannot be excluded. In this case, the pre-existing diagnosis of prostate cancer and multiple bone lesions would have led to an incorrect conclusion that the prostate cancer was the source of the pleural effusion, especially in the setting of bone metastasis. As only a small portion of metastatic prostate cancer presents with abnormalities on chest radiography, further examination of the pleural fluid was definitely warranted. This rare presentation of B-cell lymphoma underscores the reason that tissue is necessary prior to treatment.

CONCLUSIONS: n this case, large B-cell lymphoma primarily in the pleural fluid was diagnosed in a patient with another known malignancy. It is important to have a broad differential when a new pleural effusion is discovered.

Reference #1 Celikoglu F, Teirstein AS, Krellenstein DJ, Strauchen JA. Pleural effusion in non-Hodgkins lymphoma. Chest 1992;101:1357-1360

Reference #2 Antony VB, Loddenkemper R, Astoul P, Boutin C, Goldstraw P, Hott J, Rodriguez Panadero F, Sahn SA. Management of malignant pleural effusions. Eur Respir J. 2001;18(2):402-419.

Reference #3 Y. Lim, T.-Y. Kim, I. S. Choi, B.-S. Kim, T. S. Lee, J. E. Kim, M. S. Chang, and K. H. Kim Diffuse Large B-Cell Lymphoma With Germinal Center B-Cell Phenotype Mimicking Primary Effusion Lymphoma J. Clin. Oncol., April 1, 2011; 29(10): e271 - e273.

DISCLOSURE: The following authors have nothing to disclose: Irtza Sharif, Navneet Arora, Mark Regala, Christina Migliore

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Chest. 2011;140(4_MeetingAbstracts):25A. doi:10.1378/chest.1077522

INTRODUCTION: Recurrent unilateral pleural effusion in a patient with chronic pancreatitis should heighten suspicion of a pancreatico-pleural fistula. The predominance of pulmonary findings with minimal clues to pancreas as the initiator of the effusion make this diagnosis challenging.

CASE PRESENTATION: A 54-year old male with medical history of chronic alcoholic pancreatitis presented to his primary physician’s office with increasing shortness of breath and severe weakness. Since he had decreased breath sounds on left side, he was sent for a chest radiograph, which confirmed a large left pleural effusion. Pleural fluid analysis confirmed yellow exudative effusion, but no evidence of infection or malignancy. Pleural fluid analysis showed pH 8.0, protein 2.0 g/dL, LDH 8603 U/L, but no amylase or lipase levels were drawn. Despite a video-assisted thoracoscopic surgical decortication and two tube thoracostomy procedures within next nine months, he developed a loculated left effusion. He was admitted to outside hospital with acute pancreatitis, and soon developed multi-organ failure requiring ventilation, ionotropic support and dialysis. CT chest confirmed large left sided effusion with patchy airspace consolidation. Intravenous antibiotics were followed by chest tube insertion and drainage. He improved clinically with supportive measures, and was weaned off the ventilator. In the next few days, his effusion became larger and he developed a hydro-pneumothorax as a complication of multiple thoracic interventions. On transfer to our facility, he had decreased left sided breath sounds on auscultation. CBC and electrolytes were in normal range but amylase (243 U/L) was elevated. Malnutrition was confirmed with albumin (1.9 g/dL) and pre-albumin (9.0 mg/dL). Thorax tomography confirmed large loculated hydro-pneumothorax. No esophageal perforation was seen. Ultrasound guided thoracocentesis yielded grayish pleural fluid with elevated amylase (19,987 U/L) and lipase (16,125 U/L). All cultures remained negative. Pleural fluid analysis was significant for pH 7.25, triglyceride 41 mg/dL, protein - 1.6 g/dL, LDH - 3818 IU/L, cholesterol - 9 mg/dL, chylomicrons- negative, and Cytology negative. A distinct fluid collection intimately related to the tail of pancreas with a fistulous tract piercing the diaphragm was seen on MRI abdomen with MRCP. Initial non-surgical treatment included endoscopic retrograde cholangiopancreatography with pancreatic stent and octreotide. Total parenteral nutrition was initiated. Three months later, conservative measures failed to close the fistula. The fistulous tract was surgically removed with distal pancreatectomy.

DISCUSSION: This case emphasizes the importance of performing additional testing on pleural fluid if routine laboratory data fail to yield a diagnosis. Patients with chronic alcohol use may have minimal abdominal complaints except mild pain and malnutrition.

CONCLUSIONS: A high index of suspicion is required, in those with a large (usually left-sided) pleural effusion and history of alcohol abuse or chronic pancreatitis, with addition of amylase and lipase levels to pleural fluid analysis. Other causes of high amylase in pleural fluid include acute pancreatitis (small, transient, usually left sided), esophageal perforation, and certain tumors.

Reference #1 Rockey DC, Cello JP. Pancreaticopleural fistula. Report of 7 patients and review of the literature. Medicine (Baltimore). 1990;69:332-344

Reference #2 Materne R, Vranckx P, Pauls C, et al. Pancreaticopleural fistula: diagnosis with magnetic resonance pancreatography. Chest. 2000;117:912-914

Reference #3 Poddar U, Kochhar R, Singh A, et al. Pancreatcopleural fistula: successful treatment with octreotide. Indian J Gastroenterol. 1995;14:145-146 Burgess NA, Moore HE, Williams JO, Lewis MH. A review of pancreatico-pleural fistula in pancreatitis and its management. HPB Surg. 1992;5:79-86 Saeed ZA, Ramirez FC, Hepps KS. Endoscopic stent placement for internal and external pancreatic fistulas. Gastroenterology. 1993;105:1213-1217

DISCLOSURE: The following authors have nothing to disclose: Vipul Kumar, Shashank Jain, Anthony Donato

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Chest. 2011;140(4_MeetingAbstracts):26A. doi:10.1378/chest.1120055

INTRODUCTION: The pulmonary complications of inflammatory bowel disease (IBD) have been well described in the past, including the rare manifestation of pleuritis and pleural effusions. The characteristics of pleural fluid in Crohn’s pleuritis have rarely been reported. We report a case of pleuritis and myopericarditis in a patient with Crohn’s disease.

CASE PRESENTATION: A 29-year-old woman with Crohn’s disease (CD) on mesalamine therapy presented to our institution with a 3-day history of progressively worsening dyspnea and pleuritic chest pain. The patient had been in her usual health until one week prior to admission when she developed a severe sore throat. Vital signs on admission were temperature 97.3 F, blood pressure 118/70, pulse 124 beats per minute, respiratory rate 26/min and pulse-oximetry 99% on 2L/min of oxygen via nasal cannula. Pertinent physical exam included decreased breath sounds at the bases bilaterally and a normal cardiac exam. Admission labs were significant for white blood cell count 22,900/cu.mm with 91% neutrophils, C-reactive protein (CRP) 28.3 mg/dL, sedimentation rate 86mm/hr and troponin 1.29ng/mL. Electrocardiogram revealed sinus tachycardia without ST-T wave changes. Chest radiograph revealed bilateral pleural effusions. CT scan of chest revealed bilateral loculated pleural effusions and cardiomegaly with moderate pericardial effusion. No pulmonary embolism was identified. Thoracic ultrasound of the left pleural effusion identified septations and stranding within the fluid. Pleural fluid analysis was significant for a pH of 6.9, WBC = 1100/ uL with 90% neutrophils, no eosinophils, LDH = 3327 U/L, glucose< 20 mg/dl, protein = 3.5 g/dl and amylase <30 U/L. Right sided pleural fluid analysis showed similar characteristics. An extensive panel of serology for rheumatologic and viral causes were negative. Microbiological cultures from the blood and pleural fluid were also negative. The patient was started empirically on vancomycin, piperacillin/tazobactam, and metronidazole and a chest-tube was placed for drainage of the left pleural effusion. An esophagram was performed and a fistula between the gastrointestinal tract and the thoracic cavity was ruled out. Transthoracic echocardiogram showed a small pericardial effusion, normal ventricular function and no vegetations. By day 3, a peak troponin level was noted to be 8.42ng/ml. The patient did not show any improvement in her pleurisy and shortness of breath symptoms despite broad antibiotic therapy. Methylprednisone and high dose aspirin were then initiated for treatment of presumed inflammatory pleuritis and myopericarditis related to Crohn’s disease. Within two days, the patient’s dyspnea and pleurisy improved and CRP decreased to 3.4 mg/dL. Colonoscopy was performed revealing mildly congested mucosa in the recto-sigmoid colon and the pathology was consistent with mild nonspecific inflammation.

DISCUSSION: IBD is known to produce a multitude of extraintestinal manifestations. The pulmonary manifestations of IBD are more common than generally appreciated and are quite varied. The large airways are the most common site with bronchiectasis being the typical pulmonary manifestation. Pleura, myocardium and pericardium are relatively uncommon locations for manifestations of IBD and the patients are typically male, with ulcerative colitis (UC). Pleuro-myopericarditis can also occur in the quiescent phase of inflammatory bowel disease as in our patient but no case reports to our knowledge have reported the characteristics of pleural fluid in these cases. Our patient was a female with CD, as opposed to UC, and had bilateral complicated pleural effusion with myopericardial involvement. The pleural fluid characteristics were similar to pleural effusions from other rheumatologic diseases like systemic lupus and rheumatoid arthritis, however laboratory tests to support these diagnoses were all negative. Such pleural fluid characteristics can also be seen in parapneumonic effusions, however the patient had no clinical symptoms of pneumonia and did not respond to antibiotic therapy. Our patient responded dramatically to anti-inflammatory therapy with steroids and aspirin as has been described in previous reports of pleuropericarditis.

CONCLUSIONS: Pleuro-myopericarditis can occur in the dormant phase of inflammatory bowel disease and present with exudative bilateral pleural effusions. This condition usually responds well to anti inflammatory therapy.

Reference #1 Patwardhan RV, Heilpern RJ, Brewster AC, Darrah JJ. Pleuropericarditis: an extraintestinal complication of inflammatory bowel disease. Report of three cases and review of literature. Arch Intern Med. 1983 Jan;143(1):94-96

Reference #2 Michèle Faller,Bernard Gasserb,Gilbert Massardc, Gabrielle Paulid,Elisabeth Quoix: Pulmonary Migratory Infiltrates and Pachypleuritis in a Patient with Crohn’s Disease. Respiration 2000;67:459-463

DISCLOSURE: The following authors have nothing to disclose: Erwin Moy, Jaspreet Ahuja, Joseph Mathew, Pierre Kory, Arthur Sung, Patricia Walker

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Chest. 2011;140(4_MeetingAbstracts):27A. doi:10.1378/chest.1118748

INTRODUCTION: Medical thoracoscopy has proven useful in diagnosing both malignant and benign causes of pleural effusions. This technique has advantages over traditional video-assisted thoracoscopy (VATS) because of its ability to be performed under moderate sedation, obviating the need for intubation and single-lung ventilation, while achieving a high diagnostic yield and low complication rates.(1) Frequently, cryptogenic exudative effusions are later found to be related to malignancy or tuberculosis. We report an unusual case of recurrent exudative pleural effusion due to amyloid, where medical thoracoscopy was essential to the diagnosis and treatment.

CASE PRESENTATION: A 61 year-old female with a past medical history of primary AL amyloidosis with cardiac involvement diagnosed by tongue biopsy 10 years prior was referred to the interventional pulmonology clinic for recurrent pleural effusion and dyspnea occurring during the previous six months. Her amyloid had initially been treated with stem cell transplant followed by trials of melphalan and steroids with partial response. More recently, she demonstrated good hematologic response to a regimen of bortezomib, cytoxan, and dexamethasone. Four previous large volume thoracenteses had yielded temporary symptomatic relief. Fluid analysis indicated an exudative effusion in the absence of infection or malignancy. Physical exam revealed a Mallampati IV anatomy, macroglossia, as well as decreased breath sounds and decreased fremitus over the right hemithorax 2/3 of the way up. For diagnosis and management, we proceeded with medical thoracoscopy in the endoscopy suite under moderate sedation with ultrasonographic guidance. The procedure was done using a semi-rigid thoracoscope through a 10 mm trocar. The patient required only 0.5 mg of midazolam and 200 mcg of fentanyl throughout the procedure. Local anesthesia was achieved with 25mL of 1% lidocaine. 1500 ml of serous turbid fluid was drained. Most of the pleural space was visualized and revealed normal-appearing pleura. Multiple biopsies of the parietal pleura were obtained. A Pleurx catheter was placed under vision, and five grams of sterile talc was then insufflated with thoracoscopic guidance. A 24 F chest tube was also placed to ensure full lung re-expansion. Post-operatively, the intent was to pull the 24F chest tube at 24 hours, however due to inadequate lung re-expansion, the tube was removed at day 5 and she was discharged home with daily Pleurx drainage. Three days after discharge, she achieved adequate pleurodesis, and her Pleurx was removed.

DISCUSSION: Medical thoracoscopy was first described in the medical literature in the early 1900s by Jacobeus (2). Previously more popular in Europe; medical thoracoscopy has become more readily available in North America due mostly to rapid expansion of the field of interventional pulmonology and the availability of a semi-rigid thoracoscope. Nonetheless, medical thoracoscopy remains an underutilized procedure despite having significant benefits over VATS in many patients. As demonstrated in this case, the patient’s co-morbidities, in particular, macroglossia, necessitated an alternative to deep sedation and intubation. Thoracoscopy was not only instrumental in obtaining a diagnosis through pleural biopsy, but was also effective in treating the recurrent effusion by talc insufflation and pleurx catheter placement, while requiring only minimal sedation. Given the reduced morbidity and relative ease of this procedure in the hands of a trained interventional pulmonologist, medical thoracoscopy should be considered first whenever access to the pleural space is necessary for diagnosis or treatment. VATS should clearly be utilized for more complicated effusions.

CONCLUSIONS: This is a unique case of pleural amyloid successfully diagnosed and treated by medical thoracoscopy. This approach should be considered as a useful, less morbid alternative to managing cryptogenic pleural effusions.

Reference #1 Michaud G et al. Pleuroscopy for diagnosis and therapy for pleural effusions. Chest. 2010 Nov;138(5):1242-6.

Reference #2 Jacobeus HC . Ueber die Möglichkeit die Zystoskope beiuntersuchung seröser höhlungen anzuwenden . Munch Med Wochenschr . 1910 ; 40 : 2090 - 2092

DISCLOSURE: The following authors have nothing to disclose: Melhem Imad, Sonali Bose, Daniel Jamieson, Lonny Yarmus, David Feller-Kopman

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Chest. 2011;140(4_MeetingAbstracts):28A. doi:10.1378/chest.1107774

INTRODUCTION: Pneumonectomy is a treatment option for certain types of bronchogenic carcinoma and some non-malignant pulmonary diseases. There are predictable post-operative adaptive anatomic and physiologic changes which may affect cardiac, pulmonary, and/or gastrointestinal systems. In the immediate post-operative period, the post-pneumonectomy space (PPS) is largely preserved by the combination of air and pleural effusion. Over the course of 6-12 months, the mediastinum generally shifts towards the PPS as air and fluid in the cavity are partially resorbed and replaced with scar tissue.

CASE PRESENTATION: A 65 year-old man with a history of hypertension, diabetes, hyperlipidemia, and heart disease presented with a complaint of cough. He had a history of left pneumonectomy for squamous cell carcinoma of the lung 13 years ago. He reported a worsening nonproductive cough and dyspnea over 6 months. He now experienced significant dyspnea with walking as well as with activities of daily living. He had fevers, drenching night sweats, and a 25 pound weight loss over the last 2 months. He had a 50 pack-year history of smoking, quitting over twenty years ago. On examination, the patient had normal vitals without hypoxia. Physical exam was remarkable for decreased heart sounds anteriorly with preserved breath sounds bilaterally. There was dullness to percussion in the left lower lung field. Initial basic laboratory was unremarkable. The patient’s chest radiograph, however, showed aerated lung fields in the left upper hemithorax with shift of the trachea and mediastinum leftwards. Computed tomography of the chest revealed complete herniation of the right lung anteriorly into the left hemithorax. The mediastinum was shifted so far leftward and posteriorly as to occupy the most posterolateral space abutting the left hemidiaphragm with mild clockwise rotation of the heart. There was a prominent right hilar mass with postobstructive changes to the right upper lobe and a mass in the right middle lobe abutting the heart. The mediastinal window revealed an enlarged right paratracheal lymph node and subcarinal lymph node. Bronchoscopic evaluation revealed a normal appearing left mainstem post-surgical stump and a counter-clockwise rotation of the entire right bronchial tree. The anterior segment of the right upper lobe showed endobronchial stenosis from which brushings were collected. There was an endobronchial plaque-like lesion at the distal bronchus intermedius immediately proximal to the right middle lobe take-off from which endobronchial biopsies were sampled. The right middle lobe bronchus exhibited extrinsic compression which caused difficulty in canulating the middle lobe with the bronchoscope; brushings were taken from this site which were positive for small cell cancer.

DISCUSSION: Anatomic post-operative changes from pneumonectomy have been described with a somewhat predictable evolution of pleural fluid and air within PPS. The majority of patients will have loculated air or pleural fluid within the PPS; a smaller portion of patients will have complete obliteration with varying levels of herniation of the remaining lung into the PPS. The aerated lung fields seen within this patient’s PPS was unexpected. Closer examination of the radiograph’s lateral view revealed the herniation of the right lung through the anterior mediastinum with displacement of the heart posteriorly. Better detailed with computed tomography, these anatomic changes are important especially in cases of planned invasive procedures involving the PPS.

CONCLUSIONS: The usual post-pneumonectomy change in the chest involves shift of the mediastinum toward the resected side. A less-common but important post-operative variant to be aware of is the herniation of the contralateral lung into the PPS. This herniation occurs over time and is generally well tolerated by patients.

Reference #1 Bazwinsky-Wutschke I, Paulsen F, Stovesandt D, Holzhausen H, Heine H, Peschke E. Anatomical Changes After Pneumonectomy. Annals of Anatomy 2011; 193: 168-172.

Reference #2 Harmon H, Fergus S, Cole F. Pneumonectomy: Review of 351 Cases. Annals of Surgery 1976; 183: 719-721.

Reference #3 Wechsler R, Goodman L. Mediastinal Position and Air-Fluid Height After Pneumonectomy: The Effect of the Respiratory Cycle. American Journal of Roentgenology 1985; 145: 1173-1176.

DISCLOSURE: The following authors have nothing to disclose: Eric Yim, Darryl Weiman

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Topics: lung , hernias
Chest. 2011;140(4_MeetingAbstracts):29A. doi:10.1378/chest.1117018

INTRODUCTION: While cancers of the breast, colon and kidney commonly metastasize to the lungs and airways, endobronchial metastasis of ovarian cancer is extremely rare. In this report, we present a case of metastatic endobronchial ovarian carcinoma and describe the clinical challenges associated with its management.

CASE PRESENTATION: A 42 year-old Hispanic female was diagnosed with metastatic poorly differentiated papillary serous carcinoma of the ovary after presenting with abdominal pain and anorexia. She was initially treated with surgical debulking and chemotherapy resulting in symptomatic improvement. However, on surveillance CT imaging eleven months after diagnosis, she was noted to have right paratracheal, right hilar and subcarinal lymphadenopathy, which persisted despite additional chemotherapy. Eighteen months after initial diagnosis, she underwent bronchoscopy with endobronchial ultrasound which revealed normal airways, but cytology from fine needle aspiration of the enlarged lymph nodes was positive for metastatic ovarian cancer. She subsequently underwent additional chemotherapy and external beam radiation to the chest. Twenty-six months after initial diagnosis she was admitted with fever and hemoptysis. Chest imaging showed persistent mediastinal lymphadenopathy with partial collapse of the right middle lobe and a post-obstructive infiltrate. The patient underwent a second bronchoscopy which revealed an obstructive endobronchial lesion in the right middle lobe, as well as exophytic friable endobronchial lesions in the right and left mainstem bronchi. Based on their location, the endobronchial lesions likely represented extrinsic airway invasion from the malignant lymph nodes that were seen on Chest CT. As such, no additional biopsies were performed. The patient was treated with antibiotics for post-obstructive pneumonia, and external radiation was continued. Interventional pulmonary therapies including laser debridement, airway stenting and endobronchial brachytherapy were discussed in a multi-disciplinary conference. Given the location of the lesions, we felt that no intervention would provide a palliative benefit for the patient. Ultimately no further pulmonary interventions were performed, and the patient’s pneumonia resolved with antibiotics. The patient was discharged home with continued outpatient radiation and chemotherapy. The patient remained stable clinically through April 2011 with no further pulmonary complications or infections.

DISCUSSION: Although parenchymal and pleural lung metastases are often seen with ovarian carcinoma, endobronchial metastases are rare. To our knowledge, only six cases of endobronchial metastasis from ovarian carcinoma have been reported in the medical literature in the past 22 years. In these reports, the treatments of the endobronchial metastases were aggressive and heterogeneous; three patients were treated with surgery, one with laser therapy and one with a combination of radiation, chemotherapy and corticosteroids. One patient's treatment was not reported. Despite recent advances in interventional bronchoscopy and the development of new modalities to treat malignant airway obstruction, the optimal management of endobronchial metastases remains challenging. In our patient, the location and multifocal nature of the airway lesions made her a poor candidate for aggressive airway interventions. Moreover, the integration of palliative care into clinical practice over the last two decades has led to less invasive treatment approaches that prioritize symptom management over the reduction of disease burden in patients with widely metastatic cancer. Consistent with these principles of palliative care, we practiced a less invasive treatment strategy in our patient. This report represents the first case of ovarian cancer with endobronchial metastasis since 1995; the management decisions reflect the advances seen in both interventional pulmonology and palliative care over the last 16 years.

CONCLUSIONS: This case provides a rare example of endobronchial ovarian cancer, likely spread through direct invasion from mediastinal lymph nodes into the airway. Palliative management of metastatic cancer with endobronchial involvement demands thoughtful consideration to determine what treatments will truly benefit the patient.

Reference #1 Mateo F, Serur E, Smith PR. Bronchial metastases from ovarian carcinoma. Report of a case and review of the literature. Gynecol Oncol. 1992 Aug;46(2):235-8

Reference #2 Merimsky O, Greif J, Chaitchik S, Inbar M. Endobronchial metastasis of ovarian cancer. A case report. Tumori. 1990 Dec 31;76(6):614-5.

Reference #3 Wholey MH, Meyerrose GE, McGuire WP, Reinhardt MJ, Sostre S. Endobronchial lesion from metastatic ovarian carcinoma resulting in partial right mainstem obstruction demonstrated by lung scintigraphy. Clin Nucl Med. 1995 May;20(5):465-6.

DISCLOSURE: The following authors have nothing to disclose: Annie Harrington, Thomas Mahrer, Dong Chang

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Chest. 2011;140(4_MeetingAbstracts):30A. doi:10.1378/chest.1118756

INTRODUCTION: Cutaneous paraneoplastic syndromes are rare, but frequently herald the presence of an occult internal malignancy. Often documented after a diagnosis of cancer is made, skin manifestations are postulated to be of concurrent onset with the neoplastic process (1). We report a case of a primary pulmonary carcinoma in a patient with cutaneous abnormalities noted on physical examination for several years prior to diagnosis.

CASE PRESENTATION: A 64-year-old male smoker with long-standing digital clubbing was referred to a dermatologist for evaluation of actinic keratoses. Comprehensive skin examination revealed an incidental finding of ridged palmar keratoderma, or “tripe palms,” often a paraneoplastic entity associated with bronchogenic or gastrointestinal carcinoma. Computed tomography of the thorax was obtained, which was notable for severe emphysematous changes and basilar reticular interstitial thickening peripherally. Colonoscopy and esophagogastroduodenoscopy were unremarkable. No further work-up was performed. Three years later, the patient was referred for pulmonary consultation with chief complaint of mild dyspnea on exertion in the setting of a ten pound unintentional weight loss. Medications at the time of evaluation included ranitidine and aspirin. Pulmonary function testing revealed a mildly decreased FEV1 (80% predicted) with a normal FEV1/FVC ratio and a diminished diffusing capacity (52% predicted). Total lung capacity was normal, with decreased residual volume (76% predicted). Physical examination demonstrated fine basilar inspiratory crackles. Tripe palms and digital clubbing were again noted. Repeat computed tomography of the chest and abdomen was obtained. Imaging revealed a four centimeter spiculated pulmonary mass in the left lower lobe with bulky subcarinal, left hilar, and left paratracheal lymphadenopathy. Several hypodense liver lesions were additionally noted. Bronchoscopy revealed tumor studding of the left lower lobe basilar segmental bronchi. Scant tumor material from both fine needle aspiration of the subcarinal node and brushing of the left lower lobe basilar segments demonstrated a poorly-differentiated carcinoma. Immunohistochemistry staining was positive for neural cell adhesion molecule (CD56), suggestive of neuroendocrine tumor origin. Other neuroendocrine markers, including leukocyte common antigen (LCA) and synaptophysin, returned negative. Small cell carcinoma was suspected. Given the prolonged duration of paraneoplastic skin changes temporally inconsistent with a traditionally aggressive malignancy, additional tissue was requested for clarification of diagnosis. Repeat bronchoscopy with brushings from the left lower lobe again demonstrated CD56 positivity, but also focal positivity to p63 and CK5/6, suggestive of non-small cell carcinoma with squamous differentiation. Forceps biopsy of the left lower lobe segmental wall demonstrated similar staining features to the initial cytology specimen, with additional negative staining to p63 and CK5/6, consistent with the original diagnosis of a neuroendocrine carcinoma. Chemotherapy was ultimately initiated with cisplatin and etoposide for presumed small cell lung cancer.

DISCUSSION: Acanthosis palmaris, or “tripe palms,” was first described in the 1970s, likening the condition to tripe, the edible lining of the bovine foregut. It is a rare, often paraneoplastic, cutaneous syndrome thought to be associated with an excess of circulating epidermal growth factor (1). Malignancy is reported in greater than 90% of patients with tripe palms, about one-quarter of which manifest lung cancer. Clubbing in association with tripe palms appears to be more specific for lung carcinoma. The appearance of tripe palms precedes the diagnosis of malignancy in about half of reported cases (2). Tripe palms are rarely associated with neuroendocrine carcinomas (3), and we can find no published reports of tripe palms associated with a biopsy-confirmed morphologically mosaic primary lung carcinoma.

CONCLUSIONS: There are no widely-accepted recommendations regarding cancer screening in patients with documented cutaneous findings indicative of paraneoplastic syndromes. Our case importantly underscores the need for careful physical examination of all patients, with continued close surveillance for neoplastic disease in patients who manifest dermatologic findings associated with malignancies.

Reference #1 Thiers, BH, Sahn, RE, Callen, JP. Cutaneous manifestations of internal malignancy. CA Cancer J Clin 2009; 59:73-98.

Reference #2 Cohen, PR, Grossman, ME, Silvers, DN, Kurzrock, R. Tripe palms and cancer. Clin Dermatol 1993; 11:165-173.

Reference #3 Cohen, PR, Grossman, ME, Almeida, L, Kurzrock, R. Tripe palms and malignancy. J Clin Oncol 1989; 7(5):669-678.

DISCLOSURE: The following authors have nothing to disclose: Jamie Bessich, Jeffrey Munson

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Chest. 2011;140(4_MeetingAbstracts):31A. doi:10.1378/chest.1119823

INTRODUCTION: Epithelioid Hemangioendothelioma (EHE) is a vascular tumor that rarely presents of pulmonary origin. CREST Syndrome (calcinosis cutis, Raynaud phenomenon, esophageal motility disorder sclerodactyly, and telangiectasia) is associated with a modest increase in risk of lung cancer. We present a case of a patient who presents with concomitant EHE and CREST syndrome.

CASE PRESENTATION: Patient is a 59 year old Caucasian female with a past medical history of CREST syndrome, pulmonary fibrosis, pulmonary hypertension, and hypothyroidism, who presented for further workup of multiple pulmonary nodules that were found on a CT scan of the chest. Her initial diagnosis of CREST was made in 2007 during the workup of her severe pulmonary hypertension. She also was found to have pulmonary fibrosis that was considered to be most likely secondary to the CREST syndrome. During her follow up, multiple nodules were followed with an outpatient PET scan which showed the largest lesions to be hypermetabolic. Subsequently, the patient underwent VATS which histologically showed EHE. The patient was given a brief trial of Sunitinib by medical oncology, but the medication was eventually discontinued due to side effects of diarrhea and worsening of her pulmonary hypertension. Ultimately, the patient expired from complications of her pulmonary hypertension and respiratory failure.

DISCUSSION: EHE is rare cancer that was first described in 1975 and it was initially thought to be an aggressive variant of bronchoalveolar cell carcinoma. EHE can present with a variable clinical course: it can be asymptomatic, indolent with chronic cough, regress spontaneously, or be rapidly fatal with similar CT findings. It is a tumor that is usually located in soft tissue, bone or liver, but it can rarely be of primary pulmonary origin. The radiographic appearance of EHE can be easily mistaken for metastatic disease. EHE is a vascular tumor and is considered a sarcoma that does not have a well-described natural history. The literature has documented cases that were stable without progression over a decade or cases that were rapidly fatal in months even with similar CT scan findings. The clinical presentation of EHE favors women over men and is half of the patients are less than 40 years old, but EHE can present in men and women of any age. Chemotherapy and radiotherapy are generally considered ineffective and curative surgery is often not feasible since EHE often presents as multifocal bilateral lesions. In the pulmonary form of EHE, half of the cases die due to respiratory failure. Case reports and small phase 2 clinical trials have reported clinical response or regression to chemotherapy regimens that include medications that block vascular endothelial growth factor (VEGFR) such as bevacizumab, or a VEGFR Tyrosine kinase inhibitor (sunitinib, sorafenib, or pazopanib). There have also been a few case reports that describe clinical response to treatment with regimens of carboplatin plus etoposide or interferon. CREST syndrome has been associated with a modest increase in risk for certain malignancies, especially lung cancers, but no previous case reports have reported EHE in a patient with CREST syndrome.

CONCLUSIONS: To our knowledge, this is the first reported case of EHE in the English literature that presents concomitantly in a patient with the CREST syndrome.

Reference #1 Cronin P, Arenberg D. Pulmonary epithelioid hemangioen-dothelioma: an unusual case and a review of the literature. Chest. 2004; 125 (2): 789-793

Reference #2 Park MS, Ravi V, Araujo DM Inhibiting the VEGF-VEGFR pathway in angiosarcoma, epithelioid hemangioendothelioma, and hemangiopericytoma/solitary fibrous tumor. Curr Opin Oncol. 2010 Jul;22(4):351-5.

DISCLOSURE: The following authors have nothing to disclose: Jason Henderson, Mark Lega, Marvin Balaan

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Chest. 2011;140(4_MeetingAbstracts):32A. doi:10.1378/chest.1118379

INTRODUCTION: Pulmonary lymphangitic carcinomatosis (PLC) accounts for 6% of metastasis to the lung and is associated with a dismal prognosis. The most common sites of origin are the breast, lung, stomach, pancreas, and cervix. Lymphangitic spread of sarcomas, or “lymphangitic sarcomatosis” (PLS) is extremely rare, as sarcomas tend to disseminate hematogenously. Only a few cases of PLS have been reported. Here we describe the clinical, radiologic, and histologic features of pulmonary lymphangitic dissemination of a primitive neuroectodermal/Ewing’s sarcoma (PNET/ES). To our knowledge, this is the first report of the PLS due to PNET/ES.

CASE PRESENTATION: A 67 year old man presented with a 2 week history of night sweats, asthenia, subjective fevers and a nonproductive cough. He denied dyspnea, hemoptysis, and weight loss. The patient received a course of amoxicillin/clavulanate without improvement. The patient’s past medical history was significant for systemic amyloidosis remaining in remission following an autologous bone marrow transplant 10 years ago. Two years prior to presentation, he developed a poorly differentiated PNET/ES of the S2 sacral nerve root which was resected and treated with cyclophosphamide, doxorubicin and vincristine, alternating with ifosphamide and etoposide. Local pelvic recurrence was identified 5 months prior to presentation, for which he underwent external beam radiation therapy. Examination revealed new bibasilar rales, and laboratory studies were remarkable for an elevated lactic dehydrogenase level (555 U/L). Computed tomography (CT) of the chest revealed multiple diffuse micronodules and tiny, new bilateral pleural effusions. The subpleural distribution of the nodularity as well as the irregular thickening of the lymphatics studded with micronodules were consistent with possible lymphangitic metastases. Because of a former smoking history, the initial concerns were for PLC. A bronchoscopy was performed. Although the bronchoalveolar lavage was unrevealing, transbronchial lung biopsies confirmed metastases of PNET/ES. Cytokeratin and TTF-1 stains were negative and vimentin, S-100, and CD-99 stains were positive, consistent with metastatic Ewing’s sarcoma. He subsequently received cyclophhosphamide and topotecan with significant clinical and radiological improvement.

DISCUSSION: PNET/ES are rare, aggressive neoplasms that belong to the ES family of tumors (EFT). The most important prognostic factor for prognosis is the presence of clinical metastasis. Lung metastasis via hematogenous tumor embolization represents the first site of distant spread in 70-80% of cases, and are the leading cause of death for patients with ES. The most common presentation of sarcomatous metastasis to the lung is that of multiple well-circumscribed randomly-distributed pulmonary nodules. In contrast to carcinomas, only 2-5% of sarcomas metastasize to lymph nodes, and these have not been shown to present with lymphangitic spread. Most cases of PLC arise from adenocarcinomas. The CT can be highly suggestive with the characteristic irregular thickening of the interlobular septa and bronchovascular bundles, polygonal arcade formation and lack of distortion of the lung parenchyma despite extensive involvement. Pleural effusions and lymphadenopathy are also common. Because of the rarity of PLS, its behavior is difficult to characterize and diagnosis may be delayed. The limited reports of PLS have been on one primary pulmonary angiosarcoma, intravascular bronchoalveolar tumors (epitheliod hemangioendotheliomas), one limb cutaneous hemangiosarcoma, osteosarcomas, mesotheliomas with sarcomatoid features, and Kaposi’s sarcomas.

CONCLUSIONS: This case represents the first description of lymphangitic spread of a PNET/ES and underscores the fact that in patients with this diagnosis and a compatible CT of the chest, PLS should be considered in the differential diagnosis and transbronchial lung biopsy can be diagnostic.

Reference #1 1. Karosas, A. Am J Health-Sys Pharm. 2010;67:1599.

Reference #2 2. Cotterill, SJ. J Clin Oncol. 2000;18: 3108.

Reference #3 3. Liau, Chi-Ting. Jpn J Clin Oncol. 2000; 30(1):37.

DISCLOSURE: The following authors have nothing to disclose: Maria Mirant-Borde, Gerardo Colon-Otero, David Menke, Augustine Lee

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Chest. 2011;140(4_MeetingAbstracts):33A. doi:10.1378/chest.1089656

INTRODUCTION: We describe a very interesting iatrogenic complication of atrial septal defect closure device which divided the right atrium into two chambers. We name this complication as Cor Triatriatum Dextro Iatrogenica.

CASE PRESENTATION: A 29-year-old female was brought to the emergency room with complaints of sudden onset of ataxia and dysarthria. Magnetic resonance imaging of the brain revealed basilar infarct and subacute infarcts on both the medial thalami. A transesophageal echocardiogram (TEE) showed a large atrial septal defect (ASD) with shunt of 1.7:1 and a normal left ventricular function. Her neurological deficits gradually improved and she was transferred for transcatheter closure of ASD. An intracardiac echocardiogram during the procedure showed a septum secundum oval defect of 0.9-1.3cm with good septal margins that was closed using a helex device (30mm). There was no residual shunting through the defect by color flow mapping. Repeat TEE done after two months showed a very interesting finding that we have called Cor Triatriatum Dextro Iatrogenica, which means formation of iatrogenic triple atria. It was noticed that the inferior limb of the right atrial side of closure device was bent coming close to Eustachian valve, and partitioning the atrium into two parts - one part between atrial septum and Eustachian valve receiving blood from the inferior vena cava and the second part on the other side of Eustachian valve receiving blood from the superior vena cava with turbulent flow from the first to the second part through small opening between edges of Eustachian valve and the bent part of device. This functionally created third atria. No further intervention was done, as the patient was completely asymptomatic. Patient remained asymptomatic after a 9 month follow up.

DISCUSSION: Transcatheter closure of secundum ASD was first reported in 1976 (1), and now is more popular than surgery because of short learning curve, cosmetic benefits, reduced pain and reduced hospital stay. The development of transesophageal and intracardiac echocardiography has facilitated the use of transcatheter ASD closure device. These studies help in better evaluation of the defect size, shape and margins, device selection, placement and evaluation of complications post procedure.(2) The complications reported with these devices are device malposition, embolization, cardiac perforations, residual shunts, vascular trauma, thrombus formation, and sudden death. Device malposition or embolisation is the most common complication as seen in multiple studies ranging from 4% to 20% and mostly require surgery. Our patient also presented with an asymptomatic malposition diaganosed echocardiographically leading to a unique iatrogenic triatria. There have been few case reports of patients with a chamber in between the right and the left atria secondary to a rare congenital atrial septal malformations diagnosed echocardiographically. The possible hemodynamic effects of this are still not known. Although our patient was asymptomatic, it remains uncertain whether the increased turbulence due to iatrogenic triatria translates into increased chances of infective endocarditis and thrombus formation. Currently there are no guidelines regarding anticoagulation and follow up in these patients.

CONCLUSIONS: This case demonstrates an interesting iatrogenic complication of atrial septal device leading to creation of unique triatria which has not been reported so far.

Reference #1 King TD, Thompson SL, Steiner C, Mills NL. Secundum atrial septal defect. Nonoperative closure during cardiac catheterization. JAMA. Jun 7 1976;235(23):2506-2509.

Reference #2 Ewert P, Berger F, Daehnert I, et al. Transcatheter closure of atrial septal defects without fluoroscopy: feasibility of a new method. Circulation. Feb 29 2000;101(8):847-849.

DISCLOSURE: The following authors have nothing to disclose: Ankur Lodha, Mehandi Haran, Adnan Sadiq, Jacob Shani

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Chest. 2011;140(4_MeetingAbstracts):34A. doi:10.1378/chest.1116752

INTRODUCTION: Hydroxychloroquine-induced cardiomyopathy is a rare and life-threatening condition characterized by restrictive hemodynamics. Drug cessation and aggressive afterload reduction halts disease allowing for clinical recovery. We present a 50-year-old woman with hydroxychloroquine-induced restrictive cardiomyopathy and correlate clinical, echocardiographic, and right heart catheterization (RHC) findings at initial presentation and following treatment.

CASE PRESENTATION: A 50 year-old female with systemic lupus erythematosus (SLE) was referred to the pulmonary hypertension (PH) clinic for evaluation of worsening shortness of breath. She described progressive exertional dyspnea, lower extremity swelling, and increasing abdominal girth for 6 months. The patient’s medical history was remarkable for complications of SLE including serositis with pericardial and pleural effusions; ascites; mesangiopathic nephritis; Raynaud's phenomenon; leucopenia; and anemia. Medications consisted of hydroxychloroquine (200 mg twice daily for over 10 years) and corticosteroids. Physical examination revealed resting tachycardia at 100 bpm, blood pressure 123/58 mmHg, respiratory rate 12, and oxygen saturation of 96% on room air. Pertinent findings included increased jugular venous pressure, diminished breath sounds at the base of the right lung with dullness to percussion, an accentuated P2 component of the second heart sound despite muffled heart sounds, a protuberant abdomen, and 2+ pitting edema to the knees. Chest X-ray demonstrated a right basilar pleural effusion. EKG was normal voltage, sinus rhythm with normal intervals and no evidence of ischemia or infarction. Transthoracic echocardiography (2D echo) revealed mild left ventricular dilatation, mild pericardial effusion, moderate mitral valve regurgitation, moderate tricuspid valve regurgitation and an estimated pulmonary artery systolic pressure (sPAP) of 52 mmHg. Pulmonary function tests demonstrated combined obstruction and restriction with normal diffusing capacity. A ventilation/perfusion scan demonstrated no evidence of chronic thromboembolic disease. Given the longstanding use of hydroxychloroquine as well as pericardial involvement of SLE, and after evaluation for pulmonary etiology of symptoms, our differential included both restrictive cardiomyopathy and constrictive pericarditis. A cardiac MRI was pursued and was non-revealing. A left/right heart cardiac catheterization (L/RHC) was performed demonstrating mean right atrial pressure (mRAP) of 16 mmHg with pronounced “x” and “y” descents; right ventricular end diastolic pressure (RVEDP) was 23 mmHg with square root sign; mean pulmonary artery pressure (MPAP) was 29 mmHg; pulmonary capillary wedge pressure (PCWP) was 22 mmHg; left ventricular end diastolic pressure (LVEDP) was 24 mmHg. Cardiac output (CO) was 5.61 L/min by Fick. Cardiac index (CI) was 3.13 L/min/m2 by Fick. Pulmonary vascular resistance (PVR) was 1.25 Woods units. The equalization of pressures of the right ventricle, pulmonary artery, PCWP and left ventricle during diastole strongly suggested restrictive physiology. Hydroxychloroquine therapy was discontinued. Aggressive afterload reduction was instituted. The patient’s symptoms resolved. Repeat 2D echo three months following discontinuation of hydroxychloroquine also suggested resolution, demonstrating sPAP of 36 mmHg. A repeat L/RHC one year after discontinuation of hyroxychloroquine demonstrated: mRAP 6 mmHg; RVEDP 13 mmHg; mPAP 23 mmHg; PCWP 11 mmHg. CO/CI were 4.07 L/min and 2.35 L/min/m2 by Fick respectively.

DISCUSSION: Hydroxycholoquine therapy is indicated to treat connective tissue disorders including SLE. Cardiovascular complications include myocardial thickening, restrictive cardiomyopathy, and conduction disorders. The time interval between initiation of therapy and development of complications has been documented to vary from 7 months to 25 years [1]. We present a case of prolonged hydroxychloroquine use resulting in clinical heart failure and restrictive cardiomyopathy as demonstrated by RHC. Discontinuation of hydroxychloroquine therapy and aggressive afterload reduction resulted in dramatic improvement in clinical symptoms and hemodynamics.

CONCLUSIONS: It is important to consider restrictive cardiomyopathy as an etiology of heart failure in patients treated with hydroxychloroquine.

Reference #1 Cotroneo J, Sleik K, Rodriguez E, Klein A. Hydroxychloroquine-induced restrictive cardiomyopathy. Eur J Electrocardiology 2007;8,247-251

DISCLOSURE: The following authors have nothing to disclose: Daniel Urbine, Rana Awdish

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Chest. 2011;140(4_MeetingAbstracts):35A. doi:10.1378/chest.1119956

INTRODUCTION: Cardiovascular complications of carbon monoxide (CO) poisoning include myocardial ischemia, left ventricular dysfunction, or arrhythmias. We report the first case of Brugada pattern in CO poisoning in the English literature.

CASE PRESENTATION: : A 56-year-old Asian male was found unconscious in his home by his son who called emergency medical services. Patient regained consciousness while receiving 100% oxygen by means of nonrebreather reservoir face mask in the ambulance. Upon arrival to the emergency room (ER), the patient was awake, but complained of mild headache and dizziness. He denied any chest pain, palpitation or shortness of breath. Past medical history was significant for dyslipidemia treated with simvastatin. Family history was negative for sudden cardiac death. Vital signs on admission showed a temperature of 100.4 F, heart rate of 110 beats/minute, blood pressure of 129/89 mmHg, respiratory rate of 24 per minute, with oxygen saturation of 100% on non-rebreather mask delivering 100% oxygen. Arterial blood gas analysis by co-oximetry showed pH-7.48, pCO2-33 mmHg, pO2-250 mmHg, HCO3-24.6 mmol/L, and oxygen saturation of 99%. Carboxyhemoglobin level on admission was 25.2%. Creatine kinase, creatine kinase-MB isoenzyme, and troponin I was within normal limits. Electrocardiogram done in ER showed sinus tachycardia at 104 beats per minute, left axis deviation, and ST-segment elevation of 3.5 mm in V1-V2 with a saddleback appearance, characteristic of type 2 brugada electrocardiographic pattern (BEP). Portable chest radiograph was within normal limits. The patient was admitted to telemetry unit and administered 3 hyperbaric-oxygen treatments (HBOT) within a 24-hour period. Transthoracic 2-dimensional echocardiogram was within normal limits. Repeat electrocardiogram after HBOT showed resolution of the brugada pattern.

DISCUSSION: The Brugada syndrome is a clinical and electrocardiographic entity consisting of right bundle-branch block and unusual ST-segment elevation in the right precordial leads (V1 to V3) and responsible for at least 4% of all sudden deaths and at least 20% of sudden deaths in patients with structurally normal hearts. Mutations in SCN5A, a cardiac sodium channel gene, transmitted in an autosomal dominant pattern are implicated as a cause. BEP in absence of clinical symptoms can occur in variety of other conditions. This is the first report in the English literature of BEP due to CO poisoning. Myocardial injury occurs frequently in patients with CO poisoning and is a significant predictor of long term mortality. In a prospective study 1of 230 patients, mean age 47.2 years, 72% men, with moderate to severe CO poisoning, myocardial injury was reported in 37% of patients. Ischemic electrocardiographic changes were seen in 30%, and positive biomarkers in 35%. The current indications for HBOT in CO poisoning from cardiovascular standpoint are cardiac ischemia, arrhythmias, or carboxyhemoglobin level >20% with underlying coronary artery disease. Recent data demonstrate that a type I BEP pattern alone, even when other clinical criteria are not fulfilled, can be associated with sudden cardiac death during follow up. Thus, all patients who present as BEP pattern, in the setting of CO poisoning should be considered at risk, and timely HBOT may help in recovery.

CONCLUSIONS: CO poisoning should be considered one of the differentials of BEP. Even in the absence of arrhythmias or myocardial ischemia, BEP in a patient with CO poisoning may be considered for hyperbaric oxygen therapy.

Reference #1 Henry CR, Satran D, Lindgren B, Adkinson C, Nicholson CI, Henry TD. Myocardial injury and long-term mortality following moderate to severe carbon monoxide poisoning. JAMA. 2006 Jan 25; 295(4):398-402.

Reference #2 Probst V, Veltmann C, Eckardt L, et al. Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry. Circulation. 2010 Feb 9; 121(5):635-43.

DISCLOSURE: The following authors have nothing to disclose: Jaya Prakash Sugunaraj, Jung Julie Kang, Wilbert Aronow, Kausik Kar, Chandrasekar Palaniswamy, Jessica Most

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Chest. 2011;140(4_MeetingAbstracts):36A. doi:10.1378/chest.1119898

INTRODUCTION: While hemoptysis following blunt trauma is typically due to pulmonary injury, we present an unusal cardiovascular cause.

CASE PRESENTATION: Case Report: The patient is a 46 year old active duty male who presented to the Walter Reed Army Medical Center Pulmonary Clinic with two days of hemoptysis. He was in his usual state of excellent health until two days prior to presentation when he was involved in a collision during an ice hockey game. Afterwards he noted shortness of breath and cough with blood-streaked sputum. During the subsequent twenty-four hours the patient had 50cc. of hemoptysis. The next day he presented to the Emergency Department where pulmonary contusion, pneumothorax and pulmonary embolism were excluded by CT. He was provided a referral for prompt outpatient pulmonary consultation. At his outpatient appointment the next day his exam was notable for no respiratory distress, normal lung sounds, and a new harsh III/VI holosystolic murmur, that was loudest at the apex with radiation to the left axilla. Because of suspicion for a traumatic papillary muscle rupture, he was admitted to the internal medicine service for expedited evaluation. A transthoracic echocardiogram revealed severe, eccentric, anteriorly directed mitral regurgitation associated with a flail posterior mitral valve leaflet resulting in severe posterior leaflet prolapse. He was diagnosed by cardiology with traumatic rupture of the posterior chordae tendoneae resulting in acute severe mitral regurgitation. Within the first twenty four hours of admission he developed worsening dyspnea and was transferred to the medical intensive care unit, where he received intravenous lasix and nitroglycerin. He responded well to these therapies, and remained clinically stable for the duration of his admission. Prior to definitive mitral valve repair, trans-esophageal echocardiogram demonstrated severe mitral regurgitation due to rupture of the P2 valve chord. Left and right heart catheterization showed no obstructive arterial disease, mitral regurgitation; a hyperdynamic left ventricle, and mild pulmonary hypertension. The patient successfully underwent mitral valve repair without complication and subsequent resolution of hemoptysis.

DISCUSSION: Discussion: This case demonstrates an unusual presentation of traumatic posterior mitral valve leaflet rupture with severe regurgitation manifesting as submassive hemoptysis. A recent review of the literature determined that sports injuries are the third most common cause of traumatic mitral valve insufficiency. Overall, mitral valve rupture due to blunt trauma is a rare occurrence with just 82 cases found in the literature since 1964. (1) Of note, hemoptysis as a symptom of acute mitral valve insufficiency appears to be an even rarer, based on our review of the literature. The pathophysiology of traumatic severe mitral valve regurgitation is thought to occur as a result of loss of valve integrity from a sudden increase in intracardiac pressure while the heart is completing diastole and entering systole, with the ventricles dilated and the arterioventricular valves closed. Most frequently damaged is the papillary muscle, followed by the chordae tendineae. (1) Symptoms of acute mitral valve regurgitation are due increased left atrial pressure and decreased left ventricular ejection fraction. Acutely, the patient usually presents with signs of pulmonary edema such as dyspnea, cough and orthopnea. Hemoptysis is most often associated with mitral valvular stenosis, not regurgitation.

CONCLUSIONS: Conclusion: Blunt thoracic trauma occurring during contact sports can result in severe injuries such as mitral valve rupture and subsequent hemoptysis. Mitral valve insufficiency should be suspected in a patient with a new murmur and a history of trauma.

Reference #1 Pasquier M, et al. “A review of traumatic mitral valve prolapse- Traumatic mitral valve injury after blunt chest trauma: a case report and review of the literature.” J Trauma. 2010 Jan;68(1):243-6.

DISCLOSURE: The following authors have nothing to disclose: Sarah Petteys, Sean Roark, Jeffrey Kunz, John Atwood, John Thurber, Oleh Hnatiuk

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Chest. 2011;140(4_MeetingAbstracts):37A. doi:10.1378/chest.1114234

INTRODUCTION: A pseudoaneurysm of the aorta after cardiac transplantation is relatively rare but potentially life threatening complication in immunosuppressed cardiac recipients. Herein, we report a rare case of a pseudoaneurysm related to the outflow graft anastomosis site of the left ventricular assist device (LVAD) to the descending thoracic aorta.

CASE PRESENTATION: This is a 59-year-old male with past medical history of congestive heart failure, coronary artery disease status post coronary artery bypass grafting and mitral valve repair, renal insufficiency and pulmonary hypertension. He admitted to Thomas Jefferson University Hospital for severe heart failure recalcitrant to 2 inotropes. The Jarvik 2000 Flowmaker (Jarvik Heart, Inc) implantation was performed in December 2009. The outflow graft of the LVAD was anastomosed to the descending thoracic aorta with the aid of side-clamping using a 4-0 Prolene running suture. His postoperative course was unremarkable and he discharged from the hospital on postoperative day 7. In June 2010, after 6 months support with the LVAD, OHT was performed via redo-sternotomy. The outflow graft of the LVAD was densely adhered to the surrounding left lung, therefore the previous left thoracotomy had to be reentered. The outflow graft was divided with vascular staple and overswen with a 3-0 Prolene suture. The patient required an extended intensive care unit stay due to ventilator dependent respiratory failure. In July 2010, 5 week after the heart transplant, the patient experienced severe infection in which multiple culture from sputum, pleural fluids, urine and blood was positive for Pseudomonas aeruginosa, and furthermore, the patients developed necrotizing fasciitis of bilateral lower extremities required multiple course of fasciotomies and skin grafting caused by the same organism. In November 2010, the patient had an episode of hemoptysis at rehab facility. The further evaluation with bronchoscope revealed blood clot in his left lower lobe and computed tomography (CT) scan of the chest showed a pseudoaneurysm of the descending thoracic aorta at the anastomosis site of the outflow graft of the LVAD. He underwent endovascular stent graft placement urgently on the following day. However, his hemoptysis persisted and which required open repair of descending thoracic aorta using two aortic allograft. The culture of the pseudoaneurysm wall and the outflow graft material collected in the operating room were positive for Pseudomonas aeruginosa. The patient was treated with amikacin for 3 weeks and followed by tigacycline for additional 2 weeks based on the results of the culture. At 5 months follow-up, the patient is doing well with no signs of recurrence.

DISCUSSION: To the best of our knowledge, this is the first described case of a pseudoaneurysm of the descending thoracic aorta related to the outflow graft anastomosis site of the LVAD. Pseudoaneurysms of the great vessel in cardiac transplantation recipients had been reported, although most of these cases are suture lines or cannulation sites of the ascending aorta. In our experience, we have 5 consequence patients who had successfully bridged to transplantation after Jarvik 2000 LVAD implantation with its outflow graft anastomosed to the descending thoracic aorta and the presented case was the only case that experienced the pseudoaneurysm at the site of outflow anastomosis. Cohn et al. published their experience of 18 patients with Jarvik 2000 implantation followed by OHT. In their series, no patients had complication related to the remnant of the outflow graft or anastomosis site on the descending thoracic aorta. During follow-up in their patients, no evidence of infections or graft remnant related complication was noted. On the contrary, our patient suffered from systemic bacteremia of Pseudomonas aeruginosa and local evidence of Pseudomonas infection in the pleural cavity, blood stream, and outflow graft itself, which most likely contributed to the development of the pseudoaneurysm in this patient. Hemoptysis was an early and significant sign in our patient and enabled us to further investigate to diagnose this rare complication.

CONCLUSIONS: We reported a rare case of pseudoaneurysm of the descending thoracic aorta at the anastomosis site of LVAD outflow graft presented with the symptom of hemoptysis. The evidence of systemic and local infection and the immunosuppression of the cardiac transplantation recipient were thought to contribute the development of the pseudoaneurysm. Although pseudoaneurysm of the graft anastomosis site is rare, this complication should be treated in appropriate timing and strategy to avoid possibly lethal consequences after cardiac transplantation.

Reference #1 Cohn WE, Fikfak V, Gregoric ID, Frazier OH. Retention of left ventricular assist device outflow grafts after transplantation. J Heart Lung Transplant. 2008 Aug;27(8):865-8.

DISCLOSURE: The following authors have nothing to disclose: Kentaro Yamane, Hitoshi Hirose, Linda Bogar, Nicholas Cavarocchi, Atul Rao, Joshua Eisenberg, Scott Cowan, Nathaniel Evans

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Chest. 2011;140(4_MeetingAbstracts):38A. doi:10.1378/chest.1118768

INTRODUCTION: Multiple pulmonary nodules are commonly secondary to metastatic malignancy or are infectious in etiology. We describe an uncommon pulmonary disorder characterized by multiple pulmonary nodular lesions with lymphocytic invasion of vascular walls on biopsy called pulmonary lymphomatoid granulomatosis (PLG).

CASE PRESENTATION: 72 year old male with history of COPD presented with sudden onset of progressively worsening weakness since laminectomy done three weeks prior to the presentation. One week after the surgery he developed cough and low grade fever for which he was treated with antibiotics by his family physician for presumable bronchitis. Six days later he was admitted to the hospital with worsening fever and shortness of breath. He denied any headache, visual disturbances, back pain, seizure or neck stiffness. On examination, he was tachycardic, tachypneic and was febrile. Chest X-ray revealed bibasilar atelectasis. He was started on levofloxacin and a chest CT was done to evaluate for pulmonary embolism. CT showed numerous nodules scattered throughout the left lung. Bronchial washings were sent for routine cultures and cytology. While awaiting the report, he developed pancytopenia, worsening shortness of breath, abdominal pain and a repeat CT of the chest along with abdomen and pelvis was done. Results were consistent with bilateral worsening nodules both in size and in number and nodular lesions on the left adrenal gland. Pancytopenia worsened progressively. He developed 0.5 cm macular oval erythematous lesions on his left lower extremity with raised border, largest at left ankle. Given its rapidity in progression, we were highly concerned about infectious cause - fungal, particularly disseminated histoplasmosis, being a significant concern given lack of response to antibacterials, negative bacterial cultures, pancytopenia, rash and pulmonary nodules. Noninfectious causes were also entertained such as vasculitis and autoimmune phenomenon. He was started on amphotericin, vancomycin, ciprofloxacin and aztreonam empirically. Fungal serology was sent along with PCR for CMV, EBV and work up for vasculitis. Skin biopsy was performed which was nondiagnostic. CMV titre came back >600 K, EBV titre was > 400 K, though unlikely to cause pulmonary nodules, he was started on ganciclovir. Biopsy of adrenal nodule was done which revealed coagulative necrosis. Cytology from bronchial washings came back for atypical cells with hyperchromatic nuclei suspicious for malignancy. Finally Video assisted thoracoscopic surgery (VATS) gave away the diagnosis of lymphomatoid granulomatosis grade 3. Patient was started on solucortef and rituximab. Initially he did show response but soon deteriorated, developed tumor lysis syndrome, worsening renal function and family withdrew care.

DISCUSSION: PLG likely represents a lymphoproliferative disorder in the family of Epstein-Barr virus (EBV)-associated B cell lymphomas. It usually presents between the ages of 30 and 50. PLG can be seen in patients with an underlying immunodeficiency (eg, Wiskott-Aldrich, X-linked immunodeficiency, immunosuppression, organ transplant). These immune defects may lead to an abnormal host response to EBV infection, resulting in lymphomatoid granulomatosis. The lung is the most commonly involved organ; the skin and neurologic system may be affected separately or concurrently. The most common presenting symptoms and signs include cough, fever, rash/nodules, malaise, weight loss, neurologic abnormalities, dyspnea, and chest pain. Chest imaging studies typically show multiple ill-defined nodular opacities. Nodules are preferentially located along the bronchovascular structures or interlobular septa. Thin walled cystic lesions may also be present. The histopathologic diagnosis of PLG requires a triad of polymorphic lymphoid infiltrates, transmural infiltration of arteries and veins by lymphoid cells ("angiitis"), and focal areas of necrosis within the lymphoid infiltrates. The clinical course of lymphomatoid granulomatosis is variable, ranging from remission without treatment to death within 2 years from malignant lymphoma.

CONCLUSIONS: Pulmonary lymphomatoid granulomatosis (PLG) should be considered in the differential diagnosis of multiple pulmonary nodules.

Reference #1 Liebow AA, Carrington CR, Friedman PJ. Lymphomatoid granulomatosis. Hum Pathol 1972; 3:457.

Reference #2 Jaffe ES, Wilson WH. Lymphomatoid granulomatosis: pathogenesis, pathology and clinical implications. Cancer Surv 1997; 30:233.

Reference #3 Pisani RJ, DeRemee RA. Clinical implications of the histopathologic diagnosis of pulmonary lymphomatoid granulomatosis. Mayo Clin Proc 1990; 65:151.

DISCLOSURE: The following authors have nothing to disclose: Aanchal Kapoor

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Chest. 2011;140(4_MeetingAbstracts):39A. doi:10.1378/chest.1119840

INTRODUCTION: Lipoid Pneumonia is an uncommon entity characterized by pulmonary accumulation of lipids from endogenous or exogenous sources. We report a case of exogenous lipoid pneumonia following illicit subcutaneous silicone injection for cosmetic purposes.

CASE PRESENTATION: A 30-year-old previously healthy female presented to our ED complaining of dyspnea for four days. Her symptoms started shortly after liquid silicon injection to her buttocks by a non-professional individual. Two days later she noticed cough with clear sputum that subsequently turned bloody. Dyspnea and hemoptysis worsened despite prescribed inhaled steroids and azithromycin at another ED. Review of systems was otherwise unremarkable. Physical exam was pertinent for an oxygen saturation of 93% on room air and bibasilar crackles. Chest radiography showed bibasilar airspace disease. Computed tomography revealed bibasilar and peripheral ground glass opacities. Bronchoscopic exam showed diffuse bright red blood in the airways. Bronchoalveolar lavage had a sanguinous appearance that cleared with subsequent aliquots of saline. Pulmonary function tests suggested a restrictive pattern with an elevated diffusing capacity. Transbronchial biopsies showed lipoid pneumonia and silicon vacuoles. Due to worsening dyspnea, hypoxemia and hemoptysis, the patient was started on high-dose methylprednisolone and she experienced a dramatic clinical improvement within 24 hours. She was discharged five days later on prednisone and without supplemental oxygen. On a 3-week follow-up visit she reported no symptoms and her pulmonary function tests showed a significant improvement. Prednisone was successfully tapered off over four weeks.

DISCUSSION: Lipoid Pneumonia (LP) is caused by accumulation of lipids in the alveoli from endogenous or exogenous sources. Endogenous LP is seen in alveolar proteinosis and hereditary errors of metabolism. Exogenous LP is typically caused by inhalation or aspiration of oils. Injectable silicon fluid is widely used in cosmetic procedures. Animal studies have shown that it can be subsequently recovered in various organs after injection.1 Illicit injections in humans are associated with adverse effects including migration to other organs, granulomatous hepatitis, and death.2 Silicone-fluid induced embolism has been implicated as a cause of acute pneumonitis with alveolar hemorrhage. The patient we present had hypoxemia, dyspnea and hemoptysis. In a series of 33 patients, 92% had hypoxemia, 88% dyspnea, 70% fever, and 64% alveolar hemorrhage.3 Symptoms started a few minutes after the injection in 72% of patients. Although our patient had hemoptysis and elevated diffusing capacity, bronchoscopy failed to demonstrate active alveolar hemorrhage. Chest radiography showed basilar infiltrates as the previously reported patients. CT showed diffuse peripheral and bibasilar ground glass opacities, characteristic features seen on previous reports of similar cases. Transbronchial biopsies revealed lipoid pneumonia and silicon vacuoles but no evidence of alveolar hemorrhage. The proposed mechanism is delivery to the lung via blood vessels and leakage into the airspaces as silicone is notorious for not staying compartmentalized. The evidence for therapy in these patients is mostly based on case reports. The patient was deteriorating rapidly until systemic steroids were started. She showed remarkable improvement in symptoms and hypoxia. She was discharged a few days later and remained symptom free after a slow taper of prednisone.

CONCLUSIONS: Silicon injection for cosmetic purposes is potentially a lethal procedure. We present the case of a woman who developed lipoid pneumonia presumably from silicon embolization that successfully recovered with systemic steroid therapy.

Reference #1 Ben-Hur N, Ballantyne DL Jr, Rees TD, Seidman I. Local and systemic effects of dimethylpolysiloxane fluid in mice. Plast Reconstr Surg 1967;39:423-6.

Reference #2 Ellenbogen R, Rubin L. Injectable fluid silicone therapy: human morbidity and mortality. JAMA. 1975;2324:308-309.

Reference #3 Smith A, Tzur A, Leshko L, Krieger BP. Silicone embolism syndrome: a case report, review of the literature, and comparison with fat embolism syndrome. Chest 2005;127:2276-81.

DISCLOSURE: The following authors have nothing to disclose: Angel Coz Yataco, Javier Diaz-Mendoza, Chad Stone

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Chest. 2011;140(4_MeetingAbstracts):40A. doi:10.1378/chest.1113801

INTRODUCTION: Apnea testing (AT) is the standard for clinical diagnosis of brain death. Intensivists must be prepared to perform AT as part of routine Intensive care Unit (ICU) duties. The safety of AT has been a topic of debate with the most common complications observed being hypotension, hypoxemia and acidosis. We describe a case of bilateral tension pneumothoraces during AT.

CASE PRESENTATION: A 57 year old woman with past medical history of hypertension, fibromyalgia and hypothyroidism was brought to the hospital unresponsive and intubated. Initial neuro imaging was consistent with global anoxia revealing bilaterally symmetrical basal ganglia and occipital infarcts apparently secondary to fentanyl overdose. She progressively deteriorated over the following 12 hours until brainstem reflexes were absent. American Academy of Neurology pre-requisites for apnea testing were met, including euvolemia, core temperature > 36.5 degrees and normotension. Formal AT was initiated. The patient was removed from ventilator and a 12 french oxygen catheter was placed at the level of the carina within the lumen of a 7.0 millimeter endotracheal tube (ETT). After 4 minutes of observed apnea, hypotension and desaturation ensued followed by obvious subcutaneous air in the thorax and head necessitating termination of AT. Chest X-ray confirmed bilateral tension pneumothoraces and subcutaneous emphysema treated with thoracostomy. Cerebral blood flow testing performed immediately after thoracostomy confirmed brain death.

DISCUSSION: Tension Pneumothorax is an extremely rare complication encountered during AT. Predictors for its development should be identified to limit potential life threatening complications. Wijdicks et al(1) reviewed 228 cases of AT and no pneumothoraces were described. Saposnik et al(2) identified acidosis as a risk factor for developing complications during AT. The patient described here had no apparent risk factors and had normal hemodynamic and acid-base status prior to AT. Review of the AT in this case revealed that the oxygen tubing was likely not moving freely within the smaller lumen ETT (7 millimeter) nor was gas noted to be venting from the ETT. We suspect that a snug fit between oxygen tubing and ETT did not allow sufficient venting of oxygen flow in this case precipitating the pneumothoraces.

CONCLUSIONS: ETT luminal diameter, oxygen tubing outer diameter and depth of placement should be shown careful attention in AT. Vigilance with respect to presence of adequate residual ETT lumen to allow ventilation with oxygen flow rates set at physiologic minute volumes, and preferably, review and standardization of these parameters by prospective studies will lessen the risk of pneumothoraces during AT.

Reference #1 Wijdicks EF, Rabinstein AA, Manno EM, Atkinson JD. Pronouncing brain death: Contemporary practice and safety of the apnea test. Neurology. 2008 Oct 14;71(16):1240-4

Reference #2 Saposnik G, Rizzo G, Vega A, Sabbatiello R, Deluca JL. Problems associated with the apnea test in the diagnosis of brain death. Neurol India 2004;52:342-5

DISCLOSURE: The following authors have nothing to disclose: Naveed Hasan, David Landsberg

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Topics: apnea , pneumothorax
Chest. 2011;140(4_MeetingAbstracts):41A. doi:10.1378/chest.1114467

INTRODUCTION: The effects of caffeinated alcoholic beverages on patients with chronic medical conditions are not well documented. We present a case of a type I diabetic with intoxication from caffeinated alcoholic beverages.

CASE PRESENTATION: A 53-year-old man was brought to the emergency department by ambulance after being found unresponsive and surrounded by empty cans of the caffeinated alcoholic beverage 4 Loko. The patient had a history of type I diabetes mellitus, with multiple admissions to this institution for diabetic ketoacidosis. The patient was last seen 3 days prior to his presentation. On arrival to the emergency department the patient was obtunded and unable to provide history. The patient later reported that he drank approximately six 24-oz cans of 4 Loko before he lost consciousness. The patient’s past medical history was significant for type I diabetes mellitus, hypertension, hyperlipidemia, and substance abuse. He had a history of alcohol abuse and occasionally used marijuana. His reported home medications included insulin glargine, insulin aspart and lisinopril. On physical examination, the patient was obtunded on arrival to the emergency department and required endotracheal intubation with mechanical ventilation. He was afebrile, BP was 101/42, and HR was 128. His pupils were round and reactive with intact corneal reflex. His mucous membranes were dry, with an endotracheal tube in place. His lungs were grossly clear to auscultation bilaterally. His heart sounds were tachycardic but regular, with no murmurs, rubs or gallops. His abdomen was soft and non-tender. He had no clubbing, cyanosis or edema in his lower extremities. His laboratory data included a hemoglobin of 10.9 gm/dL, white blood count was 17.2 k/uL, and platelet count was 234 k/uL. His sodium was 124 mmol/L, potassium was 9.3 mmol/L, chloride was 87 mmol/L, bicarbonate was 3 mmol/L, BUN was 64 mg/dL, creatinine was 4.44 mg/dL, and blood glucose was greater than 2400 mmol/l. He was noted to have a large amount of serum ketones in his blood, and a lactic acid of 5.3 mmol/L. His chest x-ray showed a normal cardiac and mediastinal silhouette, with proper placement of endotracheal tube and clear lung fields. His serum osmolality, measured after volume resuscitation was initiated, was 384 mosmol/KG, but his serum alcohol level was non-detectable and his volatile alcohol screen was negative. His EKG showed the dynamic changes of widening of the QRS complexes and inversion of the T waves in the precordium. The patient was admitted to the intensive care unit and treated with intravenous insulin, sodium bicarbonate and aggressive intravenous fluid administration. His metabolic acidosis and hyperglycemia quickly improved, and his EKG normalized without hemodialysis. He was liberated from mechanical ventilation within 24 hours of admission. His hospital course was complicated by alcohol withdrawal, which was treated with lorazepam. He was discharged to home after refusing rehabilitation on hospital day #5.

DISCUSSION: The use of caffeinated alcoholic beverages and other non-alcoholic energy beverages has been increasing since they were first introduced approximately 20 years ago. Some studies have shown an increasing amount of risk taking behavior among individuals using alcoholic energy drinks. One study showed a 3-fold increased risk of leaving a bar highly intoxicated and a 4-fold increased risk of intending to drive in comparison with those who did not consume alcoholic drinks mixed with energy drinks. 4 Loko is just one example of a caffeinated alcoholic beverage; the alcohol content ranges from 11-12%, with 660 calories and 60 grams of sugar. 4 Loko has currently been banned in Utah, Michigan, Oklahoma, New York, and Washington. Since evaluation by the FDA, Phusion Products (the makers of 4 Loko) has removed caffeine, taurine, and guarana from their drinks. There have been no studies evaluating the side effects of these beverages on individuals with chronic illnesses, in adolescents, or in young adults. We hypothesize that our patient's high glucose load and caffeine intake allowed for excessive alcohol consumption, which induced loss of consciousness, prolonged time without insulin, and diabetic coma.

CONCLUSIONS: The use of caffeinated alcoholic beverages in those with chronic illnesses has not been studied, and their side effects may be more pronounced in these particular patients.

Reference #1 Thombs DL, O’Mara RJ, Tsukamoto M et al. Event-level analyses of energy drink consumption and alcohol intoxication in bar patrons. Addictive Behaviors; 35 (2010):325-330.

Reference #2 O'Brien, M. C., McCoy, T. P., Rhodes, S. D.,Wagoner, A., &Wolfson, M. (2008). Caffeinated cocktails: Energy drink consumption, high-risk drinking, and alcohol-related consequences among college students. Academic Emergency Medicine, 15, 453−460.

Reference #3 Press Release. Update regarding our reformulated products. Avaliable at: http://www.phusionprojects.com/media_reformulationupdate.html

DISCLOSURE: The following authors have nothing to disclose: Kyle Brownback, Krishna Rangarajan, Steven Simpson

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Chest. 2011;140(4_MeetingAbstracts):42A. doi:10.1378/chest.1117991

INTRODUCTION: DIPNECH is a rare pulmonary disease that has an insidious presentation that may lead to delayed diagnosis. We describe a case of DIPNECH in a young women with a two year history of cough and mild dyspnea.

CASE PRESENTATION: A 37 year-old woman was referred to our pulmonary clinic for evaluation of chronic non productive cough and abnormal CT scan showing a mosaic pattern. Her cough and mild dyspnea was persistent for two and a half years. The patient’s medical history was significant for GERD, eczema and asthma diagnosed after joining the military four years prior. Her medications included inhaled budesonide with formoterol, loratadine, and esomeprazol. She denied alcohol, tobacco and illicit drugs. She is currently active in the US Army and states she was deployed to the Middle East for two years where she was exposed to burn pits. On physical exam her vitals signs were normal. Pulmonary exam demonstrated bilateral wheezing. The results of the initial laboratory tests were normal. Echocardiogram was unremarkable. Pulmonary Function test demonstrate a borderline obstructive pulmonary defect with air trapping and airflow reversibility. These were unchanged from PFT’s prior to initiating budesonide/formoterol. A bronchoscopy with BAL performed six months prior demonstrated no endobronchial lesions. The BAL cultures and cytology were negative. A repeat high resolution CT scan revealed a mosaic pattern more pronounced on the expiratory images with multiple bilateral sub-centimeter nodules. The patient was referred for surgical lung biopsy which showed advanced chronic small airways disease with evidence of constrictive bronchiolitis. Numerous carcinoid tumorlets along with patchy neuroendocrine hyperplasia of airways were present.

DISCUSSION: DIPNECH is a rare primary lung disorder. In 1999, was included as an entity in the World Health Organization (WHO) classification of lung tumors and by then fully recognized and named accordingly. The cardinal pathophysiologic change is progressive narrowing and eventual obstruction of the small airways as a consequence of obliterative bronchiolitis and excessive intraluminal proliferation of the pulmonary endocrine cell. The pathologic change of airway obstruction dictates the common features in clinical presentation, pulmonary function test, and radiologic examination. Clinical presentation is often insidious, with slow onset of dry cough and breathlessness; however, some patients are asymptomatic. While clinical signs are minimal, PFTs tend to show variably severe obstructive or mixed obstructive and restrictive patterns, but may be normal in a minority of cases. The most consistent changes on high-resolution chest CT included thickening of bronchiolar walls and mosaic pattern of air trapping presumably caused by airway obstruction. Nodules are occasionally detected on chest CT. The nodules usually represent tumorlets or peripheral typical carcinoid tumors commonly associated with DIPNECH. Follow-up without treatment may be a reasonable first course of action. However, a trial of steroid therapy is warranted if pulmonary function indices deteriorate. Patients generally have slowly progressive disease over many years with a very good long term prognosis; however, occasional patients develop more rapidly deteriorating airway obstruction with extreme bronchiolar obliteration and may even die of the disease [1, 2, 3].

CONCLUSIONS: We report an extremely rare case of DIPNECH. Clinical diagnosis is extremely challenging and often delayed for many years after initial presentation due to the insidious clinical course, non-characteristic presentations, and lack of effective noninvasive diagnostic tests. Pathologic evaluation of lung tissue is the gold standard in making a definitive diagnosis.

Reference #1 [1] Y. Ge, M.A. Eltorky: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia. Ann. Diagn. Pathol. 11 (2007) 122-126.

Reference #2 [2] Davies SJ, Gosney JR: Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease. Thorax 2007, 248-252.

Reference #3 [3] C. Lim, Stanford: Diffuse idiopathic pulmonary endocrine cell hyperplasia. Pathology international 2010; 538-541.

DISCLOSURE: The following authors have nothing to disclose: Luis Chug, Heath Latham

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Chest. 2011;140(4_MeetingAbstracts):43A. doi:10.1378/chest.1117953

INTRODUCTION: Relapsing polychondritis is a chronic multisystem disease associated with recurrent inflammation and destruction of cartilaginous structures. Typical areas of involvement are the ears, nose, and airways with the lower respiratory tract becoming involved in 20-50% of cases. In those who develop respiratory tract manifestations, respiratory symptoms may be the initial presentation in up to one half of these patients. Historically, airway involvement has been associated with poor outcomes.

CASE PRESENTATION: A 44-year old female presented to the pulmonary clinic for progressive dyspnea on exertion and wheezing. She had been diagnosed with asthma on the basis of these symptoms along with severe obstruction noted on spirometry in the past. She had been treated with bronchodilators and inhaled corticosteroids with minimal response. Her initial examination was remarkable for a mild saddle nose deformity, attributed to damage following a transphenoidal surgery for a pituitary adenoma several years ago. At the time of referral, she had no wheezing on exam; however, her spirometry was significant for severe obstruction without bronchodilator reversibility. Impulse oscillometry, an effort-independent technique to determine airway resistance, was found to be normal in this patient, suggesting the possibility of dynamic airway obstruction. Bronchoscopy was performed, which demonstrated dynamic collapse of the bilateral mainstem bronchi during forced exhalation. Subsequent laboratory evaluation revealed elevated IgG to Type II Collagen which can be found with relapsing polychondirits.

DISCUSSION: Patients with relapsing polychondritis frequently develop airway complications that can be devastating, causing significant morbidity and mortality. The airway complications may consist of tracheobronchomalacia (TBM) and/or excessive dynamic airway collapse (EDAC). In normal individuals, forceful expiration or coughing can lead to 40% narrowing of the tracheobronchial lumen. Such reductions become pathologic when they exceed 50% of the lumen. With TBM, there is weakness of the walls of the lower airway secondary to cartilaginous destruction and hypotonia of the myoelastic components. However, if the narrowing is predominantly due to collapse of the posterior lumen, EDAC is the likely underlying pathophysiology. By bronchoscopic and dynamic computed tomography evaluation, this patient had predominantly EDAC related airway changes. Regardless of the predominant pathophysiology, patients with this condition are treated through a multidisciplinary approach aimed at relieving this dynamic obstruction.

CONCLUSIONS: Relapsing polychondritis with tracheobronchomalacia often presents with non-specific symptoms similar to patients with lung diseases such as COPD or asthma. The typical symptoms of dyspnea and wheezing, often made worse by exertion, may lead clinicians to treat these more commonly encountered lung pathologies without ever considering this condition. When pulmonary manifestations are the first clinically relevant signs of relapsing polychondritis, it can be appreciated how the correct diagnosis is often delayed. The diagnosis is often suspected once patients have failed to respond to escalating therapy for the more common obstructive lung diseases and when CT imaging demonstrates tracheal narrowing. In this patient’s case, an alternative diagnosis to asthma was suspected after impulse oscillometry demonstrated no increase in airway resistance during tidal volume breathing, in sharp contrast to the severe obstruction seen with the force maneuver on her spirometry.

Reference #1 Ernst A, Rafeq A, et al. Relapsing Polychondritis and Airway Involvement. Chest 2009; 135 (4):1024-30.

Reference #2 Murgu S, Colt H. Tracheobronchomalacia and excessive dynamic airway collapse. Respirology 2006; 11:388-406.

Reference #3 Rafeq S, Trentham D, Ernst A. Pulmonary Manifestations of Relapsing Polychondritis. Clin Chest Med 2010;31:513-18.

DISCLOSURE: The following authors have nothing to disclose: Nicholas Ondrasik, David Bell

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Chest. 2011;140(4_MeetingAbstracts):44A. doi:10.1378/chest.1119935

INTRODUCTION: Invasive Aspergillosis in critically ill patients in medical intensive care units (MICU) is a well recognized entity. Association with contaminated air handling systems has been reported in the past, although the epidemiological data is sparse. We report two cases of invasive pulmonary aspergillosis in critically ill patients associated with mold contamination of air handling ducts and reversal of air flow in negative pressure isolation room in MICU.

CASE PRESENTATION: Index case: A 61-year-old female with history of Chronic Obstructive Pulmonary Disease (COPD) was admitted with two week history of cough and dyspnea. At presentation, patient was found to be hypotensive, hypoxic, and in acute renal failure. The CXR revealed bilateral diffuse infiltrates suggestive of pneumonia. The patient was intubated for respiratory failure, started on broad spectrum antibiotics and methylprednisolone. The influenza A-PCR was positive for H1N1. The initial blood and tracheal aspirate cultures were negative. Hospital course was complicated by development of Acute Respiratory Distress Syndrome (ARDS), Atrial fibrillation with rapid ventricular rate (RVR), and pulmonary embolism. The patient remained hemodynamically unstable requiring vasopressor and ventilator support. On day 14, the patient developed fever of 105° F and elevated WBC of 40,000 with a differential of 36% neutrophils, 5% lymphocytes, and 12% bands. Repeat blood cultures and Clostridium difficile toxin assay were negative. Echocardiogram showed no vegetation or thrombosis. The patient underwent bronchoscopy which revealed multiple raised whitish lesions surrounded by rim of brownish discoloration in the left main stem bronchus. The endobronchial forceps biopsy of lesions was performed and pathological diagnosis of invasive aspergillosis was made. The patient was started on Micafungin and Amphotericin B. Voriconazole was not administered as the patient had Atrial fibrillation with RVR responding to Amiodarone only. Despite broad-spectrum antibiotics and anti-fungal therapy, the patient died of cardiopulmonary arrest on 17th day of hospitalization. An autopsy was not performed. Case #2: A 58-year-old male with history of Hepatitis C, alcohol abuse and COPD presented with septic shock. Course of hospitalization was complicated with severe hypoxemia and development of ARDS. Tracheal aspirate grew Aspergillosis spp. Lung biopsy was not performed due to severe hypoxemia. Patient was treated with Voriconazole and Amphotericin. Patient died after being hospitalized for 12 days.

DISCUSSION: Aspergillus species are ubiquitous molds that produce numerous spores, 2-4 µm in diameter. Despite routine inhalation of these spores, Aspergillus spp remain an uncommon cause of disease, except in individuals at particular risk for invasive disease. Given the ubiquitous nature of Aspergillus spores in the external environment, numerous reservoirs have been identified in hospitals. Contaminated central air handling systems can become breeding grounds for mold. Lutz (2003) reports an outbreak of invasive Aspergillus infection in post-surgical patients due to a contaminated air-handling system in an operating theater, reportedly with 3-1000-fold increased concentrations of ≥ 3 µm particles. In our cases, both patients were admitted to the same negative pressure isolation room. Despite aggressive medical therapy, their conditions continued to deteriorate, and subsequently diagnosis of aspergillosis was made. Cluster of cases involving Aspergillus spp within a span of 4 weeks in MICU lead to an investigation. As a surrogate for Aspergillus spp infestation particle counts were performed using Zephon Bio pump. A visual inspection of air ducts using digital photographs was also consistent with mold infestation. Spore trap samples revealed significant increase in >0.3 µm particles approximately 2 million and for particles ≥1 µm 41,000 particles.

CONCLUSIONS: Aspergillus species can cause infrequent but serious nosocomial infection in MICU, and our cases point towards a possible association with contaminated air handling ducts, which warrants further studies. Introduction of comprehensive air quality guidelines in critical areas such as ICU may prevent outbreaks.

Reference #1 Lutz BD, Jin J, Rinaldi M, Wickes B. Outbreak of Invasive Aspergillus Infection in Surgical Patients, Associated with a Contaminated Air-Handling System. CID. 2003:37.

Reference #2 Srinivasan A, Beck C, Buckley T, GehA. The ability of hospital ventilation systems to filter Aspergillus and other fungi following a building implosion. Infection Control Hosp Epidermiol. 2002 Sep;23(9):520-4.

DISCLOSURE: The following authors have nothing to disclose: Mitra Sahebazamani, Edmundo Rubio, Umar Sofi

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Chest. 2011;140(4_MeetingAbstracts):45A. doi:10.1378/chest.1113810

INTRODUCTION: A 70 year old African American woman with history of smoking presented with worsening of asthma associated with productive cough and hemoptysis of 3 days duration.

CASE PRESENTATION: Her medications included Fluticasone/Salmeterol, bronchodilators and Omalizumab. On examination, she was not in distress. Blood Pressure was 125/80 mmHg; heart rate, 68/min; respiratory rate, 22/min; temperature, 98 F and SaO2 of 98% on room air. Auscultation revealed scattered wheezes more prominent on the right lung field. Computed tomography images of the chest are shown. Flexible bronchoscopy revealed a polypoid lesion arising from the right bronchus intermedius close to secondary carina occluding 90% of the bronchial lumen. Multiple biopsies and bronchial brushings were negative for carcinoma. Bronchoalveolar lavage and cultures were negative for acid fast bacilli and fungi. Pathologic specimen of the excised mass, by rigid bronchoscopy, is shown.

DISCUSSION: Benign polypoid lesions are uncommon findings and present a diagnostic dilemma. Fibroepithelial polyp of the tracheobronchial tree is a slow-growing tumor but can produce obstructive complications such as chronic bronchitis and bronchiectasis, recurrent pneumonia. Benign tracheobronchial tumors represent 1.9% of pulmonary tumors. Fibroepithelial polyps are rare with unclear etiology. The most accepted hypothesis is chronic inflammation due to factors such as foreign body aspiration, prolonged mechanical ventilation, asthma, smoke inhalation and mycobacterial infections. Histopathologically they are fibro-inflammatory and lined with respiratory epithelium with edematous stroma. Inflammatory polyps are solitary benign endobronchial lesions with stromal configurations consisting of well-formed fibrous connective tissue with or without inflammation and covered with stratified squamous epithelium or normal bronchial mucosa. The silent nature of these lesions often leads to a delayed diagnosis. Diagnosis is essential to rule out malignancy and avoid parenchymal resection. Fiber-optic bronchoscopy is the procedure of choice to diagnose clinically-suspected endobronchial lesions but excisional biopsy by rigid bronchoscopy is required for definitive diagnosis and to exclude bronchogenic carcinoma. Occasionally surgical excision is required. Treatment is eradication of the lesion in order to relieve complications secondary to airway obstruction and conserve functioning lung parenchyma. For localized endobronchial lesions without extraluminal extension, endoscopic treatment modalities like bronchoscopic curettage, Nd-YAG laser, electrocautery and cryosurgery are available which avoid the morbidity and mortality associated with thoracotomy and pulmonary resection. The choice between these endobronchial therapies varies from center to center depending on the equipment available and the preferences and experience of the physician. Surgical resection is required when the lesion has extended beyond the bronchial lumen and there is a high likelihood of recurrence. Proximal lesions which cannot be excised by endoscopy require surgical excision of involved bronchus with brochoplasty to avoid resection of functional lung parenchyma. In the surgical treatment, tissue-sparing techniques with minimally possible parenchymal resection should be performed. For this reason, excision of the bronchial part, where the lesion takes its origin (bronchoplasty), should be tried first. The most frequent indication for bronchoplastic resection is the presence of a lesion in the main bronchus or lobar bronchus. Our patient underwent rigid bronchoscopy. The pedunculated polypoid mass was removed. On six-month follow up, there was a marked improvement in asthma symptoms with fewer exacerbations and the use of inhaled bronchodilators and corticosteroids had declined substantially.

CONCLUSIONS: Benign Fibroepithelioma is rare and difficult to distinguish from malignant lesion based on endobronchial appearance. Flexible Fiber-optic bronchoscopy with small tissue samples is insufficient to diagnose fibroepithelioma. Rigid bronchoscopy with excision is required to avoid sampling error, exclude malignancy and confirm fibroepithelioma. Majority of patients can be treated with therapeutic endoscopic procedures like laser, elctrocautery or mechanical excision. Rarely, conservative surgical excision of fibroepithelioma along with bronchus and bronchoplasty with preservation of functioning lung parenchyma may be required.

Reference #1 Leiro-Fernandez et al J Bronchol Intervent Pulmonol _ Volume 17, Number 1, January 2010 56-58 58 | J Bronchol Intervent Pulmonol 2010;17:56-58

Reference #2 Ushiki A, Yasou M, Tanabe T, et al. A rare case of a tracheal polyp treated by endobronchial resection. Intern Med. 2008;47:1723-1726.

Reference #3 Strachan P, Multz AS, Esposito MJ, et al. Bronchoscopic image: benign bronchial polyp. J Bronchol. 2007; 14:111-112.

DISCLOSURE: The following authors have nothing to disclose: Saleem Shahzad, Manoj Suryanarayanan, Sasikanth Nallagatla, Vishal Verma, Viswanath Vasudevan, Farhad Arjomand, Rana Ali, Scott Reminick

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Topics: skin tag
Chest. 2011;140(4_MeetingAbstracts):46A. doi:10.1378/chest.1119686

INTRODUCTION: Stongyloides Stercoralis is an enteric nematode that usually causes asymptomatic eosinophilia in immunocompetent hosts and can persist in the body for long periods. It can, however, cause fatal infections in immunocompromised hosts. It has been rarely reported as a cause of COPD exacerbation, as well as of interlobular fibrosis. We report a patient with chronic intermittent gastrointestinal symptoms and recurrent COPD exacerbations along with CT scan of chest showing pulmonary infiltrates and ground glass opacities. After extensive workup, the patient was found to have S. Stercoralis infection and showed significant improvement of pulmonary and gastrointestinal symptoms after treatment with Ivermectin.

CASE PRESENTATION: A 62 year old Caucasian male with a known history of COPD presented with dyspnea, wheezing and abdominal pain for a few days. The patient reported episodic abdominal pain, vomiting and diarrhea intermittently for six months. During that time period, he was hospitalized several times for acute COPD exacerbations. Previous work up included routine blood work, peripheral eosinophil count of 7100, negative sputum and stool cultures and spirogram revealing airflow obstruction with FEV1 53% predicted. Bronchoscopy was unremarkable with a non-diagnostic BAL. Transbronchial biopsy demonstrated non-specific inflammatory changes. Endoscopic biopsies demonstrated evidence of eosinophilic infiltration in the gastric mucosa. For a presumptive diagnosis of hypereosinophilic syndrome, the patient was then treated with Prednisone with only partial improvement. On this admission, repeat spirogram showed FEV1 of 37% predicted. CT scan of abdomen was unremarkable. CT scan of chest showed bilateral pulmonary infiltrates with air space and ground glass opacities. Antibiotics and corticosteroids yielded little improvement. The patient was then tested for S. Stercoralis by serum antibody titer and stool culture, which were positive. Upon further questioning, we found he had been a surveyor in Louisiana for a few years. Treatment with Ivermectin resulted in significant improvement in both pulmonary and gastrointestinal symptoms. He continued to feel well without any re-admissions for COPD exacerbations during the following six months.

DISCUSSION: S. Stercoralis is an intestinal parasite predominantly found in tropical and subtropical areas (e.g. southeastern United States). The primary mode of transmission occurs when larvae from contaminated feces penetrate the skin. Immunocompetent hosts remain asymptomatic (with peripheral eosinophilia), even for years, while immunosuppressed can develop disseminated strongyloidiasis or hyperinfection syndrome, both considered systemic strongyloidiasis(1). Disseminated strongyloidiasis occurs when the organism, in the larval form, is found outside the usual migration pattern. Hyperinfection is an augmentation of the life cycle, resulting in a heavy infestation of worms in the lungs(1) . Patients with COPD, who are infected by this organism and have altered cellular immunity or on therapy with corticosteroids, could develop hyperinfection and dissemination of the larvae from the gastrointestinal tract to the bloodstream (2). In such context there should be search for rhabditiform larvae in stool. It is not routinely tested and has to be requested separately. It is important to consider Strongyloidaisis since it is an easily treatable condition, which if left untreated can be fatal. If intestinal infection with S. Stercoralis is suspected, detected, and treated before immunosuppressive therapy is initiated, morbidity and mortality from the disease can be prevented.

CONCLUSIONS: The unexpected presence of enteric symptoms in the context of recurrent COPD exacerbation with unexplained chronic eosinophilia should prompt investigation for S. Stercoralis, which if treated early, can prevent further morbidity

Reference #1 Keiser PB, Nutman TB Strongyloides Stercoralis in imunocompromised population. Clin Microbiol Rev. 2004;17(1):208

Reference #2 Sen P, Gil C, Estrellas B, Middleton JR. Corticosteroid-induced asthma: a manifestation of limited hyperinfection syndrome due to Strongyloides stercoralis. South Med J. 1995;88(9):923

DISCLOSURE: The following authors have nothing to disclose: Mohammad Syed, Swapna Devanna, Muhammad Siddique, Patricia Hopkins-Price, Haitham Bakir

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Chest. 2011;140(4_MeetingAbstracts):47A. doi:10.1378/chest.1094245

INTRODUCTION: Scytalidium is a fungus which belongs to group of dematiaceous coelomycete which is a large and heterogeneous group of molds that causes mostly cutaneous, subcutaneous and corneal infections. These organisms are widespread in the environment and they are mostly found in soil, wood and decomposing plant debris. Human infection occurs mainly after traumatic implantation.

CASE PRESENTATION: A 70 year old male presents with recurrent pneumonia. His medical history includes unilateral vocal cord paralysis following meningioma resection, bladder and prostate cancer in 2002 for which he had surgical resection and received chemotherapy. His chest CT scans over the years were significant for right lung patchy opacities with fleeting appearance and mediastinal adenopathy. Radiographic findings were consistent with recurrent pneumonia. One year ago, the Chest CT showed a 0.8 x 1.2 cm nodule in the right middle lobe. Six month follow up scan showed mild increase in size of the nodule. A PET scan was negative for right middle lobe pulmonary nodule, however two right and one left hilar lymph nodes were mildly positive on PET scan. For the last two months, patient had complaints of worsening dyspnea on exertion and occasional wheezing. He received 2 courses of oral antibiotics and bronchodilators without improvement. He underwent a VATS procedure without adverse events. Pathological review showed lung parenchyma with a granulomatous lesion characterized by central necrosis and peripheral palisading histiocytes with multinucleated giant cells. Special staining with GMS was positive for fungal organisms. The fungus was identified as Scytalidium species. Fungal cultures grew same pathogen. Our patient received 4 weeks of voriconazole treatment. Patient’s symptoms improved and he is currently asymptomatic.

DISCUSSION: The Scytalidium species were first reported in 1970 to cause superficial dermatomycosis and onychomycosis clinically undistinguishable from typical dermatophyte infections. Most frequent clinical presentations of invasive infection include brain abscess and abdominal abscesses. This case of invasive Scytalidium infection is unique in organ involved and clinical presentation with recurrent pneumonia and lung nodules. Other distinctive features of this case are absence of immunodeficiency and chronicity of symptoms.

CONCLUSIONS: Optimal therapy of deep Scytalidium infections is not known. Our patient responded well to treatment with voriconazole. Amphotericin B and voriconazole are effective in vitro, however surgical debridement is frequently necessary. In spite of improvement in therapy, this condition is still associated with high morbidity and mortality.

Reference #1 JOURNAL OF CLINICAL MICROBIOLOGY, 0095-1137/97/$04.0010 Feb. 1997, p. 433-440

Reference #2 JOURNAL OF CLINICAL MICROBIOLOGY, Aug. 2004, p. 3789-3794 Vol. 42, No. 8 0095-1137/04/$08.00[|#1#|]0 DOI: 10.1128/JCM.42.8.3789-3794.2004

DISCLOSURE: The following authors have nothing to disclose: John Youssef, Andreea Antonescu-Turcu, Rade Tomic

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Topics: lung infection
Chest. 2011;140(4_MeetingAbstracts):48A. doi:10.1378/chest.1115151

INTRODUCTION: Pasteurella multocida is a rare cause of pneumonia mainly occurring in patients with underlying lung disease and contact with domestic animals. Symptoms of P. multocida range from mild cough to hemoptysis and can present acutely or as a chronic infection. Aberrant bronchial arteries have been described in chronic lung infections and are associated with massive hemopytsis. Pulmonary sequestrations can be congenital or acquired, are normally found in the left lower lobe; and typically should be sent for resection. Anomalous arteries supplying lung tissue with normally communicating bronchial airways has been termed Pryce type 1 anomaly, and pseudosequestration when associated with consolidation.

CASE PRESENTATION: An 80 year old female presented to the emergency department with a two day history of hemoptysis. Her past medical history was significant for chronic bronchitis, breast cancer, and previous pneumonia. She was a former 20 pack-year smoker and her daughter frequently visited with a small dog. Over the previous 5 months she had noted weight loss, increasing dyspnea, and fatigue. Upon presentation she was afebrile, however had a leukocytosis of 11,400 K/UL. The patient continued to cough blood and was intubated for airway protection. Computed tomography (CT) of the chest showed a left lower lobe dense consolidation distal to bronchial narrowing. Interventional radiology performed aortic angiography revealing a dilated and tortuous bronchial artery feeding the left lower lobe consolidation and an abnormal parenchymal blush. The artery was embolized and cessation of distal flow was confirmed. A subsequent bronchoscopy showed inflamed and edematous left-sided bronchi and cessation of bleeding. Given her prior cancer history, there was some concern for recurrent cancer with post obstructive pneumonia. Due to an increasing white blood cell count and febrile episodes a bronchoalveolar lavage (BAL) was performed and she was started on therapy for severe pneumonia. BAL cultures showed heavy growth of P. multocida and the patient was treated with a 10 day course of a respiratory fluorquinolone. A 6 week follow-up chest CT showed resolution of the consolidation and bronchoscopy revealed mildly stenotic left lower lobe airways without mucosal abnormality. The patient’s fatigue and dyspnea had improved at follow-up.

DISCUSSION: We present a case of an elderly female with a history of chronic bronchitis presenting with massive hemoptysis associated with P. multocida pneumonia and pseudosequestration. P. multocida is often a colonizer in individuals with an appropriate exposure history, but may present as pneumonia in the elderly or those with chronic lung disease. Severity of respiratory infection is directly related to a patient’s comorbidities, and is associated with a mortality rate as high as 30%. Cases of hemoptysis from this infection have been described previously, but typically with acute infections.

CONCLUSIONS: This case illustrates some usual presenting features for a chronic P. multocida lung infection with an unusual finding of pseudosequestration and hemoptysis. This high mortality rate, and risk for the elderly patient should make this an increasingly concerning cause of community acquired infection. Additionally, prior to sending a patient for resection of a pulmonary sequestration or mass, one should consider bronchoscopy or empiric treatment for chronic pneumonia. A clue for pseudosequestration is the arterial supply comes from a normally present but hypertrophied systemic artery rather than an anomalous artery. Disclaimer: The opinions and assertions contained herein are those of the authors and are not to be construed as official or as reflecting the views of the Department of Defense, the Department of the Navy, or the naval services at large.

Reference #1 Singh SP, Nath H. Chest 2001; 120: 298-301.

Reference #2 Kimura R, Hayashi Y, et al. J Infect Chemother 2004; 10: 250-2.

DISCLOSURE: The following authors have nothing to disclose: Jimmy Suvatne, Melissa Butts, Joel Nations

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Chest. 2011;140(4_MeetingAbstracts):49A. doi:10.1378/chest.1120114

INTRODUCTION: Histoplasmosis is the most common mycotic infection in the United States and is especially prevalent in the Ohio and Mississippi river valleys. Most cases are asymptomatic benign pulmonary infections incidentally found in immune competent hosts. Disseminated histoplasmosis infections are usually found in patients that are immune compromised by medications, cancer, and HIV infection. Fungal particles found in dust and soil are in inhaled. As a result the lower respiratory tract the most common site for infection with extrapulmonary histoplasmosis often affecting the tongue and buccal mucosa. This case illustrates an unusual presentation of disseminated histoplasmosis initially presenting as oral bleeding from raised lesion of the epiglottis.

CASE PRESENTATION: This is a 72 year old male with history of coronary artery disease, valvular heart disease and chronic kidney disease stage III initially presented to his local emergency department after spontaneously developing painless oral bleeding. He denied any trauma, constitutional abnormalities, cough or dyspnea. No lesions or source of bleeding were found at that time. He did have an elevated INR that was corrected and the bleeding subsided. During the following week he redeveloped intermittent oral bleeding and returned for evaluation. His INR was 2 and again oropharyngeal examination did not reveal the source of bleeding. A nasopharyngoscopy was performed that showed epiglottic thickened with ulceration and a right true vocal fold with a mounded soft tissue mass concerning for malignancy. The patient was taken to the operating room for biopsies of these lesions and surrounding regions of the posterior pharynx. All of the biopsies revealed extensive infiltration of histoplasmosis. The patient continued to have oral bleeding post-operatively and remained intubated for airway protection. Over the course of the next three days he continued to have extensive oral bleeding requiring repeated packing despite a normal coagulation profile. He was alert and oriented during this time and tolerated his antifungal therapy. On his fourth post-operative day his mental status declined and eventually became obtunded off of any sedative medications. A head CT and brain MRI did not show any abnormalties responsible for the patient's condition. A lumbar puncture was performed for further evaluation of his mental status change. The cerebral spinal fluid (CSF) had no organisms seen and the white blood cell count was 3, but his histoplasmosis antigen was positive. Urine, serum, and bronchoalveolar lavage specimens were negative for histoplasmosis and its antigen. HIV testing was also negative. The patient’s mental status did not improve and a repeat lumbar puncture was performed to confirm the diagnosis of central nervous system (CNS) histoplasmosis. The CSF now revealed acute inflammation with leukocytosis and the histoplasmosis antigen was again positive. Despite treatment, his clinical condition worsened and the patient expired.

DISCUSSION: Histoplasmosis is a common organism in the United States with positive histoplasmin skin testing in as many as 90 percent of the people living endemic areas. It usually does not cause disseminated infection in immune competent patients and in those that do develop active infection, presenting symptoms often include malaise, low grade fevers, and dyspnea. The respiratory tract is the most common site for lesions since the organism enters the body by inhalation. Non bleeding oral lesions are more common the HIV positive patients, but can occur in any patient. Usually the tongue and buccal mucosa are affected and the lesions can be misdiagnosed as malignant on first appearance. The organism migrates through the bloodstream to involved many organ systems. Central nervous system infection occurs in approximately 5 percent of cases with disseminated disease and yields a poor prognosis. Histoplasmosis needs to be considered in endemic regions since special testing is often required. Antigen testing from urine and serum as well as other body fluids can support to the diagnosis of disseminated infection. Early and aggressive antifungal therapy with prolonged duration is necessary in disseminated infection, especially with CNS involvement.

CONCLUSIONS: The diagnosis of disseminated histoplasmosis can be difficult in an immune competent patient since symptoms may be vague and mimic many disease processes. The organism is not often reveal itself on standard cultures. A hightened index of suspicion for infection with histoplasmosis is necessary in endemic regions, since aggressive antifungal therapy is required.

Reference #1 Oropharyngeal histoplasmosis: report of eleven cases and review of the literature. Rev Soc Bras Med Trop. 2011 Jan-Feb;44(1):26-9.

Reference #2 L. J. Wheat, et al. Diagnosis and Management of Central Nervous System Histoplasmosis. Clin Infect Dis. (2005) 40 (6): 844-52.

DISCLOSURE: The following authors have nothing to disclose: Brian Mieczkowski, Matthew Exline

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Chest. 2011;140(4_MeetingAbstracts):50A. doi:10.1378/chest.1120192

INTRODUCTION: Mucormycosis is a fungal disease with high mortality and especially a rapid progression in pulmonary infections. Due to the high mortality, early and appropriate treatment is critical in the therapeutic success of patients with mucormycosis. Limited data are available regarding the ideal treatment for patients with mucormycosis. The following case illustrates a mucormycosis infection in a poorly controlled diabetic with a non ketoacidotic state treated with surgical resection and a combination of liposomal amphotericin B and echinocandin, which has not been previously reported for pulmonary mucormycosis.

CASE PRESENTATION: A 29 year old male with a medical history of smoking and type I diabetes mellitus of 10 years duration, was seen in the emergency department with an 8-week history of progressive shortness of breath, left sided pleuritic chest pain and progressive hemoptysis. He denied any fever, chills or night sweats. No intravenous drugs use or close contacts with persons with tuberculosis was reported. On exam he was afebrile with normal blood pressure, heart rate and respiratory rate. Oxygen saturation was 99% on room air. He had egophony and decreased breath sounds in the middle and lower left lung fields. Abdomen was soft, with the presence of a colostomy in the left lower quadrant. Laboratory data: normal complete blood cell count and urinalysis. Chemistry unremarkable, except for glucose 587 mg/dl and carbon dioxide 34mmol/L. Serum ketones negative. Hemoglobin A1C 10.2% and serum pH of 7.46. Coccidiomycosis, histoplasmosis, aspergillosis and crytococcus serologies were negative. Sputum acid fast bacillae, anti-neutrophil antibody and HIV were negative. Clinical course: Chest radiograph and computerized tomography (CT) revealed left upper lobe (LUL) cavitation and left lower lobe (LLL) consolidation. Bronchoscopy did not showed endobronchial lesions but LUL bronchial alveolar lavage (BAL) and transbronchial biopsies revealed non septated hyphae consistent with Rhizopus spp. CT scan of the head and sinus were normal and the patient was started on liposomal amphotericin B and micafungin intravenously on day 2. On day 4 he underwent LUL lobectomy plus LLL wedge resection with cultures from LUL positive for Rhizopus spp. There were no hyphae growth from the LLL resection and he completed 19 days of amphotericin B and micafungin. On day 20, he developed acute kidney injury, elevation of liver enzymes and pericardial tamponade. Antifungal therapy was stopped and a pericardial window was performed. There was no evidence of hyphae on the pericardial fluid. He recovered well and was started on posaconazole alone on day 25 to complete 4 additional weeks of oral antifungal therapy as outpatient.

DISCUSSION: Mucormycosis is a rare infection usually in patients with underlying immunocompromise. Diabetes mellitus especially with ketoacidosis is considered the most common underlying condition that favors this infection; however it is a less common association in pulmonary mucormycosis. The clinical presentation of fever and hemoptysis plus tissue infarction and necrosis are characteristic of pulmonary mucormycosis. Radiographic findings include focal consolidation, pleural effusions and multiple nodules, but cavitary lesions with an air crescent sign are rare. The standard therapy of pulmonary mucomycosis is the combination of surgical debridement and early antifungal therapy. Among antifungal, amphotericin B monotherapy has been the drug of choice before transitioning to oral agents. Rizhopus spp the most common form found in humans express the target enzyme for echinocandins. Therefore, combination of amphotericin B with echinocandins has been suggested from animals studies and a recent retrospective study performed in patients with rhino-orbital mucormycosis. However, no human data or case reports are available about the role of this antifungal combination in pulmonary mucormycosis.

CONCLUSIONS: Since the mortality of pulmonary mucormycosis is high, this case illustrates a successful outcome with an early aggressive combination of dual antifungal therapy and surgery.

Reference #1 Spellberg B, Ibrahim AS. (2010) Recent advances in the treatment of mucormycosis. Curr Infect Dis Rep 12(6):423-9.

Reference #2 Lee FY, Mossad SB, Adal KA. (1999) Pulmonary mucormycosis : the last 30 years. Arch Intern Med 159(12):130-9.

Reference #3 Reed C, Bryant R, Ibrahim AS, Edwards J Jr, Filler SG, Goldberg R, et al. (2008) Combination polyene-caspofungin treatment of rhino-orbital-cerebral mucormycosis. Clin Infect Dis 47(3):364.

DISCLOSURE: The following authors have nothing to disclose: Juan Fernandez, Tamara Simpson, Marcos Restrepo

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Chest. 2011;140(4_MeetingAbstracts):51A. doi:10.1378/chest.1113543

INTRODUCTION: Pulmonary arterial hypertension (PAH) in pregnancy carries high maternal mortality. In non-pregnant patients with PAH, inhaled treprostinil has been demonstrated to sustained effect on pulmonary vascular resistance with a longer duration of action. We report a successful maternal-fetal outcome in a pregnant woman with PAH treated with inhaled treprostinil.

CASE PRESENTATION: A 17 year-old female with Mixed Connective Tissue Disease presented to the emergency department with dyspnea at 33 weeks gestation. Medications included plaquenil, prednisone, and albuterol. Physical exam revealed a respiratory rate of 22 and an oxygen saturation of 91%. She had mild bibasilar crackles, regular heart tones, no peripheral edema, and a gravid uterus. Chest x-ray showed bilateral hilar prominence. Computed tomography of the chest showed enlargement of the pulmonary arteries. Transthoracic echocardiogram (TTE) showed normal left ventricular (LV) function, with mildly dilated right atrium (RA) and right ventricle (RV). Estimated systolic pulmonary artery (PA) pressure was 60 mmHg. She was admitted to the hospital, and a right heart catheterization with vasoreactivity was performed, revealing moderate fixed pulmonary hypertension, along with Class III symptoms. Treatment was initiated with diuretics and inhaled treprostinil. Her symptoms improved, and she was discharged home. She was re-admitted 12 days later in active labor at 36 weeks gestation. Inhaled nitric oxide and supplemental oxygen were initiated for desaturations during early labor. Inhaled treprostinil and furosemide were continued. TTE showed normal LV and RV function. Labor and delivery was uneventful, with birth of a healthy baby girl via vaginal forceps-assisted delivery. Postpartum, the inhaled nitric oxide and oxygen were quickly discontinued. She remained on furosemide and inhaled treprostinil, and was discharged on Postpartum Day 4. TTE at 3 months showed improved RA and RV size, normal RV systolic function, and an improved systolic PA pressure. At 6-month follow-up she had Class I symptoms, and six-minute walk distance (6MWD) had improved from 351 to 410 meters.

DISCUSSION: PAH is a devastating complication of Mixed Connective Tissue Disease. Normal pregnancy causes a wide range of physiologic and hemodynamic changes including volume overload. Patients with PAH have a limited ability to compensate for these changes. Maternal mortality in pregnant patients with severe PH has been reported as high as 56%. Management of PAH during pregnancy must evaluate possible toxic effects of medications on the mother and fetus. Aggressive diuresis is key to maintaining appropriate RV volume postpartum. There is a paucity of data regarding management of this patient population, and no reported cases using inhaled treprostinil, which was chosen for our patient due to its longer duration of action, and safer pregnancy category. Management of our patient was successful as evidenced by peripartum adherence to therapy, successful maternal-fetal outcome, improved RA and RV size, improved PA pressure, and improvement in 6MWD.

CONCLUSIONS: PAH in pregnancy carries high mortality, and there is limited data regarding management of this patient population. Our patient was successfully managed using inhaled treprostinil.

Reference #1 Bedard E, Dimopoulos K, Gatzouls MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J 2009; 30(3)256-265.

Reference #2 Voswinckel R, Enke B, Reichenberger F, et. al. Favorable effects of inhaled treprostinil in severe pulmonary hypertension. J Am Coll Cardiol 2006; 48:1672-1681.

Reference #3 Weiss BM, Zemp L, Seifert B, et. al. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol 1998; 31: 1650-1657.

DISCLOSURE: The following authors have nothing to disclose: Carmel Goudzwaard, Rajive Tandon, Elaine Chen

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Chest. 2011;140(4_MeetingAbstracts):52A. doi:10.1378/chest.1119514

INTRODUCTION: Sarcoidosis is characterized by multiorgan involvement of granulomatous inflammation. It rarely affects female genital tract and even rarer to cause fistulizing disease. Majority of cases of female genital tract sarcoidosis have been seen in uterus with sporadic cases involving ovaries, fallopian tubes, cervix and vagina.

CASE PRESENTATION: A 30 year old African American woman with no past medical histroy presented to our hospital with lower abdominal pain of few months duration. She was diagnosed with fibroids and underwent myomectomy .The pathology revealed areas of hyalinization with foci of necrosis. Her abdominal symptoms persisted and she got readmitted to the hospital. She was started on antibiotics with presumed diagnosis of pelvic inflammatory disease (PID). CT abdomen and chest done at that time showed hilar, bilateral axillary and retroperitoneal lymphadenopathy. Cervical lymph node biopsy was done which revealed well formed granulomas with negative GMS and AFB stains. She was diagnosed with pulmonary sarcoidosis and was started on low dose of steroids. Her abdominal symptoms resolved simultaneously. On tapering her steroids she started to experience recurrent episodes of pelvic pain. On examination she had cervical motion tenderness. CT scan pelvis revealed free fluid in cul-de-sac with multiseptated left ovarian mass. She was treated for PID with antibiotics without much relief. Blood, urine, and cervical cultures also came back negative. She continued with symptoms and soon underwent exploratory laparotomy with left salpingoopherectomy. The pathology reported granulomatous salpingitis with granulomatous oophoritis. A biopsy taken from pelvic wall bowel nodule revealed nodular fibrous and fibrovascular tissue with granulomatous inflammation along with vegetable material in the sample which raised concerns of gastrointestinal perforation. She underwent colonoscopy, small bowel series and lower GI studies which failed to reveal any abnormalities. Soon after her discharge from the hospital she again developed abdominal pain and was found to have right sided tubo-ovarian abscess. She underwent repeat exploratory laparotomy with total hysterectomy and right salpingoophorectomy. The biopsy was consistent with generalized granulomatous disease in the ovary, fallopian tube and the uterus with negative microbiology. Her post operative course got complicated by enterocutaneous fistula followed by enterovaginal fistula. Repeat colonoscopy ruled out any pathological evidence of Inflammatory Bowel Disease. Patient was started on high dose steriods. Patient's stool output from the fistula decreased markedly over a span of one week on treatment suggesting sarcoidosis as the culprit of the fistulas.

DISCUSSION: Sarcoidosis also called Boeck's disease was first identified over 100 years ago in Norway. It is very crucial that before the patient is diagnosed with genital tract sarcoidosis more obscure causes of the granulomatous inflammation are ruled out including coccidiomycosis, lymphogranuloma inguinale, foreign body reaction, tuberculosis and leprosy. Thus microbiological proof is vital to differentiate sarcoidosis from other forms of granulomatous inflammation. Here we present a unique case of sarcoidosis which fulfills the criteria of diagnosis of on the basis of histopathology and clinical response of the patient to the anti-sarcoid chemotherapy. The fistulizing form of sarcoidosis in the female genital tract has not been reported yet. There has been one case report of anal fistula due to sarcoidosis. The fistulizing form also responds adequately to steriods as noted in our case, therefore adequate time should be taken before decision to reoperate the patient is made.

CONCLUSIONS: Non- infectious cases for recurrent tubo-ovarian abscesses should be kept in mind. Sarcoid although rare in the female genital tract can present with features of pelvic inflammatory disease. Fistulizing disease has not been clearly reported in association to sarcoidosis. We report a unique case of female genital tract sarcoidosis with extensive involvement of ovaries, fallopian tubes and uterus, later complicating with fistulas which showed excellent response to steriods.

Reference #1 Boakye, K; Omalu, B; Thomas, L."Fallopian tube and pulmonary sarcoidosis. A case report". J Reprod Med 1997; 42 (8):533-5.

Reference #2 Allen,SL; Judson, MA."Vaginal Involvement in a Patient with Sarcoidosis". Chest 2010; 137:455-456.

Reference #3 Jouannaud, V et col."Is sarcoidosic anal fistula a real entity?".Gastroenterologie Clinique et Bilogique 2004; 28 (1):88-90.

DISCLOSURE: The following authors have nothing to disclose: Maximiliano Tamae Kakazu, Ashima Sahni, Aiyub Patel

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Chest. 2011;140(4_MeetingAbstracts):53A. doi:10.1378/chest.1117717

INTRODUCTION: The presence of multiple bilateral pulmonary nodules on chest imaging has an expansive differential diagnosis that includes metastatic disease, autoimmune disorders, and infectious etiologies. The differential tends to encompass less common etiologies in the setting of younger and asymptomatic patients. One such entity is benign metastasizing leiomyoma (BML).

CASE PRESENTATION: A 43 year old female presented to her primary care physician with complaints of nasal stuffiness, cough for approximately four weeks, and low back pain without associated fevers. Her medical history was notable for congenital deafness, cognitive delay, hepatitis of unclear etiology treated with low dose prednisone since childhood, and history of abdominal myomectomy followed by subsequent hysterectomy with unilateral oophorectomy, She was prescribed antibiotics for the cough, but due to lack of improvement presented to the Emergency Department two weeks later with worsening low back pain and continued non-productive cough. Initial imaging of the chest demonstrated innumerable bilateral pulmonary nodules without evidence of lymphadenopathy. Evaluation of the nodules included CT scan which confirmed the finding of multiple bilateral pulmonary nodules of varying sizes suggestive of metastases seen on the chest x-ray. Patient underwent CT scan of the abdomen and pelvis to examine for possible primary neoplastic lesions, of which none were found. A CT guided fine needle aspiration was subsequently performed which yielded only macrophages and clusters of benign bronchial cells. PET scan was also performed and demonstrated no hypermetabolic foci within the lungs. She was discharged from the hospital as symptoms had resolved and eventually underwent left lower lobe wedge resection approximately three and a half months following her initial presentation. Pathologic examination of the tissue demonstrated smooth muscle proliferation with entrapped epithelial clefts consistent with benign metastasizing leiomyoma. Pathology from the patient’s prior myomectomy and hysterectomy was reviewed and felt to be consistent with leiomyoma without evidence of leiomyosarcoma. Patient was started on leuprolide and received 3 doses with stabilization of nodules on subsequent chest x-rays. She remained asymptomatic during this period. Follow up chest x-ray 12 months following cessation of leuprolide demonstrated minimal enlargement of some of the nodules with repeat chest x-ray 6 months later demonstrating clear increase in size and number of lung lesions. At this time, the patient underwent salpingo-oophorectomy of her remaining ovary. Follow up chest imaging demonstrated significant improvement in the size of her nodules and she remains asymptomatic.

DISCUSSION: Benign metastasizing leiomyoma is an infrequent cause of asymptomatic pulmonary nodules which is generally seen in pre-menopausal females following myomectomy or hysterectomy for uterine fibroids. The lesions are most commonly seen in the lung as well demarcated nodules scattered throughout both lungs fields. These lesions are usually asymptomatic, but can occasionally present with chest discomfort or cough. The etiology of the lesions remains unclear, however, it is felt that they most likely represent hematogenous spread of benign uterine tumors. The diagnosis of BML is made following biopsy either by fine needle aspiration or wedge resection. Pathology typically demonstrates a smooth muscle phenotype with rare mitotic figures, as well as frequent estrogen and progesterone receptor positivity. Management of the disease is mediated through hormonal manipulation through the use of medical or surgical oophorectomy.

CONCLUSIONS: This case highlights a unique cause of multiple pulmonary nodules to be kept in mind in pre-menopausal patients without overt evidence infection or malignancy. It also demonstrates the use of both treatment modalities to achieve regression of the patient’s multiple pulmonary nodules.

Reference #1 Robboy SJ, Bentley RC, Butnor K, et al. Pathology and pathophysiology of uterine smooth-muscle tumors. Environ Health Perspect 2000;108(Suppl 5):779 - 84.

Reference #2 Abramson S, Gilkeson RC, Goldstein JD, Woodard PK, Eisenberg R., and Abramson N. Benign Metastasizing Leiomyoma: Clinical, Imaging, and Pathologic Correlation. AJR 2001; 176:1409-1413.

Reference #3 Pitts S, Oberstein EM, Glassberg MK. Benign metastasizing leiomyoma and lymphangioleiomyomatosis: sex-specific diseases? Clin Chest Med 25 (2004) 343 - 360

DISCLOSURE: The following authors have nothing to disclose: Kendra Hammond, Nidhi Undevia

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Chest. 2011;140(4_MeetingAbstracts):54A. doi:10.1378/chest.1107446

INTRODUCTION: Pulmonary arteriovenous malformations (AVMs) have shown to clinically deteriorate during pregnancy, frequently during the second and third trimester(1). We report the case of a young woman in her first trimester of pregnancy presenting with spontaneous abortion and profound hypoxemia due to diffuse pulmonary AVMs.

CASE PRESENTATION: An eighteen-year old African American woman at 12th week of her first pregnancy presents with vaginal bleeding, abdominal pain and chronic shortness of breath. She denied smoking or current medication use. She suffered from mild intermittent asthma treated with inhaled albuterol as needed since childhood. Her family history was non-contributory. Initial evaluation revealed patient to be tachycardic to 110 beats per minute, with a blood pressure of 138/68 mm/Hg, and oxygen saturation (SaO2) of 85-87% on room air that increased to 93% on 3 liters nasal cannula. Her physical exam revealed mild suprapubic tenderness and blood in vaginal vault. Missed abortion was confirmed by ultrasonography and treated with emergent dilation and curettage. The patient’s hypoxemia persisted despite supplemental oxygen and albuterol and ipratropium nebulizers. Chest radiograph showed no consolidation, effusion or pneumothorax. Computed tomography of the chest with intravenous contrast revealed several areas of small ground glass opacities some with vessels in their central region. There were no discrete nodules or obvious pulmonary arteriovenous malformations. A trans-esophageal echocardiogram with agitated saline showed left atrium opacification after 3.5 cardiac cycles. Bilateral selective and segmental pulmonary angiogram demonstrated diffuse innumerable pulmonary AVMs concentrated predominantly in the left lower lobe measuring between 1.0 and 3mm. The patient was medically managed with supplemental oxygen via nasal cannula. She declined any contraceptive method. Over the following 3 years, the patient had a second spontaneous abortion during her first trimester of pregnancy and three consecutive live births. Her respiratory status remained unchanged.

DISCUSSION: Pulmonary AVMs are abnormal communications between pulmonary arteries and veins that lead to right-to-left blood shunting. They could clinically manifest as persistent hypoxemia, hemoptysis, pleuro-pulmonary hemorrhages or as neurologic complications due to paradoxical embolization. Dyspnea could be masked as a pregnancy-related symptom, but evidence of hypoxemia is always pathologic. Progression of pulmonary AVMs during the second and third trimester has been linked to physiologic changes of pregnancy such as increase in total plasma volume and cardiac output. These changes promote blood flow across the pulmonary AVMs and its further dilation leading to increased shunting and hypoxemia. Out of twenty-six cases of pregnant patients with pulmonary AVMs reported(2), two patients developed symptoms during their first trimester. Enlargement of pulmonary AVMs during the first trimester has been linked to the vasodilating effects of progesterone(1). Our patient’s angiogram showed diffuse pulmonary AVMs, defined as AVMs in all sub-segmental arteries of at least one pulmonary lobe(3). This presentation is not often seen and difficult to detect by initial imaging studies. Patients with diffuse pulmonary AVMs have been able to conceive but the rate of fetal loss is increased(3) as showed in our patient. It is difficult to determine if our patient developed new arterio-venous malformations during pregnancy, or if her pregnancy-related hormonal changes dilated the clinically quiescent pulmonary AVMs.

CONCLUSIONS: Diffuse pulmonary AVMs may become clinically evident during the first trimester of pregnancy as unexplained hypoxemia in the setting of a spontaneous abortion.

Reference #1 Swinburne, A J Fedullo, R Gangemi and J A Mijangos. Hereditary telangiectasia and multiple pulmonary arteriovenous fistulas. Clinical Deterioration During Pregnancy. Chest 1986;89;459-460.

Reference #2 Andrea S. Gershon, Marie E. Faughnan, Kenneth S. Chon, et al. Transcatheter Embolotherapy of Maternal Pulmonary Arteriovenous Malformations During Pregnancy. Chest 2001;119;470-477

Reference #3 Marie E. Faughnan, Yvonne W. Lui, Joel A et al. Diffuse Pulmonary Arteriovenous Malformations. Chest 2000;117;31-38

DISCLOSURE: The following authors have nothing to disclose: Mauricio Danckers, Carlos Alviar, Ruth Minkin

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Chest. 2011;140(4_MeetingAbstracts):55A. doi:10.1378/chest.1119599

INTRODUCTION: Use of in vitro fertilization (IVF) has increased in recent years. Treatment with intramuscular (IM) progesterone in IVF is standard due to its relative safety and high success rates. We report a case of acute eosinophilic pneumonia (AEP) secondary to IM progesterone.

CASE PRESENTATION: A 27 year old female nonsmoker who was six weeks pregnant with twins after IVF presented with three days of fever, chills, progressive dyspnea and cough. The patient denied exposures to birds, molds or sick contacts. She had recently traveled to India. Her only medications were perinatal vitamins and once daily IM progesterone. Upon admission, the patient was febrile to 100.2°F with a respiratory rate of 30 and an oxygen saturation of 88% on room air. Labs were notable for a leukocytosis of 14,700 and an absolute eosinophil count of 900. Computed tomography of the chest revealed bilateral patchy peripheral opacities and pleural effusions. She was admitted to the ICU and given supplemental oxygen and antibiotics for community acquired pneumonia. Despite mild improvement in her dyspnea, labs showed persistent leukocytosis and eosinophilia. She underwent a bronchoscopy. Bronchoalveolar lavage (BAL) demonstrated 70% eosinophils without organisms on cytology. Bacterial, fungal and AFB cultures were negative, as were stool ova and parasites. Antibiotics were discontinued and progesterone treatments were changed to vaginal suppositories. She was discharged on hospital day four with normal oxygen saturation, no dyspnea and significant improvement on chest x-ray.

DISCUSSION: AEP is an uncommon disease characterized by an acute febrile illness, cough, dyspnea and pulmonary infiltrates which can progress to hypoxemic respiratory failure. Although its etiology is unknown, it has been suggested that AEP may be a hypersensitivity reaction to various inhaled antigens. The diagnosis is confirmed by presence of eosinophilia (>25%) on BAL and exclusion of infectious and systemic causes of pulmonary eosinophilia. Treatment of AEP includes elimination of the suspected underlying cause and systemic corticosteroids. Observational studies show rapid clinical improvement with corticosteroids. Success of IVF depends in part on the maintenance of the luteal phase with progesterone. IM and vaginal progesterone have both been used and there is no definite consensus regarding the optimal route of administration. The IM preparation is dissolved in sesame oil with a benzyl alcohol preservative. We believe that our patient had AEP secondary to IM progesterone. The etiology has been proposed to be a hypersensitivity reaction to the sesame oil or the benzyl alcohol. Compared to similar reports in the literature, a unique aspect of this case is the patient's rapid clinical improvement after discontinuation of the drug without initiation of corticosteroids.

CONCLUSIONS: We present the sixth published case of acute eosinophilic pneumonia caused by IM progesterone injections after in vitro fertilization. We believe that this may be an underreported complication. Our patient's rapid clinical improvement without corticosteroids suggests that close observation after discontinuation of IM progesterone is a feasible option in patients who are not severely ill. It is important for physicians and patients who use IM progesterone to be aware of this serious side effect.

Reference #1 Khan A, Jariwala S, Lieman HJ, Klapper P. Acute eosinophilic pneumonia with intramuscular progesterone after in vitro fertilization. Fertility and Sterility 2008; 90: e3-e6.

Reference #2 Jantz MA, Sahn SA. Corticosteroids in acute respiratory failure. Am J Respir Crit Care Med 1999; 160: 1079-100.

DISCLOSURE: The following authors have nothing to disclose: Cyrus Shariat, Derrick Raptis, Audrey Pendleton, Michael Fingerhood, Ronald Goldenberg

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Case Reports: Monday, October 24, 2011

Chest. 2011;140(4_MeetingAbstracts):56A. doi:10.1378/chest.1120048

INTRODUCTION: Trastuzumab, a humanized monoclonal antibody against the extracellular domain of the human epidermal growth factor receptor 2 (HER2), is indicated for the treatment of HER2-positive early or metastatic breast cancer. Common pulmonary complications include dyspnea and cough. We report a case of probable trastuzumab related adverse reaction in a breast cancer patient with clinical manifestations resembling sarcoidosis and characterized by noncaseating granulomas in skin, bone and hilar lymph nodes.

CASE PRESENTATION: A 48 year-old female presented to the hospital with a one month history of shortness of breath, fevers, malaise, nonproductive cough, and violaceous painful plaques on her knees. Ten months prior to presentation, the patient was diagnosed with HER2 positive infiltrating ductal carcinoma of the right breast and underwent mastectomy. One of 20 lymph nodes excised was positive for tumor, and she was staged at T1 N1 M0. Her chemotherapy regimen consisted of a combination of doxorubicin plus cyclophosphamide for 12 weeks, followed by paclitaxel plus concurrent trastuzumab for 12 weeks, and then followed by trastuzumab alone to complete a total of 52 weeks of therapy. On admission, her chest CT revealed enlarged subcarinal, sub-aortic and bilateral hilar lymph nodes along with multiple sub centimeter intraparenchymal and juxta-pleural nodules throughout both lungs with no lobe spared. Given concern for metastatic disease, the patient underwent a positron emission tomography (PET) CT scan revealing hypermetabolic activity within multiple small pulmonary nodules and bilateral hilar lymph nodes. There was also increased tracer uptake in the right anterior and left posterior iliac bones, all suggestive of metastatic disease. The increased tracer uptake in the iliac bones was confirmed with a technetium bone scan. A skin punch biopsy from the lesions on her right knee was performed revealing noncaseating granulomatous nodular dermatitis, consistent with sarcoidosis. Transbronchial needle aspiration of the subcarinal lymph node revealed noncaseating granulomas. CT guided biopsy of the left ilium was performed, again revealing noncaseating granulomas suggestive of osseous sarcoidosis. Subsequently, a one month trial of high dose corticosteroids provided no significant symptom relief. After careful discussion with the oncologist, trastuzumab was discontinued given our concern that the patient’s symptoms and imaging findings may represent an adverse drug reaction. Three months after discontinuation of trastuzumab, the patient’s symptoms including dyspnea and skin rash resolved. Repeat chest CT revealed near complete resolution of the mediastinal and hilar adenopathy as well as the pulmonary nodules. Repeat PET CT also revealed resolution of the abnormal hilar, mediastinal and lung parenchymal lesions along with near complete resolution of the increased tracer uptake in the iliac bones.

DISCUSSION: Trastuzumab is indicated for the treatment of HER2-positive early or metastatic breast cancer. Common pulmonary complications include dyspnea and cough. Granulomatous dermatitis has been recently reported as a potential adverse effect of trastuzumab. Drug induced sarcoidosis has been reported in patients treated with interferon- . Similarly, multiple other medications including bleomycin and etanercept have been reported to cause granulomatous lung lesions that resemble sarcoidosis. To our knowledge, this is the first report of trastuzumab associated granulomatous reaction involving hilar and mediastinal lymph nodes with pulmonary nodules resembling sarcoidosis. The patient’s symptoms and radiologic abnormalities resolved after the discontinuation of trastuzumab.

CONCLUSIONS: In patients receiving trastuzumab, the presence of enlarged lymph nodes and pulmonary nodules might represent a potential granulomatous reaction resembling sarcoidosis and not metastatic disease. Further workup to rule out metastatic disease in these patients is warranted.

Reference #1 Martín G, Cañueto J, Santos-Briz A, Alonso G, Unamuno PD, Cruz JJ. Interstitial granulomatous dermatitis with arthritis associated with trastuzumab.J Eur Acad Dermatol Venereol. 2010 Apr;24(4):493-4.

Reference #2 Romond EH, Perez EA, Bryant J, et al. Trastuzumab plus adjuvant chemotherapy for operable HER2-positive breast cancer. N Engl J Med 2005; 353:1673.

DISCLOSURE: The following authors have nothing to disclose: Rabih Halabi, Catherine Grossman

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Chest. 2011;140(4_MeetingAbstracts):57A. doi:10.1378/chest.1119493

INTRODUCTION: Scleritis is often the first clinical manifestation of a systemic disease. Identifying the underlying disease is critical for proper treatment and prognosis. Approximately 43% of scleritis cases are idiopathic, 48% are related to skeletal and connective tissue diseases and 7% are infection related. Malignancy is rarely associated with scleritis. Two previous reports of malignancy (direct scleral involvement with choroidal melanoma and metastatic adenocarcinoma of unknown origin) were associated with an initial presentation of scleritis. The following case illustrates the importance of suspecting malignancy as a paraneoplastic cause of resistant scleritis.

CASE PRESENTATION: An 81 year old man with COPD on supplemental oxygen, hypertension, hyperlipidemia, diabetes mellitus, osteoarthritis and necrotizing scleritis, was referred for further evaluation and management of worsening bilateral eye pain, despite treatment with daily prednisone for two months. He denied fever, weight loss, cough, hemoptysis or other pulmonary symptoms. The tobacco history was significant for 50 pack-years of use. On exam, he was afebrile, with normal heart and respiratory rates, a blood pressure of 150/58 mmHg, and an oxygen saturation of 95% on 2L of supplemental oxygen via nasal cannula. Lung examination revealed a prolonged expiratory phase and decreased breath sounds. Laboratory results including ANCA, ANA, RF, C3, and C4 were within normal limits. Cultures, cytology and histopathology from scleral biopsies were negative for infection and malignancy. A chest radiograph revealed a mass confirmed by CT scan, which showed a 4.3cm spiculated irregular mass in the right upper lobe with multiple areas of lucency and abnormally enlarged lymph nodes throughout the mediastinum. During bronchoscopy, the endobronchial exam was unremarkable, and endobronchial ultrasound guided fine needle aspiration demonstrated malignancy consistent with adenocarcinoma.

DISCUSSION: His paraneoplastic scleritis required acute treatment with bilateral scleral patches with amniotic membrane grafting to both eyes to stabilize the necrotic areas and oral prednisone. Metastatic survey studies were negative. Significant improvement was noted after chemotherapy was started for the lung adenocarcinoma. Currently, three out of twelve chemotherapy treatment sessions have been given. Prednisone has been discontinued. The bilateral eye pain has resolved, vision continues to improve and a steroid sparing agent was never needed.

CONCLUSIONS: Scleritis is an infrequent and rare inflammatory disorder that can present a diagnostic challenge. In half of the cases it is the initial manifestation of a systemic problem. The most common systemic diseases associated with scleritis are rheumatoid arthritis, Wegener’s granulomatosis, polyarteritis nodosa, systemic lupus erythematosus, relapsing polychondritis, and inflammatory bowel disease. Infections with organisms such as pseudomonas, aspergillus, herpes (simplex or zoster), or tuberculosis may cause severe scleritis that is difficult to treat. Rarely, malignancies can present as scleritis either with direct metastatic involvement or as a paraneoplastic syndrome as demonstrated by this case. Effective therapy is dictated by the diagnosis of the associated systemic, infectious, or, as in this case, malignant etiology. An evaluation for malignancy should be considered in a patient presenting with what appears to be idiopathic scleritis that is resistant to treatment.

Reference #1 Kafkala, et al. Masquerade scleritis. Ocular Immunology and Inflammation. 2005;13:479-482.

Reference #2 Fraser Jr DJ, Font RL. Ocular inflammation and hemorrhage as initial manifestations of uveal malignant melanoma: incidence and prognosis. Arch Ophthalmol. 1979;97:1482-1486.

DISCLOSURE: The following authors have nothing to disclose: Hector Payan, Sergio Burguete, Stephanie Levine

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Chest. 2011;140(4_MeetingAbstracts):58A. doi:10.1378/chest.1119907

INTRODUCTION: Dyspnea is a common symptom in cancer patients, but pulmonary hypertension (PH) is not well defined in this population. Acute cor pulmonale in a cancer patient may be due to cardiac causes, thromboembolism or pulmonary tumor embolism.

CASE PRESENTATION: A 50 year old woman was diagnosed with inflammatory breast cancer metastatic to the contralateral chest and pelvic bones. She received neoadjuvant chemotherapy with docetaxel, doxorubicin and cyclophosphamide followed by modified radical mastectomy. Unfortunately, she was found to have significant residual disease and was started on adjuvant chemotherapy with capecitabine along with radiation therapy to the chest wall. Further staging showed no evidence of disease, and chemotherapy was stopped. Two months later, the patient developed progressive dyspnea and hypoxemia. Work-up at an outside hospital was negative. At our institution, she was found to be tachycardic and tachypneic. Her initial oxygen saturation was 94% on 45% FiO2. On physical examination, she was alert and had clear lung sounds. Her abdominal exam revealed a firm, enlarged liver. No jugular venous distention or pitting edema was present. Laboratory data showed a mild leukocytosis (12,200/UL), carcioembryonic antigen at 596.8 u/mL, aspartate aminotransferase 518 IU/L, alanine aminotransferase 206 IU/L, alkaline phosphatase 175 IU/L, lactate dehydrogenase was noted to be >42,000 IU/L, and arterial blood gas performed on room air showed a pH 7.49, pCO2 33 mmHg and pO2 32 mmHg with 68% saturation. Chest radiography was normal, and CT angiogram of the chest showed no pulmonary embolus, but extensive metastases within the liver. A PET/CT scan demonstrated a mottled appearance of FDG-avid metastases within the liver. The patient was initiated on ixabepilone along with corticosteroids. The patient had progressive hypoxemia, and orthodeoxia with a supine oxygen saturation of 94% that decreased to 85% on standing and a nadir of 67% on ambulation for ten feet. A ventilation-perfusion scan revealed normal ventilation with multiple peripheral, non-segmental perfusion defects in both lungs. An electrocardiogram showed right axis deviation, and a transthoracic echocardiogram revealed a hyperdynamic left ventricle with a severely dilated right ventricle with paradoxical septal motion and elevated right ventricular systolic pressure. The echocardiogram was repeated with agitated saline but did not show right-to-left shunt. Abdominal ultrasound showed no evidence of portal hypertension. Right heart catheterization (RHC) was performed. Pulmonary artery pressure was 77/34 mmHg with a mean pulmonary pressure of 49 mmHg and a pulmonary artery occlusion pressure of 15 mmHg. Further therapy was discussed, but the patient rapidly deteriorated and arrested. Autopsy showed extensive hematogenous dissemination of metastatic carcinoma in bilateral lungs.

DISCUSSION: Acute PH in cancer patients may often suggest thromboembolic disease, but severe PH as noted in our patient suggest either acute on chronic PH or tumor embolus. Tumor embolism to the lung has been reported primarily in autopsy series, but mostly in gastric adenocarcinoma. Pulmonary tumor thrombotic microangiopathy (PTTM) may also manifest with severe PH and is clinically indistinguishable from tumor embolism. PTTM may only be differentiated histologically from tumor embolism, where pathology specifically demonstrates pulmonary vascular obstruction and infiltration with tumor. Additional workup such as a ventilation perfusion scan may reveal multiple subsegmental mis-matched defects. Treatment options are limited, and the condition is often fatal.

CONCLUSIONS: This case illustrates the diagnostic dilemma posed by a patient with metastatic breast cancer that presents with severe acute PH. A high clinical suspicion may facilitate prompt diagnosis and intervention. The effectiveness of chemotherapy, anticoagulation and vasoactive medications, however, has not been well studied in this patient population.

Reference #1 Roberts KE, Hamele-Bena D, Saqi A, Stein CA and Cole RP. Pulmonary Tumor Embolism: A Review of the Literature. Am J Med 2003;115:228-232.

DISCLOSURE: The following authors have nothing to disclose: Mark Warner, Saadia Faiz, Kimberly Koenig, Lavinia Middleton, Peter Kim, Elie Mouhayar, Bela Patel, Lara Bashoura

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Chest. 2011;140(4_MeetingAbstracts):59A. doi:10.1378/chest.1117151

INTRODUCTION: Metastatic skin lesions has been rarely presented as an early sign of advanced lung cancer and often misdiagnosed. We present a case of metastatic non small cell carcinoma of the lung who presented with painful zosteriform skin rash.

CASE PRESENTATION: A 59 year old Caucasian female with history of 40 pack year smoking , presented with a painful, blistering skin rash in her chest which was initially diagnosed with Shingles in December 2010. The skin rash has progressively worsened and extended to her left axillary and breast and she started developing hoarseness, dyspnea and neck swelling over the next few months. In April 2011, the CT of neck revealed multiple cervical lymphadenopathy causing deviation of the trachea. Chest x-ray showed a left upper lobe mass. A whole body CT confirmed a 5.9 x 7 x 5 cm mass involving the anterior segment of the left upper lobe, extensive mediastinal and supraclavicular adenopathy which displaces the trachea to the right, also large bony metastasis to the manubrium with lytic destruction, multiple hepatic lesions, and bilateral adrenal metastasis. A CT guided biopsy of sternal mass was consistent with adenocarcinoma of lung. The skin biopsy showed extensive expansion of the dermis by multiple nests of a moderately differentiated lung adenocarcinoma with associated mucin.

DISCUSSION: Cutaneous metastases are rare and they are usually diagnosed in patients with a known primary malignancy. Skin nodules or masses are the most common patterns however an inflammatory pattern mimicking infection, condyloma, epidermal inclusion cyst, chancre, and herpes zoster are not uncommon. Although cutaneous metastases in lung cancer without obvious primary lesions are very rare, this diagnosis should still be considered, particularly in patients with history of smoking. The percentage of patients with lung cancer that develop cutaneous metastases ranges from 1 to 12 percent. In 20-60 percent of cases the skin lesions present before or synchronously with the diagnosis of the primary tumor. Histologically, cutaneous metastases from the lung are frequently moderately or poorly differentiated adenocarcinoma. Immunohistochemistry markers including anti-thyroid transcription factor (TTF) and CK7/20 are usually helpful to distinguish the primary tumor. Treatment of solitary cutaneous metastases usually includes surgery alone or combined with chemotherapy, and/or radiation. If multiple cutaneous lesions or internal metastases exist, chemotherapy is the primary option.

CONCLUSIONS: Zosterioform rash is a rare presentation of cutaneous metastases in non small cell carcinoma of the lung and suggests multiple distant metastases with a poor prognosis. Persistent zosteriform lesions without response to treatment should raise the probability of underlying lung cancer particularly in smokers.

Reference #1 1.Saeed S, Keehn C, Morgan M. Cutaneous metastasis: a clinical, pathological, and immunohistochemical appraisal. J Cutan Pathol 2004: 31: 419-430

Reference #2 2.Mego M, Sycova-Mila Z, Martanovic P, Liskova S, Obertova J, Mardiak J. Inflammatory skin metastasis as a first sign of progression of lung cancer--a case report. Klin Onkol. 2010;23(6):449-51.

Reference #3 3.Bianchi L, Orlandi A, Carboni I, Costanzo A, Chimenti S. Zosteriform metastasis of occult bronchogenic carcinoma. Acta Derm Venereol 2000; 80: 391.

DISCLOSURE: The following authors have nothing to disclose: Mostafa Tabassomi, Haifaa Abdulhaq, Eyad Almasri, Nastran Hashemi

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Chest. 2011;140(4_MeetingAbstracts):60A. doi:10.1378/chest.1118864

INTRODUCTION: Cardiac involvement secondary to malignant lymphoma has rarely been described as a presenting feature of this disease. We describe an unusual case of a patient with advanced T-cell lymphoma presenting with dyspnea due to a restrictive cardiomyopathy resulting from endocardial tumor infiltration.

CASE PRESENTATION: A 29-year-old Hispanic female with no medical history presented with a 3-month history of fevers, chills, night sweats, and weight loss. Physical exam revealed generalized lymphadenopathy and splenomegaly. The patient underwent an excisional axillary lymph node biopsy confirming the diagnosis of stage IV peripheral T-Cell lymphoma. One month later, the patient presented with worsening shortness of breath. A chest radiograph revealed mild cardiomegaly and increased interstitial markings. Chest CT was notable for smooth and nodular intra- and interlobular septal thickening in lower lung fields, most consistent with lymphangitic carcinomatosis. At the time of our examination, the patient was noted to be tachycardic, tachypneic, and mildly hypoxic. Pulmonary exam revealed bibasilar rales; cardiac exam was significant for an S3 gallop, a soft systolic murmur, and jugular venous distention estimated at 12 cm H20; abdominal exam revealed splenomegaly; trace lower extremity edema was also noted. Echocardiogram demonstrated an irregularly thickened left ventricle with trabeculations, resulting in severe diastolic dysfunction; however, the systolic function was preserved. Further work-up with contrast enhanced chest CT was notable for hypodensity of the myocardium corresponding to the area of irregularity seen on the echocardiogram. This was followed by a cardiac MRI, with short Axis Phase Sensitive Inversion Recovery Delayed enhancement, showing increased signal throughout the endocardium of the left ventricle, as well as pronounced thickening of the left ventricle consistent with an infiltrative disease. The patient improved dramatically after treatment with diuresis, beta-blockers, and ACE inhibitors. Follow-up chest imaging, including repeat chest CT, showed complete resolution of the intra- and interlobular septal thickening. Furthermore, post-chemotherapy echocardiography demonstrated complete resolution of the endocardial infiltrative process.

DISCUSSION: Historically, cardiac metastases have been reported in anywhere from 8.7% to 20% of patients with lymphoma post-mortem, the vast majority of which are of B-cell origin [1,2]. Despite the development of advanced imaging modalities, this disease is seldom detected clinically. We describe a very rare presentation of T-cell lymphoma manifesting as diffuse endocardial infiltration with resultant diastolic heart failure. Dyspnea in patients with advanced cancer and interstitial infiltrates commands an extensive differential diagnosis, including a variety of infectious processes, pulmonary edema, lymphocytic interstitial pneumonia, and lymphangitic carcinomatosis. In this patient, the nodular pattern of the intra- and interlobular septal thickening was most concerning for lymphangitic carcinomatosis, particularly as some literature suggests an increased risk of pulmonary involvement in patients with endocardial infiltration of lymphoma [3]. Nonetheless, the physical exam was consistent with heart failure, prompting the appropriate investigation and diagnosis of restrictive cardiomyopathy due to endocardial invasion.

CONCLUSIONS: Cardiac involvement due to lymphoma is a rare ante-mortem finding as this disease seldom presents as heart failure. The differential diagnosis for dyspnea in a patient with advanced lymphoma and interstitial edema is broad and all potential causes must be entertained. To our knowledge, this is the first reported case of stage IV peripheral T-cell lymphoma presenting with pulmonary edema due to endocardial tumor invasion. Furthermore, the presence of delayed endocardial enhancement on cardiac MRI has never been reported in patients with cardiac involvement due to lymphoma.

Reference #1 O’Mahony D., Piekarz R., Bandettini P. et al. Cardiac involvement with lymphoma: a review of the literature. Clin Lymphoma Myeloma. 2008 August; 8(4): 249-252.

Reference #2 Giunta R., Cravero R., Granata G. et al. Primary cardiac T-cell lymphoma. Ann Hematol. 2004; 83: 450-454.

Reference #3 O’Mahony D., Debnath I., Janik J., et al. Cardiac involvement with human T-cell lymphotrophic virus type-1-associated adult T-cell leukemia/lymphoma: The NIH experience. Leuk Lymphoma. 2008 March; 49(3): 439-46.

DISCLOSURE: The following authors have nothing to disclose: Tarik Ngab, Nidhi Nikhanj, Mariam Thomas, Robin Wachsner, Nader Kamangar

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Chest. 2011;140(4_MeetingAbstracts):61A. doi:10.1378/chest.1112642

INTRODUCTION: PET-CT scans are used to clinically stage cancers; however clinicians must know the causes of false positive and negative results to fully interpret the test results. We report a case of synchronous head and neck and lung cancer in a patient with a false positive PET scan in the larynx due to excessive muscle activity of the right vocal cord as it compensates for the paralyzed left vocal cord.

CASE PRESENTATION: A 64-year-old female smoker presented with a two month history of hoarseness and a chronic dry cough. Despite treatment with oral antibiotics her symptoms persisted. Endoscopic examination revealed a non motile left vocal cord with a mass. Biopsy revealed squamous cell carcinoma. A CT scan of the head and neck revealed no nodal disease in the neck but showed a left upper lobe lung mass. This led to a fused CT/PET (positron emission tomography) scan which showed increased fluorodeoxyglucose (FDG) uptake in the left hilar mass, mediastinal lymphadenopathy and the right vocal cord, but none in the left vocal cord where the lesion was found. Bronchoscopy with EBUS biopsy of an enlarged lower paratracheal lymph node was positive for squamous cell carcinoma. The patient was diagnosed with synchronous primary lung cancer (stage IIIA) and T1 N0 M0 laryngeal carcinoma. There was no pathologic correlate to the FDG uptake on the right vocal cord. The patient was not a candidate for lung cancer surgery and underwent chemoradiation to the lung and larynx. After three months she had an improvement in her phonation and repeat imaging revealed a decrease in the size of the lymphadenopathy

DISCUSSION: This case illustrates a false positive PET scan in the larynx due to excessive muscle activity of the functional right vocal cord as it compensates for paralysis of the left vocal cord(1). Compression of the left recurrent laryngeal nerve by the pathologic mediastinal lymphadenopathy led to left vocal cord paralysis. Symmetrically increased FDG muscle uptake in the neck and thoracic paravertebral region is attributed to physiological uptake due to symmetrical brown fat activation. Nonpathologic asymmetrical increased muscle uptake occurs with muscle contractions, uncomfortable positions after radiotracer injection, arthritis, or surgery(2). In instances of recent biopsy or in areas of muscle activity due to swallowing or vocalization, the PET scan may be falsely positive(2). False positives are seen in patients with active infection and inflammation where there is increased glycolysis. False negative PET scans are seen where there is impaired blood flow and minimal radiotracer can reach the area(2). In areas of tumor necrosis there is less metabolically active tissue so PET scans are falsely negative(2). It is reported that 14% of patients with head and neck cancers will develop lung cancer, and 31% of these will be synchronous(3). There are no pathological stains or genetic markers that identify the primary site in patients with synchronous squamous cell cancers(3). It is dependent on the clinical history and radiographic presentation of the patient that assist in determining which arose first and which is considered metastatic. This case is interesting as there is a clinical and radiologic pattern of synchronous primary squamous cell carcinomas of the larynx and lung instead of metastatic disease from one primary. Treatment involves resection of the lung cancer, if feasible, followed by treatment of the head and neck cancer with chemotherapy and concurrent radiation(3).

CONCLUSIONS: Awareness of the conditions and mechanisms by which false positive and negative results occur will assist in the interpretation of PET scans. Such knowledge may help clinicians decide whether or not acquisition of tissue is needed to confirm PET-CT scan findings suggestive of metastatic disease.

Reference #1 Lee M, Ramaswamy MR, Lilien DL, Nathan CA. Unilateral vocal cord paralysis causes contralateral false-positive positron emission tomography scans of the larynx. Ann Otol Rhinol Laryngol 2005;114:202-206.

Reference #2 Koppula D, Rajendran JG. PET-CT in head and neck cancer. Appl Radiol 2010;4:20-27.

Reference #3 Douglas WG, Rigual NR, Loree TR, Wiseman SM, Al-Rawi S, Hicks WL. Current concepts in the management of a second malignancy of the lung in patients with head and neck cancer. Curr Opin Otolaryngol Head Neck Surg 2003; 11: 85-88.

DISCLOSURE: The following authors have nothing to disclose: Luca Paoletti, Hiren Mehta, Leonie Gordon, Boyd Gillespie, Nicholas Pastis

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Chest. 2011;140(4_MeetingAbstracts):62A. doi:10.1378/chest.1119639

INTRODUCTION: Intravascular large B-cell lymphoma (IVL) is a rare, diagnostically challenging, and usually fatal disease classified as a high-grade malignant lymphoma and characterized by proliferation of malignant cells within the lumen of small blood vessels. While IVL can involve the vasculature of any organ, primary pulmonary involvement is uncommon and secondary pulmonary arterial hypertension has only rarely been described(1,2). We present a case of fatal IVL causing hypoxemic respiratory failure and right ventricular failure due to occlusion of the pulmonary vasculature with a monoclonal B-cell population; e.g. IVL.

CASE PRESENTATION: A sixty-eight year old male was evaluated at our institution with a 3 month history of progressive dyspnea, fatigue, and low grade fevers. His review of systems was positive for anorexia, 40 lb weight loss in 3 months, occasional night sweats, mild headache and depression. Physical exam revealed a grade 3/6 systolic ejection murmur. Laboratory studies were performed and significant for hemoglobin 9.6 g/dL, MCV 84.7, ESR 120 mm/hr, CRP 159 mg/L, LDH 576 U/L and Ferritin 1313ug/L. Initial concerns were for a malignant or infectious process. All cultures remained negative. The patient continued to be more dyspneic and pulmonary function test were done. This showed a nonspecific reduction in his FVC 3.38 (76%), marked reduction of his DLCO 12.5 (48%), and desaturation with exercise (87%). An echocardiogram demonstrated a bicuspid aortic valve, normal ejection fraction (63%) and pulmonary hypertension (right ventricular systolic pressure (RVSP) 41mmHg). A bone marrow biopsy, FDG PET and CT Chest were unrevealing. He was empirically treated with both antibiotics and steroids. Despite this he developed worsening hypoxia, fatigue, delirium and was readmitted to the hospital. On hospital day eight, the patient developed shock and respiratory failure and was transferred to the intensive care unit. An urgent echocardiogram was performed which demonstrated acute changes when compared to his prior echocardiogram. He had developed worsening pulmonary hypertension with a RV failure, D-shaped left ventricle and RVSP of 56mmHg (SBP 73 mmHg). A pulmonary embolism was suspected and thrombolytics were administered. The patient continued to worsen and developed multiorgan failure and died despite multiple attempts at CPR and resuscitation. Autopsy was requested and revealed diffuse intravascular large B-cell lymphoma.

DISCUSSION: Intravascular large B-cell lymphoma is a rare malignant lymphoma characterized by selective growth of neoplastic cells within blood vessel lumina, particularly small vessels, e.g. capillaries. It commonly affects the vessels of the skin and central nervous system although blood vessels in other organs may be occluded(1). Due to its varied and non-specific clinical manifestations, antemortem diagnosis is often difficult. Our case of IVL was not diagnosed despite extensive outpatient evaluation and prolonged symptoms. He presented to the ICU with hypoxic respiratory failure, hemodynamic compromise and echocardiographic findings suggesting acute worsening of right heart failure which progressed despite support and resulted in his death. Post-mortem examination did not reveal PE, rather histopathologic analysis demonstrated obstruction of the pulmonary arterioles by large lymphoma cells. We conclude this was the mechanism leading to decompensated acute right heart failure and death.

CONCLUSIONS: Intravascular lymphoma remains a diagnostic challenge and is commonly unrecognized pre-mortem. Preferential involvement of the lumen of the pulmonary vasculature leading to hypoxia and decompensated right heart failure is a rare presentation of a rare disease. Clinicians should consider IVL within the spectrum of etiologies of acute and subacute right heart failure/PAH in the context of systemic inflammatory illness.

Reference #1 Aouba A, Diop S, Saadoun D, et al. Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: case report. Am J Hematol. 2005;79:46-49.

Reference #2 Snyder LS, Harmon KR, Estensen RD. Intravascular lymphomatosis (malignant angioendotheliomatosis) presenting as pulmonary hypertension. Chest. 1989;96:1199-1200.

DISCLOSURE: The following authors have nothing to disclose: Darlene Nelson, Craig Daniels

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Chest. 2011;140(4_MeetingAbstracts):63A. doi:10.1378/chest.1117113

INTRODUCTION: Inhaled nitric oxide (iNO2) is an effective pulmonary vasodilator in treatment of pulmonary hypertension. Prior studies demonstrated a decrease in pulmonary arterial pressure and pulmonary vascular resistance and increase in cardiac output at a dosage between 20-80ppm. However, the effect of lower dosage in conjunction with high flow oxygen has not been well-studied.

CASE PRESENTATION: A 63 year-old woman with obese hypoventilation syndrome, severe pulmonary hypertension, patent foramen ovale with right-to-left shunt, coronary artery disease with RCA/LAD stents, and diabetes was admitted for diarrhea. She was transferred to MICU for oxygen desaturation to 88% on 5L nasal cannula, hypotension, and decrease urinary output. Her right heart catheterization prior to admission showed pulmonary arterial pressure of 95/22mm and mean pulmonary arterial pressure (MPAP) of 56mm Hg on 100% FiO2. The MPAP improved to 42-45mg with 20-40ppm of iNo2. Her cardiac index was 2.0 L/min/m2 on 100% FiO2 and improved to 3.2 L/min/m2 on 20-40ppm of iNO2. Upon arrival to MICU, her vitals were pulse 74, blood pressure 82/65, respiration rate 24, and O2 saturation of 95% on 4 liters nasal cannula. A right internal jugular triple lumen catheter was inserted and showed elevated CVP of 19-20mm Hg. Physical exam showed morbid obesity, right sided S3 gallop, clear lungs, and 2+ bilateral leg edema. Laboratory findings were significant for HCO3 18, Cr 1.6, NT-BNP 771, ABG 7.31/36/62/18 at room air, and SvO2 42%. CXR showed mild cardiomegaly without focal consolidation or pulmonary edema. The patient was started on dobutamine and norepinephrine for obstructive shock. 20ppm of iNO2 with 4L O2 was initiated and CVP improved from 19-20mm Hg to 11-14mm Hg, which rebounded to 18-20mm Hg when the iNO2 was accidentally turned off. iNO2 was reinstated and subsequently weaned down to 10ppm on hospital day 3 and high flow oxygen at 30L/min with 60% FiO2 was added on hospital day 4. iNO was weaned off slowly at 1.5ppm/day and high flow O2 was titrated down to 4L nasal cannula over 9 days. Sildenafil was restarted at 20mg po bid and titrated up to 40mg tid by the time iNO2 was completely weaned off.

DISCUSSION: The treatment of pulmonary hypertension involves targeting various mechanisms of the pathophysiology, including pulmonary vasoconstriction, endothelial and smooth muscle cell proliferation, and thrombosis. These pathophysiological parameters involve the endothelin, nitric oxide, and prostacyclin pathways. Prior to FDA approval of iloprost, inhaled NO2 was the only available inhaled therapy for pulmonary hypertension. It is a vasodilator restricted to pulmonary vasculature without affecting the systemic circulation. In a pilot study involving patients with pulmonary hypertension and right heart failure, iNO2 increased cardiac output and stroke volume and decreased pulmonary vascular resistance at a mean dosage of 35ppm without affecting systemic arterial pressure1. Another study demonstrated similar findings as well as decreased flow through PFO at a dosage of 80ppm2. Other studies demonstrated similar findings at a dosage of 40ppm. These studies suggest that iNO2 is effective in treating pulmonary hypertension with hemodynamic instability by selectively targeting pulmonary vasculature and decreasing right ventricular afterload, resulting in overall improvement in cardiac output.

CONCLUSIONS: The concomitant administration of high flow oxygen and iNO2 in the treatment of pulmonary hypertension has not been well studied. Our patient’s obstructive shock physiology improved at a dosage of iNO much less than what had been published previously. We postulate that the addition of high flow oxygen may drastically decrease the iNO2 requirement and the combination of high flow oxygen and low dose iNO2 may be as effect as iNO2 alone at a higher dose.

Reference #1 Sangeeta B, Christensen J, O’Connor M et al. Response to Inhaled Nitric Oxide in Patients with Acute Right Heart Failure. Am J Respir Crit Med 1999; 159: 571-579.

Reference #2 Inglessis I, Shin J, Lepore J et al. Hemodynamic Effect of Inhaled Nitric Oxide in Right Ventricular Myocardial Infarction and Cardiogenic Shock. J Am Coll Cardiol. 2004 Aug 18;44(4):793-8.

Reference #3 Hoeper MM, Olschewski H, Ghofrani HA et al. A comparison of the acute hemodynamic effects of inhaled nitric oxide and aerosolized iloprost in primary pulmonary hypertension. German PPH study group. J Am Coll Cardiol. 2000 Jan;35(1):176-82

DISCLOSURE: The following authors have nothing to disclose: Joseph Huang, David Fridman

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Chest. 2011;140(4_MeetingAbstracts):64A. doi:10.1378/chest.1117995

INTRODUCTION: Cardiogenic shock from pulmonary embolism (PE) is often fatal. Therapies include systemic thrombolysis, catheter thrombolysis, surgical embolectomy, and catheter embolectomy. Extracorporeal membrane oxygenation (ECMO) has sporadically been used in treating hemodynamically significant PE. We report the case of a 38 year-old man with PE and refractory shock after systemic thrombolysis who was supported with veno-arterial (VA) ECMO with hemodynamic improvement. This case demonstrates the successful use of ECMO in massive PE with refractory shock after systemic thrombolysis.

CASE PRESENTATION: A 38 year-old man presented to the hospital with bilateral leg pain and dyspnea. Three years prior he had deep venous thrombosis (DVT) following knee arthroscopy, treated with surgical thrombectomy and systemic anticoagulation. An inferior vena cava (IVC) filter was placed. For one year he had not taken systemic anticoagulation. His mother and brother had a history of DVT. Physical examination revealed a man in no distress with normal vital signs and bilateral lower extremity edema. Lower extremity ultrasound revealed acute DVT in the right common femoral and left common iliac veins. Abdominal computed tomography (CT) demonstrated clot extending through the IVC filter. Chest CT demonstrated filling defects in the distal right main pulmonary artery and left lower lobe pulmonary artery. Transthoracic echocardiography revealed mild right ventricular dysfunction. Electrocardiogram, troponin, and pro-brain natriuretic peptide were normal. The patient received heparin infusion and underwent pharmacomechanical thrombectomy of the IVC DVT. The following day he suffered acute respiratory failure and shock while ambulating. Despite mechanical ventilation with an FiO2 of 1.0, fluid resuscitation, and vasopressor support, he did not improve. Transthoracic echocardiography demonstrated acute pulmonary hypertension, severe right ventricular dilatation and hypokinesis but preserved contractility of the apex. He received alteplase 100 mg intravenously for presumed massive PE, but remained hypotensive four hours later. VA ECMO was instituted through an 8mm Gelweave graft sewn to the femoral artery and a 22Fr vacuum assist cannula passed into the right atrium via the femoral artery. Bleeding at cannulae sites due to recent thrombolysis and anticoagulation necessitated multiple transfusions. After 24 hours, transesophageal echocardiography showed improved right ventricular function, and ECMO was discontinued. The patient recovered fully and was discharged on low molecular weight heparin. Thrombophilia workup was unrevealing.

DISCUSSION: Mortality from cardiogenic shock after a massive PE approaches 75%.(1) Anticoagulation, which relies on intrinsic fibrinolysis, might not restore systemic circulation quickly. Case reports and series have explored ECMO as a means of unloading the right ventricle and supporting systemic circulation in massive PE. ECMO has been utilized mainly in patients with contraindications to other interventions or as a bridge when profound shock precluded their immediate use. Fewer reports describe ECMO in patients with refractory shock after treatment.(1-3) Our case demonstrates the use of ECMO in a patient with massive PE and refractory shock despite systemic thrombolysis. ECMO allowed our patient time in which right ventricular function recovered and shock reversed. This case illustrates ECMO as a feasible salvage therapy in massive PE with refractory shock after treatment.

CONCLUSIONS: ECMO should be considered for the treatment of PE with refractory cardiogenic shock after systemic thrombolysis.

Reference #1 Arlt M, Philipp A, Iesalnieks I, Kobuch R, and Graf BM. Successful use of a new handheald ECMO system in cardiopulmonary failure and bleeding shock after thrombolysis in massive post-partal pulmonary embolism. Perfusion. 2009;24:49-50.

Reference #2 Misawa Y, Fuse K, Yamaguchi T, Saito T, and Konishi H. Mechanical circulatory assist for pulmonary embolism. Perfusion. 2000;15:527-29.

Reference #3 Deehring R, Kiss AB, Garrett A, and Hillier AG. Extracorporeal membrane oxygenation as a bridge to surgical embolectomy in acute fulminant pulmonary embolism. Am J Emerg Med. 2006;24:879-80.

DISCLOSURE: The following authors have nothing to disclose: Jennifer Howes, Michael Khilkin, Joseph DeRose, Peter Dicpinigaitis, Alina Dulu

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Chest. 2011;140(4_MeetingAbstracts):65A. doi:10.1378/chest.1118734

INTRODUCTION: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare arrhythmogenic disorder characterized by stress induced ventricular tachyarrhythmia resulting in syncope or sudden death. We present a case of a young girl with CPVT presenting in cardiogenic shock treated with cardiac sympathetic denervation.

CASE PRESENTATION: A 9-year old girl presented after a near drowning even in cardiogenic shock secondary to refractory ventricular tachycardia (VT). Despite multiple attempts at cardioversion as well as lidocaine and high-dose beta-blockers, the patient remained in VT necessitating ECMO support to maintain circulation and hemodynamics. She was then transferred to our institution for further management. Further questioning revealed a history of episodic exercise induced syncope and questionable seizures and inspection of her EKG showed bidirectional ventricular tachycardia characteristic of CPVT. She was taken to the operating room for left cardiac sympathetic denervation consisting of resection of the T1-T4 ganglia via left thoracoscopy. Following this procedure she remained in NSR and was weaned from ECMO two days later. Intravenous administration of a beta-blocker was initially continued while the patient made a satisfactory recovery from surgery. The following hospital course was complicated by cholecystitis eventually requiring cholecystectomy and a prolonged recovery from a neuropsychological perspective. On multiple occasions during this complicated hospital course the patient developed cardiac tachyarrhythmias although she always remained hemodynamically stable and had no further episodes of sustained VT. Yet, in view of the underlying disease and risk of lethal arrhythmias implantation of an ICD as a preventive measure was warranted. She was ultimately discharged home 32 days after her VATS with left cardiac sympathetic denervation. An uneventful further recovery followed at home. During the subsequent 18 months of follow up she remained asymptomatic from a cardiovascular standpoint, had an unrestricted, age-appropriate physical activity level and no recorded events of VT or shocks fired by the ICD.

DISCUSSION: CPVT, first described in 1975, is a rare genetic arrhythmogenic disorder with an estimated prevalence of 1:10,000. It is a disorder of abnormal myocardial calcium homeostasis characterized by life-threatening ventricular arrhythmias triggered during states of high sympathetic output. The mainstay of treatment is protection of the unstable heart both pharmacologically with beta blockers and mechanically with placement of an ICD [1]. Even with these interventions, episodes of VT can occur and can result in the phenomenon of “electrical storms” in which the first ICD shock restores NSR. However, the pain and fear associated with this can cause another surge of catecholamines triggering further arrhythmic episodes followed by ICD shocks resulting in a vicious cycle. In these cases, cardiac sympathetic denervation consisting of division of the left sympathetic chain from T1-T4 has proven useful [2], and in our case life-saving. This surgery can be done thoracoscopically via 2 or 3 small (1cm) incisions with minimal morbidity [2].

CONCLUSIONS: CPVT, although rare, should be considered in all young patients presenting with ventricular arrythmias or with a history of stress/exercise induced syncope. Beta blockers and ICD placement are the mainstays of treatment, however, thoracoscopic cardiac sympathetic denervation, a procedure with minimal morbidity, is a valuable surgical adjunct in difficult to control cases or in life-threatening situations.

Reference #1 Sumitomo N, Harada K, Nagashima M, Yasuda T, Nakamura Y, Aragaki Y, Saito A, Kurosaki K, Jouo K, Koujiro M, Konishi S, Matsuoka S, Oono T, Hayakawa S, Miura M, Ushinohama H, Shibata T, Niimura I. Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death. Heart. 2003 Jan;89(1):66-70

Reference #2 Wilde AA, Bhuiyan ZA, Crotti L, Facchini M, De Ferrari GM, Paul T, Ferrandi C, Koolbergen DR, Odero A, Schwartz PJ. Left cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia. N Engl J Med. 2008 May 8;358(19):2024-9.

DISCLOSURE: The following authors have nothing to disclose: Moritz Wyler Von Ballmoos, Ghulam Murtaza, James Tweddell, Mario Gasparri

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Chest. 2011;140(4_MeetingAbstracts):66A. doi:10.1378/chest.1119859

INTRODUCTION: Reports of adverse events resulting from the injection of silicone for cosmetic purposes have been increasing in the scientific literature and popular media, emphasizing the importance of diagnostic consideration, disease recognition and understanding of pathophysiology. Here we present a previously unreported cardiopulmonary manifestation of systemic silicone embolization.

CASE PRESENTATION: A 22-year-old woman was brought to the hospital for shortness of breath that began hours before arrival. She denied chest pain, cough, fever, and recent illness. She had no medical history and did not use tobacco or illicit drugs; her only medication was an oral contraceptive. Initial exam revealed temperature 98.4 F, pulse 151 beats/min, blood pressure 112/60mmHg, and respirations 42/min with oxygen saturation 92% while on 100% oxygen via non-rebreather mask. She was diaphoretic with labored respirations. Her chest was clear to auscultation. She had no adventitious heart sounds, jugular venous distension, or extremity edema; the remainder of her exam was unremarkable. Electrocardiogram confirmed sinus tachycardia and revealed evidence of right heart strain. Chest radiography showed diffuse bilateral infiltrates. Various laboratory panels were sent and were notable for: leukocytosis of 29,900/microliter, bicarbonate 21mmol/L, D-dimer 1,514ng/mL, lactate 7.1mmol/L, and troponin 5.16ng/mL. Her respiratory status further declined requiring endotracheal intubation; venous blood gas prior to intubation revealed pH 6.97, PCO2 78mmHg, and PO2 57mmHg. Bedside echocardiogram revealed acute right ventricular (RV) failure. CT pulmonary angiogram showed no pulmonary embolism but confirmed bilateral alveolar opacities and interstitial prominence. Central venous access was obtained and vasopressors initiated for hypotension despite intravenous fluid administration. A friend of the patient later gave additional history: the patient had cosmetic material injected into her buttocks earlier that day by “doctors from Mexico.” The patient remained acidemic, hypercapnic and hypoxemic despite high minute ventilation with high FiO2 and PEEP. She also exhibited renal dysfunction with increasing creatinine, hyperkalemia and anuria, in addition to profound acidosis. Hemodialysis was attempted, but further cardiovascular collapse ensued with profound hypotension and bradycardia leading to cardiac arrest; the patient expired despite extensive resuscitative measures.

DISCUSSION: Existing case reports of illness following silicone injection display a variety of clinical presentations, with the majority of patients suffering neurologic and respiratory complications. The most recognized pulmonary complications include embolus, pneumonitis, alveolar hemorrhage and acute respiratory distress syndrome. The precise mechanism of pulmonary injury is unclear, but is unlikely limited to the physical embolic phenomenon, particularly given the evidence of parenchymal inflammation despite the relatively inert nature of silicone. Our patient presented with acute RV failure, which was most likely induced by pulmonary vascular injury due to silicone embolization. Elevated right sided pressures measured via pulmonary arterial catheterization have been noted in prior case reports and were presumed to be secondary to large pulmonary emboli, though the presence of these emboli was not confirmed by visualization on imaging. The possibility exists that acute pulmonary arterial hypertension and right ventricular failure may play a larger role in this disease process than previously suspected.

CONCLUSIONS: Soft tissue injection of silicone for cosmetic purposes is dangerous and often deadly, resulting in varied complications throughout the body, most notably neurologic and respiratory. Understanding the pathophysiology of this disease process may prevent the loss of young lives in the future. To our knowledge, this is the first reported case of cosmetic silicone injection resulting in acute RV failure and cardiovascular collapse.

Reference #1 Chung KY, et al. Clinicopathologic review of pulmonary silicone embolism with special emphasis on the resultant histologic diversity in the lung: a review of five cases. Yonsei Medical Journal. 2002 April;43(2):152-9.

Reference #2 Schmid A, et al. Silicone embolism syndrome: a case report, review of the literature, and comparison with fat embolism syndrome. Chest. 2005 June;127(6):2276-81.

DISCLOSURE: The following authors have nothing to disclose: Christina Rager, Dan Naim, Susan Stein, Dennis Yick, Nader Kamangar

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Chest. 2011;140(4_MeetingAbstracts):67A. doi:10.1378/chest.1118818

INTRODUCTION: Pulmonary vein stenosis (PVS) is an uncommon cause of hemoptysis. We report a case of severe pulmonary vein stenosis in a patient who had undergone catheter ablation for atrial fibrillation who later presented with hemoptysis.

CASE PRESENTATION: A 51-year-old man was referred for evaluation of sudden onset daily hemoptysis, dyspnea and progressive anemia. He had no fever, chills, night sweats or weight loss. Medical history included positive tuberculin test and atrial fibrillation for which he had undergone three catheter ablation procedures, latest 8 months ago. At presentation he was in no acute distress, afebrile, and vital signs were stable. His physical examination was unremarkable. He was admitted to the hospital and subsequently underwent bronchoscopy which showed irregular mucosa in the anterolateral wall of the left upper bronchus and ongoing active oozing. Endobronchial biopsies of the irregular mucosa showed reactive follicles and germinal center but no malignancy and cultures (bacterial, mycobacterial and fungal) were negative. Contrast-enhanced computed tomography (CT) of the chest showed non visualization and near complete occlusion of the superior pulmonary veins. Quantitative perfusion scan showed markedly decreased perfusion in the left upper lobe. Cardiac catherization was performed via transeptal approach. Angiogram revealed severe stenosis of the left superior pulmonary vein. Balloon dilatation followed by stent placement was performed. His hemoptysis resolved and he was discharged home.

DISCUSSION: Pulmonary vein stenosis (PVS) is a relatively rare condition and can be congenital or acquired. Congenital PVS has been proposed to be secondary to abnormal incorporation of the common pulmonary vein into the left atrium in the later stages of cardiac development. Affected patients most often become symptomatic in the first few months to years of life and frequently have one or more additional cardiac anomalies (30-80%). The most commonly associated congenital heart defects are septal defects. The most common cause of acquired PVS in adult patients is radiofrequency ablation procedures for treatment of atrial fibrillation. PVS secondary to neoplasm growth, sarcoidosis, or fibrosing mediastinitis has been reported. The frequency of PVS post ablation procedure varies and had been reported to be as high as 42.4% in small early case series but recently due to improved technique and improved experience reported to be as low as 1.3 %. Patients can be asymptomatic, but more extensive and severe stenosis may cause dyspnea, hemoptysis, cough or chest pain. Due to the variability of symptoms diagnosis can be missed. Chest radiographs may show oligemia but this is not diagnostic. Quantitative perfusion scan is useful in assessing the extent of flow in the distribution of PVS. CT angiography and magnetic resonance imaging can provide accurate location and extent of stenosis. Suboptimal angiography can often overestimate the severity. Untreated congenital PVS carries a poor prognosis. Repair of PVS has low success rates and patients usually require multiple repairs. Even with newer techniques the freedom from reoperation or death at 5 years is still only 50 %. Pneumonectomy may be required for patients with massive hemoptysis. In patients with severe pulmonary hypertension, lung transplantation has been successful. Acquired PVS has been treated almost exclusively by transcatheter techniques. Balloon angioplasty of the involved vessels usually leads to a reasonably good initial result but restenosis is reported in more than 50% of patients within 1 year. Stent implantation has been associated with better success rates.

CONCLUSIONS: Clinicians should be aware of the possibility of PVS as a cause of hemoptysis in a patient who has had ablation procedure for atrial fibrillation.

Reference #1 Latson LA, Prieto LR. Congenital and aquired pulmonary vein stenosis. Circulation. 2007 Jan 2; 115(1):103-8

Reference #2 Holmes DR Jr, Monahan KH, Packer D. Pulmonary vein stenosis complicating ablation for atrial fibrillation: clinical spectrum and interventional considerations. JACC Cardiovascular Interv.2009 Apr; 2(4):267-76.

Reference #3 Devaney EJ, Chang AC, Ohye RG, Bove EL. Management of congenital and acquired pulmonary vein stenosis. Ann Thorac Surg. 2006; 81: 992-995

DISCLOSURE: The following authors have nothing to disclose: Neeraj Desai, Sara Greenhill, Edward Diamond, Andrei Pop, Kevin Kovitz

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Chest. 2011;140(4_MeetingAbstracts):68A. doi:10.1378/chest.1108837

INTRODUCTION: Airway-centred interstitial fibrosis (ACIF) is a relatively new pattern of lung injury described by Churg et al. (1) but has not yet been added to the list of Idiopathic Interstitial Pneumonias. We present a case of a non-smoking Hutterite woman with Common Variable Immunodeficiency (CVID) and ACIF. ACIF has not yet been described in association with CVID.

CASE PRESENTATION: A 52-year-old woman living in a Hutterite community in Alberta, Canada presented with a 2-year history of persistent non-productive cough, wheeze and dyspnea. She had a history of recurrent, radiographically-proven bacterial pneumonias that resolved with antibiotics. She was a lifetime non-smoker and was taking no medications. Apart from subjective worsening around household cleaners including bleach and Pinesol, she had no other exposures. Her symptoms did not improve with bronchodilators or inhaled corticosteroids. High resolution computed tomography scan revealed a pattern of tree-in-bud nodularity and no honeycombing. Pulmonary function testing showed normal spirometry with reduced DLCO. Bronchoalveolar lavage (BAL) revealed 20% polymorphonuclear cells, 1% eosinophils, 3% lymphocytes, 76% macrophages. Pertinent laboratory investigations revealed a negative antinuclear antigen (ANA), IgA 0.66 g/L (0.75-4.55 g/L), IgG 5.63 g/L (6.8 - 18 g/L) and IgM <0.21 g/L (0.5 - 3g/L). Flow cytometry showed no evidence of a B-cell lymphoproliferative disorder. She was diagnosed with common variable immunodeficiency (CVID) and treated with monthly injections of intravenous immunoglobulins (IVIG). Precipitin analysis was not performed due to the CVID. Open lung biopsy revealed prominent bronchiolar fibrosis around isolated bronchi with marked bronchiolar metaplasia which appeared to link up. Only sparse chronic inflammation was present and no granulomata were identified. A diagnosis of ACIF was made. There was no improvement in symptoms or spirometry with 8 weeks of prednisone 50 mg/d. She has remained dyspneic but clinically stable since 2008.

DISCUSSION: Airway-centred interstitial fibrosis (ACIF) is one of several patterns of lung injury predominantly involving airways described in recent years. Others include centrilobular fibrosis, idiopathic bronchiolocentric interstitial pneumonia and peribronchial metaplasia (2). It is unclear whether ACIF represents a distinct disease process or a manifestation of an environmental exposure. In our case, our patient had no prolonged contact with substances known to cause hypersensitivity pneumonitis (HP). Also, lack of granulomas, minimal inflammation on histology and the paucity of lymphocytes on BAL argue against a diagnosis of HP. ACIF following exposure to cleaning products, however, has been reported previously (3). Interstitial lung disease is known to occur in up to 25% of patients with CVID. Granulomatous-lymphocytic interstitial lung disease (GL-ILD) is a term used for lymphoid hyperplasia, follicular bronchiolitis and LIP and is seen in CVID but the absence of granulomas and paucity of lymphocytes in our case makes this diagnosis unlikely. ACIF has not previously been reported in association with CVID. CVID may be unrelated to the diagnosis of ACIF in our patient. Alternatively, CVID may have contributed to the development of ACIF due to immune dysregulation which might provide clues to the pathogenesis of this relatively new pattern of lung injury.

CONCLUSIONS: As ACIF becomes better understood we will hopefully learn the pathogenesis of ACIF, whether there is an association between CVID and ACIF and whether ACIF should be added to the list of Idiopathic Interstitial Pneumonias.

Reference #1 Churg A et al. Airway-centred interstitial fibrosis. A distinct form of aggressive diffuse lung disease. Am J Surg Pathol 2004; 28: 62-68.

Reference #2 Cordeiro CR. Airway involvement in Interstitial lung disease. Curr Opin Pulm Med 2006; 12: 337-341

Reference #3 Serrano M et al. Airway centred interstitial fibrosis associated with exposure to fumes from cleaning products. Arch broncopneumol 2006; 42 (10): 557 - 559.

DISCLOSURE: The following authors have nothing to disclose: Tara Lohmann, John Chan, Margaret Kelly

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Chest. 2011;140(4_MeetingAbstracts):69A. doi:10.1378/chest.1113561

INTRODUCTION: Novel immunosuppressive therapies create a diverse set of immune deficits that are a substrate for opportunistic infections and noninfectious etiologies for pulmonary infiltrates including drug allergy or toxicity, pulmonary hemorrhage, malignancy and pulmonary edema.

CASE PRESENTATION: We present the case of a 61-year-old male with Psoriasis and psoriatic arthritis on methotrexate for the past 6 years and golimumab for 1 year. He had been on other immunosuppressive agents in the past including infliximab, etanercept, and adalimumab. He had pneumonia in April 2010 and a follow up chest x-ray and CT revealed non-calcified 1.3 x 1cm size nodule in the right upper lobe in May 2010. PPD was negative and the nodule was stable on a repeat CT in June. The patient then developed cough with night sweats and weight loss over the next 1 month. Repeat CT revealed a spiculated lesion in the right upper lobe with a new soft tissue mass in the anterior right hemithorax contiguous with the pleural surface and pericardium with multifocal ground glass opacities in both lungs. Bronchoscopy with brushings and transbronchial biopsy were inconclusive. Sputum for AFB and PPD was again negative. Patient underwent a CT guided lung biopsy. The pathology revealed lymphoplasmacytic infiltrate with fibrosis, most suggestive of an iatrogenic immunodeficiency associated lymphoproliferative disorder (LPD). EBV studies were negative. This was thought to be related to Methotrexate which was stopped. His symptoms have significantly improved over the last 12 weeks. Repeat CT shows a marked improvement in the multifocal lung disease, complete resolution of changes in RLL and significant resolution of changes in the other lobes.

DISCUSSION: Pulmonary toxicity develops in 2-8% of patients receiving methotrexate, but some reports suggest an incidence as high as 33%. The mechanisms causing the side effects of methotrexate (MTX) include mutation of the genotype, inhibition of transport, MTX-polyglutamates and P-glycoprotein binding with methotrexate. The p38 MAPK-signaling pathway is especially associated with a pulmonary inflammatory response. Hypersensitivity pneumonitis is the most common pulmonary toxicity although cases of bronchiolitis obliterans with organizing pneumonia (BOOP), acute lung injury with noncardiogenic pulmonary edema and pulmonary fibrosis have also been reported. Methotrexate-related LPD is an extremely rare complication of the drug. The spectrum of Methotrexate-related LPD includes both Hodgkin’s and Non-Hodgkin's lymphoma. Most regresses after discontinuation of methotrexate therapy but high grade lymphoma may require treatment. This suggests that diminished immune surveillance due to methotrexate may facilitate the proliferation of malignant lymphoid clones. There is evidence of an EBV association in a few cases as seen in immunosuppression in the setting of organ transplantation or AIDS. There are no prospective trials of therapy for methotrexate-induced pulmonary toxicity. The current strategy of methotrexate discontinuation and glucocorticoid administration is based largely upon anecdote.

CONCLUSIONS: Methotrexate-related LPD should be a differential of new onset pulmonary lesions in an immunosuppressed patient on the drug.

Reference #1 Bragg DG, et al. Lymphoproliferative disorders of the lung: histopathology, clinical manifestations, and imaging features. Am J Roentgenol. 1994;163(2):273-81.

Reference #2 Ebeo CT, et al. Methotrexate-induced pulmonary lymphoma. Chest. 2003;123(6):2150-3.

Reference #3 Kim YJ, Song M, Ryu JC. Mechanisms underlying methotrexate-induced pulmonary toxicity. Expert Opin Drug Saf. 2009;8(4):451-8.

DISCLOSURE: The following authors have nothing to disclose: Pankaj Mehta, Girish Trikha

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Chest. 2011;140(4_MeetingAbstracts):70A. doi:10.1378/chest.1113856

INTRODUCTION: Respiratory involvement in Primary Sjogren’s Syndrome (pSS) ranges from 9-75%(1). Diffuse lung disease (DLD) is a common manifestation with non-specific interstitial pneumonia (NSIP) being most frequently identified. pSS DLD is typically diagnosed in clinical practice when sicca symptoms are present in conjunction with elevated titers of anti-SSA/SSB antibodies. However, situations arise when the suspicion for pSS DLD is high but the antibody titers are normal and the diagnosis remains elusive. We describe a case of a patient with a high clinical concern for pSS DLD but with normal antibody titers that required a minor salivary gland biopsy (MSGB) to confirm the diagnosis.

CASE PRESENTATION: A 37-year-old African American woman with a history of post traumatic stress disorder (PTSD) presented with a complaint of 3 months of an intermittent, non-productive cough and two years of progressive dyspnea on exertion. She was an active duty service member and deployed to Iraq three years prior to presentation, with symptoms beginning after she returned from deployment. While in Iraq, she was also in close proximity to a “burn pit”, but denied any specific exposures. She endorsed dry eyes and mouth, GERD related symptoms and dysphagia, as well as numerous dental cavities. She quit smoking 1 year prior to presentation. She was taking sertraline for PTSD. Her vital signs on initial evaluation showed a heart rate of 97 beats/min, BP of 104/64 mmHg, a temperature of 97.1°F, an oxygenation saturation of 100% on room air and a respiratory rate of 16. Her overall physical examination was normal. Her complete blood count, chemistry and liver studies were normal. Her anti-SSA, anti-SSBA, anti-dsDNA, anti-smith, anti-Jo1, anti-centeromere and anti-scl-70 antibiodies were negative. The anti-nuclear antibody (ANA) and rheumatoid factor (RF) were both positive at 1:1280 (<1:160) and 20.3 (<14 IU/ml), respectively. A chest x-ray was unremarkable. Spirometry with full lung volumes showed moderate restrictive lung disease (FVC 62%, FEV1 63%, FEV1/FVC 85%, TLC 54%). Her DLCO was reduced at 51%. A HRCT showed bilateral, lower lobe reticular and ground glass opacities. A surgical lung biopsy showed fibrosing NSIP. Due to the concern for pSS related NSIP but normal SS specific antibody titers, she underwent a MSGB that showed a lymphocytic focus score ≥ 1 which was consistent with a diagnosis of pSS. She had a moderate reduction in her 6 minute walk test but no evidence of pulmonary hypertension by a right heart catheterization. Given her functional impairment, she began treatment with prednisone and azathioprine but has not noticed a significant improvement to date in her functional status or spirometric values.

DISCUSSION: Primary Sjogren’s Syndrome (pSS) is described histopathologically as a lymphocytic infiltration of the exocrine glands leading to eventual mucosal dryness. Reports of respiratory involvement in pSS vary widely but NSIP is the most common diffuse lung disease. An updated diagnostic classification system for pSS includes a MSGB as a component of the diagnostic criteria(2). Sicca-related symptoms are common, but serologic studies might not be consistently elevated. As such, a MSGB might be needed in selected cases to confirm the diagnosis(3). The traditionally recommended treatment is prednisone and azathioprine with variable results. The prognosis of connective tissue disease-related DLD has been reported as more favorable than the idiopathic interstitial pneumonias and is one of the major reasons to confirm the etiology of the diffuse lung disease.

CONCLUSIONS: A MSGB should be considered in high pre-test probability cases of pSS even in the absence of positive serological studies. NSIP is the most common diffuse lung disease related to Sjogren’s syndrome. Sjogren’s NSIP may have a better overall survival than idiopathic NSIP. Prednisone and Azathioprine are recommended as initial treatment for NSIP causing clinical impairment.

Reference #1 Sarkar P, Patel N, Furie R, et al. Pulmonary manifestations of primary sjogren’s syndrome. Indian journal of chest diseases and allied sciences 2009 Apr-Jun;51:93-101

Reference #2 Vitali C, Bombardieri S, and Jonsson R et al. Classification criteria for Sjogren’s Syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 2002;61:554-558

Reference #3 Fishcer A, Swigris J, and du Bois R et al. Minor salivary gland biopsy to detect pSS in patients with ILD. Chest 2009;136:1072-1078

DISCLOSURE: The following authors have nothing to disclose: Matthew Aboudara, John Sherner, George Underwood, Russell Harley

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Topics: lung diseases
Chest. 2011;140(4_MeetingAbstracts):71A. doi:10.1378/chest.1117358

INTRODUCTION: Sorafenib is an oral multikinase inhibitor used in the treatment of unresectable renal cell and hepatocellular carcinoma (HCC). While pneumonitis is listed as an uncommon (<1%) adverse event in the full prescribing information there are only rare post-market reports of interstitial lung disease (ILD) attributable to sorafenib, most without accompanying histologic evidence. This case details the development of biopsy proven desquamative interstitial pneumonia in an individual with HCC after treatment with sorafenib.

CASE PRESENTATION: A 59 year old African American male was referred to pulmonary clinic for complaints of non-productive cough, worsening exertional dyspnea, and pleuritic chest pain that progressed over 4 months. His history is significant for active smoking of 3-5 cigarettes daily (40 pack-years), metastatic prostate cancer and HCC. The latter progressed despite chemoembolization and was subsequently treated with sorafenib 4 months prior to presentation. There were no prior respiratory complaints. Also of note, abdominal CT scan five months prior to sorafenib commencement showed that the lung bases were clear. Abdominal CT scan ten days after sorafenib initiation revealed ground-glass opacities in the lung bases bilaterally. One month after starting sorafenib, the patient experienced dry cough associated with bilateral pleuritic chest pain which only mildly improved on dextromethorphan and guaifenesin. The patient was then treated with azithromycin and codeine, but the cough became more productive and was associated with low grade fevers. CT of the chest demonstrated extensive perihilar and bibasilar ground glass infiltrates (left side greater than right) with air bronchograms. At this time, he was referred to pulmonary clinic where he was also found to be hypoxic (87% SaO2 on room air at rest). Bronchoscopy was performed and revealed erythematous airways but cultures, cytologic and pathologic specimens were non-diagnostic. Due to clinical decline (increased dyspnea and involuntary weight loss) , sorafenib was held. A repeat chest CT revealed worsening ground-glass infiltrates as well as mild bronchiectasis. In order to establish a definitive diagnosis, the patient underwent a lung biopsy which revealed desquamative interstitial pneumonia. When symptoms persisted he was started on prednisone with subsequent decrease in cough and dyspnea, weight gain, and decreasing oxygen requirements after two weeks of steroid treatment.

DISCUSSION: While ILD has rarely been described with sorafenib, this case details the development of desquamative interstitial pneumonia (DIP) after the initiation of sorafenib. While DIP is an uncommon entity affecting cigarette smokers in their 40’s, the temporal relationship of this patient’s symptoms and radiographic findings, his improvement after cessation of the offending agent and initiation of steroids strongly imply that this oral multikinase inhibitor was the causative source. The pathogenesis of its pulmonary toxicity is unknown, but may be related to its inhibition of the VEGF signaling pathway, the reduction of which and subsequent improvement after recovery has been shown in several ARDS trials. Histologic confirmation of DIP was obtained and displayed the key features of alveolar macrophage accumulation, fibrotic thickening of alveolar septa, and interstitial chronic inflammation, in the absence of extensive fibrosis, smooth muscle proliferation and eosinophils.

CONCLUSIONS: Desquamative interstitial pneumonia is a potential sequela of sorafenib use. Respiratory symptoms after treatment should prompt the appropriate diagnostic modalities to obtain tissue diagnosis so that the therapeutic modalities can be initiated to thwart further pulmonary complications.

Reference #1 Myung H, Jeong S, et al. Sorafenib-Induced Interstitial Pneumonitis in a Patient with Hepatocellular Carcinoma: A Case Report. Gut Liver. 2010 December; 4(4): 543-546.

Reference #2 Carrington CB, Gaensler EA, Coutu RE, et al. Natural history and treated course of usual and desquamative interstitial pneumonia. N Engl J Med 1978; 298:801.

DISCLOSURE: The following authors have nothing to disclose: Jorge Dolojan, Allen Blaivas, Jin Choe

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Chest. 2011;140(4_MeetingAbstracts):72A. doi:10.1378/chest.1120101

INTRODUCTION: The most serious and dose limiting side effect of Bleomycin is induction of pulmonary toxicity. When clinical Bleomycin Induced Pneumonitis (BIP) occurs, the most widely used treatment agents are corticosteroids in high doses. The data on the efficacy of corticosteroids for BIP are rather conflicting and only a few studies have shown minimal response to corticosteroids. We report a case of BIP improved on treatment with Pentoxyfylline in addition to corticosteroids.

CASE PRESENTATION: A 38 year old woman with Hodgkins Lymphoma presented to our clinic with insidious onset of shortness of breath. The patient’s diagnosis of Hodgkins Lymphoma was made five months prior to her presentation, and she had received 4 cycles of Adriamycin, Bleomycin, Vincristine, and Dacarbazine (ABVD). She underwent pulmonary function tests (PFT’s) which revealed a FEV1 of 64% of predicted, FVC of 56% predicted with a normal FEV1/FVC ratio. Her diffusion capacity was severely reduced at 24% of predicted. PFT’s prior to starting chemotherapy were within normal limits. A chest CT performed at this time revealed bilateral subpleural reticulation with patchy ground glass interstitial changes. BIP was suspected and the patient underwent fiberoptic bronchoscopy with transbronchial biopsies. All cultures including bacterial, viral, AFB, PCP and fungal stains were negative. Pathology showed peribronchial collections of foamy histiocytes without granulomas or evidence of lymphoma. The patient was started on Prednisone 60 mg daily with minimal improvement. Due to worsening shortness of breath, she presented to the emergency department three weeks later. Exam was significant for a temperature of 97.4F, blood pressure of 94/57 mm hg, pulse rate of 120 beats per minute, respiratory rate of 36 per minute and oxygen saturation of 84% on room air. Physical exam included bibasilar, fine dry crackles on auscultation. Admission laboratory data was significant for a white cell count of 3200 per cu mm. Repeat CT scan done at this time showed worsening parenchymal opacities and interstitial fibrosis with no evidence of pulmonary embolism. The patient required 100% Fio2 delivered by face mask with oxygen saturations between 92-94% and a respiratory rate of 44 per minute. The patient was started on Vancomycin, Cefepime, Oseltamavir, Trimethoprim-Sulfamethoxazole and methylprednisolone and methylprednisolone one gram IV daily for three days. Thereafter, she was maintained on solumedrol 60 mg IV every 6 hours. Despite this regimen, her clinical course worsened with deteriorating hypoxemia and diaphoresis over the next 8 days, requiring non-invasive positive pressure ventilation (NIPPV). She was then started on Pentoxyfylline 400mg every along with high dose steroids. The patient’s clinical course gradually improved over the next 3 days with liberation from NIPPV. Over the next two weeks, she showed progressive improvement in symptoms with reducing oxygen requirements to 6L per minute via nasal cannula. A Chest CT scan performed 14 days later revealed improved ground glass opacities and fibrosis.

DISCUSSION: BIP occurs in 0 to 46% of patients treated with bleomycin-containing chemotherapy, depending on the criteria used for the diagnosis. Our patient had developed severe BIP, and despite treatment her symptoms were minimally improved. After the addition of oral Pentoxifylline to her regimen, clinical symptoms, oxygen saturation and radiographic abnormalities were all improved. The mechanism of bleomycin-induced lung injury is not entirely clear, but likely includes components of oxidative damage, relative deficiency of the deactivating enzyme bleomycin hydrolase, genetic susceptibility, and elaboration of inflammatory cytokines. Since Pentoxifylline inhibits activation of neutrophil granulocytes and formation of reactive oxygen species, this may be the mechanism responsible for the improvement.

CONCLUSIONS: Few case reports have reported improvement of BIP on addition of Pentoxyfylline but the data is still very limited. Addition of Pentoxyfylline in management of these refractory cases should be considered if the clinical course is worsening.

Reference #1 John Goffin, Harvey Kreisman, Victor Sandor: Bleomycin-Induced Lung Toxicity and Pentoxifylline. Journal of Clinical Oncology, Vol 19, Issue 2 (January), 2001: 597-598

Reference #2 Stefan Sleijfer: Bleomycin-Induced Pneumonitis. Chest 2001;120;617-624

Reference #3 D A White, D E Stover: Severe bleomycin-induced pneumonitis. Clinical features and response to corticosteroids. Chest 1984;86;723-728

DISCLOSURE: The following authors have nothing to disclose: Jaspreet Ahuja, Mohammed Sharif, Edwin Annan, Samuel Acquah

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Chest. 2011;140(4_MeetingAbstracts):73A. doi:10.1378/chest.1120134

INTRODUCTION: Lipoid Pneumonia is a pulmonary disorder that results from the deposition of lipids in the alveoli, causing acute or chronic inflammation. Albeit rare, the most common form of this disease occurs from unintentional inhalation or aspiration of exogenous oils found in oral laxatives, balms, mineral oils or aerosolized droplets.

CASE PRESENTATION: A previously healthy 33 year-old woman presented with acute onset of pleuritic chest pain, palpitations and severe dizziness. She also reported gradual onset of exertional dyspnea in the weeks leading up to the presentation. Her medical and family history was otherwise unremarkable. She worked as a hair stylist throughout her career. Her initial physical examination was remarkable for mild tachycardia, loud P2 and desaturation up to 82% after minimal exertion on room air. Her routine labs, EKG and two-view chest radiograph were all negative. A computer tomography (CT) scan of the chest with intravenous contrast was done to rule out pulmonary embolism (PE) and showed ground-glass consolidations in both lower lung fields and peripherally in the upper lung fields with no evidence of PE. Trans-thoracic echocardiography revealed a hypokinetic and dilated right ventricle with estimated pulmonary artery pressure 75-80mmHg. Pulmonary function testing was consistent with moderate restrictive lung impairment with evidence of air-trapping. A decision was made that the patient’s diagnosis would be best elucidated via lung biopsy using video-assisted thoracoscopic surgery. A wedge resection of lung was obtained and demonstrated evidence of lipoid pneumonia with acute interstitial pneumonitis and alveolar cell hyperplasia. A Trichrome stain showed minimal increase in fibrosis. Referring back to her history, she admitted using several types of spray-on hair sheens in her salon, which create substantial potential for aerosolized inhalation of oil-based droplets and was considered the mode of exposure in her case. Treatment was initiated with intravenous steroids followed by a tapering dose of oral prednisone. The patient was instructed to avoid oil-based aerosolized hair sheen applications. On follow up visit she reported complete resolution of her symptoms. Repeat Pulmonary function testing revealed only minimal restrictive lung impairment with normal diffusion, her forced vital capacity increased from 1.55L to 2.62L. Repeat Trans-thoracic echocardiography revealed normalization of right ventricle function with improved pulmonary artery pressure to 43mmHg.

DISCUSSION: Most cases of lipoid pneumonia occur from aspiration or inhalation of mineral oil, especially in patients with impaired protective mucociliary clearance. The source of the oil commonly is oil-based laxatives, excessive use of lip balm or flavored lip gloss, or traditional folk remedies using oil based product for various purposes. Other unusual sources, such as smoking black fat tobacco in Guyana and excessive use of industrial lubricants and more peculiar ones, such as inhaled burning fat in a fire fighter and fire-eater have also been reported. Our case is unique in the sense that it affected predominantly peripheral area of lung in contrast to literature supporting peripheral sparing in cases of exogenous lipoid pneumonia.

CONCLUSIONS: Exogenous lipoid pneumonia should be included in differential diagnosis of patient presenting with respiratory complaints, positive occupational exposure history and ground glass infiltrate on CT images.

Reference #1 Anderson Spickard III, MD, J. V. Hirschmann, MD. Exogenous Lipoid Pneumonia. Arch Intern Med. 1994;154(6):686-692.

Reference #2 A. Gondouin, Ph. Manzoni, E. Ranfaing, J. Brun, J. Cadranel, D. Sadoun, J.F. Cordier, A. Depierre, J.C. Dalphin. Exogenous Lipid Pneumonia: a retrospective multicentre study of 44 cases in France. Eur Respir J, 1996, 9, 1463-1469.

Reference #3 3.Foe RB, Bigham RS. Lipoid pneumonia following occupational exposure to oil spray. JAMA 1954;155:33-34.

DISCLOSURE: The following authors have nothing to disclose: Matthew Michaels, Ajaykumar Patel, Zaza Cohen

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Chest. 2011;140(4_MeetingAbstracts):74A. doi:10.1378/chest.1120052

INTRODUCTION: We report an unusual case of embolic stroke in a middle-aged male.

CASE PRESENTATION: A 63-year-old male smoker presented to an outside hospital with aphasia, headache, and blurry vision. Initial head CT revealed a hypodense lesion in the right temporal lobe. MRI of the brain showed areas consistent with multiple acute or subacute infarcts in the right temporal and occipital regions and left temporal and cerebellar regions. A transesophageal echocardiogram revealed a large (36 x 6 mm) pedunculated echogenic left atrial mass, adjacent to the posterior leaflet of the mitral valve and prolapsing into the left ventricular outflow tract. The patient was transferred to our hospital for further evaluation. A Chest radiograph revealed a 4.3 cm left lower lobe mass. A CT of chest confirmed a 3.7 x 3.8 cm lobulated mass in the superior segment of the left lower lobe. A CT-guided transthoracic needle biopsy showed abundant necrosis with malignant appearing epithelial and chondroid cells. A PET scan revealed intense uptake of the mass with a maximum SUV of 6 and linear extension of activity from the mass toward the left atrium corresponding with the course of the inferior pulmonary vein. There was no evidence of distant metastasis. The left atrial mass along with the left inferior pulmonary vein was excised via sternotomy on cardiopulmonary bypass. Subsequently, a left lower lobectomy was performed by video-assisted thoracoscopy. The pathology revealed high grade malignancy with both epithelial and sarcomatous components, consistent with carcinosarcoma. Immunohistochemical stains show focal positivity of the epithelial component for cytokeratins AE1/AE3, CK5/6 and CK7, and negativity for CK20. The tumor also showed diffuse positivity for vimentin and focal positivity for p63. The patient is awaiting chemotherapy.

DISCUSSION: Carcinosarcoma is a rare malignancy representing less than 0.3% of all lung tumors. Carcinosarcomas are extremely aggressive biphasic tumors composed of an admixture of malignant epithelial and sarcomatous components. Carcinosarcomas most commonly present in men in their fifth to seventh decades of life. A strong association with smoking has been observed. Controversy exists as to whether carcinosarcomas represent the collision of two distinct primary tumors or a single tumor derived from a pluripotential cell that undergoes sarcomatous and carcinomatous differentiation. The tumor necrosis that carcinosarcomas commonly undergo makes attempts at percutaneous needle or transbronchial biopsy techniques difficult. Patients with carcinosarcomas have a poor prognosis. The median duration of survival after diagnosis is six months, with 6 percent of patients surviving five years. Patients should be considered for curative resection whenever possible, although extension to regional lymph nodes portends a poor surgical result. The value of adjuvant radiation therapy or systemic chemotherapy for patients with unresectable disease is unclear.

CONCLUSIONS: To our knowledge, this is the first case of a carcinosarcoma of the lung with vascular invasion of the inferior pulmonary vein and left atrium and presenting as an embolic stroke. This case demonstrates that an accurate preoperative diagnosis of carcinosarcoma of the lung is rarely made because of the difficulty in sampling both histologic components of the tumor.

Reference #1 Pulmonary carcinosarcoma: diagnostic problems and determinants of the prognosis. Huwer H, Kalweit G, Straub U, Feindt P, Volkmer I, Gams E. Eur J Cardiothorac Surg. 1996;10(6):403-7.

Reference #2 Pulmonary carcinomas with pleomorphic, sarcomatoid, or sarcomatous elements: a clinicopathologic and immunohistochemical study of 75 cases. Rossi G, Cavazza A, Sturm N, Migaldi M, Facciolongo N, Longo L, Maiorana A, Brambilla E. Am J Surg Pathol. 2003 Mar;27(3):311-24.

Reference #3 Molecular pathogenesis of pulmonary carcinosarcoma as determined by microdissection-based allelotyping. Dacic S, Finkelstein SD, Sasatomi E, Swalsky PA, Yousem SA. Am J Surg Pathol. 2002 Apr;26(4):510-6.

DISCLOSURE: The following authors have nothing to disclose: Raghukumar Thirumala, Klaus Lessnau, Valavanur Subramanian, Larry Di Fabrizio

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