Abstract: Case Reports

Chest. 2004;126(4_MeetingAbstracts):924S. doi:10.1378/chest.126.4_MeetingAbstracts.924S

INTRODUCTION:  High-frequency oscillatory ventilation (HFOV) is an alternative mode of ventilatory support for patients with severe adult respiratory distress syndrome (ARDS) failing conventional ventilation. Reported complications include pneumothorax, pneumomediastinum, and subcutaneous emphysema. While necrotizing tracheobronchitis (NTB) has been widely reported in the pediatric literature with a reported incidence between 4–44%, it is rare in adults. We report a case of NTB associated with HFOV.

CASE PRESENTATION:  A 41-year-old previously healthy Ecuadorian male was taken to the operating room with signs and symptoms consistent with an acute appendicitis. He was found to have a normal appendix. On the fifth hospital day, broad spectrum antibiotics were initiated for a fever to 103 and diffuse bilateral infiltrates on his chest radiograph. A chest CT scan revealed diffuse interstitial infiltrates with mediastinal adenopathy. A biopsy of an enlarged supraclavicular lymph node was performed. On the 8th hospital day, he was transferred to the Intensive Care Unit and intubated for hypoxemic respiratory failure. On the same day, his lymph node biopsy revealed anaplastic T-cell lymphoma for which chemotherapy was initiated. He remained on the ventilator through five days of neutropenia with no improvement. With the resolution of his neutropenia, his condition deteriorated with worsening hypoxemia despite optimal ventilator support. After an unsuccessful trial of prone ventilation, High Frequency Oscillatory Ventilation (HFOV Sensor Medics Adult HFOV 3100B) with Nitric Oxide (NO) was initiated. On the 28th hospital day the patient developed hypercapnea prompting an urgent bronchoscopy which revealed a bronchial mucosa replaced by dry, hemorrhagic, eschar-like debris nearly occluding the airway. Extensive bronchoscopic debridement was required on a daily basis. Numerous evaluations of tissue and bronchoalveolar specimen were negative for bacterial, fungal, viral etiologies. Cytology was negative for lymphoma. As inadequate humidification was postulated to be causing this condition, changes were made to the ventilator circuit to optimize humidification. Over the next seven days, surveillance bronchoscopy revealed dramatic improvement of the tracheobronchial mucosa. Unfortunately, the patient’s ARDS and overall clinical condition was unremitting despite aggressive measures. He continued to decline and died after 30 days of mechanical ventilation.

DISCUSSIONS:  Only eleven adult cases of NTB have been reported, with nine associated with High Frequency Jet Ventilation (HFJV) and two with conventional ventilation. A combination of inadequate humidification, sub-mucosal ischemia, and high operating pressures has been implicated as an etiology of this disorder. In neonates, NTB has been associated with life-threatening airway obstruction and death with most cases identified at autopsy. In recent review of HFOV by Derdak (1), NTB is not listed as a potential complication. To our knowledge, this is the first case of NTB reported in association with HFOV. Perhaps as HFOV is utilized more frequently as an alternate mode, NTB will become better recognized as a potential complication.

CONCLUSION:  In patients being placed on HFOV, maintaining an adequately humidified circuit is essential. An urgent bronchoscopy is indicated for a sudden onset of unexplained hypercapnea. Reversal of this disorder is possible with identification and management of the precipitating factors. Once identified, frequent bronchoscopy may be necessary secondary to distal bronchial obstruction caused by the sloughing of necrotic debris. This diagnosis seems to portend a grim prognosis. All of the adult cases reported died as a result of or shortly after detection of NTB.

DISCLOSURE:  K.K. Chung, None.

Chest. 2004;126(4_MeetingAbstracts):924S-a-925S. doi:10.1378/chest.126.4_MeetingAbstracts.924S-a

INTRODUCTION:  Tracheomediastinal Fistula (TMF) is uncommon and has never, to our knowledge, been reported as a complication of mediastinal lymph node excisional biopsy. We report such a case and its successful management using a Self Expandible Metallic Stent (SEMS).

CASE PRESENTATION:  A 69 year old female with a history of breast cancer requiring radiation in 1982, non-small cell lung cancer requiring left upper lobectomy in 1989, and small cell lung cancer requiring right lower lobectomy in 1996 developed PET scan positive subcarinal lymphadenopathy. Left thoracotomy and excisinal biopsy was performed; however, during the surgery a communication between left main stem bronchus (LMB) and mediastinum was noted. Following an attempt at intercostal muscle flap repair she was transferd to our institutuion. On examination she was hemodynamically stable with diffuse subcutaneous emphysema requiring 40% FIO2 via mechanical ventilation. Flexible bronchoscopy (FB) revealed dehiscence of the carina with TMF involving both main stem bronchi (figure 1). The left and right main stem bronchi were 80 and 50% detached from the carina, respectively. The FB was passed through the defect in to the mediastinum and a large amount of mucopurulent material was evacuated, the culture of which grew Pseudomonas aeruginosa. 14x40 mm and 12x20 mm uncovered Ultraflex stents were placed in the left and right main stem bronchi respectively. The patient was excubated the next day and discharged five days later. FB, 5 and 10 weeks after the stent placement, revealed dramatic healing of the fistulae. At 13 weeks the RMB stent was removed using FB revealing complete closure of the fistula on the right. A small medial defect was still present on the left hence only the portion of the stent extending above the carina was removed using laser. Presence of fresh granulation tissue around the fistula suggested that the area was still healing and the defect would close (figure 2). The patient is alive and well one year post incidence.

DISCUSSIONS:  Bronchomediastinal fistula has been described in association with mediastinal tuberculous lymphadenitis (1), necrotic mediastinal lymphadenopathy from metastatic oral cancer (2), mediastinitis after esophageal perforation (3), and with brachytherapy (4). Review of the English-language literature did not reveal any information regarding TMF fistula as a complication of mediastinal lymph node excision. The factors contributing to fistula formation in our patient likely were many. First, some event during the biopsy may have created a rent in one or both mainstem bronchi. Second, previous radiation therapy for breast cancer may have altered the bronchial architecture, weakening the integrity of the bronchus. Finally, and likely much more importantly, the presence of an infected fluid collection below the carina may have caused direct tissue destruction.

CONCLUSION:  Self Expandable Metallic Stents (SEMS), by promoting the formation of granulation tissue, are efficacious in the treatment of TMF. This case is notable because the patient, even with severely distorted airway architecture, demonstrated a quick response to treatment with SEMS, with notable improvement on the first surveillance bronchoscopic visualization and near complete fistula closure by three months. This case demonstrates that large airway fistula can be treated with SEMS, obviating the need for invasive surgical approaches.

DISCLOSURE:  J.L. Ranes, None.

Chest. 2004;126(4_MeetingAbstracts):925S. doi:10.1378/chest.126.4_MeetingAbstracts.925S

INTRODUCTION:  Bronchial artery malformations are rare entities that have been described as aneurysmal or as racemose hemangiomas (1). Previously described etiologies include bronchiectasis, cystic fibrosis, Osler Weber Rendu Syndrome, sarcoidosis (2), silicosis, pulmonary artery agenesis, conditions causing high flow states such as athersclerosis or surgically created shunts, (3) and infections by tuberculosis and syphilis. Reported treatments have included endovascular embolization and surgical resection. Brachytherapy remains unexplored for treating bronchial artery malformations.

CASE PRESENTATION:  This 48-year old male presented with two episodes of massive hemoptysis. At the intial presentation, fiberoptic bronchoscopy revealed a friable 1mm “cone shaped” exophytic lesion on the posterior wall of the proximal left upper lobe bronchus. This was treated with local injection of epinephrine; however, the patient refused further evaluation or treatment at that time. He was admitted ten months later with hemoptysis and expectorated >400 ml during the initial 24 hours of his admission. He had no bleeding diathesis, bronchiectasis, malignancy, or tuberculosis. Physical exam revealed diffuse inspiratory crackles in the lower and anterior left lung. Laboratory studies and echocardiogram were unremarkable. Chest radiograph and CT scan showed patchy infiltrates, consistent with blood, but no vascular abnormality. Rigid bronchscopy revealed adherent clot that was removed by suction and the exophytic lesion began bleeding bright red blood in a pulsatile fashion. The tip of lesion was treated with Nd:YAG laser (380 joules) and the bleeding stopped. The proximity of the pulmonary artery limited the amount of laser therapy we applied. Exhaustive bronchial arteriography did not reveal bronchial or intercostal arteries feeding this area or any other vascular abnormalities. He had no further hemoptysis and was discharged two days later. Ten days later, fiberoptic bronchoscopy revealed erythema, but no evidence of the exophytic lesion, at the bleeding site. We were not confident that the Nd:YAG laser therapy had completely treated the underlying vessel. After careful consideration of the therapeutic options, the lesion was treated with high dose rate afterload intraluminal radiation (brachytherapy, 8 Gy at 1cm, 4 cm length centered at the lesion). Bronchoscopy 10 weeks later showed complete resolution of the vascular abnormality.

DISCUSSIONS:  Endobronchial brachytherapy involves the placement of radioactive material within a catheter that is localized to the lesion being treated. This therapy has been almost exclusively described for palliation of malignancies that cause bronchial obstruction. Massive hemoptysis and fistula formation are the two most reported serious complications and range in incidence from 0-42% (4). Histopathologically, both early and late radiation effects support our rationale for using endobronchial radiation to treat this bronchial vascular lesion. The early vascular effects involve constriction of the microvasculature, arterioles and even small arteries and venules in response to endothelial cell injury (5). Radiation-induced vascular sclerosis, a late effect, is caused by intimal proliferation in medium and small arteries and arterioles that constricts blood vessel lumens (5).

CONCLUSION:  Endobronchial brachytherapy appears to be a viable alternative to sclerose small localized bronchial artery malformations. While the risk-benefit ratio must be evaluated on a case by case basis, this noninvasive approach may help avoid surgical exploration with the possibility of pneumonectomy.

DISCLOSURE:  M.A. Rasmus, None.

Chest. 2004;126(4_MeetingAbstracts):926S. doi:10.1378/chest.126.4_MeetingAbstracts.926S

INTRODUCTION:  Abciximab, a platelet glycoprotein IIb/IIIa receptor blocker, has significant and well established clinical benefits both short tem and long term, during high risk percutaneous coronary intervention. These benefits are probably due to the antithrombotic effects of abciximab; however, there is an increased risk of hemorrhage associated with its use. Diffuse alveolar hemorrhage is a rare but potentially fatal complication. We report a case of serious pulmonary hemorrhage after the use of abciximab therapy and the possible therapeutic role of iced saline lavages in controlling the bleed.

CASE PRESENTATION:  We present a 78 year old male patient with a history of coronary artery disease status post coronary artery bypass grafting in the past, who presents with unstable angina. The patient was subsequently started on aspirin, plavix, standard dose heparin and nitroglycerine. The patient was then transferred to the coronary catheterization lab for percutaneous coronary angioplasty. During the procedure, and after administration of 9.8 mg of Reopro (Abciximab), the patient complained of shortness of breath. He was then put a 100% non-rebreather mask with an arterial blood gas measurement showing the following: pH –7.28, PCO2 –50mmHg and PO2 –45mmHg. The patient suddenly started to cough up pink frothy secretions and was subsequently intubated in an atraumatic manner by the anesthetic team approximately 15 minutes after the administration of Reopro. Post intubation the patient was noted to have profuse bloody tracheal secretions and a bronchoscopy was performed. Bronchoscopic evaluation using an Olympus T200 scope showed diffuse and profuse bleeding from all broncho-pulmonary segments. Iced saline lavage was then performed using a tamponade technique whereby the iced saline was allowed to stay within the broncho-pulmonary segments for a few seconds. This was followed by an immediate cessation of bleeding from the lavaged segments. The patient required vasopressor agents to maintain hemodynamic stability and was transferred to the coronary care unit. Further endo-tracheal suctioning revealed only minimal bloody secretions. The hemoglobin decreased from 11.4g/dL to 9.7g/dL. The patient received two units of packed red blood cells and platelet transfusion. Follow up bronchoscopy in 24 hours showed no further bleeding.

DISCUSSIONS:  Dyspnea after PCI have causes.These include pain, pulmonary edema, transient aspiration, diffuse alveolar hemorrhage, or a combination of these. Identifying the etiology of dyspnea is imperative as the appreciation of early alveolar hemorrhage and the consequent discontinuation of anticoagulation may be life saving. Transient hypoxemia and new radiological infiltrates even in the absence of hemoptysis in a patient in the immediate post per cutaneous coronary intervention period should entertain the diagnosis of alveolar hemorrhage. An increased risk for this complication is seen in older patients, female sex, lower weight and a complicated or prolonged PCI. Bronchoscopy may help in the early diagnosis of this condition and may have both a diagnostic and therapeutic role.

CONCLUSION:  Percutaneous coronary revascularization is being performed in increasing numbers. Each year about one million procedures are performed worldwide. Diffuse alveolar hemorrhage is a rare but potentially life threatening complication that is associated with its use. Iced saline lavage maybe an important therapeutic option or a bridge to adequate oxygenation until other interventions can be undertaken.

DISCLOSURE:  S. Amanullah, None.

Chest. 2004;126(4_MeetingAbstracts):926S-a-927S. doi:10.1378/chest.126.4_MeetingAbstracts.926S-a

INTRODUCTION:  Tumors of the Trachea constitute 2% of the upper respiratory tumors. Dyspnea cough and wheezing are common symptom as the airway becomes narrow. Tracheal tumors can be easily missed on CXR and are usually discovered on Bronchoscopy or CT scan.

CASE PRESENTATION:  A 65-year-old man with 100 pack year smoking history presented with shortness of breath and cough. He had been seen with similar complaints on multiple occasions in the ER and discharged on Proventil and Advair with diagnosis of COPD. He was using accessory muscles of respiration, breathing 28 per minute. CXR showed no abnormality. CT scan showed a Tracheal mass obstructing the lower trachea. Bronchoscopy confirmed an endotracheal mass just above the carina, obliterating the airway almost completely(picture 1,2,3). PET scan revealed no metastases. After consultation with surgery and oncology, resection of the tumor was done with the Rigid Bronchoscope, knife/snare Cauterization and ND YAG LASER with no complication. The pathology showed Squamous cell carcinoma in the background of Squamous papilloma with clear margin. A follow-up Bronchoscopy showed residual thickening in the posterior wall of the trachea (Picture 4). Photodynamic therapy was indicated for treatment of possible microscopic residual malignancy. The patient received Photofrin II, 2mg/kg and 2 days later the area was treated with PDT LASER and the debris in the Trachea was cleared 2 days later. (picture 5). The patient has been followed with CT scan and bronchoscopy for more than 8 months so far and there is no evidence of recurrent tumor and the patient remains symptom free. (Picture 6).

DISCUSSIONS:  Interventional Pulmonology is a new field within Pulmonary Medicine. Advanced Bronchoscopic techniques such as Rigid Bronchoscopy, Laser therapy, Dilatation, Cauterization, Cryotherapy, Brachytherapy, Stenting and Photodynamic therapy are commonly used in Interventional Pulmonary Medicine. The number of centers and pulmonologists with experience in all of these procedures are limited. Laser produces a beam of monochromatic, coherent light. This light energy is transformed into heat as the laser interact with living tissue causing vaporization, coagulation, hemostasis & necrosis. Photocoagulation with ND Yag laser can be performed through a rigid or flexible Bronchoscope. Complications of laser therapy includes fistula formation, perforation of the airway, pneumothorax, hemorrhage, hypoxemia, or Endobronchial fire Photodynamic Therapy is used for the palliative treatment of endoluminal non-small cell cancer and for curative treatment of carcinoma In-situ. This procedure involves a photosensitizing agent (Photofrin II) which, when exposed to light of a proper wavelength, forms toxic oxygen radicals that result in cell death. PDT can destroys malignant cells while sparing adjacent normal tissues. The selective effect of photodynamic therapy is due to the greater uptake and retention of photosensitizing agents in neoplastic cells than in normal cells. The effect appears to be more pronounced 24 to 48 hours after infusion of the photosensitizing agent. The Bronchoscopic light therapy is performed 1-2 days after the injection of the agent. Electro Cauterization converts electrical energy to heat that is used to destroy tumors.

CONCLUSION:  Laser therapy is minimally invasive procedure with low morbidity and mortality. Combining different modalities of Laser, Cautery and Photodynamic therapy in Interventional Pulmonology have shown the best outcome in the treatment of airway involvement with primary and metastatic lung cancer. The interventional modalities are also considered safer and cost-effective as major surgery can be avoided.

DISCLOSURE:  A.R. Shad, None.

Topics: neoplasms
Chest. 2004;126(4_MeetingAbstracts):927S. doi:10.1378/chest.126.4_MeetingAbstracts.927S

INTRODUCTION:  Common variable immunodeficiency (CVID)predisposes to recurrent sinopulmonary infections, but other thoracic manifestations are unusual. We report a patient with CVID and bronchus associated lymphoid tissue (BALT).

CASE PRESENTATION:  27-year-old female presented with fever, shortness of breath, pleuritic chest pain and productive cough. This patient had asthma since childhood and recurrent ear infections in the last five years. Two years prior to admission, she had a left-sided pneumonia. Twenty days prior to admission she presented to the Emergency Department (ED) with worsening asthma and fever. A chest x-ray (CXR) done at that time showed a lingular pneumonia, and she was discharged on oral moxifloxacin, fluticasone/salmeterol and prednisone. The patient returned to the ED three weeks later with no improvement in symptoms, and the CXR showed extension of the pneumonia into the left lower lobe. The patient never smoked, used illicit drugs or used alcohol. On physical exam, the patient appeared comfortable. The temperature was 37°C, pulse 72 beats/minute, blood pressure 108/65, respirations 18/minute, O2 saturation 96% on room air. The head, ears, eyes, nose and throat were normal. The heart had no murmurs, rubs or gallops. She had diminished breath sounds at the left base with crackles in the left lower and mid-lung zones, with diffuse mild end-expiratory wheezing. The abdomen and extremities were normal. The white blood count was 14,500/mm3 with 80% granulocytes. Serum chemistries and liver function tests were normal. She was HIV seronegative. Computerized tomography of the chest showed multifocal airspace infiltrates, some with a nodular appearance and others, showing variable degrees of consolidation and ground glass opacity. The lingula and basal segments of the lower lobe are affected asymmetrically. No definite underlying anatomic abnormalities were seen nor, was there evidence of bronchiectasis or congenital deformities. Immunoglobulin assays were performed and serum IgA, IgE, IgM and IgG (including all subclasses) were all markedly reduced. Complement ( CH50 ) and Tcell abnormalities (CD4, CD8, CD4/CD8 ratio and Candidal Anergy panel) were negative. As patient has recurrent sinopulmonary infections with depressed immunoglobulin levels, a diagnosis of CVID was established. Flexible bronchoscopy revealed a diffuse bilateral cobblestone appearance of the airway at and below the level of the main carina and, purulent secretions in the lingula. Endobronchial biopsy revealed BALT.

DISCUSSIONS:  CVID is characterized by low levels of immunoglobulins and an increased susceptibility to infections. It typically occurs in the second or third decade of life, presenting with recurrent sinopulmonary infections that my lead to bronchiectasis. CVID is associated with lymph node and spleen enlargement and occasionally with enlargement of gut-associated lymphoid tissue (Peyer’s patches). CVID is also associated with an increased incidence of lymphoma, gastric cancer, inflammatory bowel disease, and autoimmune disorders. Sarcoid-like granulomas can occur in the skin, gut and other viscera; in this case, the bronchoscopic appearance of the airways looked like that of sarcoidosis. BALT is a localized collection of lymphocytes in the subepithelial area of the bronchi. Unlike Peyer’s patches, which occur in normal gut mucosa, BALT is not present in normal adult lungs, but may appear in patients with chronic respiratory infection and autoimmune disorders. BALT is associated with the development of extranodal low-grade B-cell lymphomas.

CONCLUSION:  To our knowledge, this is the only report of diffuse endobronchial BALT in association with CVID.

DISCLOSURE:  A. Tsegaye, None.

Chest. 2004;126(4_MeetingAbstracts):927S-a-928S. doi:10.1378/chest.126.4_MeetingAbstracts.927S-a

INTRODUCTION:  Lung malignancies reported in association with neurofibromatosis are sparse. We present the case of a patient with neurofibromatosis, a lung mass and a misleading transbronchial biopsy.

CASE PRESENTATION:  A 55 year-old smoker male with history of neurofibromatosis involving the skin, colon and presumably the lungs was evaluated for a new lung mass. Chest radiograph (CXR) and High Resolution Chest Tomogram (HRCT) done in 2000 for evaluation of dyspnea revealed extensive upper lobe bullous disease and diffuse interstitial lung disease (ILD). Pulmonary function tests were consistent with moderated restrictive pattern and decreased diffusion capacity. CXR in 2002 was unchanged; in early 2003 another CXR revealed a 1.3 cm density in the right upper lobe that increased to 3.4 cm in a following film at the end of 2003. The patient had no new respiratory symptoms. Chest CT showed a 4 x 5 cm mass with no lymphadenopathy. Fiberoptic bronchoscopic biopsy suggested neurofibroma (Fig 1A). In view of the atypical presentation, the risk factors for malignancy and the rapidly enlarging mass, the patient underwent lung resection. The pathologic report revealed adenocarcinoma of the lung (Fig 1B). Staging of lung cancer was T2N0M0.

DISCUSSIONS:  The most common pulmonary manifestations of neurofibromatosis consist of diffuse interstitial fibrosis and bullae. The prevalence of interstitial fibrosis and bullae is about 10 and 20 percent respectively. Neurofibromas are benign, slow-growing neoplasm that frequently arise from a spinal nerve root but may involve any thoracic nerve; radiologically they are usually sharply marginated, spherical, and lobulated paraspinous masses. Neurofibromin, the protein product encoded by the neurofibromatosis1 gene, is expressed in many tissues; gene mutations result in loss of functional protein, causing the wide spectrum of clinical findings, including neurofibromatosis-associated tumors. Neurofibromin belongs to a family of GTPase-activating proteins that downregulate a cellular proto-oncogene, p21-ras, an important determinant of cell growth and regulation. In most studies, Ras mutations are predominantly associated with adenocarcinoma; a causal relationship between smoking, K-ras mutation and the development of lung cancer have being suggested, but remains speculative [1]. The estimated overall risk of malignancy in neurofibromatosis is 2 to 10 percent of affected individuals; rate higher than that of the general population. Mediastinal atypical carcinoid as well as non-small and small cell cancer has been reported in patients with neurofibromatosis. We found 11 reports of neurofibromatosis associated with lung cancer in the Japanese literature. Adenocarcinoma was observed in 72.9% of cases, and poorly-differentiated tumor was observed in 7 out of 8 patients [3].

CONCLUSION:  In our opinion this case illustrates the intricacy of management in patients with neurofibromatosis and pulmonary involvement. The etiology of the bullae in our patient is most probable multifactorial due to NF1 and tobacco use; the ILD due to NF1. Development of carcinoma has been associated with emphysematous bullae, with ILD, tobacco use and NF1. As routine screening for gliomas is part of the care of patients with NF1, we suggest screening for lung cancer in this population. If a thoracic mass is identified, aggressive diagnostic intervention should be entertained keeping in mind that transbronchial specimens can be misleading.

DISCLOSURE:  L. Varadarajalu, None.

Chest. 2004;126(4_MeetingAbstracts):928S-929S. doi:10.1378/chest.126.4_MeetingAbstracts.928S

INTRODUCTION:  The complications associated with silicone breast mammoplasty have been previously described in the medical literature. However, the potential thoracic manifestations are not well known. We report a case of a patient who presented with a left hilar mass and pulmonary nodule twenty-five years after bilateral silicone injection breast augmentation.

CASE PRESENTATION:  A 46-year-old asymptomatic female with no history of cigarette smoking or known exposures to carcinogens presented initially for breast reconstruction utilizing saline bag prosthesis. The preoperative chest radiograph revealed a left hilar density, a left lung nodule, and a pleural effusion (Figure 1). Twenty-five years previously, she underwent cosmetic, bilateral mammoplasty with direct liquid silicone injection. Workup of the thoracic abnormalities proved to be non-diagnostic, and subsequent thoracotomy was performed. Biopsies of the left hilar mass showed benign granulomatous response presumably to the silicone, which was also present in the hilar nodes. A wedge resection of the pulmonary nodule revealed thromboembolic vascular occlusion with silicone and an associated nodular pulmonary infarction. Eighteen months later, the patient presented with hoarseness and mild dyspnea on exertion. A repeat chest radiograph showed an enlarging left hilar mass (Figure 2). Fiberoptic bronchoscopy showed a newly paralyzed left vocal cord and a lobulated endobronchial lesion nearly completely occluding the left upper lobe orifice. Subsequent biopsies of this lesion revealed a small cell lung carcinoma. The patient received multiple courses of systemic chemotherapy but eventually succumbed to widespread extrathoracic metastases.

DISCUSSIONS:  The potential health hazards of the silicone implant led to a ban of its use for augmentation mammoplasty by the Food and Drug Administration (FDA) in 1992 (1). A known complication of silicone breast augmentation is silicone lymphadenopathy, an infrequent and benign consequence of mammary augmentation utilizing either direct injection or bag-gel prosthesis technique (2,3). The majority of reported cases have involved axillary or supraclavicular nodes and had been considered an incidental finding without clear clinical significance (1,4). To our knowledge, only one other report has described hilar adenopathy as a consequence of either silicone injection or prosthesis placement, and no other report has described the development of a pulmonary nodule (5). Anecdotal case reports have described a possible link between silicone mammoplasty and connective tissue diseases (1). Malignant lymphomas have been reported in orthopedic patients with silicone metacarpophalangeal joint implants in association with silicone granulomas in lymph nodes, but whether silicone is the causative agent remains uncertain (6). According to a review of the possible health implications of silicone breast implants, there was no association between silicone mammoplasty and the subsequent development of breast carcinoma (1). To our knowledge, no other case report has described the subsequent development of a small cell lung carcinoma in the site of silicone lymphadenopathy.

CONCLUSION:  This discovery in a non-smoking patient is suggestive of a causal relationship. Our observations in this case should stimulate further studies regarding potential sequelae of direct silicone migration.

DISCLOSURE:  J.A. De Olazabal, None.

Chest. 2004;126(4_MeetingAbstracts):929S. doi:10.1378/chest.126.4_MeetingAbstracts.929S

INTRODUCTION:  Benign thymic hyperplasia (BTH), more commonly reported in children, can also occur in adults with various cancers concurrently or following chemotherapy.(1) It is thought to be an immunological rebound phenomenon either related to the malignancy or the chemotherapy administered.(2) To our knowledge, this is the first report of BTH following chemotherapy for metastatic uterine leiomyosarcoma.

CASE PRESENTATION:  In March 2001, a 47-year-old woman with pelvic pain was diagnosed with high-grade leiomyosarcoma of the uterus and underwent radical hysterectomy followed by local radiation therapy. A chest CT scan at this time was unremarkable. 3 months later, surveillance CT scans detected bilateral lung nodules, and one measuring 1.1-cm in the right upper lobe. She had no respiratory symptoms at that time. A CT-guided needle aspirate of a right upper lobe nodule confirmed the metastatic nature of her uterine sarcoma. Between September 2001 and March 2002, she received chemotherapy consisting of doxorubicin, ifosfamide, carboplatin and paclitaxel with almost complete resolution of the lung nodules. PET scan in October 2002 did not show any hypermetabolic foci but a CT scan of the chest (Fig 1B) obtained in June 2003 showed a new anterior mediastinal mass measuring 4.3 cm x 3.7 cm not seen in a CT scan from September 2002 (Fig 1A). In July 2003, she underwent a median sternotomy and resection of the mass. The specimen weighed 41 grams and was diagnosed to be BTH (Fig 2). She had an uneventful post-operative course.

DISCUSSIONS:  Appearance of an anterior mediastinal mass in a patient with malignancy most often is an ominous finding that leads to further invasive tests or surgery to exclude recurrence of disease or a new primary cancer. BTH was described in children recovering from thermal burns and later on recognized as thymic rebound in children following chemotherapy. It is rare in adults and is reported after chemotherapy for lymphomas, testicular germ cell tumors, and sarcomas. BTH can present and co-exist with lymphoma and newly diagnosed testicular germ cell tumors. BTH usually occurs within the first year after chemotherapy, but it can present even 5 years later. Data on nuclear imaging of BTH is limited. BTH can have positive uptake of F-18 fluorodeoxyglucose (FDG) on PET scan (3) and uptake of gallium-67 citrate scintigram, but usually negative on thallium-201 imaging. Tissue confirmation of cancer relapse should be obtained prior to initiating further chemotherapy to avoid the morbidity of unnecessary treatment.(4)

CONCLUSION:  Chest physicians should consider BTH in patients who present with a new anterior mediastinal mass after completion of chemotherapy for a variety of malignancies including metastatic leiomyosarcoma of uterus. Before resorting to median sternotomy and resection, in appropriate patients whose primary cancer is in remission and the CT imaging is suggestive of BTH, nuclear imaging studies could be considered to further evaluate the anterior mediastinal mass. Clinical or radiographic suspicion of BTH in adult cancer patients may allow for a minimally invasive tissue procurement and diagnosis, and obviate the need for surgical resection.

DISCLOSURE:  S.K. Vora, None.

Chest. 2004;126(4_MeetingAbstracts):929S-a-930S. doi:10.1378/chest.126.4_MeetingAbstracts.929S-a

INTRODUCTION:  Monoblastic sarcoma, a subtype of myelocytic sarcoma, is an extramedullary manifestation of myelocytic leukemia. We report a case with monoblastic sarcoma involving lung presenting with mediastinal lymhadenopathy, tracheal compression, pleural effusion and superior vena cava (SVC) syndrome which was responsive to chemotherapy.

CASE PRESENTATION:  A 54 year-old white male with hypertension presented with profound dyspnea at rest and non-productive cough. Chest roentogram and computed tomogram (CT) of the thorax showed right pleural effusion with right mediastinal and paratracheal masses. The mediastinal mass was encasing the distal trachea with near complete collapse of the right lung. Thoracentesis revealed an exudative effusion negative for malignant cells. A cervical lymph node biopsy was consistent with monoblastic sarcoma (flow cytometry: MPO-, CD 68+, CD 34-). Bone marrow biopsy was negative for AML. The patient exhibited clinical signs of superior vena cava obstruction and continued to be dyspneic at rest requiring 4 liters of oxygen. Flexible bronchoscopy revealed extensive submucosal disease with extrinsic compression of the tracheobronchial tree. Intravenous steroids to decrease mucosal edema were started, and within one week his room air saturation was 94% on ambulation. Chemotherapy induction with danorubicin, etoposide and Ara-C was initiated. A repeat CT thorax at day 16 demonstrated resolution of the pleural effusion and adenopathy. A repeat bronchoscopy performed 28 days after the initial one showed no extrinsic compression or submucosal disease of the airways. Five months after treatment the patient has had cranial recurrence of disease with cervical adenopathy, but there has been no recurrence of disease in the lung.

DISCUSSIONS:  Intrathoracic myelocytic sarcoma is rare with only 27 previously reported cases. Of these, 6 were associated with superior vena cava syndrome. Myelocytic sarcoma is further subdivided into granulocytic myeloperoxidase, monoblastic, myelomonoblastic and megakaryoblastic variants. Of the 28 cases 13 patients (46%) responded to treatment and 11 (39%) died. Of the cases with SVC syndrome three survived and three died. The mediastinum was the most common site of involvement (66%) of intrathoracic myelocytic sarcomas. SVC syndrome is an even rarer presentation, seen in only 21% of the cases. This is the first documented case of the monoblastic subtype involving lung. Acute dyspnea in our patient was secondary to pleural effusion, superior vena cava syndrome and tracheobronchial compression by the mediastinal lymphphadenopathy. Thoracentesis assisted in relieving the effusion related dyspnea. It was initially planned to treat the tracheobronchial compression with endobronchial stent placement, but the response to chemotherapy in the intervening period was impressive. A repeat bronchoscopy and CT scan revealed resolution of the tracheobronchial compression and thus stent placement was not deemed necessary. As the SVC syndrome subsided his dyspnea improved. At one week post chemotherapy the patient was off supplemental oxygen. SVC syndrome always portends poor prognosis; except possibly in intrathoracic myelocytic sarcomas in which the response rate to treatment has improved over the years. Pleural effusion in myelocytic sarcoma is distinctly rare and is reported in one other case in addition to ours.

CONCLUSION:  Though the intrathoracic presentation of myelocytic sarcoma is rare, this condition needs to be considered in the differential diagnosis while evaluating patients with mediastinal lymhadenopathy. The quick resolution of the SVC syndrome and extensive tracheobronchial compression highlights the fact that even in cases with significant intrathoracic involvement, early diagnosis and chemotherapy may ultimately improve outcome.

DISCLOSURE:  A.d. Corla-Souza, None.

Topics: sarcoma
Chest. 2004;126(4_MeetingAbstracts):930S-931S. doi:10.1378/chest.126.4_MeetingAbstracts.930S

INTRODUCTION:  Bronchial carcinoid is a rare tumor, making up 0.5 to 1.0% of all primary lung tumors. There is no known relationship to smoking or other environmental factors. Carcinoid tumors commonly present with hemoptysis, wheezing, stridor, recurrent pneumonia, or lung abscess due to bronchial obstruction. Many patients, however, are asymptomatic and tumor is an incidental radiographic finding. They spread by either the lymphatics or the bloodstream. Prognosis depends on the histologic appearance. Typical (well-differentiated) tumors are usually indolent with survival exceeding 90% after resection. Carcinoids with atypical histologic features (architectural disorganization, necrosis, or a mitotic rate of 2-5 mitoses per 10 high power fields) have a worse prognosis. They are much more likely to recur locally or to have distant metastases.

CASE PRESENTATION:  A 68-year-old male former smoker, originally presented with hemoptysis and was found to have a right middle lobe endobronchial carcinoid. A bronchoscopic biopsy revealed cells with bland nuclei, less than two mitotic figures per high power field, and no areas of necrosis. A right middle lobectomy was performed. Surgical margins and sampled lymph nodes were free of tumor. The patient did well postoperatively but did not follow-up in the pulmonary clinic. Two years later, the patient was seen with a 6 month history of hemoptysis and dyspnea on exertion. He denied any flushing, diarrhea, or abdominal cramping. Physical exam revealed wheezing localized over the right lung field and was otherwise normal. Computed tomography of the chest demonstrated probable endobronchial lesions in the upper trachea and the right mainstem bronchus. Bronchoscopy demonstrated numerous endobronchial lesions in the subglottic space, the upper trachea, and throughout the right mainstem bronchus and bronchus intermedius. Biopsies were obtained from the distal bronchus intermedius and the pathology revealed atypical histologic features with seven mitoses per ten high power fields.

DISCUSSIONS:  Bronchial carcinoid is usually a slow growing, locally invasive malignant tumor. Chemotherapy and radiation therapy are ineffective treatments; resection is the only means of cure. Many techniques have been shown to be successful: bronchoscopic resection, bronchial sleeve resection, wedge resection, lobectomy, and pneumonectomy. In histologically typical carcinoid with small tumor size and complete resection (like our patient initially), 10-year survival rates are in excess of 95%. Carcinoid tumors generally metastasize to the liver, bones, brain, and adrenal glands. Recurrence after resection is generally local to the area of resection or to regional lymph nodes. Although treatment for endobronchial carcinoid is often successful, they have a highly varied clinical course. Aggressive metastatic disease with survival less than one year has been reported. Our patient was admitted following his bronchoscopy. Further work-up revealed distant metastases to the liver, spleen, and vertebral bodies. Resection of the tracheal tumors using carbon dioxide laser was planned but the patient developed a myocardial infarction. He supsequently expired from respiratory failure deciding against further treatment.

CONCLUSION:  Patients with bronchial carcinoid have a variable course and require regular follow-up evaluation, although whether this will allow earlier detection of recurrence and improve outcomes requires further study.

DISCLOSURE:  M.J. Rossing, None.

Topics: carcinoid tumor
Chest. 2004;126(4_MeetingAbstracts):931S-932S. doi:10.1378/chest.126.4_MeetingAbstracts.931S

INTRODUCTION:  Primary sarcomas of the lung are exceedingly rare. They usually represent cases of fibrosarcoma, leiomyosarcoma, and hemangiopericytoma. Less commonly synovial sarcoma, malignant peripheral nerve sheath tumor, malignant fibrous histiocytoma and osteosarcoma have been described. Primary pulmonary myxoid leiomyosarcoma has only been reported once presenting as a peribronchial mass (1). However, myxoid leiomyosarcoma arising in other sites including the pulmonary vein (2) has been reported. We report a case of primary myxoid leiomyosarcoma presenting as a loculated pleural effusion.

CASE PRESENTATION:  A 71 year old non-smoking female presented with left shoulder pain. Her medical history was only remarkable for a hysterectomy performed 20 years ago with pathologic findings consistent with leiomyomas. On initial evaluation a chest X-ray (Figure 1) and a CT scan of the chest revealed a loculated right pleural effusion. No parenchymal abnormalities were observed. A bone scan was performed which demonstrated enhancement of the pleural effusion with no skeletal abnormalities. A thoracocentesis yielded exudative fluid with negative cytology. A closed pleural biopsy was nondiagnostic. Subsequently, the patient underwent a thoracotomy. Frozen section revealed malignant cells and a right lower lobectomy with mediastinal lymph node dissection was performed. A mass measuring 15.0 x 14.5 x 7.0 cm. composed of soft mucoid material was identified in the lung parenchyma extending to and involving the visceral pleural. Microscopically, this lesion was composed of spindle cells surrounded by a loose myxoid stroma (Figure 2) with focal hypercellular areas dysplaying a fascicular pattern.Immunohistochemical stains were positive for smooth muscle actin (SMA)(Figure 2 - insert), muscle specific actin (MSA), desmin, and vimentin. Electron microscopy revealed plaques, thin filaments and dense bodies. All resected mediastinal lymph nodes were negative for tumor. The postoperative course was unremarkable. The patient was offered but declined postoperative chemotherapy. She has remained clinically free of recurrence one year following thoracotomy.

DISCUSSIONS:  Primary myxoid leiomyosarcoma of the lung is an extremely rare entity with only one previously reported case (1). Radiologically, myxoid leiomyosarcomas usually appear as sharply demarcated masses with homogeneous density. We present a case in which no mass could be identified by plain film or CT imaging. The mucous character of the neoplasm combined with the presence of pleural involvement precluded the preoperative identification of parenchymal involvement. Frequently conventional pulmonary leiomyosarcoma is diagnosed by fine needle aspiration cytology (3). Although in the present case there was spillage of mucinous matrix into the pleural cavity, aspiration cytology failed to identify neoplastic cells. This histologic variant is known to be deceptively hypocellular with lower mitotic rates as compared to conventional leiomyosarcomas. Despite their hypocellularity, extrapulmonary myxoid leiomyosarcomas tend to behave aggressively with a high rate of local recurrence. This case does not follow this pattern. Despite a large primary tumor with pleural involvement the patient has shown no evidence of recurrence one year following resection.

CONCLUSION:  We describe the second case of primary myxoid leiomyosarcoma of the lung reported in the medical literature. Our patient’s course following resection has been distinctly superior to that reported with myxoid leiomyosarcomas of extrapulmonary origin. The role of adjuvant chemotherapy and radiation therapy remains uncertain.

DISCLOSURE:  J.I. Mora, None.

Topics: lung , leiomyosarcoma
Chest. 2004;126(4_MeetingAbstracts):932S-933S. doi:10.1378/chest.126.4_MeetingAbstracts.932S

INTRODUCTION:  Giant left atrium associated with mitral valve disease may cause morbidity from compression of adjacent structures. We present a case of recurrent left lung collapse caused by massive left atrial enlargement.

CASE PRESENTATION:  A 53-year-old man with a history of mitral valve prolapse and severe mitral regurgitation presented in acute pulmonary edema and required immediate intubation. He had several preceding admissions for heart failure and had become increasingly breathless (New York Heart Association class IV). He had previously declined mitral valve replacement surgery. Echocardiography performed two months ago showed a giant left atrium (13x12 centimeters), normal left ventricular function, severe pulmonary hypertension (pulmonary artery systolic pressure 65 millimeters of mercury), severe mitral and tricuspid regurgitation. He improved with diuretic therapy and was extubated two days later. The following day, he developed respiratory distress and was re-intubated. Chest radiographs showed left lung collapse that re-expanded with positive-pressure ventilation (Figure 1). He developed a second episode of left lung collapse after being weaned off the ventilator and was intubated again. Bronchoscopy revealed pulsatile extrinsic compression of the distal end of the left main bronchus (Figure 2) and the scope could not be passed into the upper or lower lobar bronchi. The patient underwent mitral and tricuspid valve replacement. In addition, left atrial reduction by plicating the posteroinferior wall using a trans-septal approach was done, thereby relieving bronchial compression. There were no further episodes of lung collapse and post-operative bronchoscopy showed patent bronchi. After a post-operative period complicated by brainstem hemorrhage, prolonged intubation requiring tracheostomy and nosocomial infection, he was weaned off the ventilator and successfully rehabilitated. Echocardiography showed reduction of left atrial size to 7x5 centimeters.

DISCUSSIONS:  Giant left atrium are defined as those measuring more than 8 centimeters in diameter on echocardiography(1) and are typically found in patients with severe mitral regurgitation from rheumatic heart disease or mitral valve prolapse. It causes morbidity by compressing adjacent structures, for example Ortner’s syndrome and esophageal compression. Less common presentations include hyperlucent lung(2) and atelectasis after pericardial decortication(3). However, to the authors’ knowledge, recurrent collapse of the lung has not been reported in the English-language medical literature. In our patient, having established on bronchoscopy that the cause of recurrent lung collapse was due to extrinsic compression by a giant left atrium, we considered the treatment options. Endobronchial stenting was excluded as there were concerns about the risk of perforation into the left atrium with continuing external pressure. In addition, the site of compression was at the distal end of the main bronchus and involved the bifurcation of the lobar bronchi. As such, stenting was not appropriate. We concluded that definitive surgical treatment with prosthetic valve replacement was necessary to correct the underlying pathology. In addition, left atrial reduction surgery, first advocated by Kawazoe(4), was required to reduce bronchial compression.

CONCLUSION:  Recurrent left lung collapse caused by giant left atrium compressing on left main bronchus should be surgically treated with mitral valve replacement and left atrial reduction surgery.

DISCLOSURE:  G. Phua, None.

Chest. 2004;126(4_MeetingAbstracts):933S. doi:10.1378/chest.126.4_MeetingAbstracts.933S

INTRODUCTION:  Desiccated porcine thyroid preparations have been used as an alternative to synthetic thyroxine (1). The perceived benefits of desiccated porcine thyroid (DPT) are mood elevation, improved cognitive performance and increased activity level. “Holistic” physicians have touted DPT for conditions ranging from Major Depression to infertility. Accordingly, the abuse of DPT may be an under-recognized phenomenon.

CASE PRESENTATION:  A 48 year-old female nonsmoker with a history of restrictive lung disease, fibromyalgia, and chronic fatigue syndrome presented with dyspnea,hemoptysis, and massive lower extremity edema. Previous pulmonary function testing revealed total lung capacity of 3.33 liters (78% predicted) and diffusing capacity of 11.8 mL/mmHg/min (56% predicted). For ten years she had been taking prescription DPT for fibromyalgia and chronic fatigue syndrome. Examination revealed tachypnea, tachycardia, and labored breathing. B-type Natriuretic Peptide(BNP) was 508 pg/mL. Lactate was 2.6 mmol/L. Troponin-I assay was <0.02 ng/mL. TSH was consistently <0.04 uIU/mL during DPT treatment, free thyronine was currently 2.9 pg/mL, free thyroxine was 1.29 ng/dL, and antithyroglobulin and antimicrosomal antibodies were undetectable. Spiral CT revealed a segmental right lower lobe filling defect and multiple small basilar nodules [Fig 1]. Subsequent ventilation-perfusion scan demonstrated multiple bilateral moderate-sized unmatched perfusion defects. Echocardiogram showed severe right-side dilation with paradoxical septal motion and left ventricular ejection fraction of 55%. Cardiac catheterization revealed pulmonary arterial pressure of 84/28 mmHg (mean 51 mmHg). Pulmonary capillary occlusion pressure was 6 mmHg, cardiac index 1.3 L/min/m2, and pulmonary vascular resistance 1789 dynes-sec(-1)-cm(-5). Limited angiogram excluded chronic thromboembolic disease. Adenosine challenge demonstrated 15.8% reversibility in Total Pulmonary Resistance. Prior surgical lung biopsy was then reviewed, and it revealed stellate fibrosis and arteriolar thrombosis with recanalization. Eosinsophilic tissue infiltration was not evident, and S-100 stain was negative [Fig 2].

DISCUSSIONS:  Prior reports suggest that Grave’s thyrotoxicosis is associated with isolated pulmonary hypertension (234). It has been hypothesized that antithyroglobulin is a marker of generalized immune activation leading to both pulmonary hypertension and the hyperthyroid state (5,6). Our patient tested negative for antithyrotropin. Therefore if her pulmonary hypertension was due to hyperthyroidism, it cannot be due to the proposed antibody-mediated mechanism. Our patient had some features consistent with pulmonary Langerhans Cell Histiocytosis (LCH), such as nodules on CT and histiologic findings of stellate fibrosis. Pulmonary hypertension has been described in a series of patients with pulmonary Langerhans Cell Histiocytosis (LCH) (7). Our patient’s presentation was not fully consistent with LCH, because the nodules were predominantly basilar and S-100 staining of her biopsy was negative. Her disease may represent a yet unreported variant of LCH with manifestations of pulmonary hypertension and stellate fibrosis.

CONCLUSION:  Abuse of DPT may induce pulmonary hypertension and/or interstitial lung disease. Caution should be used when prescribing this treatment for euthyroid patients with disorders such as fibromyalgia.

DISCLOSURE:  M.J. Emerick, None.

Chest. 2004;126(4_MeetingAbstracts):933S-a-934S. doi:10.1378/chest.126.4_MeetingAbstracts.933S-a

INTRODUCTION:  Tako tsubo cardiomyopathy(Octopus trap) is an enigmatic cardiomyopathy, characterized by marked apical asynergy in the absence of significant coronary disease.

CASE PRESENTATION:  A 77-year-old Caucasian female with history of bovine aortic valve replacement 5 years ago, recently presented to the Emergency Room(ER) with severe typical anginal pain, following the news of her brother’s death. At the time of presentation to the ER, she was hemodynamically stable and her physical exam was unremarkable except for 1+ bilateral ankle edema. Her significant laboratory work up included a positive troponin T of 0.83 ng/ml (normal <0.1 ng/ml); EKG showed first degree AV block, left axis deviation and 0.5mm-1mm ST elevations in V2 to V4; an urgent transthoracic echocardiogram done at the peripheral hospital showed extensive anteroapical akinesis. An emergency coronary angiography at our hospital revealed only a minimal atherosclerotic disease. Left ventriculography demonstrated significantly depressed left ventricular(LV) function with extensive akinesis of the apex, anterior apical, mid anterior, inferoapical and mid inferior segments with an EF estimated at 30%. Basal segments were vigrously contracting. Given the extent of the wall motion abnormality to the lack of significant coronary artery disease, a diagnosis of Tako-tsubo was entertained. Echocardiogram performed the next day demonstrated a severe dilatation and akinesis of the apical and mid segments. Perfusion echocardiogram using Optison showed absence of perfusion in the apex and distal septum while perfusion was preserved in the mid septum and mid lateral wall(graph 1). Repeat Perfusion study 72 hours later showed improved perfusion in all walls except the apex (graph 2). The LV function had only marginally improved in the interim.

DISCUSSIONS:  Tako tsubo cardiomyopathy has been characterized by reversible apical ballooning of the left ventricule(1). It is common in elderly females. Emotional or physical stress has been recognized as a triggering factor(1). Transient ST-T segment changes is seen on EKG with only minimal evidence of epicardial coronary artery stenosis. It was first described in Japanese patients (123), but has recently been recognized in Caucasians and other too(456). The exact mechanism is unknown but microvascular dysfunction is suspected. Simultaneous multivessel spasm is also considered as possible etiology. Evidence of decreased blood flow in the apical segments has been shown by nuclear and PET perfusion studies. Perfusion echocardiogram is a new technique and literature review shows perfusion echocardiogram been performed in this condition in only one prior patient. We performed perfusion echocardiogram in this rare but increasingly recognized condition.

CONCLUSION:  Perfusion echocardiogram gives us insight about the possibility of microvascular dysfunction as the cause of this transient apical stunning in Tako - tsubo cardiomyopathy.

DISCLOSURE:  S.P. Upadya, None.

Chest. 2004;126(4_MeetingAbstracts):934S-935S. doi:10.1378/chest.126.4_MeetingAbstracts.934S

INTRODUCTION:  Aneurysms of coronary artery grafts though rare have been well reported in literature. Most of these reports however describe patients with single aneurysms. We would like to describe a patient presenting with three simultaneous Saphenous Vein Graft (SVG) aneurysms and an aortic pseudoaneurysm.

CASE PRESENTATION:  A 71 year old female presented with shortness of breath, orthopnea, pedal edema and retrosternal chest pain. Past medical history was significant for hypertension and coronary artery disease for which she underwent saphenous vein bypass graft to the Right Coronary Artery (RCA), Left Anterior Descending Artery (LAD) and Left Circumflex Artery (LCX). Physical examination revealed an elderly woman with a respiratory rate of 24, pulse rate of 130 and a blood pressure of 117/78. She had jugular venous distension 3 cm above the sternal angle, bilateral basal rales on chest examination and pitting edema in both lower extremities. Laboratory revealed anemia with hemoglobin of 8.8, normal white cell count and electrolytes. Serial cardiac enzymes were normal and electrocardiograms showed no evidence of acute ischemia. Chest x-ray (CXR) revealed some vascular prominence and mediastinal widening compared to her prior CXR done 15 months before. A CT scan of her chest ordered to rule out aortic dissection showed pseudoaneurysms of the SVG to the RCA (3.4 cm), true aneurysms of LAD (2 cm) and LCX (1.6.cm) and also a large pseudoaneurysm of the Aorta (7.7cm). A cardiac catheterization confirmed the findings. Options of surgical and percutaneous procedures were discussed but the patient opted for conservative management. The patient was treated for congestive heart failure, discharged home, is currently asymptomatic and doing well at one year follow up.

DISCUSSIONS:  Aneurysms of the saphenous vein grafts, though rare must be considered in the differential diagnosis of mediastinal masses in patients who have undergone coronary artery bypass surgery. Aneurysm of saphenous vein grafts (SVG) is a rare late complication of coronary bypass surgery with reported incidence of about 0.07 %. Pseudoaneurysm formation of saphenous vein grafts have been attributed to technical factors such as disruption of suture lines or infection while true aneurysms are related to the progression of degenerative atherosclerotic disease. Hyperlipidemia and hypertension remains a significant contributor to the pathogenesis. Clinical presentation is usually secondary to incidental mediastinal mass, angina or myocardial infarction due to progressive lumen occlusion, distal embolization and compression of surrounding structures, including grafts and coronary arteries. Rarer presentations include fistula formation between aneurysm and right atrium or ventricle, and hemoptysis secondary to rupture into right middle bronchus. Though chest x-ray is usually abnormal, CT remains the mainstay of diagnosis. It differentiates between true and pseudoaneurysms, estimates size and can describe the presence of intraluminal thrombus. Other imaging modalities include echocardiography, magnetic resonance imaging and angiography. Therapeutic options include surgical exclusion or resection of the aneurysm with or without revascularization. Percutaneous coil embolization and covered stent placement has been well described. Conservative management may also have a role. Almost all prior case reports have described single graft aneurysms and multiple SVG aneurysms are extremely rare. Our patient had three concomitant saphenous vein graft aneurysms,a large aortic pseudoaneurym and is doing well with conservative management. A similar presentation has not been described before to the best of our knowledge.

CONCLUSION:  Aneurysms of the saphenous vein grafts, though rare must be considered in the differential diagnosis of mediastinal masses in patients who have undergone prior coronary artery bypass surgery.The role for conservative management in these patients need to be explored further.

DISCLOSURE:  T. Majumdar, None.

Chest. 2004;126(4_MeetingAbstracts):935S-936S. doi:10.1378/chest.126.4_MeetingAbstracts.935S

INTRODUCTION:  Pulmonary hypertension (PH) is a life-threatening disease causing an increase in pulmonary arterial pressure. Sarcoidosis is an idiopathic multi-system granulomatous disorder. There is no approved treatment for sarcoidosis-associated PH. We describe a patient with this condition who improved remarkably in both symptomatology and hemodynamic parameters with bosentan, an endothelin antagonist approved for treatment of pulmonary arterial hypertension (PAH).

CASE PRESENTATION:  A 55 year-old African-American woman with severe pulmonary sarcoidosis (biopsied in 1979) presented to the hospital in October 2001 with worsening dyspnea (NYHA Class III-IV), fatigue, and hacking cough. Past medical history included systemic hypertension and dyslipidemia. She had never smoked or used recreational drugs. She had a core temperature of 98.5° F, pulse of 79/minute, blood pressure of 140/84 mmHg, and respiratory rate of 20/minute with oxygen saturation of 95% on 2 liters/minute oxygen via nasal cannula. Physical examination revealed a jugular venous pressure of 4 cm at 30°. Chest exhibited bibasilar coarse crackles and scoliosis. Cardiac exam showed tricuspid regurgitation murmur with right ventricular heave and a loud P2. Chest x-ray confirmed scoliosis, and a CT showed prominent interstitial lung changes (Figures 1and 2). She underwent a right heart catheterization (RHC) with mean pulmonary arterial pressure (mPAP) of 40 mmHg, pulmonary vascular resistance (PVR) of 580 dynes•sec•cm-5 (7 Woods Unit), and cardiac output (CO) of 3.85 L/min. Hemodynamics did not improve with adenosine. She was discharged on diuretics and seen in clinic where her 6-minute walk was 390 m. She was started on bosentan 62.5 mg twice daily in January 2002, and the dose was increased to 125 mg after one month. Aminotransferases were monitored monthly. Her dyspnea began to improve after 3-4 months and she regained her physical activity. The 6-minute walk improved to 550 m by November 2002. After 2 years of therapy, her dyspnea improved to NYHA Class II and she began to live independently. She had taken sildenafil intermittently when available as samples from the clinic. A repeat RHC in December 2003 showed improvement in hemodynamics. PVR had fallen from 7 to 5.1 Wood units (408 dynes/sec-1.m-5), CO increased from 3.9 to 7.4 L/min, and mPAP increased to 47 mmHg, but in view of the markedly improved CO and reduced PVR, does not represent worsening of her underlying disease.

DISCUSSIONS:  The mechanism of sarcoidosis-related PH has not been elucidated. In idiopathic PAH, release of NO by endothelial NO synthase plays an essential role in the maintenance of normal blood flow. Endothelial disruption causes decreased expression of endothelial NO synthase and consequent defective release of NO. Alterations in the synthesis and release of prostacyclin and endothelin-1 also occur and likely cause an imbalance of endothelium-derived vasoactive mediators. These changes cause vasoconstriction and subsequent remodeling. A similar mechanism may contribute to PH in sarcoidosis. Bosentan is a nonspecific endothelin receptor antagonist used in the treatment of PAH. Bosentan is not approved for the treatment of PH secondary to sarcoidosis. However, the increase of plasma endothelin-1 concentration in sarcoidosis, and decline with disease remission, support the idea that bosentan may be helpful in this condition(1,2). Our data show that bosentan improved our patient’s symptoms and hemodynamics. A randomized controlled trial is needed to establish therapeutic efficacy in this otherwise lethal disease.

CONCLUSION:  This is the first case report of sarcoidosis-related PH that improved both symptomatically and hemodynamically with bosentan.

DISCLOSURE:  K. Sirithanakul, None.

Chest. 2004;126(4_MeetingAbstracts):936S. doi:10.1378/chest.126.4_MeetingAbstracts.936S

INTRODUCTION:  Aortobronchial fistula is a rare but frequently lethal cause of hemoptysis. The most common causes are expanding descending thoracic aortic aneurysm, previous aortic surgery or neoplasm.

CASE PRESENTATION:  We present a case of a 60 year-old gentleman who, in 1960, had a left lower lobectomy for extensive bronchiectasis. Multiple, recurrent pneumonias of his remaining left lung resulted in bronchomalacia and chronic collapse of his left upper lobe. The bronchomalacia was treated with endoluminal bronchial nitinol stents because of his inability to tolerate any further resection secondary to his medical comorbidities. Approximately 2 years after the stent placement, he developed hemoptysis. Initial bronchoscopy revealed granulation tissue at the distal end of the stents. With the idea that the granulation tissue was the cause of his hemoptysis laser and PDT therapy were attempted without success. Bronchial artery embolization was then considered; on the initial aortogram an aortobronchial fistula was identified. Because of his poor overall medical condition and inability to tolerate an operation an endovascular stent graft was placed across the fistula with subsequent successful resolution of his hemoptysis.

DISCUSSIONS:  Although aneurysmal disease, previous aortic surgery and neoplasm are the most common causes of aortobronchial fistula, other causes exist as well. In our patient the chronic inflammation from the in dwelling endobronchial stents as well as the bronchomalacia from recurrent infections predisposed him to fistulazation. Formal graft repair of the aorta with or without pulmonary resection is the classic treatment. Most patients with this condition are too high risk for such an extensive procedure. Although there is risk of infection, endovascular exclusion of the fistula provides a safe and effective alternitive for high risk patients.

CONCLUSION:  Aortobronchial fistula can be successfully treated with less invasive, endovascular techniques in high risk patients.

DISCLOSURE:  G.G. Smaroff, None.

Chest. 2004;126(4_MeetingAbstracts):936S-a-937S. doi:10.1378/chest.126.4_MeetingAbstracts.936S-a

INTRODUCTION:  Mechanical ventilation has undergone many changes, particularly with the creation of additional modes of ventilation. Automatic tube compensation (ATC) is one of the newer, more unique modes. It is designed to precisely overcome the calculated resistance of the airway by adjusting the delivered pressure in accordance to flow rates generated by the patient. We report a case of respiratory myoclonus with unusual results while utilizing ATC.

CASE PRESENTATION:  A thirty-six year old male presented to the emergency department in cardiopulmonary arrest and was successfully resuscitated after thirty minutes. He developed multiple organ failure. Head CT did not reveal any acute neurological process. Examination revealed minimal brainstem activity. Within twenty-four hours, vasopressors were weaned off and hepatic transaminases normalized, but his renal failure required hemodialysis and his respiratory failure necessitated continued mechanical ventilatory support. Seizure activity was noted on the second hospital day, confirmed by EEG, and resolved with anticonvulsant therapy. Medically, the patient stabilized, but continued to have severe anoxic encephalopathy. A tracheostomy (8.0 mm) was performed for airway protection and maintenance of mechanical ventilatory support. Four weeks after his admission, he developed myoclonus involving his right sternocleidomastoid and intercostal muscles and diaphragm. These were refractory to benzodiazepines, dantrolene, and phenytoin. While receiving pressure support ventilation of 10 cm H2O, tidal volumes of 350 ml were produced by the myoclonic jerks. This continued for several days while efforts were made to wean mechanical ventilation. Unexpectedly, during a spontaneous breathing trial utilizing automatic tube compensation, the myoclonic contractions produced peak inspiratory pressures (PIP) of 70 cm H2O and tidal volumes in excess of one liter. When the % support was reduced from 100% to 20%, the PIP decreased to 25 cm H2O and tidal volumes of 700 ml.

DISCUSSIONS:  Respiratory myoclonus is a rare phenomenon. It is also known as Leeuwenhoek’s disease, as Antoine van Leeuwenhoek was the first to describe it in 1723. Fewer than one hundred cases have been reported and only four of which were involving patients on mechanical ventilation. In most cases, discomfort due to diaphragmatic “fluttering” is described, but actual impairment of ventilation is not. ATC is designed to deliver positive pressure proportional to the inspired flow. This pressure overcomes the estimated resistance of an artificial airway, which is proportional to the tube length and internal diameter as well as the flow rate. We postulate that in the ATC mode, sudden respiratory mycoclonic contractions can produce an abrupt, marked increase in flow rate that generates very high airway pressure and correspondingly large tidal volume. A literature review and discussion with Nellcor Puritan Bennett representatives suggests ours is the first reported case of this phenomenon.

CONCLUSION:  Respiratory myoclonus can produce high inspiratory flow rates that generate excessive pressure and tidal volumes when utilizing automatic ube compensation.

DISCLOSURE:  J.C. Perry, None.

Chest. 2004;126(4_MeetingAbstracts):937S-938S. doi:10.1378/chest.126.4_MeetingAbstracts.937S

INTRODUCTION:  Polyostotic fibrous dysplasia is a rare developmental disorder with focal areas of abnormal bony architecture. We present a case of progressive rib involvement causing severe restrictive pulmonary disease and respiratory failure.

CASE PRESENTATION:  A 59-year-old woman with a history of polyostotic fibrous dysplasia with thoracic cage involvement presented with acute on chronic hypercapnic respiratory failure requiring mechanical ventilation. On physical examination, she was obese with a weight of 201 lbs. The chest wall appeared smaller than expected for patient size, however, no gross deformity was apparent. Breath sounds were reduced over the right lung field. Chest radiographs and thoracic computed tomography scanning revealed marked bony deformities of the rib cage, worse on the right, encroaching on the pulmonary parenchyma. (Images 1: AP chest roentgenogram showing extensive bony abnormalities of the chest with marked decreases in lung volumes. Image 2: Axial computer tomographic image showing massively enlarged irregular ribs impinging on thoracic structures.) The patient improved with intravenous antibiotic coverage for acute bacterial bronchitis, was successfully extubated and discharged home on supplemental oxygen. During outpatient follow-up her pulmonary function tests revealed a severe restrictive ventilatory defect. Total lung capacity (TLC) of 1.53 liters (35% predicted) and vital capacity (VC) of 0.79 liters (30% predicted). Room air pulse oximetry showed a saturation of 92% at rest with symptomatic desaturation to 85% with ambulation. She was managed with continued supplemental oxygen at 3 liters/minute.

DISCUSSIONS:  Fibrous dysplasia is a sporadic developmental condition affecting bony architecture. Woven bone and calcified cartilage forms in areas where lamellar bone would usually develop. This is accompanied by intense marrow fibrosis and a high rate of bone turnover. Affected bones demonstrate poor mechanical strength leading to pathologic fractures and progressively enlarging deformities that impinge on adjacent structures. Monostotic involvement occurs more commonly than the polyostotic form. The triad of polyostotic fibrous dysplasia, distinctive café-au-lait skin pigmentation and precocious puberty characterizes the McCune-Albright Syndrome. Genomic analysis of patients with McCune-Albright Syndrome has demonstrated mutations in the genes encoding the alpha subunit of the stimulatory guanine-nucleotide–binding protein (Gs-alpha).1 The mutations cause increased activation of the cyclic AMP-protein kinase A signal transduction pathway. It is postulated that the somatic mutations are postzygotic, occurring during early embryogenesis. Mutations at that juncture cause a mosaic pattern where some tissues become affected while others develop normally. Bony involvement in polyostotic fibrous dysplasia typically involves facial bones, skull base, long bones and occasionally ribs. Clinically, patients present with localized pain, pathologic fractures, cranial nerve abnormalities, and rarely high-output cardiac failure resembling Paget’s disease. Malignant transformation occurs with a frequency of less than 1 percent. Typical radiological changes include increased bony radiolucency, focal thinning of the cortex, bony enlargement and deformity. In our review of the literature only two other cases of polyostotic fibrous dysplasia causing restrictive lung disease and respiratory insufficiency have been described.2,3 Surgical resection was employed in both cases to relieve dyspnea and improve the restrictive ventilatory defect.

CONCLUSION:  Polyostotic fibrous dysplasia is a rare cause of progressive chest wall related restrictive lung disease with distinctive radiological findings. In severe cases surgical resection has been attempted for palliation.

DISCLOSURE:  M.D. Suskin, None.

Chest. 2004;126(4_MeetingAbstracts):938S. doi:10.1378/chest.126.4_MeetingAbstracts.938S

INTRODUCTION:  While a mainstay for inflammatory lung disease, corticosteroids are avoided in patients suspected of having infectious lung diseases. Steroid use in fungal lung diseases in particular is concerning for fear of suppressing the host immune response and promoting spread of the pathogen. However, there are case reports of clinical responses to corticosteroid therapy in critically ill patients with invasive fungal lung disease such as histoplasmosis and coccidioidomycosis1,2. We report a case of hypoxemia due to disseminated histoplasmosis, in a patient who was not critically ill, with a dramatic response to steroid therapy.

CASE PRESENTATION:  31-year-old white male with a history of Crohn’s disease, receiving routine infliximab infusions, presents with four weeks of fever, non-productive cough, dyspnea, and abdominal pain. He also reported fatigue and night sweats. He was seen by his primary care physician and started on a seven day course of levofloxacin. His symptoms significantly worsened over two weeks prior to presentation including a 20 pound weight loss, increased dyspnea and development of jaundice. Physical exam: Temperature of 104.6 degrees Fahrenheit, heart rate 96 beats per minute and blood pressure 110/76, SpO2 86% on room air. Lung exam demonstrated bilateral rales, right greater than left, with significant egophony throughout his right lung base. His abdomen was tender to palpation and the liver was palpable approximately 3 cm below his right costal margin. Skin exam revealed jaundice. Laboratory evaluation revealed white blood cell count 4.1, hemoglobin 12.1, platelet count 145, total bilirubin 3.6, direct bilirubin 2.2, alkaline phosphatase 1006, ALT 116, AST 207. Arterial blood gas showed a PaO2 of 54 on two liters of oxygen. Histoplasmosis urinary antigen was strongly positive. PPD was negative. Chest radiograph and CT scan of chest showed bilateral alveolar infiltrates, right greater than left. Bronchoalveolar lavage analysis revealed a profound lymphocytosis (61%) with a lymphocyte helper/suppressor ratio of 0.5. Blood cultures revealed moderate growth of filamentous fungus consistent with Histoplasmosis Capsulatum. Treatment with liposomal amphotericin B was begun. After 4 days of therapy there was minimal improvement in his oxygenation. Prednisone 40 mg once a day was begun and within 24-36 hours his hypoxemia resolved with a SpO2 of 95% on room air, and his liver dysfunction and pancytopenia continued to normalize.

DISCUSSIONS:  Few patients with acute disseminated pulmonary histoplasmosis with hypoxemia have been prescribed corticosteroids, and all other known reported cases were patients with ARDS.1,3 Experiments have shown that exposure of lymphocytes and alveolar macrophages to H. capsulatum results in T cell proliferation and secretion of pro-inflammatory cytokines which further amplify the inflammatory response. We hypothesize that steroid therapy improved our patient’s hypoxemia by retarding the pulmonary inflammatory response to the histoplasmosis organism in the lung. Steroids have been demonstrated to limit chemotaxis and phagocytosis in pulmonary inflammatory cells, as well as reduce the complement-mediated neutrophil activation and reduce surfactant degradation.

CONCLUSION:  This case demonstrates a dramatic clinical response to corticosteroid therapy in a patient with moderate hypoxemia and disseminated histoplasmosis. While steroids should be used with caution in fungal infections, this case suggests a possible beneficial role of steroids in the treatment of disseminated histoplasmosis with significant lung involvement, particularly in patients with hypoxemia.

DISCLOSURE:  J.P. Parsons, None.

Chest. 2004;126(4_MeetingAbstracts):938S-a-939S. doi:10.1378/chest.126.4_MeetingAbstracts.938S-a

INTRODUCTION:  Pulmonary complications after hematopoietic stem cell transplant have a wide differential diagnosis. We report a patient with diffuse nodular disease three months after autologous bone marrow transplant.

CASE PRESENTATION:  27 year old man with Hodgkin’s lymphoma presented with acute febrile illness with dry cough. He originally presented 15 months prior to admission with fevers and cough, and was found to have mediastinal adenopathy involving the chest wall and several lung nodules; pathology revealed nodular sclerosing Hodgkin’s lymphoma. He underwent chemotherapy with doxorubicin, bleomycin, vinblastine, and dacarbazine but social issues limited him to 2 of 28 planned radiation doses. He relapsed and received ifosfamide, carboplatin, and etoposide, but recurred 3 months prior to admission. He underwent an autologous peripheral bone marrow transplant after high dose BCNU, etoposide, cytarabine, and melphalan with apparent clinical remission. One week before admission, he was well and playing basketball until the acute onset of fevers, chills, dry cough then progressive hypoxemia, despite antibacterials. Chest CT revealed innumerable coalescent nodules and mediastinal adenopathy involving the left chest wall with pleural effusion (figure 1). He was started on treatment for fungal, bacterial, PCP and mycobacterial etiologies with adjuvant steroids. Pathologic specimens included transbronchial biopsy which was negative for infection but with lymphocyte aggregates of uncertain significance, and chest wall biopsy was consistent with recurrent Hodgkin’s lymphoma (figure 2demonstrates Reed-Sternberg cell surrounded by characteristic mixed cellular infiltrate). After a month of mechanical ventilation with progressively higher levels of oxygen and lower compliances, he expired. At autopsy, the lung was found to be infiltrated with sheets of anaplastic cells consistent with a syncytial varient of Hodgkin’s lymphoma.

DISCUSSIONS:  The infectious pulmonary complications of autologous bone marrow transplant are similar to, but less common than, those of allogeneic transplants because of persistent t-cell defects. We suspected that the randomly distributed diffuse nodular pulmonary infiltration in this patient could represent milliary tuberculosis or fungal disease. Although recurrent Hodgkin’s disease involving the lung is common, it rarely causes respiratory failure due to infiltration of the lung parenchyma, as can be seen in NHL. In Hodgkin’s lymphoma, autopsy series show equal numbers of patients who die from tumor progression as from infection, with significant numbers of patients with clinically missed infections (1). Involvement of the lung in Hodgkin’s lymphoma occurs with increasing frequency as the disease advances from initial diagnosis (20%) to autopsy (60%) (2). Pulmonary manifestations of Hodgkin’s disease during relapse of disease include nodules (sometimes cavitating), consolidation, often with mediastinal nodal and chest wall involvement (3). Increased aggressiveness of tumor is often observed after failed hematopoietic transplantation, perhaps because of decreased immune surveillance. Transformation of Hodgkin’s disease into an aggressive variant infiltrating the lung is rare. A Japanese (4) case series recently documented the progression of 6 patients with Hodgkin’s disease into anaplastic large cell lymphoma with an aggressive clinical course. One patient had lung involvement and 5 of 6 died within 13 months.

CONCLUSION:  Progressive pulmonary infiltrates leading to acute respiratory failure in patients who are post bone marrow transplantation for Hodgkin’s lymphoma are most commonly related to infectious etiologies. We report a rare case of transformation into an aggressive syncytial varient leading to hypoxemic respiratory failure.

DISCLOSURE:  S.C. Jacoby, None.

Chest. 2004;126(4_MeetingAbstracts):939S. doi:10.1378/chest.126.4_MeetingAbstracts.939S

INTRODUCTION:  We present a case of post-traumatic neurological deficit due to Guillain-Barre syndrome. This case is the first presentation described to the best of our knowledge.

CASE PRESENTATION:  A 37 year-old male was admitted to the ICU after a motor vehicle collision where he was found head trapped underneath the car, agitated and confused. Glasgow Coma Scale (GCS) at the scene was 13. At admission the patient was agitated, with multiple superficial abrasions over scalp and forehead and swelling over the right temporal region. Remainder of the initial physical examination was normal. The admission computed tomography (CT) revealed a C6 spinous process fracture without spinal cord compression. Head CT was normal. The blood toxicology screen was positive for opiates. Patient was admitted for observation to the surgical ICU. The past medical history was significant for hypertension, depression, recent upper respiratory infection, chronic back pain and a L4-5 laminectomy. Thirty-six hours following the admission, the patient complained of “tingling” sensation and weakness in his lower extremities, progressively involving the upper extremities and difficulty breathing. The patient was stridorous, hypertensive with new onset atrial fibrillation. He was emergently intubated, and lorazepam, metoprolol, nitroprusside, and methylprednisolone were started. On repeat physical examination, he was able to open his eyes spontaneously, with a new right facial droop. The pupils were equal bilaterally. The cough and gag reflexes were weak. The patient was unable to move extremities. There was no rectal tone. The deep tendon reflexes were absent in four extremities. He was still able to communicate with his facial muscles. The patient underwent a repeat head and neck CT showing no evidence of cord compression. Magnetic resonance imaging (MRI) of the head and neck were also normal. The magnetic resonance angiography (MRA) was normal except for an occluded right vertebral artery. A lumbar puncture was performed. The Lyme and Campylobacter serologies were negative. Lumbar puncture (LP) results were abnormal (4 WBC, 4 RBC, protein 65mg/dl, glucose 105) suggestive of a non-bacterial inflammatory process. The electromyography (EMG) showed severe, generalized sensorimotor polyneuropathy with both axonal and demyelinating features. Based on the clinical findings, ascending nature, EMG and LP results, Guillain-Barre syndrome(GBS) was diagnosed. The patient underwent exchange plasmapheresis for 5 consecutive days with significant improvement and was discharged to a rehabilitation center 10 days after admission with improved motor strength in all extremities.

DISCUSSIONS:  GBS is an idiopathic acute inflammatory demyelinating polyneuropathy with progressive muscle weakness, areflexia, and with spontaneous remission. The incidence is 0.6-2.4/100.000 population. Both sexes and all ages are affected. Demyelination in GBS is believed to be immunologically mediated. It has been reported following infections (URI, GI infections, Camphylobacter, Lyme disease, H. influenza, Cytomegalovirus, HSV), vaccinations, epidural anesthesia, thrombolytic agents, and systemic diseases (e.g. Hodgkin’s, SLE, and Sarcoidosis). In our patient, the presentation was unusual; it is possible that a previous URI was the initial culprit, which caused impaired motor functions leading to the MVC, hospitalization and delayed respiratory distress. Or the trauma was the sole reason for the presentation of GBS. If trauma is the sole reason, this case is one of the few in the literature. Regardless of the cause, we want to stress the importance of having a wide spectrum of differential diagnosis in post-traumatic neurologic deficits.

CONCLUSION:  This case presents an unusual manifestation of GBS in the setting of post-traumatic neurologic deficit. The intensivists should keep this diagnosis as part of their differential diagnosis in patients with post-traumaic neurologic deficits.

DISCLOSURE:  A.R. Barakat, None.

Chest. 2004;126(4_MeetingAbstracts):940S. doi:10.1378/chest.126.4_MeetingAbstracts.940S

INTRODUCTION:  There are fewer than a hundred reported cases of lead toxicity from retained bullet fragments within the medical literature despite more than 475,000 nonfatal firearm related injuries in the US between 1993 and 1998. Symptoms may begin days to decades after the initial injury. Plumbism may go unrecognized until encephalopathy, with or without seizures, develops requiring admission to the Medical Intensive Care Unit (MICU). Increased lead absorption has been reported with intravertebral disc involvement, a large surface area of distribution, and osseous remodeling. We describe a case of plumbism complicated by encephalopathy and seizures from 20 year old retained bullet fragments with the aforementioned risk factors.

CASE PRESENTATION:  A 47 year-old female was transferred from an outside hospital with status epilepticus. Prior to initial presentation she reported abdominal pain, nausea, vomiting, and developed mental status changes for several months. She was diagnosed with Acute Intermittent Porphyria (AIP) and hemin therapy was initiated prior to transfer. Her status epilepticus required midazolam coma and mechanical ventilation. Repeat evaluation for porphyria was negative. Subsequently, basophilic stippling was noted on a peripheral smear. A serum lead level was markedly elevated at 111.1 μgm/dL (0.0 to 24.9 μgm/dL). Chelation therapy with Ethylene Diamine Tetra-Acetic Acid (EDTA) resulted in prompt improvement of neurological symptoms. The source for elemental lead was determined to be the 20 year old retained bullet fragments following a negative environmental evaluation of her home. Retained bullet fragments within the lateral left chest wall with surrounding osseous reaction were demonstrated on chest radiograph. Computerized tomography of the neck disclosed bullet fragments in close proximity to the carotid plexus. The bullet fragments within her chest wall were removed however those within her neck were inoperable. Maintaining a decreased serum lead level of 31 μgm/dL and improvement in her dysarthria, confusion, and abdominal pain required continued chelation treatments due to the retained bullet fragments within her neck.

DISCUSSIONS:  Although plumbism from retained bullet fragments is rare several risk factors for increased lead absorption from bullets including intravertebral disk penetration, a large surface area of involvement, and osseous remodeling with increased osteocyte activity have been described in the medical literature. Our patient’s initial presentation and laboratory evaluation prior to transfer led us to continue treatment for porphyria. Lack of further clinical improvement directed us to reanalyze the diagnosis of AIP. Determination of her lead level resulted in appropriate therapy with rapid improvement in symptoms. Additionally, this case also illustrated the limited availability of treatment methods for persistent lead exposure from an inoperable bullet.

CONCLUSION:  Prompt recognition of the risk factors for and symptoms of plumbism from bullet fragments are essential in expediting appropriate therapy including chelation in conjunction with possible surgical removal of the fragments especially when their clinical condition requires MICU admission.

DISCLOSURE:  F.R. Quijano, None.

Chest. 2004;126(4_MeetingAbstracts):940S-a-941S. doi:10.1378/chest.126.4_MeetingAbstracts.940S-a

INTRODUCTION:  We report a case of recurrent massive hemoptysis in a 27-year-old man with Proteus syndrome. Proteus Syndrome is a rare, sporadic, congenital disorder characterized by overgrowth of multiple tissues [1]. It is caused by the sporadic genetic mutation of a somatic gene, which distributes in a mosaic pattern. This mutation results in sporadic growth of varying body tissues occurring primarily in the postnatal period [2]. The phenotypic presentation is varied, depending on the mosaic distribution of the genetic mutation. Typically overgrowth of the skin, vasculature, skeleton, as well as other soft tissues, results in asymmetric deformities of the extremities. Only several hundred patients in the United States and Western Europe are affected with this disease, with less than 150 total reported cases in the world’s literature [2]. Previous reports of pulmonary involvement in Proteus syndrome are limited to PE resulting from DVT in Proteus patients with vascular malformations. Hemoptysis has not previously been reported in Proteus syndrome.

CASE PRESENTATION:  A 27-year-old man with Proteus syndrome presented for evaluation of massive hemoptysis. He was previously diagnosed with Proteus syndrome characterized by asymmetric growth of his lower extremities, venous ectasia of the lower extremities, lipomas, and epidermal nevi (Figure 1). Within one year prior to presentation, he had two episodes of massive hemoptysis requiring hospitalization and transfusion. These episodes occurred during periods of anticoagulation for suspected, but undocumented, PE. Prior work-up included CT scan of the chest, V/Q scan, pulmonary angiogram, and thoracic and bronchial arteriography. The diagnosis at referral was massive hemoptysis secondary to PE. On arrival, he was in his usual state of health without hemoptysis, cough, or shortness of breath. Physical exam was remarkable for phenotypic abnormalities consistent with Proteus syndrome (Figure 1). Echocardiography showed left ventricular ejection of 65%. CT of the chest with contrast (pulmonary angiogram protocol) showed asymmetric adipose growths in the chest wall, no evidence for PE, and no infiltrate or mass. Abdominal and lower extremity ultrasound was negative for acute or chronic thrombosis. Flexible fiberoptic bronchoscopy was performed (Figure 2). Bronchoscopy showed prominent submucosal vessels at RC1 with hypervascularity of the submucosa visible from RC1 through the length of bronchus intermedius. Left sided hypervascular lesions with prominent vessels were also apparent, but limited to the left upper lobe. Based on the bronchoscopic finding of neovascularization of the bronchial submucosa, it was felt this patient’s hemoptysis was secondary to bronchitis in combination with anticoagulation. With no evidence for PE and no current evidence for venous thrombosis, we decided to withhold anticoagulation.

DISCUSSIONS:  Our patient had massive hemoptysis while on anticoagulation for suspected PE. PE was suspected because of dyspnea in combination with his lower extremity venous ectasias, and previous reports of DVT/PE in Proteus syndrome. Eventually, bronchoscopic examination demonstrated neovascularization of the tracheobronchial tree with prominent submucosal vessels as the likely source for hemoptysis. Neovascularization of the bladder and life threatening hematuria has been reported with Proteus syndrome [3]. However, this is the first case report of massive hemoptysis, and the first time vascular abnormalities of the tracheobronchial tree have ever been described in Proteus syndrome.

CONCLUSION:  This case underscores the important role for bronchoscopy in evaluation of hemoptysis and adds neovascularization of the tracheobronchial tree as a known complication resulting from Proteus syndrome.

DISCLOSURE:  C.E. Daniels, None.

Chest. 2004;126(4_MeetingAbstracts):941S. doi:10.1378/chest.126.4_MeetingAbstracts.941S

INTRODUCTION:  Mediastinal benign teratomas are rare germ cell tumors most commonly found in the anterior mediastinum, and constitute about 3-12% of all mediastinal tumors. They grow slowly and are usually diagnosed incidently, when the patient is still asymptomatic. This is a rare presentation of a mediastinal mature cytstic teratoma(dermoid cyst) presenting with respiratory failure requiring intubation and mechanical ventilation.

CASE PRESENTATION:  A 29-year old white female, postpartum day nine, was transferred with a three day history of fever, cough, and increasing dyspnea. Shortly after arrival she developed respiratory failure and was intubated and placed on mechanical ventilation. Physical examination after intubation revealed decreased breath sounds in the left and basilar crackles in the right hemithorax. Routine laboratory tests showed an elevated white blood count of 19,200 with a left shift. Chest roentgenogram demonstrated a peripherally calcified rounded mass occupying a large portion of the left hemithorax, right mediastinal shift, and consolidation of the right hemithorax. Computed tomographic examination showed a 9.9cm x 12.4cm x 12.4cm peripherally calcified cystic mass occyping a majority of the left hemithorax. Several areas of fat attentuation were seen within the lesion. Also demonstrated was a shift of the mediastinum to the right, infiltrative process involving the right hemithorax, atelectasis of the right lower lobe, and a small right pleural effusion. The patient was treated for pneumonia and successfully extubated on day six. Our differential diagnosis was bronchogenic cyst, teratoma, dermoid cyst, and hamartoma. Two days after extubation a posterolateral thoracotomy was performed for histological diagnosis and removal of the cyst. Intraoperatively the cyst was located between the fissure of the left upper and lower lobe and was lateral to and compressing the heart. The cyst was opened and approximately 1.5 liters of turbid fluid was removed. The cyst was then dissected sharply off of the pericardium, pulmonary vessels, and remaining lung parenchyma. The pathology was consistent with a mature cystic teratoma (dermoid cyst) with the pleural cyst wall demonstrating mature epidermis, dermal appendages, pilosebaceous follicles, ciliated epithelia, mucous glands, and smooth muscle. The cystic contents demonstrated lamellated keratin debris and amorphous proteinaceous material consistent with dermoid cyst contents. The patient had an uneventful course after surgery.

DISCUSSIONS:  Benign teratomas, also known as teratodermoids, are rare germ cell tumors thought to originate from multipotent embryonic cells near the third branchial cleft. They are divided into 3 groups; epidermoid cysts, dermoid cysts, and teratomas based on their histological appearance. Dermoid cysts contain only ectodermal layer elements, such as skin, tooth, hair and sebaceous glands. They are usually solitary and cystic, but may be multilobulated. About 95% are found in the anterior mediastinum, and mean age at diagnosis is between twenty and thirty years, affecting men and women equally. They grow slowly and are usually diagnosed incidently. About 60% are asymptomatic at the time of diagnosis. The most common symptoms when present are chest pain, dyspnea, and cough. Calcification is one of the most distinctive characteristics of benign teratoms. In this case, the CT scan demonstrated fat density in the lesion and aided in establishing the diagnosis preoperatively.

CONCLUSION:  It is extremely rare for an adult with a mediastinal mature cystic teratoma(dermoid cyst)to present with respiratory distress requiring mechanical ventilation. These tumors are generally bengin and surgery is the treatment of choice.

DISCLOSURE:  N. Cossaart, None.

Chest. 2004;126(4_MeetingAbstracts):941S-a-942S. doi:10.1378/chest.126.4_MeetingAbstracts.941S-a

INTRODUCTION:  Zygomycosis is an opportunistic fungal infection primarily affecting immunocompromised hosts: patients with diabetes, metabolic acidosis, hematologic malignancies, iron overload states, and transplant patients. Pulmonary zygomycosis is a relatively rare entity, usually manifesting as parenchymal infiltrates or cavities, and is a diagnosis often made at autopsy. We present a case of zygomycosis isolated to the trachea, presenting as an airway emergency.

CASE PRESENTATION:  A 52 y/o man with poorly controlled diabetes mellitus presented with stridor for one day, and blood-tinged sputum associated with progressive dyspnea for 6 weeks. He had no fevers, chills, or weight loss. For the past five years, he had been taking prednisone 5 mg per day for rheumatoid arthritis. On examination, he was afebrile, stridorus, and using accessory muscles to breathe. His chest examination revealed transmitted sounds from his upper airway. Notable laboratory studies include a WBC count of 10,100, a serum bicarbonate level of 19 without an elevated anion gap, and a glucose of 239. CT of the chest showed a thickened trachea with mediastinal lymphadenopathy, without parenchymal lesions. The patient underwent emergent tracheostomy for acute upper airway obstruction. Bronchoscopy showed circumferential gray white membranes with necrotic debris extending from the fourth tracheal ring down to 2 cm above the carina (Graphic 1). Tracheal biospies revealed broad, nonseptate hyphae consistent with mucor (Graphic 2). The patient was not a candidate for surgical resection due to the extensive tracheal involvement. He underwent multiple tracheal debridements and was treated with liposomal amphotericin B. He received tight glycemic control and was titrated off prednisone. He was also treated with eleven sessions of hyperbaric oxygen, each consisting of 100% oxygen at two atmospheres for 90 minutes. Seven months after his initial presentation, the patient is off of amphotericin B, and is doing well with a tracheostomy tube which bypasses an area of tracheal stenosis resulting from tracheal fibrosis. Follow up chest CT shows improvement of his mediastinal adenopathy.

DISCUSSIONS:  Pulmonary zygomycosis has a very poor prognosis. It carries a 55 percent mortality rate with medical treatment alone, and a 27 percent mortality rate with combined surgical and medical treatment. Only two case reports of tracheal zygomycosis exist in the literature. Both patients presented in diabetic ketoacidosis. One patient was treated with tracheal resection with primary reanastamosis and survived (1), while the other died of overwhelming infection before surgery was contemplated (2). Unfortunately, our patient was not a surgical candidate due to the extensive length of trachea involved. He was given maximal medical therapy including tight glycemic control, tapering off steroids, as well as amphotericin B. Hyperbaric oxygen has been studied and used in rhinocerebral and cutaneous zygomycosis. It is believed to increase oxygen tension in tissues, to enhance cidal action of PMN’s, and to promote angiogenesis. There is no data on its utility in pulmonary zygomycosis. Due to the favorable risk benefit profile, we decided to try hyperbaric oxygen treatment on our patient. It is uncertain to what extent it has helped in our patient’s recovery.

CONCLUSION:  Tracheal zygomycosis is a rare cause of acute upper airway obstruction. This is the first report of successful treatment of tracheal zygomycosis with medical therapy alone. This is also the first reported use of hyperbaric oxygen in treating pulmonary zygomycosis.

DISCLOSURE:  V.Y. Kwan, None.

Chest. 2004;126(4_MeetingAbstracts):942S-943S. doi:10.1378/chest.126.4_MeetingAbstracts.942S

INTRODUCTION:  Gastrobronchial fistula (GBF) is an extremely rare occurrence. Several cases of GBF have been described in the English literature but none have occurred secondary to bariatric surgery. We describe a case of GBF occurring after laparoscopic gastric banding.

CASE PRESENTATION:  A 31-year-old morbidly obese female with a body mass index of 47 underwent laparoscopic gastric banding. The surgery was successful and the patient lost 100 lbs over eight months at which time she developed a left pneumothorax and pleural effusion. A tube thoracostomy was placed with 1800 ml of purulent fluid removed. Cultures contained gram-positive cocci and gram-negative rods. The patient required a left thoracotomy with decortication. Despite several courses of antibiotics she continued to complain of left sided chest pain, dyspnea, and productive cough. On physical exam, she was hemodynamically stable, afebrile, with decreased breath sounds and dullness to percussion on the left side. The remainder of the exam was normal. Computer Tomography (CT) of the chest revealed an intrathoracic stomach, consolidated left lower lobe, and air-bronchograms. Upper gastrointestinal series (UGIS) demonstrated a displaced gastric band with a large portion of gastric fundus within the left hemithorax and extravasation of contrast into the left thoracic cavity. (Fig 1) Flexible bronchoscopy revealed thick secretions in the left lower lobe and esophagogastroduodenoscopy (EGD) revealed the herniated fundus with an area of inflammation in the superior aspect. No ulcer or perforation was appreciated. The patient was taken to the operating room electively where an exploratory laparotomy and left thoracotmy were performed. A fistula was identified between the fundus of the stomach and the left lower lobe bronchus measuring 5 mm in diameter and 4 cm in length. (Fig 2) Removal of the band, repair of the stomach, diaphragm, and left lower lobectomy were performed.

DISCUSSIONS:  This is the first case report of a GBF secondary to a laparoscopic gastric band. Regardless of the etiology, GBF is a rare condition. A total of 35 cases have been reported in the literature. Postoperative complications following foregut surgery constitute the most common cause of GBF.(123) Trauma is the second leading cause.(4) Patients can present with expectoration of gastric contents, recurrent fever, hemoptysis, and pulmonary infections such as bronchitis, pneumonia, lung abscess, or bronchiectasis.(4) GBF should be suspected in any patient presenting with the above following surgery of the foregut or trauma. The investigation of choice is the esophagram or UGIS as in this case. Other diagnostic modalities include measurement of bronchial pH(2), instillation of methylene blue, bronchoscopy, EGD, and CT scan. We performed a lobectomy with complete excision of the fistulous tract.

CONCLUSION:  We have described a case of gastrobronchial fistula secondary to laparoscopic gastric banding, a type of bariatric surgery. To our knowledge, there have been no previously reported cases. With the increase in the number of laparoscopic banding procedures performed in this country this complication may occur more often and awareness of this complication should be heightened.

DISCLOSURE:  D.J. Rassias, None.

Chest. 2004;126(4_MeetingAbstracts):943S. doi:10.1378/chest.126.4_MeetingAbstracts.943S

INTRODUCTION:  Tracheal laceration is a rare but potentially devastating complication of endotracheal intubation. History of trauma or difficulty during intubation in combination with the clinical findings of hemoptysis, subcutaneous emphysema, and new stridor may lead towards the diagnosis of tracheal injury. Traditional treatment has been surgical repair, however a non-operative approach is appropriate under certain conditions.

CASE PRESENTATION:  A 19-year-old female presented to an outpatient facility for an elective abortion. During the dilation and curettage the patient received intracervical injection of .25% marcaine and 2 units of vasopressin. Following the procedure the patient became dyspneic and was given metaproterenol without relief. Progressive respiratory distress ensued with subsequent respiratory and cardiac arrest. CPR was initiated and subcutaneous epinephrine was administered for anaphylaxis. The patient was ventilated using bag-mask ventilation only. Spontaneous cardiac activity returned and the patient continued to be ventilated utilizing bag-mask ventilation. Orotracheal intubation was then attempted unsuccessfully. Oxygenation became suboptimal and a combitube was placed with improvements in oxygenation. Following transport to the emergency department, physical exam revealed diminished breath sounds on the left side. A chest x-ray confirmed right main stem intubation therefore a 7.0 endotracheal tube was placed with appropriate positioning and improvements in oxygentation. The patient was then transferred to the MICU. Physical exam revealed subcutaneous emphysema at the neck anteriorly and bilateral breath sounds. Fiberoptic evaluation was performed to evaluate for airway trauma. This revealed a 2.5 x 1.0 x 0.5 cm laceration in the posterior membranous portion of the trachea extending to the carina. Following evaluation by thoracic surgery it was determined that operative repair of the trachea was not indicated. Subsequently, the patient underwent nasopharyngoscopy as well as gastrograffin swallow, both of which were normal. Ampicillin/sulbactam was started due to a concern for mediastinitis. Bronchoscopy performed on hospital day two revealed no extension of tracheal laceration or glottic edema and the patient was extubated without complications. Over the ensuing four months the patient underwent fiberoptic bronchoscopy three additional times that confirmed complete wound healing.

DISCUSSIONS:  Tracheal laceration has traditionally been managed surgically, however non-operative management of tracheal laceration has been described. The patient should exhibit hemodynamic stability without difficulty in ventilation. Furthermore, there should be no evidence of esophageal injury, mediastinitis or progressive pneumomediastinum.1 Conditions which may actually favor non-operative management include chronic corticosteroid use, potential surgical traumitization and old age.2 Transmural lacerations or tears involving the paracarinal region have generally been surgically repaired, and surgical repair of lacerations greater than two cm has been advocated although not uniformly.3 Placement of the endotracheal tube distal to the tear appears to be agreed upon as well as early extubation, if possible. No consensus of single or double-lumen endotracheal tubes exists. Further characterization of the extent of injury is important and includes evaluation for esophageal leak and progressive pneumomediastinum. Additional recommendations include the use of liberal sedation and/or paralysis to prevent coughing and low-pressure ventilation as well as the use of prophylactic antibiotics to prevent the development of mediastinitis.

CONCLUSION:  Tracheal laceration can be managed in a non-operative manner in carefully selected cases. Systematic evaluation may allow for appropriate patient selection. Non-operative management in these cases may optimize patient outcome.

DISCLOSURE:  J.P. Gagermeier, None.

Chest. 2004;126(4_MeetingAbstracts):943S-a-944S. doi:10.1378/chest.126.4_MeetingAbstracts.943S-a

INTRODUCTION:  Early squamous cell head and neck cancer traditionally has been treated with radiotherapy and surgery. Proponents of radiotherapy believe that the quality of voice post radiotherapy surpasses that following surgery. This belief spurred development of different reconstructive techniques using various kinds of grafts.

CASE PRESENTATION:  A 73-year-old man noted episodes of coughing up hair over several weeks. He also had slowly progressive shortness of breath, daytime somnolence, fatigue, hoarseness and wheezing. His past history included laryngeal surgery 15 years ago for a benign laryngeal tumor. He gave up cigarette smoking after his laryngeal surgery. He was noticed to have an audible inspiratory wheeze at the time of admission. Vital signs were within normal limits. On examination, he had extensive hair growth of the skin all over the body including the neck area. An old midline neck scar was seen without keloid formation, discharge or sinus tract. Examination of the chest revealed transmitted inspiratory wheezing with no rhonchi or crackles. The remainder of the examination was normal. Chest X-ray was normal. Fiberoptic bronchoscopy showed abnormal anatomy due to previous laryngeal reconstructive surgery. There was extensive meshwork of hair filaments below the epiglottis obscuring the vocal cord area. Some hair filaments were as long as 6 cm and were covered with mucus. Bronchial washing was negative for malignant cells. The patient did not return for follow up.

DISCUSSIONS:  This patient illustrates trichoptysis (coughing up hair), a complication of reconstructive surgery for laryngeal tumors. Many patients who undergo flap reconstruction require postoperative external beam radiation therapy, a technique to reduce ectopic hair growth. Our patient underwent reconstructive surgery with a mucosal flap and did not receive radiation therapy. This problem may be obviated by utilizing partial thickness skin graft (without hair follicles). Once the problem develops, hair removal by laser or epilation may be necessary. Management of this problem includes hair removal by laser or epilation therapy.

CONCLUSION:  Trichoptysis is seen rarely nowadays due to improved techniques of reducing hair growth in flaps and grafts for reconstructive surgery. The diagnosis is readily established by bronchoscopy. Hair fulguration techniques are usually successful in treating this condition.

DISCLOSURE:  M.B. Bakry, None.

Topics: hoarseness
Chest. 2004;126(4_MeetingAbstracts):944S-945S. doi:10.1378/chest.126.4_MeetingAbstracts.944S

INTRODUCTION:  Inhibition of tumor necrosis factor-alpha (TNF-alpha) with either infliximab or etanercept has been beneficial in multiple diseases. Infliximab is a TN-alpha antibody approved by the FDA for rheumatoid arthritis and Crohn’s disease. Etanercept is a soluble TNF-alpha receptor approved for rheumatoid arthritis, psoriatic arthritis, and ankylosing spondylitis.

CASE PRESENTATION:  A seventy-two year old male presented with dyspnea, purulent sputum, night sweats, and weight loss. He was taking etanercept and leflunomide for rheumatoid arthritis, and etanercept had been taken for at least three years. Physical examination was normal except for multiple nodules and joint deformities. Chest computed tomography performed that morning as part of a lung cancer screening trial revealed a seven by eight centimeter thick-walled cavity in the superior segment of the left lower lobe(figure 1). The differential diagnosis included lung abscess, tuberculosis, and bronchogenic carcinoma, and the patient was empirically treated with levofloxacin and clindamycin. Etanercept and leflunomide were discontinued, and sputum samples for AFB were ordered. On hospital day #4 he developed massive hemoptysis and required mechanical ventilation. Bronchoscopy revealed bright red blood originating from the superior segment of the left lower lobe. Soon after completion of the bronchoscopy, it was revealed that there were many acid fast bacilli seen on direct smear of sputum obtained the previous day. Isoniazid, rifampin, ethambutol, and pyrazinamide were started. Over the following 3 days, the patient had fevers up to 41.3 degrees celcius, and he became hypotensive on day #7. Despite being treated with vasopressors, the patient died that evening. Subsequent microbiologic identification revealed many mycobacterium avium complex (MAC) from multiple respiratory cultures, and no other pathogenic organisms were identified from cultures of blood, urine, and sputum.

DISCUSSIONS:  TNF-alpha plays an important role in the response to intracellular pathogens such as mycobacteria, candida, and listeria. Since the introduction of infliximab and etanercept, there have been at least 82 cases of tuberculosis associated with infliximab; 11 cases of tuberculosis with etanercept; and 6 cases of MAC, one of M.kansasii, and one of M.marinum reported with etanercept(1). The details of etanercept related NTM infections are not readily available as there are no individual case reports published in the medline-referenced literature. Our patient was also taking leflunomide, and we cannot conclude that this medication did not contribute to the MAC infection. Leflunomide suppresses T-cell and B-cell proliferation and inhibits the proliferation of smooth muscle cells. Pseudomonal lung abscess has been described in a patient receiving both leflunomide and infliximab (2), but neither tuberculous nor nontuberculous mycobacterial infection has been described with leflunomide alone. Unfortunately in our case, mycobacterial blood cultures were not obtained, and the autopsy was declined, so we do not know with certainty if the development of septic shock was related to disseminated MAC. Nevertheless, the hemoptysis was clearly related to the lung cavity, and multiple smear positive respiratory specimens are convincing evidence that the cavity represents true NTM pulmonary disease. The resulting respiratory failure was clearly a contributing factor towards the patient’s death.

CONCLUSION:  Anti-TNF therapy has been implicated as a risk factor for tuberculosis, but the risk of serious NTM infection is rarely contemplated. Our case suggests that anti-TNF therapy may contribute to MAC infection. Complications of MAC, including hempotysis and dissemination, can be life-threatening. Currently, many physicians screen patients for latent tuberculosis prior to the initiation of anti-TNF therapy, but screening for NTM is not routinely performed. Obtaining sputum and screening for NTM may help identify patients at risk of developing NTM disease during treatment with TNF-alpha inhibitors. In addition, NTM should be considered in the differential diagnosis of lung cavity, and early empiric treatment may potentially be beneficial.

DISCLOSURE:  M. Carrillo, None.

Chest. 2004;126(4_MeetingAbstracts):945S. doi:10.1378/chest.126.4_MeetingAbstracts.945S

INTRODUCTION:  We report a case of tubular adenoma of the colon presenting as empyema necessitatis of the left chest wall.

CASE PRESENTATION:  A 44 year old male presented with left sided anterior chest pain of one month duration. Associated symptoms included nocturnal dry cough and tenderness to touch. Symptoms were relieved with ibuprofen. He denied fever, chills, dyspnea, weight loss, abdominal pain, nausea, vomiting, change in bowel habits, constipation or history of trauma. Past medical history was unremarkable, but he abused alcohol and smoked 2 cigarettes/day. He denied illicit drug use. Medications included ibuprofen as needed for pain relief. On examination, he was afebrile, blood pressure 97/73 mmHg, heart rate 89 beats/minute and respiratory rate 18 breaths/minute. He appeared well without discomfort. His oropharynx was remarkable for poor dentition. Chest examination revealed an area of tenderness in the left midaxillary lower chest wall, 10 centimeter (cm) in diameter and raised 1-2 cm at the highest point. There was possible fluctuance but no crepitus. Lung examination was unremarkable. He had a non-distented soft abdomen with normal bowel sounds and no tenderness. The remainder of his exam was unremarkable. Laboratory data indicated a normal leukocyte count and differential but revealed a microcytic anemia with hematocrit 26% and mean corpuscular volume of 64 fl. Chest radiograph revealed left pleural effusion and consolidation of the lingula. Selected images from a follow up contrast-enhanced computerized tomography are shown below. He was diagnosed with pneumonia and empyema necessitatis and started on antibiotic regimen to cover for actinomyces, nocardia, streptococcus and anaerobic infections. Sputum did not reveal acid fast bacilli on 3 occasions. He underwent thoracentesis that drained air but no fluid. Surgical drainage removed 2 liters of pus and revealed tracking down to the abdomen with large abscess cavity adjacent to the transverse colon. The abdomen was felt to be the source of the infection. Cultures grew Escherichia coli and Staphylococcus aureus. A gastrografin enema was obtained and revealed a small fistula in the transverse colon extending to the chest wall abscess cavity. Flexible sigmoidoscopy showed a non-obstructing 2 cm sized mass in the left transverse colon at 60 cm. Clinical impression was of a locally advanced colonic adenocarcinoma, and he underwent surgical resection. The pathological diagnosis of the mass indicated a tubular adenoma and no evidence of inflammatory bowel disease. The patient is doing well 6 months after discharge.

DISCUSSIONS:  Empyema necessitatis is a rare entity in the antibiotic era and is usually caused by actinomycoses, mycobacterial tuberculosis or streptococcus. It has rarely been reported to extend to the retroperitoneal space or cause mastitis. Our case involves the reverse pathway: from the abdominal cavity to the pleural space, lingula and chest wall. To our knowledge, this is the first case report involving an intraperitoneal infection which migrated into the pleural space then into the lung and chest wall. The scout film of the CT scan (not illustrated) demonstrates a collection of air in the abdominal wall and examination of select mediastinal views indicate the infection communicating between the pleural space and intraperitoneal cavity.

CONCLUSION:  We report a infectious process initiated in the abdominal cavity and secondarily crossing into the pleural space and chest wall.

DISCLOSURE:  T.E. Duggan, None.

Chest. 2004;126(4_MeetingAbstracts):945S-a-946S. doi:10.1378/chest.126.2.518

INTRODUCTION:  Pulmonary sporotrichosis is an uncommon pulmonary infection caused by the thermally dimorphic fungus Sporothrix schenckii. We report a case of “primary” pulmonary sporotrichosis diagnosed by bronchoalveolar lavage (BAL). The patient had no dermatologic manifestations.

CASE PRESENTATION:  A 55 year old housewife was referred for evaluation of worsening left lower lobe alveolar infiltrates. She complained of low grade fever, frequent sweats, nasal congestion, ongoing cough, and shortness of breath of six months duration. Her past medical history was significant for asthma, hypothyroidism, nephrolithiasis, and inflammatory bowel disease. She quit smoking eight years previously and only consumed alcohol occasionally. She had traveled to Europe, China and Caribbean in the past year. She reported a hobby of building English hay racks with wet moss and planting flowers in them. Her examination was normal except for wheezing in the left lower lung field. There were no skin lesions or lymphadenopathy. A CT scan revealed patchy alveolar infiltrates in the right lung with bronchiectatic changes and new nodules in the left lower lobe and lingula. (figure 1.) Her basic laboratory studies were normal and a PPD was negative. She underwent bronchoscopy where transbronchial biopsy and BAL were performed. Culture of the BAL specimen grew a mould identified as Sporothrix schenckii (figure 2).

DISCUSSIONS:  Sporotrichosis is caused by the thermally dimorphic fungus Sporothrix schenckii, the yeast form of which infects human tissues. S. schenckii is found worldwide in decaying vegetation such as rotting wood, sphagnum moss, rose thorns and rich humus soil. Sporotrichosis is an occupational illness of gardeners, forestry workers and others who receive minor trauma from contaminated thorns, branches and wood splinters. Infections occur sporadically and are associated with trauma during outdoor work.1 The lymphocutaneous form of sporotrichosis is the most common, but pulmonary, osteoarticular, meningeal and disseminated forms have been described.2 The pulmonary form can occur as primary infection when S. schenckii conidia, aerosolized from soil or decaying vegetation, are inhaled.3 Pulmonary sporotrichosis usually presents as chronic cavitary fibronodular disease involving the upper lobes. The diagnosis is usually made accidentally when Sporothrix is cultured from the sputum of a patient suspected of having tuberculosis or chronic cavitary histoplasmosis.4 The clinical syndrome mimics tuberculosis with cough, dyspnea, low grade fever, night sweats, and weight loss. Radiologically, pulmonary sporotrichosis may present as cavitary disease or tracheo-bronchial lymphadenopathy.5 Diagnosis is usually achieved by sputum culture or bronchoscopic biopsy revealing granulomatous pneumonia. Itraconazole is the recommended therapy for patients with non-life-threatening pulmonary sporotrichosis, while amphotericin B is indicated for life-threatening or extensive pulmonary lesions. Surgical removal of the infected tissue along with antibiotic therapy appears to be the most effective treatment.5

CONCLUSION:  Pulmonary sporotrichosis is an unusual infection which may mimic tuberculosis or histoplasmosis. Diagnosis is achieved either by culture of the organism or by transbronchial biopsy. Consideration to the diagnosis of pulmonary sporothichosis should be given in patients with cavitary parenchymal disease or unexplained intrathoracic lymphadenopathy, particularly those with a history of environmental exposure.

DISCLOSURE:  M.M. Budev, None.

Topics: lung , sporotrichosis
Chest. 2004;126(4_MeetingAbstracts):946S-947S. doi:10.1378/chest.126.4_MeetingAbstracts.946S

INTRODUCTION:  Cryptococcal infections pose a clinical challenge in immunocompromised patients. Following the central nervous system, the respiratory system is most commonly affected. Pulmonary involvement includes localized nodular lesions with or without cavitation, segmental pneumonic infiltrates, patchy interstitial or alveolar infiltrates, pleural effusions, hilar masses, and thoracic lymphadenopathy. Clinical symptoms are cough, dyspnea, fever, night sweats, weight loss, and hemoptysis. Herein we describe our experience with an unusual case of endobronchial cryptococcosis.

CASE PRESENTATION:  A 72-year-old man was hospitalized following a syncopal episode related to sustained ventricular tachycardia. Coronary angiography disclosed global myocardial dysfunction, an ejection fraction of 20%, and normal coronary arteries. Myocardial biopsy revealed giant cell myocarditis. He was treated with intravenous methylprednisolone cyclosporine and azathioprine. One week after admission workup of sudden onset dyspnea revealed the diagnosis of pulmonary embolism and therapeutic anticoagulation was initiated. Four weeks into immunosuppressive therapy repeat myocardial biopsy and echocardiography showed improvement of his giant cell myocarditis. Five weeks after admission pulmonary consultation was requested to evaluate fever, worsening dyspnea, wheezing, and moderate hemoptysis. Past medical history included adult onset asthma, chronic lymphocytic leukemia, celiac disease, asbestos related pleural disease and hypothyroidism. Physical examination showed only mildly decreased breath sounds and basilar crackles bilaterally. His immunosuppressive regimen at this time included oral cyclosporine and prednisone, 5 mg per day. Other medications were warfarin, trimethoprim/sulfamethoxazole, ipratropium, albuterol, fluticasone/salmeterol, lisinopril, levothyroxine, and lansoprazole. Laboratory tests showed: hemoglobin of 10.5 g/dl, WBC 41.000/microl, platelet count 134.000/microl, INR 2.6, PTT 32 s and creatinine 1.7 mg/dl. Blood cultures were negative and chest CT revealed mild nodularity around the major fissures but was otherwise unchanged. Bronchoscopy to investigate for the cause of hemoptysis and infectious pathogens disclosed extensive pseudomembranes covering the posterior wall of the distal trachea extending into the left and right mainstem bronchi to the takeoff of the upper lobe bronchi bilaterally. Upon removal friable, nodular mucosa with shallow ulcerations was revealed Figure 1. Endobronchial biopsy and bronchoalveolar lavage showed benign bronchial tissue with fungal organisms consistent with cryptococcus. Figure 1 Cultures of lavage fluid grew Cryptococcus neoformans. The diagnosis of endobronchial cryptococcosis was established. Serum cryptococcal antigen was elevated with a titer of 1:4. Cerebrospinal fluid examination including cryptococcal antigen was negative. Liposomal amphotericin was initiated but was changed to fluconazole, 400 mg per day because of drug intolerance. Antifungal therapy was continued at this dose for 3 months followed by longterm maintenance therapy with Fluconazole 200 mg per day.

DISCUSSIONS:  Endobronchial involvement is a rare manifestation of pulmonary cryptococcosis. It can occur in immunocompromised and immunocompetent individuals. In addition to our patient 7 cases have been reported in the literature. Clinical symptoms are frequently suggestive of airway disease. Radiographic findings commonly show postobstructive atelectasis but are usually non-specific. Bronchoscopic evaluation frequently shows endobronchial masses but endobronchial involvement can also manifest as pseudomembranous tracheobronchitis similar to that described in association with aspergillus infection. Considering the high prevalence of documented CNS involvement in patients with endobronchial cryptococcosis [123456] their evaluation should include a lumbar puncture.

CONCLUSION:  Endobronchial cryptococcosis needs to be considered in the differential diagnosis of immunocompromised patients with airway related symptoms or hemoptysis. Bronchoscopy is a valuable tool to detect endobronchial disease and establish the diagnosis. Because of the low incidence of endobronchial cryptococcosis, no prospective data is available to guide management. Most authors suggest aggressive therapy with either amphotericin B or fluconazole. [1234567].

DISCLOSURE:  T. Peikert, None.

Topics: cryptococcosis
Chest. 2004;126(4_MeetingAbstracts):947S. doi:10.1378/chest.126.4_MeetingAbstracts.947S

INTRODUCTION:  Acanthamoeba is a protozoan producing keratitis in the immunocompetent host and disseminated infection with encephalitis in immunocompromised individuals. Diagnosis and treatment of acanthamoeba infections are difficult given the rarity of the infection. We describe a case of disseminated acanthamoebiasis in a previously healthy bilateral lung transplant recipient.

CASE PRESENTATION:  A 60 year-old bilateral lung transplant recipient presented with multiple, non-tender skin nodules over the trunk and extremities. Four weeks prior to presentation, he experienced a laceration on his hand while retrieving a catfish and subsequently developed a subcutaneous nodule on his upper arm. Over the next three weeks, eight other nodules appeared over trunk and extremities. He denied fever, dyspnea, cough or headaches. Prior to this illness, he had experienced an uncomplicated post-transplant course. Medications included tacrolimus, prednisone, mycophenolate and itraconazole. Initial examination revealed a nontoxic-appearing gentleman with firm, nontender, subcutaneous nodules measuring 1-3 cm over the trunk and extremities. A skin punch biopsy was performed revealing granulomatous inflammation and no organisms. Antibacterial agents were initiated for a presumed bacterial skin infection. A complete blood count, tissue cultures, echocardiography and chest radiographs were nondiagnostic. On hospital day four, he developed a focal seizure involving right arm and leg. Magnetic resonance imaging demonstrated areas of increased signal intensity in the frontal and parietal lobes. A lumbar puncture revealed a low glucose, 5 white blood cells and no organisms. On day # 7, he became febrile and developed respiratory failure requiring mechanical ventilation. Bronchoscopy demonstrated intact anastomoses with scant secretions. Bronchoalveolar fluid revealed no organisms. Antibacterial therapy was broadened and antifungal agents prescribed. Several skin lesions developed an eschar and a wide excisional skin biopsy revealed coagulative necrosis. He remained febrile, developed multiorgan failure and expired on hospital day 17. Post mortem examination revealed ulcerative skin lesions without purulence distributed on the trunk and extremities. Gross examination of the lungs revealed multiple nodules and inspection of the brain demonstrated hemorrhagic infarcts. Microscopic examination of lung and brain tissue revealed trophozoites and cysts identified as Acanthamoeba. Meticulous microscopic examination of the skin sections demonstrated Acanthamoeba trophozoites.

DISCUSSIONS:  Disseminated acanthamoebiasis is characterized by granulomatous infiltration of the skin and brain. The route of transmission appears to be hematogenous after introduction through the skin or airways. Acanthamoeba infection has been described in transplant recipients and HIV positive patients with skin lesions being a frequent initial manifestation. Skin lesions appear as papules, nodules or non-healing ulcers varying 0.5 to 3 cm in size. Microscopic examination characteristically reveals granulomatous inflammation that may also be seen with tuberculosis, tertiary syphilis, sporotrichosis, leprosy and coccidiomycosis. However, granuloma formation may be absent in severely immunocompromised patients. Trophozoites may be mislabeled as large histiocytes and Calcofluor stain allows correct identification. Identification of the cyst form is possible with Gomori Methamine Silver (GMS) staining cysts black and Periodic acid-Schiff (PAS) staining cysts red. Combination therapy with pentamidine, ketoconazole, sulfadiazine, 5-fluorocytosine has been reported, but a standard regimen remains elusive.

CONCLUSION:  Acanthamoebiasis is a rare, infectious complication following transplantation. Transplant recipients with a syndrome of cutaneous lesions and meningoencephalitis should be evaluated for disseminated acanthamoebiasis. Deep skin biopsies using PAS, GMS and Calcofluor stains improve identification. Early recognition and treatment with combination therapy is required for successful treatment.

DISCLOSURE:  F. Sattar, None.

Chest. 2004;126(4_MeetingAbstracts):947S-a-948S. doi:10.1378/chest.126.4_MeetingAbstracts.947S-a

INTRODUCTION:  Botulism can occur naturally as a food-borne illness caused by ingestion of pre-formed botulinum toxin, typically types A, B or E from Clostridium botulinum. Clostridium baratii as a causitive agent of human botulism has been reported only rarely in the medical literature(1). We report an unusual case of botulism caused by type F toxin produced by C. baratii.

CASE PRESENTATION:  A 26 year old, previously healthy white man awoke the morning of admission with a complaint of diplopia, then developed shortness of breath and mild dysarthria. He was transferred to our facility after intubation for hypercapneic respiratory failure at another institution. The patient arrived awake and able to follow commands. He remained intubated with no respiratory effort over the ventilator. Physical exam revealed bilateral opthalmoplegia, with bilateral abducens palsies, loss of upgaze, non-reactive 6mm pupils, mild ptosis and weakness of eye closure. Biceps and triceps strength were decreased to 4/5, but all other muscle testing was normal. Sensory and reflex examinations were normal. Admitting laboratories and brain imaging by computed tomography and magnetic resonance were normal. Lumbar puncture revealed elevated protein at 50mg/dL but no other abnormalities. Nerve conduction studies with electromyography found markedly decreased muscle compound action potentials in all muscle groups with normal sensory action potentials and normal repetitive stimulation. The differential diagnosis included Botulism and Miller-Fisher Syndrome. The patient was treated with botulinum anti-toxin against toxins A,B and E. Additionally he received 5 days of IVIG. Stool and serum samples were sent for botulism toxin assay and anti-ganglioside GQ1B antibody. The patient progressed to bilateral upper extremity flacid paralysis but began to recover extremity motor function on day 4. His course was complicated by acute renal failure secondary to osmotic nephrosis from IVIG and a prolonged ileus. He was extubated on hospital day 9 and discharged home on day 20. Botulism toxin assay was positive for type F toxin. Culture suggested C. baratii. Confirmatory testing by the CDC is pending. Anti-GQ1B antibody tests were negative.

DISCUSSIONS:  Botulism is caused by neurotoxins produced by the organisms C. botulinum, C. baratii or C. butyricum against proteins involved in synaptic vesicle release. Food-borne illness from botulinum toxin is rare, with only 33 reported cases in the United States in 2001(2). Food-borne illness from C. baratii type F toxin is even more unusual, with only 4 reported cases(1). Compared with classic Botulism from C. botulinum type A toxin, type F toxin may produce a much shorter clinical course, approximately 7 days, as noted in our patient and in previously published case reports(1). This difference may be due to the differential rates of replacement of the cleaved neuronal proteins. C. baratii type F toxin cleaves synaptobrevin, while type A toxin cleaves synaptosomal-associated protein of 25 kilodaltons (SNAP-25). Both proteins participate in synaptic vesicle release. In experimental rat cerebellar neuron preparations, synopatobrevin function is restored within 7 days while restoration of SNAP-25 function requires greater than 30 days(3). Finally, anti-toxin against type E toxin may provide benefit in type F toxin disease due to toxin heavy-chain sequence homology(1).

CONCLUSION:  This patient developed Botulism secondary to a rare toxin. The unique characteristics of type F toxin and its site of action provide insight into the clinical course of this patient.

DISCLOSURE:  D.S. Hagg, None.

Chest. 2004;126(4_MeetingAbstracts):948S. doi:10.1378/chest.126.4_MeetingAbstracts.948S

INTRODUCTION:  Embriologic abnormalities often occur in the lung. We present a case of an unusual intrathoracic mass.

CASE PRESENTATION:  A 34 year-old man with no significant past medical history presented to the emergency room with dyspnea that started three days before. He had fever, chills and a cough productive of yellow sputum. There was no history of chest or abdominal trauma. The physical examination revealed a 34 year old man in no acute distress, having low grade fever (100.8F). The chest examination showed no signs of consolidation or wheezing. The abdominal exam didn’t reveal any organomegaly or hernia. The rest of the examination as well as the CBC and chemistry were within normal limits. The presumptive diagnosis was pneumonia. A chest roentgenogram revealed a right lower lobe well defined mass contiguous to the right hemi-diaphragm. (fig.1). Computer tomogram with intravenous contrast was performed. (fig.2) The patient was treated with oral antibiotics for 5 days after he was discharged from the emergency department. He was seen in the pulmonary clinic for a follow up two weeks at which time he was asymptomatic.

DISCUSSIONS:  The computer tomogram of the chest showed a right intrathoracic ectopic kidney. The contrast substance was taken up and excreted by the ectopic kidney. The kidney appeared to be functional with no signs of obstructive uropathy. During fetal life the kidneys form from the metanephros in the pelvis. The hilum of the new kidney faces anteriorly. During the fifth or sixth week of gestation the kidneys begin to migrate caudally and rotate so that by week 9 the kidney are usually at the L1 level and the hilum are now facing antero-medially. An abnormally high ascent of the metanephros will generate a diaphragmatic defect and subsequently an ectopic kidney in the thorax. If the kidney does not ascend at all a pelvic or horseshoe kidney results. Intrathoracic ectopic kidneys may be congenital or acquired. This condition is rarely bilateral and occurs mostly on the left side. There is a marked preponderance in males (4,5). True intrathoracic ectopic kidney presents during fetal life and has four characteristics: 1) rotation anomaly, 2) long ureter, 3) anomalous high derivation of the renal vessels from the thoracic aorta, 4) medial deviation of the lower pole of the kidney (4). The etiology of true ectopic kidney is not well known. Acquired intrathoracic ectopic kidney occurs due to a diaphragmatic hernia that may be caused by a congenital diaphragmatic defect, in which case the vascular pedicle has a normal morphology. It can also be caused by trauma. In most cases it is asymptomatic. The diagnosis of intrathoracic kidney is one of the unusual causes of mediastinal mass on chest roentgenogram and is confirmed by intravenous urography (1,3), or computer tomography (1). Based on literature review, the incidence of intrathoracic ectopic kidney is very low. Almost 60 cases have been reported. Apart from its rare complications such as respiratory distress in newborn babies, this anomaly does not require any specific treatment (2).

CONCLUSION:  Although this is a rare condition, the presence of an intrathoracic mass needs to be carefully evaluated before performing invasive diagnostic procedures.

DISCLOSURE:  m.e. iliescu, None.

Chest. 2004;126(4_MeetingAbstracts):948S-a-949S. doi:10.1378/chest.126.4_MeetingAbstracts.948S-a

INTRODUCTION:  Solitary pulmonary papillomas are rare tumors in adults. The glandular papilloma is the rarest subtype. We describe a case of solitary glandular pulmonary papilloma and review the current literature in this disease.

CASE PRESENTATION:  A 65-year-old Caucasian woman was found to have a 9 mm lesion in the right lower lobe by a commercial CT screening examination 1.5 years prior. The patient deferred a short-term follow-up and/or diagnostic evaluation. Now, a chest CT demonstrated a 16 mm nodule in the right lower lobe with irregular margins. She was asymptomatic. The patient had a remote cumulative 25 pack-year smoking history. Her physical examination and laboratory parameters were unrevealing. A positron emission tomography showed an isolated focus of increased FDG activity (SUR 4.8) corresponding to the location of the nodule on the chest CT. A bronchogenic carcinoma was strongly suspected. Therefore, the patient underwent a resection of the nodule with an intraoperative histologic examination, which showed glandular pulmonary papilloma without evidence of malignancy.

DISCUSSIONS:  In adults a solitary pulmonary papilloma represents a rare benign neoplasm accounting for only 0.38% of all lung tumors and 7-8% of all benign lung tumors. Unlike multiple papillomatosis seen in young people, frequently in children, the solitary pulmonary papilloma commonly appears in the 5th and 6th decades of life. The clinical manifestations are: hemoptysis, dyspnea, wheezing, post-obstructive pneumonia, or clinically asymptomatic radiographic abnormalities. Three histologic types have been recognized: squamous papillomas, glandular papillomas, and mixed squamous and glandular papillomas. Squamous papillomas and mixed papillomas are predominantly seen in male smokers. Squamous papillomas are the most common type of solitary pulmonary papillomas and are strongly associated with human papilloma virus. Squamous papillomas demonstrate a high rate of malignant transformation ranging from 8% to 40%. The risk of transformation increases with cigarette smoking, age greater than 40, and infections with HPV serotype 16 or 18. The glandular papilloma, as seen in our patient, represents a very rare subtype of pulmonary papillomas. Only 13 cases have been reported in English literature from 1954 to the present. Eight were found in males and 5 in females. Eleven of the 13 cases presented with endobronchial disease and associated clinical symptoms. Only 1 patient presented as an incidental peripheral pulmonary nodule. As opposed to the squamous and the mixed papillomas the glandular papilloma tends to occur in non-smokers and in an older patient population. In contrast to the squamous papillomas, there have been no reports of malignant transformation of glandular papillomas though the number of reported cases remains limited. No recurrence has been reported after either lobectomy (6 patients) or bronchoscopic removal (4 patients).

CONCLUSION:  Solitary pulmonary papillomas are rare benign neoplasms. Among the papillomas the glandular subtype is the rarest. Patients usually present with endobronchial lesions; a peripheral location, as seen in our case, is uncommon. In contrast to squamous papillomas, malignant transformation has not been reported for glandular papillomas. Endoscopic ablation for endobronchial locations and limited surgical resection of peripheral lesions seem to be justified based on the current evidence in the medical literature. Table:

Patient characteristics, symptoms, histology, treatment and follow-up information on reported cases of glandular papilloma including our case report

AuthorAgeSexSmokeLocationSymptomsEpithelial cell typeTreatmentFollow upAshmore851F-RLLHemoptysisCiliated columnar--Ashley962MYesRLLPneumoniaCiliated columnarBronchoscopic removalNo recurrence after 18moSpencer1060M-LLL-Cuboidal multilayered nonsquamousLobectomy-59M-RLL-Cuboidal multilayered nonsquamousBronchoscopic removalNo recurrence after 2.5 yrs-----Ciliated cuboidal--26F-LUL-Columnar+ cuboidalLobectomy-Roviaro757MYesRLLRecurrent pneumoniaCiliated columnar/cuboidalLobectomy-Basheda1174MYesRLLHemoptysisColumnar-Lost to follow upFlieder374MNoLULHemoptysisColumnarLobectomyNo recurrence after 1.5 yrs65FNoR mainWheezing, Chest tightnessCiliated columnarBronchoscopic removalDied with no evidence of disease after 5yrs69FNoLULHemoptysisCiliated columnarLobectomyNo recurrence after 11yrs68MNoLULIncidentalColumnarLobectomyNo recurrence after 4yrsSari1247MNoRLLHemoptysisMultilayered columnarBronchoscopic removalNo recurrence after 12moOur case65FYesRLLIncidentalCiliated multilayered columnarWedge resection-

DISCLOSURE:  R. Tanaka, None.

Chest. 2004;126(4_MeetingAbstracts):949S-950S. doi:10.1378/chest.126.4_MeetingAbstracts.949S

INTRODUCTION:  Pulmonary complications following bone marrow transplantation are common - ranging from infectious pneumonias to venous occlusive disease. Determining the correct diagnosis depends on integrating clinical data, tissue pathology, and radiographic patterns.

CASE PRESENTATION:  A twenty-nine year old African American male presented to the emergency room 45 days after receiving an autologous bone marrow transplant (BMT) for refractory non Hodgkin’s lymphoma; he had received BCNU, etoposide, cytarabine and melphalan (BEAM) chemotherapy prior to transplant. His complaints centered around dyspnea on minimal exertion progressing to dyspnea at rest over a one week time period; he denied cough, sputum production, or dyspnea with change in position. His vitals were remarkable for tachypnea with a rate in the thirties, temperature of ninety-nine degrees Fahrenheit, pulse rate of one hundred thirty, and requirement of a non-rebreather to keep his oxygen saturations above ninety percent. Pulmonary exam was remarkable for inspiratory crackles at the bases bilaterally and velcro rales of the apices bilaterally. The rest of the physical exam was notable for a high work of breathing, skin changes from prior radiation to his neck/mantle area, and for the absence of edema or jugular venous distention. The patient was admitted to the intensive care unit where bronchoscopy was performed and broad spectrum antibiotics, antivirals and antifungals were started. Bronchoscopy was remarkable for clear return from multiple bronchoalveolar lavages. Samples were sent for multiple studies including cytology and cultures (viral, acid fast, fungal, and bacterial.) The patient was intubated for respiratory fatigue on the second hospital day and afterwards a contrast chest CT was performed which demonstrated multilobar infiltrates and evidence of radiation fibrosis in the apices. A wedge biopsy from the lingula was performed on the third hospital day;pathology returned as diffuse alveolar damage. Cultures and cytology from the lung biopsy were also negative for infectious pathogens or malignant cells.

DISCUSSIONS:  Pulmonary infiltrates after BMT are usually divided into those that occur in the first hundred days post transplant, and those that occur beyond that time frame. They are further divided into infectious and non-infectious etiologies. Infectious pulmonary infiltrates result from a multitude of causes including bacteria, viruses (especially respiratory syncytial virus, herpes simplex, and cytomegalovirus), fungi, and pneumocystis. Non-infectious causes include pulmonary edema, diffuse alveolar hemorrhage, radiation pneumonitis, mediastinal emphysema, cytolytic thrombi, acute graft versus host disease, and idiopathic pneumonia syndrome (IPS.) The patient’s lung biopsy was only remarkable for diffuse alveolar damage - no infections, no venoocclusive changes, no signs of malignancy. Our patient had two negative cultures from his lungs - from bronchoscopy and open lung biopsy. He had not received any radiation therapy in conjunction with his bone marrow transplant. Idiopathic pneumonia syndrome was formally defined by a 1993 NHLBI forum as diffuse lung injury (evident by CT and/or altered pulmonary physiology) occurring after BMT for which an infectious etiology is not identified. The patient described met these criteria. Treatment with steroids at 2mg/kg/day was started after the pathology returned and cultures from the lung biopsy remained negative for over three days. Improvements in the patient’s chest radiograph and in his pO2 to fiO2 ratio were dramatic.

CONCLUSION:  The diagnosis of idiopathic pneumonia syndrome confers a poor prognosis - the one-year mortality is over eighty percent. The mechanism of injury also continues to be poorly understood but is thought to be the result of multiple insults - pretreatment chemotherapy, T lymphocyte activation, and oxidative stresses. Treatment with high dose steroids is still regarded as the standard of care by many experts - although the benefit from the treatment is controversial. Investigational treatments with tumor necrosis factor blockers and antioxidants are currently being studied.

DISCLOSURE:  C.E. Grossman, None.

Chest. 2004;126(4_MeetingAbstracts):950S-951S. doi:10.1378/chest.126.4_MeetingAbstracts.950S

INTRODUCTION:  Pulmonary hyalinizing granuloma (PHG) is a rare fibro-sclerosing inflammatory lung tumor of unknown etiology. Patient presentation varies from asymptomatic to vague chest-related symptoms. Most of the lesions are incidental radiological findings. The nature of PHG presents diagnostic difficulties for clinicians.

CASE PRESENTATION:  A 75 year-old man with past medical history of smoking and treated tuberculosis presented with failure to thrive. He complained of generalized weakness, cough associated with yellow sputum as well as forty-seven pounds weight loss over the last year. The physical examination revealed a cachectic male in no acute distress with normal vital signs. The chest examination showed decreased bilateral air entry at the upper lung fields. A chest roentgenogram revealed bilateral opacification of both upper lung zones (fig. 1). Computer tomogram was performed (fig. 1) and showed no changes compared with the previous CT obtained nine months earlier. Fiberoptic bronchoscopy revealed no endobronchial lesions and the transbronchial biopsy as well as the bronchioalveolar lavage were negative for malignancy or active tuberculosis. CT-guided biopsy was performed and three core specimens from upper lobes were obtained (fig 2). The biopsies showed a serpentine array of dense hyalinized collagen bundles accompanied by few lymphocytes and plasma cells. The histological picture of lamellar fibrosis with chronic inflammation favored PHG.

DISCUSSIONS:  PHG is a rare pathology that presents most commonly as slowly enlarging nodules in lung parenchyma. PHG was first described in 1977 to define an unusual pulmonary tumor characterized by numerous regularly arranged lamellae of hyalinized collagen (1). This condition is uncommon and no more than 70 cases have been reported so far (1,2,3). PHG affects adults in the age range of 24 to 77 years without sex predilection (3). The etiology and pathogenesis remain unclear. However, it has been suggested that the nodules represent an exaggerated host reaction to a number of agents, such as Histoplasma capsulatum, Tuberculosis or Aspergillus (1,2,3). Many patients are asymptomatic, however, cough, dyspnea, chest pain, and hemoptysis may occur. PHG usually appears as bilateral nodular lesions 2 to 4 cm in diameter, simulating metastatic tumors. Large lesions with or without cavitation have been reported as well (1,2,6). The nodules are well demarcated and often “shell out” easily from surrounding lung parenchyma. Histologically, they consist of numerous inter-connecting lamellae of homogeneous, hyalinized collagen. Plasma cells, occasional lymphocytes and multinucleated giant cells are present in small numbers between the lamellae and adjacent to blood vessels. There is no effective treatment for this condition. Surgery was the most effective treatment for most patients with single PHG’s (3). Two recent case reports described successful treatment with corticosteroids (4,5).

CONCLUSION:  PHG is a rare explanation for pulmonary nodules. Our case stands out due to larger size and single bilateral dense apical lesions. Only one similar case was reported by Russell (6).

DISCLOSURE:  D. Lvovsky, None.

Chest. 2004;126(4_MeetingAbstracts):951S. doi:10.1378/chest.126.4_MeetingAbstracts.951S

INTRODUCTION:  Symptomatic obstruction of the esophagus can result from a variety of benign[3] and malignant[2] lesions. Endoscopic dilatation of the obstruction can provide relief of symptoms in many patients. However, in a small subset of patients, antegrade passage of a guidewire across the obstruction is not possible. Here we report a case where retrograde endoscopy via a gastrostomy failed to identify a lumen through the obstruction. A radiofrequency probe was used to bore through the obstruction and allow for safe dilation.

CASE PRESENTATION:  A 72 year old patient presented with complete obstruction of the esophagus secondary to squamous cell carcinoma. The patient experienced dysphonia after her radiation therapy that has persisted. A blind pouch was encountered behind the larynx. The gastrostomy lumen was dilated to accommodate a pediatric esophagoscope which demonstrated a very smooth, blunt tunnel ending in the cervical esophagus. Using fluoroscopy, the obstruction was estimated at 9-10 mm in length. Multiple images at various angles were obtained to confirm that the tips of both scopes were collinear. A radiofrequency probe was introduced into the distal esophagus via the gastrostomy, and was used with fluoroscopic guidance to tunnel a hole through the obstruction. A guidewire was passed through the tunnel. Sequential dilatation was performed and the scope was able to be passed through the obstruction. A Dobbhoff tube was placed through the nose and out the gastrostomy site and was anchored to the new gastrostomy tube to insure that the lumen would remain patent. On post-operative day one the patient was noted to have mild stridor. She was treated with a seven day steroid taper and racemic epinephrine nebulizers. A swallow study was performed, which was negative for any pooling or extravasation at the site of dilatation. The patient was discharged four days later able to swallow thin liquids and manage her own secretions.

DISCUSSIONS:  The use of radiofrequency ablation represents a new technique to manage complex esophageal obstruction. This technique allows for the development of a tunnel through the obstruction to allow for subsequent passage of a guidewire. The risk of perforation is kept to a minimum as the subsequent passage of dilating devices is safely done over a guidewire. In our reported case, the patient experienced stridor pre-operatively that worsened after use of the ablation procedure. It seems unlikely that the radiofrequency probe would cause any damage to neighboring structures based on it’s physics of energy dissipation, which decreases proportionally to the fourth power. It is more likely that the direct radiation of the larynx led to nerve damage and paralysis.

CONCLUSION:  Here we extend the capability of endoscopy to treat complex obstructions by using radiofrequency ablation to safely bore a path through the stricture under fluoroscopic guidance. This technique has the advantage of avoiding any delay in treatment that is encountered when a complete obstruction is encountered on upper endoscopy, as the physician can move directly to a combined antegrade and retrograde procedure.

DISCLOSURE:  R.C. Fields, None.

Chest. 2004;126(4_MeetingAbstracts):951S-a-952S. doi:10.1378/chest.126.4_MeetingAbstracts.951S-a

INTRODUCTION:  Sinus histocytosis with massive lymphadenopathy (SHML) is a rare pathological diagnosis of Mediastinal lymphadenopathy in adults. It is a lymph nodes disease recognized in 1969 (1), which reported to be associated with different diseases and extranodal presentations (2).

CASE PRESENTATION:  A 42 year old morbidly obese, white male found to have right hilar Lymphadenopathy and bilateral pulmonary nodules, on pre-surgical evaluation for bariatric surgery; Six weeks later presented with shortness of breath, repeated chest x-ray and computerized tomography showed rapidly developing mediastinal lymphadenopathy, Positron Emission Tomography showed increased uptake in the mediastinal lymph nodes and negative uptake in the lung nodules. Patients denied any weight loss, fever, night sweat and cough. White cell count and complete metabolic profile were normal. Patient underwent mediastinoscopy with biopsies, which showed focal Sinus Histocytosis without evidence of malignancy or infection.

DISCUSSIONS:  Radiological deferential diagnosis of rapidly progressing mediastinal lymphadenopathy includes lymphoma, metastatic cancer and infectious etiology. The histological differential diagnosis includes metastatic melanoma, metastatic carcinoma, malignant histiocytosis, Hodgkin disease, infectious processes, Langerhans cell histiocytosis, and if focal, SHML. Factors favoring SHML are large histiocytes strongly positive for S100 and negative for CD1a, and lymphophagocytosis. (3) (4) SHML commonly presents as massive, painless, bilateral lymph node enlargement in the neck with fevers. Most cases occur in the first or second decade of life and have a predilection for blacks. Rarely sites other than the lymph nodes can be involved including the central nervous system, eyes, upper respiratory tract, skin, and head and neck region. Interestingly, the spleen and bone marrow have been spared. These extranodal cases occur in up to 25% of cases. The association is dependent upon the site of the extranodal disease (5) (6). The cause is still unknown although a viral etiology is suspected. Molecular studies have found no evidence of clonal rearrangement implying that this disease is a reactive rather than neoplastic condition. SHML is reported in association with Lymphoma, chronic renal insufficiency, Human Immunodeficiency Virus, Amyloidosis, Autoimmune Hemolytic Anemia. Some cases have responded to chemotherapy but many times the disease undergoes spontaneous resolution. In others, an insidious course develops for years or decades. This is more common in the extranodal cases. Surgical indication is for life or function-threatening obstruction. Chemotherapy response rates are inferior to those seen in other hematopiotic diseases such as malignant lymphoma or Histiocytosis X. Radiotherapy responses were inferior to those expected with malignant hematopiotic neoplasms (7).

CONCLUSION:  SHML is pathological diagnosis can present as rapid developing mediastinal Lymphadenopathy and positive uptake on Positron Emission Tomography.

DISCLOSURE:  K. Kutmah, None.

Chest. 2004;126(4_MeetingAbstracts):952S. doi:10.1378/chest.126.4_MeetingAbstracts.952S

INTRODUCTION:  Pulmonary artery (PA) stricture occurs most often in the setting of congenital malformation. PA stricture is uncommon in the adult population, and PA stricture following radiation therapy is exceedingly rare.

CASE PRESENTATION:  A 57-year-old male presented to the pulmonary clinic with progressive dyspnea. Past medical history was significant for small cell lung cancer treated with radiation therapy and chemotherapy three years prior to presentation. On physical exam, he was a well-nourished male in no acute distress. Room air SaO2 was 91% at rest and 87% with minimal exertion. He was afebrile with normal vital signs. Neck and heart exams were within normal limits. Breath sounds were decreased bilaterally. There was no edema. Oxygen was prescribed and the patient was referred for testing. Pulmonary function testing revealed normal spirometry with a flow-volume loop suggestive of mild airflow obstruction. Diffusing capacity was within normal limits. Cardiopulmonary exercise testing demonstrated a mild ventilatory limitation with desaturation, but no evidence of a circulatory limitation. The patient returned to the outpatient office complaining of moderate chest pain without radiation or associated symptoms. Sublingual nitroglycerin completely relieved the pain and subsequent EKG revealed nonspecific changes and p-pulmonale. The patient was then evaluated by cardiac catheterization, which revealed normal coronary vessels with elevated PA pressures (48/26). A bilateral lower extremity duplex scan was negative. Ventilation-perfusion scanning, however, demonstrated complete lack of perfusion to the left lung. (Figure 1) Given the history of cancer and radiation therapy and lack of perfusion to the left lung, the differential diagnosis included intrinsic and extrinsic compression of the left PA. A spiral CT revealed a left PA stricture without evidence of thrombus or extrinsic compression; this finding was confirmed by pulmonary angiography. (Figure 2) Of note is the fact that this PA abnormality had not been observed on a CT obtained prior to radiation therapy. A stent was placed in the area of stricture with resulting 100% PA patency and improvement in dyspnea.

DISCUSSIONS:  Pulmonary artery stricture/stenosis is rare in the adult population. It may present as an isolated lesion but is more often a feature of a complex congenital heart disease. Reported cases in the literature attribute PA stenosis to extrinsic compression secondary to mediastinal fibrosis, tumor of the lung or esophagus, aortic aneurysm, or as a consequence of surgical intervention.

CONCLUSION:  Pulmonary artery stricture is a rare but possible complication of radiation therapy which can be a cause of unexplained dyspnea.

DISCLOSURE:  F.E. Loftus, None.

Chest. 2004;126(4_MeetingAbstracts):952S-a-953S. doi:10.1378/chest.126.4_MeetingAbstracts.952S-a

INTRODUCTION:  First described in 1936, Wegener’s granulomatosis (WG) is a clinical syndrome of unknown etiology characterized by necrotizing granulomatous vasculitis of the upper respiratory tract (74%), lungs (70%), and kidneys (46%). It affects all ages but is common in middle-aged men. Although renal involvement is common, lower urogenital involvement WG is rare.

CASE PRESENTATION:  A 31 year old man was admitted to the hospital with a 4-month history of night sweats, anorexia, and 30 lb weight loss and one-month history of hemoptysis and progressive dyspnea. One month prior, he had been diagnosed with necrotizing prostatitis by TURP after experiencing rectal pain, dysuria, and hematuria. After the TURP, he experienced transient blurred vision and hearing loss. He had emigrated to the US 12 years ago from Ecuador and had not traveled since then. At the time of admission, he was in hypoxemic respiratory failure and required support with mechanical ventilation. The initial examination of the chest was normal but rapidly diffuse crackles were appreciated. Lateral episcleritis was noted in both eyes. Admitting laboratory data revealed a hemoglobin of 8.2, ABGs on room air of pH 7.51, pCO2 28.5 and pO2 52.9. The BUN was 8, creatinine of 0.7, and the urinalysis showed “large” blood, “trace” protein, 40 RBCs, and 18 WBCs. A chest radiograph showed the development of bilateral upper lobe infiltrates, new from one week prior. The initial differential diagnosis included pneumonia and vasculitis. He was started on broad-spectrum antibiotics, antituberculous medications, and systemic corticosteroids. Bronchoalveolar lavage (BAL) revealed frank blood and a predominance of neutrophils. BAL bacterial, fungal, mycobacterial, and viral cultures, special stains and serum antinuclear, anti-GBM antibodies, and rheumatic factor were all negative. A PPD was negative. C-ANCA was positive. Review of the prostate tissue showed a leukocytoclastic vasculitis, fibrinoid necrosis, and giant cells consistent with WG. Cyclophosphamide was added and antimicrobials were discontinued. The patient improved rapidly over 14 days, was extubated, and ultimately discharged breathing on room air on a corticosteroid taper and continued cyclophosphamide.

DISCUSSIONS:  Involvement of the lower urogenital tract in WG is rare. Prostatitis is the most common manifestation causing frequency, dysuria, hematuria, and urinary retention. To our knowledge, only 28 cases of symptomatic prostatitis due to WG have been reported in the literature. Walton reported granuloma involving the prostate in 7.4% of cases. Stillwell et al. reported in 1987 only 2.3% (four of 174 patients) cases with WG with prostatic involvement. We report a case of WG that initially presented with extrapulmonary manifestations including prostatitis, transient vision and hearing loss followed by fulminant hypoxemic respiratory failure secondary to diffuse alveolar hemorrhage. Tuberculosis was initially considered but felt excluded by the negative PPD and BAL findings. He responded well to corticosteroids and cytotoxic therapy.

CONCLUSION:  WG is a multisystem disorder that is usually, but not exclusively, limited to the respiratory tract and kidneys. Symptomatic involvement of the lower genitourinary tract is rare but may be more frequent than previously thought. Symptoms of the urogenital system may precede the onset of pulmonary disease. Because tuberculosis, fungal and bacterial infections may cause a similar granulomatous inflammation and vasculitis, microscopic stains and cultures should be reviewed before initiating immunosuppressive therapy. Urogenital manifestations of WG seem highly sensitive to corticosteroid and cyclophosphamide therapy.

DISCLOSURE:  C.Q. See, None.

Chest. 2004;126(4_MeetingAbstracts):953S. doi:10.1378/chest.126.4_MeetingAbstracts.953S

INTRODUCTION:  Post-traumatic pulmonary pseudocyst (PPP) is an uncommon result of blunt trauma to the chest, presenting as one or more cavitary lesions within the lung parenchyma. We present the case of young man diagnosed with PPP following a motor vehicle accident.

CASE PRESENTATION:  A 20-year-old man was a restrained passenger in a car which struck a tree after the driver fell asleep while traveling approximately 20 mph. The patient walked some distance from the car before collapsing. He was evaluated at the local hospital, where a CT of the abdomen showed a liver laceration with hematoma and cavitary lesions of the lower lungs with air-fluid levels and surrounding ground glass opacity (see figure). He was transferred to a level one trauma center for management of the liver laceration, and pulmonary consultation was requested to evaluate the lesions in the lungs. The differential diagnosis for the cavitary or cystic lesions was organized as follows: 1) lesions unrelated to the trauma: blebs, bullae, congenital cysts, coccidioidomycosis, PCP, tuberculosis, hydatid disease, cavitary pneumonia, and 2) lesions related to the trauma: cavitating hematomas, lung lacerations, traumatic pseudocysts. The young man was a current smoker, with no known pulmonary disease. He had no known contact with tuberculosis, but reported a 2-pound weight loss and occasional cough. PPD had been negative several years before. He had had a normal chest radiograph 1 year earlier, at the time of a clavicular fracture. Physical exam revealed a young man in no distress. Vital signs were normal. The lungs were clear to auscultation. There was mild abdominal tenderness over the lacerated liver. Inpatient studies included induced sputum and bronchial washings negative for acid-fast bacilli but positive for Staphylococcus aureus. Blood cultures were negative. Fungal serology was negative. The diagnosis of PPP was made after granulomatous infection had been excluded. Repeat CT chest several days after admission showed the cavities were smaller, with persistent air-fluid levels. A percutaneous drain was placed into the largest cavity, and approximately 100 mL of hemorrhagic fluid was removed. Culture of the fluid yielded S. aureus. The drain was removed after 2 days, and the patient was discharged home on prolonged antibiotic treatment with amoxicillin/clavulanate. Follow up CT imaging showed resolution of the cavities over a period of 10 weeks.

DISCUSSIONS:  PPP is a cystic lesion (i.e. no epithelial lining) which forms in the lungs following significant blunt trauma. The mechanism of injury involves both shear forces, as in traumatic deceleration, and burst forces, as in thoracic compression with a closed or narrowed glottis. It is more common in young patients, whose more flexible chest wall may transmit the force of the injury to the lungs more efficiently. PPP generally resolves without specific intervention. The principle complication is infection, as in our patient, which occurs in up to 40% of patients in some series, and often requires percutaneous drainage or surgery.

CONCLUSION:  PPP should be considered in the differential diagnosis of cystic or cavitary lung lesions following significant blunt trauma, particularly in younger patients. No specific intervention is needed, but infectious complications must be ruled out, or treated aggressively if they develop.

DISCLOSURE:  R.W. Ashton, None.

Chest. 2004;126(4_MeetingAbstracts):953S-a-954S. doi:10.1378/chest.126.1.322-a

INTRODUCTION:  Wegener’s Granulomatosis (WG) is a necrotizing small vessel vasculitis predominantly affecting the sinuses, lungs and kidneys. Although the diagnosis is suggested by chronic upper airway infections, WG is a protean disease and can affect any organ in the body (1). This case is an example of WG masquerading as a metastatic lung carcinoma.

CASE PRESENTATION:  A 38-year-old male smoker presented with four months of severe right ear pain, right eye ptosis, right facial droop, dysphagia, cough, and hoarseness, with an associated forty pound weight loss. He also noted migratory, episodic swelling of his elbows, wrist, knees and ankles. Symptoms did not improve after a prolonged course of oral antibiotics and low dose corticosteroids. Physical examination: Right facial droop and ptosis, right lateral gaze paralysis. Purulent material in the right external auditory canal. The lungs were clear. Laboratory/Radiographic Findings: Serum sodium of 132. CBC: mild, normocytic anemia. Computed tomography (CT ) of the sinuses and head revealed an infiltrating mass in the right post-styloid parapharyngeal space compressing the facial nerve. CT of the chest: 4.3 x 3.2 cm cavitary lesion in the right lung apex, 1 cm nodule in the left lung apex, enlarged azygos lymph nodes, and lytic and sclerotic changes of the right clavicle. The right adrenal gland was enlarged at 3.5 cm. A diagnostic bronchoscopy revealed normal airways without endobronchial lesions. Histopathology from transbronchial biopsies: peri-vascular caseating granulomas consistent with Wegener’s Granulomatosis. Cytoplasmic-anti-neutrophil cytoplasmic (C-ANCA) and anti-protease 3 (anti- PR 3 Ab) antibodies were positive. The patient was admitted to the hospital and begun on high dose systemic corticosteroids and intravenous cyclophosphamide. He was continued on prednisone and oral cyclophosphamide. Over the next two months, he had resolution of the cranial neuropathies and systemic symptoms.

DISCUSSIONS:  Wegener’s Granulomatosis (WG) is a small vessel vasculitis that most commonly affects the airway, upper and lower, and kidneys. It is uncommon, with a reported incidence of three per 100,000 (1). The most common areas of involvement at presentation are the sinuses, lungs, and kidneys. Neurologic symptoms are less common, but have been reported in approximately one third of patients, usually as a sensorineural hearing loss (2). Otolaryngologic symptoms, the most common presenting complaint, are manifested as chronic otitis media (3) or sinus disease (3). WG manifesting as a skull-based mass is rare, however has been reported (2,4,5). It is presumed a result of extension of disease from the Eustachian tube into the temporal bone (5). WG has rarely been reported to mimic metastatic bronchogenic carcinoma, as in this case (6,7). Diagnosis may be delayed by problematic tissue sampling which relates directly to the organ sampled (1).

CONCLUSION:  Wegener’s Granulomatosis may present with misleading radiographic findings. An appropriate index of suspicion and expeditious evaluation can prevent a critical delay in therapy.

DISCLOSURE:  C.M. Chang, None.

Chest. 2004;126(4_MeetingAbstracts):954S-955S. doi:10.1378/chest.126.4_MeetingAbstracts.954S

INTRODUCTION:  Many risk factors have been identified for the development of thromboembolic disease. Prolonged immobilization, malignancy, trauma, surgery, and hematologic hypercoagulable states such as protein C, protein S and antithrombin III deficiencies, factor V laiden and anticardiolipin antibodies. Will present a case of a young man, who had hepatitis C and while on interferon-alpha and ribavirin therapy developed a pulmonary embolism.

CASE PRESENTATION:  A 26 year old man with history of high blood pressure, intravenous drug use, and recent diagnosis of hepatitis C presented with shortness of breath, fever and chills of two days of evolution with general malaise, dyspnea on exertion and decreased exercise capacity. No history of productive cough or chest pain. No past history of thromboembolic disease, and negative PPD history. The previous month he received therapy for a lower respiratory tract infection. Symptoms started on the twelve week of therapy with peggylated interferon-alpha (150mcg/week) and ribavirin (1200mg/day). Physical exam showed a patient in mild respiratory distress, tachycardic without murmurs or gallops and inspiratory crackles at right lung base. No stigmata of chronic liver disease was present. Pertinent laboratories showed hypoxemia (p02 76mmHg) at room air, normal cell blood count and chemistries. Chest-X-ray showed right basilar linear atelectatic changes. Chest Computed Tomography Scan with intravenous contrast was suggestive for the presence of thrombi at the distal right pulmonary artery. Venous Doppler was negative for Deep Venous Thrombosis in the lower extremities and a Ventilatio-Perfusion Scan was low probability for Pulmonary Embolism (decreased ventilation at right lung base and two moderate to large mostly matched perfusion defects at the superior and posterior basal segments of right lower lobe). A digital substraction angiography showed large right main pulmonary artery thrombi extending into proximal segments of upper, middle and lower lobe arteries. 2D echo showed no vegetations, and no pulmonary hypertension. A hypercoagulable state workup showed normal levels of protein C and protein S, but decreased levels of antithrombin III (58). He had undetectable anticardiolipin antibodies or lupus anticoagulant activity and normal factor V activity. Patient received six months of uneventful anticoagulation therapy. Interferon-alpha was discontinued upon diagnosis of PE. Liver biopsy showed liver fibrosis with out cirrhosis, and prior to anti HCV therapy he had a PCR-HCV RNA of 145,000 copies, that after twelve weeks of therapy had decreased to < 50 copies. At six month of anticoagulation a chest CT Scan with IV contrast showed resolution of thrombi. Antithrombin III levels had returned to normal limits, and PCR-HCV RNA had increased to 29,900 copies.

DISCUSSIONS:  It has been shown that patients with cirrhosis have decreased synthesis of clotting factors, hyperfibrinolysis, and thrombocytopenia which predispose them for hemorrhagic complications. They also show decreased levels of antithrombin III, protein C and protein S and in patients with hepatitis C, there has been an association with increased levels of antiphospholipid antibodies, which predispose them for thrombosis. Although our patient had no evidence of cirrhosis by liver biopsy, he developed a transitory antithrombin III deficiency, that after interferon-alpha therapy was discontinued returned to normal levels. Interferon-alpha effect on the respiratory system includes bronchitis, cough, dyspnea, pleural effusion, lung fibrosis and interstitial pneumonitis among others. Interferon therapy has been implicated as a cause for central retinal vein obstruction (1). Interferon-gamma has been implicated as a possible cause for the development of DVT (2). Ribavirin has no known thrombogenic effect.

CONCLUSION:  Although patients with hepatitis C can develop thrombosis by other mechanisms, this is the first known case in which interferon-alpha has been associated with thromboembolic event in a patient with hepatitis C.

DISCLOSURE:  G. Santos, None.

Chest. 2004;126(4_MeetingAbstracts):955S. doi:10.1378/chest.126.4_MeetingAbstracts.955S

INTRODUCTION:  The care of patients with pulmonary arterial hypertension (PAH) has been greatly advanced over the recent years with high dose calcium channel blockers, epoprostanol, trepostinil, and bosentan.1 Much of this work has concentrated on the vascular endothelial and smooth muscle response to the various mediators of pulmonary vasodilation and constriction. Recent work has also implicated the erythrocyte (RBC) as an active participant in pulmonary vasodilation.2,3 Based on this work, we would like to present a case report of a patient with pulmonary arterial hypertension who received extensive RBC transfusions and experienced near complete remission of her pulmonary arterial hypertension.

CASE PRESENTATION:  A 39 y/o lady with a remote history of lupus and hemolytic anemia diagnosed at age 8 was treated with steroids and splenectomy. Since age 16, she had been in remission and off all rheumatalogic drugs. The patient was asymptomatic, had no evidence of cardiovascular disease, had 3 normal pregnancies, and had no history of smoking, drug or alcohol abuse. Two years ago, she developed new symptoms consistent with PAH. Right heart catheterization documented pulmonary arterial hypertension with pulmonary artery pressure 87/31 (mean 55), mean right atrial pressure 5, and cardiac index of 3.2 (by thermodilution, 2.45 by Fick). Exercise tolerance worsened to New York Heart Association Class (NYHA) IIIb, despite only mild obstruction on pulmonary function and unremarkable radiographic studies. Physical examination was remarkable for accentuated P2 and 1+ jugular venous distension. Continuous intravenous epoprostanol therapy (Flolan) was initiated with good results. The patient had blood loss of 4 and 6 units within 2 months and received equivolume packed RBC transfusions. Afterwards, her symptoms returned to NYHA I-II. She was successfully weaned off Flolan without worsening of her symptoms. Within 2–3 months, the patient’s symptoms and physiology of PAH recurred.

DISCUSSIONS:  Recent research has implicated that the RBC plays an active role in pulmonary vasodilation.1,2 RBCs contain millimolar concentrations of adenosine triphosphate (ATP) and are able to release it in response to various physiological stimuli including deformation and stimulation of beta-adrenergic and prostacyclin receptors. The released ATP stimulates nitric oxide (NO) synthesis by endothelial cells and vasodilation in the pulmonary vasculature. RBCs from subjects with PAH did not release ATP in this manner and thus would fail to stimulate NO synthesis.

CONCLUSION:  Knowing this background, our ability to wean the Flolan may have been associated with and due to the multiple blood transfusions replacing the patient’s defective RBCs with normally behaving ones. This would suggest that RBC “exchange transfusion” might help in acute therapy for patients who have acute exacerbations of PAH as has been done for sickle cell disease patients with acute chest syndrome. Recent genetic research has implicated the bone morphogenetic protein receptor type 2 gene (BMPR2) as a common cause for PAH. We have speculated that BMPR2 may be present on the RBC membrane, potentially linking the RBCs inability to release ATP to the genetic defect in PAH.

DISCLOSURE:  G.E. Liang, None.

Chest. 2004;126(4_MeetingAbstracts):955S-a-956S. doi:10.1378/chest.126.4_MeetingAbstracts.955S-a

INTRODUCTION:  Orthodeoxia, arterial desaturation during upright posture improved by recumbency, is a rare physical finding. We present an unusual case of orthodeoxia caused by an aortic aneurysm compressing the right atrium, resulting in an intermittent right to left shunt through an inter-atrial septal aneurysm with reversal of the shunt after myocardial infarction.

CASE PRESENTATION:  A 78-year-old male presented to the emergency room with several months of dizziness, which worsened whenever he stood up and bent over to pick up his morning paper. He denied any loss of consciousness, chest pain, or shortness of breath. A CT of the head and cardiac enzymes were negative. During his hospitalization, he acutely desaturated, requiring a 100% non-rebreather mask with a PaO2 of 38 mmHg. Further testing revealed a negative V/Q scan; a helical chest CT was negative for pulmonary embolism but showed a thoracoabdominal aortic aneurysm; an echocardiogram showed preserved left ventricular function and a possible inter-atrial aneurysm. He was transferred to a tertiary care center for possible repair of his aortic aneurysm. The patient’s exam was significant for absent jugular venous distention, clear lungs, regular heart rate without murmurs, and no lower extremity edema or calf swelling. He was discovered to have orthodeoxia, with oxygen saturations of 95% supine and 78% sitting upright while receiving 5 L/min nasal oxygen. The orthodeoxia was postulated to be caused by extrinsic compression of his dilated aortic arch causing intermittent, positional shunting through the interatrial aneurysm. While undergoing right heart catherization, the patient developed ventricular tachycardia with hypotension requiring electrical cardioversion and intubation. No pulmonary hypertension was present. The patient continued to be unstable and underwent emergent cardiac catheterization that showed a 98% occlusion of his left anterior descending artery, which required angioplasty and stent placement. A subsequent transesophageal echocardiogram showed an enlarged left atrium and left ventricle, and a newly decreased ejection fraction of 35%. The patient no longer had orthodeoxia and was discharged without further surgical intervention.

DISCUSSIONS:  Orthodeoxia is arterial desaturation with an upright posture, which is improved by recumbency. The orthodeoxia-platypnea syndrome is very rare with only about 50 case reports in the literature. The differential diagnosis includes pulmonary arteriovenous malformations, intracardiac shunt, hepatopulmonary syndrome, recurrent pulmonary emboli, chronic lung disease, and post-pneumonectomy syndromes[1].Normally, small inter-atrial defects create a left to right shunt due to the higher filling pressures of the left atrium as compared to the right atrium. However, there have been reports of an elongated aorta, aortic aneurysm, or dilated aorta causing extrinsic compression of the right atrium, which elevated the right atrial pressure resulting in a right to left shunt in the presence of an interatrial defect. These patients underwent surgical correction of the aortic and interatrial defects with subsequent resolution of their orthodeoxia[2,3]. Our patient was unique, because his shunt was corrected by an acute myocardial infarction, which resulted in decreased left ventricular function and an increased left atrial pressure, thus reversing the right to left shunt. Since he was subsequently asymptomatic and no longer produced a positional right to left shunt, surgical repair of his atrial defect was deferred at this time.

CONCLUSION:  Orthodeoxia can be caused by an extrinsically compressing aorta combined with an inter-atrial defect in the setting of normal pulmonary artery pressures. We report a novel case of reversal of orthodeoxia after myocardial infarction, without surgical intervention.


Chest. 2004;126(4_MeetingAbstracts):956S. doi:10.1378/chest.126.4_MeetingAbstracts.956S

INTRODUCTION:  Polymethylmethacrylate (PMMA) is a bone cement used in various orthopedic procedures. Over the last two years it has become the most common material used for the treatment of compression fractures with percutaneous vertebroplasty (PV). Because PMMA is a low-viscosity substance that is forcibly injected into collapsed vertebral bodies, there is a significant risk of extravasation. Some degree of local leakage is reported in up to 65% of the vertebra injected (1). We report a case of a clinically significant PMMA cement pulmonary embolism following PV.

CASE PRESENTATION:  A 76 year-old Asian male with a history of atrial fibrillation and osteoporosis was admitted for PV of two compression fractures. Under fluoroscopy, extravasation of PMMA into to the paravertebral veins and pulmonary artery was observed during injection, and the procedure was discontinued. Shortly thereafter, the patient had decreasing oxygen saturation and was placed on 100% FiO2. Post-procedure he remained intubated and was transferred to the neurosurgical ICU. He was hemodynamically stable with an oxygen saturation of 96% on 100% FiO2. Physical examination was remarkable only for an irregular cardiac rhythm and trace pitting edema bilaterally of the lower extremities. A stat echocardiogram revealed pulmonary artery pressures (PAP) of 55 mmHg, a mildly dilated right atrium (RA) and hyperechoic material in the RA and right ventricle (RV). Pre-procedure PAP was 30-40mmHg. Over the next twenty-four hours his oxygen requirements decreased significantly, and he was extubated and titrated to 6 liters/minute of oxygen via nasal cannula. The chest CT demonstrated bilateral hyperdensities in the pulmonary vasculature and RV consistent with PMMA embolism. There was a small right lower lobe linear density with a peripheral wedge-shaped opacity suggestive of pulmonary infarction (Figure 1). A repeat echocardiogram demonstrated worsening RV systolic function, mild to moderate RV dilation, and hyperechoic material in the RV. Full anticoagulation was started, and over the course of one week supplemental oxygen requirements decreased to room air. He was discharged home on full anticoagulation with coumadin. Four months following the event, he still complains of persistent exertional dyspnea, and his PAP has increased to 80mmHg. His repeat chest CT demonstrates large bilateral cement emboli, subcentimeter residual cement in the RV, and cement in the azygous and hemiazygous vasculature (Figure 2).

DISCUSSIONS:  The use of PMMA for PV is increasingly common. There have been several adverse events reported related to its use such as hypotension, asystole, bradycardia, bronchospasm, spinal impingement, and pulmonary emboli (123). There have been eleven prior case reports of cement pulmonary embolization associated with PV, all in the last two years. Management has consisted of full anticoagulation, embolectomy, or no treatment. Most patients have been treated with full anticoagulation unless contraindicated, as PMMA cement has been shown to be significantly thrombogenic (4). There is no data on the long-term outcomes of the patients that have had a PMMA pulmonary embolus.

CONCLUSION:  Percutaneous vertebroplasty has become a very common procedure. We suspect there will be a significant increase in the occurrence of associated adverse events in the future, including cement pulmonary embolization, which can lead to significant morbidity and mortality. It is important to be aware of the procedural complications and managements that have been implemented to treat PMMA cement embolism thus far.

DISCLOSURE:  J. Simon Grahe, None.

Chest. 2004;126(4_MeetingAbstracts):956S-a-957S. doi:10.1378/chest.126.4_MeetingAbstracts.956S-a

INTRODUCTION:  Primary pulmonary hypertension is uncommon and a diagnosis of exclusion. This patient’s occupational history provided the key to his diagnosis.

CASE PRESENTATION:  A 59-year-old male noted dyspnea while playing golf in 2001. He ultimately had a pre-syncopal episode associated with chest pressure at work in 2002. He was hospitalized and an echocardiogram revealed normal left ventricular function and a dilated right ventricle. He underwent left and right heart catheterization that showed a pulmonary artery pressure of 63/29 mmHg and a mean of 43 mmHg. His pulmonary capillary wedge pressure was 10 mmHg. He had no coronary artery disease. An extensive investigation as to the cause of his pulmonary hypertension was unrevealing. Spirometry and lung volumes were normal. However, he had a reduced diffusion capacity of 37% predicted. His radiographic evaluation included a negative ventilation/perfusion lung scan and a high-resolution chest computed tomogram was without evidence of parenchymal lung disease. Work-up for an underlying connective tissue disease was negative. Serologies for HIV and hepatitis were negative. A sleep study was normal. His past medical history was non-contributory. His occupational history was notable for heavy exposure to a laminate containing toluene. For 15 months he worked with this material in a small, poorly ventilated room. Visitors to his work site often commented on the strong fumes present. He observed a temporal relationship between his symptoms and working with the glue. He was diagnosed with pulmonary arterial hypertension (PAH) due to toluene exposure. Currently, he is being treated with bosentan, diltiazem, and coumadin. He requires oxygen therapy for exertional desaturations. His pulmonary arterial pressure remains elevated although his chronic sinus symptoms resolved when he left his work site. His exercise tolerance and echocardiographic findings have improved on bosentan and away from toluene.

DISCUSSIONS:  This patient’s occupational history reveals important diagnostic clues about the cause of his PAH. He had heavy exposure to a laminate adhesive containing 5-15% toluene by weight. Review of the medical literature between 1966-2004 found no reports of PAH due to occupational toluene exposure. However, toxicologic information on toluene inhalation is available in the context of glue sniffing; these health effects are separated into acute and chronic toxicities. This patient’s history is consistent with both. Acutely the patient developed dyspnea while at work and this prompted his medical evaluation. Several reports of glue sniffers presenting with acute shortness of breath are published (Cronk 1985, Schikler 1984). An additional case report describes an infant who developed acute respiratory failure associated with echocardiographic pulmonary hypertension following ingestion of paint thinner containing toluene (Dauger 2003). This case is also consistent with chronic toxicity from glue inhalation. Eighteen adolescents who chronically abused adhesives containing toluene underwent extensive medical evaluations. Reduced diffusion capacity was seen in 33% (6 of 18 people) and right ventricular dilation on echocardiogram was noted in 28% (5 of 18 people) (Devathasan 1984). This patient manifests both these findings.

CONCLUSION:  This is the first case of occupational-associated PAH due to toluene exposure reported in the literature. It underscores the necessity of obtaining a thorough occupational history when evaluating patients with pulmonary hypertension.

DISCLOSURE:  D.W. Ford, None.

Chest. 2004;126(4_MeetingAbstracts):957S. doi:10.1378/chest.126.4_MeetingAbstracts.957S

INTRODUCTION:  Though Neisseria sicca is part of the normal oral flora, in rare instances it can be a serious pathogen causing endocarditis, meningitis, osteomyelitis, etc. Neisseria sicca endocarditis is characterized by large vegetations and destruction of the valve. Of the various Neisseria species, Neisseria sicca most often results in embolic complications. Since the first report of Neisseria sicca endocarditis in 19181, only 15 other cases have been reported (available upon request) and the last was 6 years ago2. We present a 17th case of endocarditis due to Neisseria sicca, accompanied by multiple embolic complications.

CASE PRESENTATION:  A 39-year-old male without any history of substance use presented with “flu-like” symptoms for one week, mental status changes, and sudden left-sided weakness for one day. He was lethargic and blood pressure was 90/40 mm Hg. Non-blanching, 1cm, purplish macular lesions on the skin, icterus, and conjunctival petechaie were noted. Cardiac examination revealed no murmurs. Left hemiparesis was present. CAT scan of the brain showed multiple non-enhancing parenchymal focal lesions (see Figure). Shortly after admission, he became more lethargic, developed increased respiratory distress, and required intubation. Chest X-ray showed bilateral diffuse alveolar infiltrates. On transesophageal echocardiogram (TEE) left ventricular size was normal and left ventricle function was moderately depressed. There was a sclerotic bicuspid aortic valve with a mobile vegetation on the right cusp prolapsing into the left ventricle (see Figure). Blood cultures grew Neisseria sicca, sensitive to ceftriaxone. He was treated with IV ceftriaxone, and carious teeth were extracted. TEE 8 days later showed a calcified vegetation (whose size had increased) prolapsing in the outflow tract. Severe aortic insufficiency was noted. He improved with intravenous antibiotics and was extubated within 8 days. Three days later, left calf and toes soreness developed, with increasing purpura. Magnetic resonance angiography of the left lower extremity showed significant thrombus in the left distal common iliac artery, left external iliac artery, and left hypogastric artery. Left iliac embolectomy and excision of vegetation were done, and a St. Jude’s mechanical aortic valve was placed. He was discharged home on intravenous ceftriaxone and warfarin. Six weeks later, sharp epigastric pain and vomiting developed. Right upper quadrant abdominal ultrasound showed a hepatic artery mycotic aneurysm with compression of gallbladder, common bile duct, and splenic vein. CAT scan showed an infra-renal abdominal aortic dissection into the left common iliac artery and a hepatic artery mycotic aneurysm with common bile duct obstruction and portal/splenic vein thrombosis from mechanical compression. Under ultrasound guidance, thrombin was injected into the pseudoaneurysm via the celiac artery. The patient was finally discharged home on intravenous antibiotics. He was doing well on outpatient follow-up.

DISCUSSIONS:  Neisseria species other than Neisseria gonorrhoeae and Neisseria meningitides are generally not considered pathogenic. Endocarditis due to Neisseria sicca may be associated with serious embolic complications including cerebral, hepatic, and peripheral embolism. Treatment should include a 6-week or longer course of intravenous antibiotics and possibly surgical correction of underlying structural heart disease and dental extraction.

CONCLUSION:  Neisseria sicca isolation from blood should be taken seriously rather than attributed to contamination, and should prompt evaluation for possible endocarditis. The index of suspicion should be greater in patients using intravenous drugs and in those with structural heart disease or dental disease. Secondly, if patients with any embolic phenomena or endocarditis have a blood culture that reveals Neisseria sicca, it should be treated as the responsible organism unless proven otherwise.

DISCLOSURE:  N. Tripathi, None.

Chest. 2004;126(4_MeetingAbstracts):958S. doi:10.1378/chest.126.4_MeetingAbstracts.958S

INTRODUCTION:  Aortic dissection is a catastrophic, though, relatively uncommon illness. Forty percent of acute cases are classified as Type B (descending) aortic dissections. Hypotension and shock on presentation is seen in about 3% of cases. Aortic rupture, a frequent cause of hypotension in aortic dissection, causes massive hemorrhage and associated high mortality rate (> 50%). Due to the location and anatomic relations of the descending aorta, aortic rupture at this level usually causes a left hemothorax. In this report, we present a case of ruptured Type B aortic dissection causing a right hemothorax and draw attention to this relatively uncommon clinical clue to rupture.

CASE PRESENTATION:  A 55 y/o black male with a history of hypertension presented with nausea and progressively worsening abdominal pain with radiation to the back for 36 hours. His blood pressure on presentation was 224/120 mm Hg. CT scan revealed Type B dissection extending to the common iliac arteries, but not affecting the visceral perfusion. Patient was placed in the medical ICU and blood pressure was controlled with intravenous beta-blockers and nitropresside then oral medications. Six days later, the patient began complaining of abdominal and back pain associated with shortness of breath. A chest x-ray at that time showed a new large right pleural effusion. A CT of chest, abdomen and pelvis was performed and confirmed the right pleural effusion with extravasation of contrast into right chest with no further extension of dissection compared to previous CT scan. Even though the patient had remained hemodynamically stable prior to surgery, he was emergently taken to the OR for repair of a contained ruptured thoracic aortic dissection. Intra-operative transesophageal echocardiogram revealed a pseudoaneurysm of the mid-thoracic aorta with a periaortic hematoma. A right chest tube was placed in the OR and 1000 cc of blood was drained. A lumber drain was placed after induction of anesthesia. For repair of aorta the patient was placed on axillary femoral bypass. Though a left thoracoabdominal incision, aorta was replaced with a graft starting at the left subclavian to the celiac artery with reimplantation of intercostals T8-L2. At surgery a free rupture of the mid thoracic aorta at the level of inferior pulmonary vein contained by the posterior mediastinal tissue was seen with extension across the midline into right pleural cavity. Postoperatively the patient suffered paraplegia, renal failure and pneumonia and died on the 25th postoperative day from sepsis.

DISCUSSIONS:  Rupture of the descending thoracic aorta results in bleeding into the left pleural cavity. A right hemothorax secondary to rupture of aortic dissection is rare. To our knowledge only four cases - excluding cases of ruptured non-dissecting aortic aneurysms- have been reported in the English literature. The right hemothorax has been described in most of the cases to develop from a medial tear in the aorta at the level of the mid thoracic spine that bleeds into the posterior mediastinum and crosses the mid line to rupture into the right pleural space. Establishing a prompt and accurate diagnosis in these cases is important to prevent early death through surgical intervention. 50% of the previously reported cases died intraoperatively due to delay in diagnosis.

CONCLUSION:  Type B aortic dissection with rupture into the right hemithorax is a rare but potentially fatal disorder that should be diagnosed rapidly. This uncommon presentation should always be considered in patients with chest pain and right pleural effusion. This report emphasizes the fact that despite the anatomic location of descending aorta close to the left pleural cavity, aortic rupture can lead to right sided hemothorax.

DISCLOSURE:  M.S. Abu-Fadel, None.

Chest. 2004;126(4_MeetingAbstracts):958S-a-959S. doi:10.1378/chest.126.4_MeetingAbstracts.958S-a

INTRODUCTION:  While cutaneous reactions to epoprostenol (EPO) may occur, leukocytoclastic vasculitis (LCV) has not previously been reported. We present a case of biopsy proven leukocytoclastic vasculitis secondary to continuous intravenous epoprostenol therapy.

CASE PRESENTATION:  A 48 year-old female with fenfluramine-phentermine induced primary pulmonary hypertension was initiated on continuous intravenous epoprostenol therapy. Upward titration of EPO was associated with the development of a centripetal rash, beginning at the lower extremities. Exam demonstrated partially blanchable erythematous papules and nonblanching violaceous purplish macules on the lower extremities bilaterally and confluent across the abdomen and buttocks. Skin biopsy revealed injured dermal blood vessels with perivascular infiltration of neutrophils, karyorrhetic debris and erythrocytes consistent with leukocytoclastic vasculitis. Extensive hematologic, biochemical, and rheumatologic serologies were unrevealing. Infection and other drugs were ruled-out as causative agents. LCV was only somewhat ameliorated by moderate doses of prednisone, prompting the addition of methotrexate and hydroxychloroquine. Despite months of immunosuppressive therapy, increasing doses of EPO correlated with a worsening rash and the formation of eruptive lesions. New microscopic hematuria and proteinuria heralded possible renal involvement. The patient was transitioned off intravenous epoprostenol to combination therapy with bosentan and sildenafil with complete resolution of her rash.

DISCUSSIONS:  Leukocytoclastic vasculitis is a hypersensitivity reaction involving the small vessels most commonly of the skin, but visceral organs may be severely affected. Palpable purpura, maculopapular rash, and skin biopsy showing perivascular neutrophils are hallmarks of the disease. In at least 10 percent of cases, LCV is thought to be drug-induced. In these cases, treatment involves discontinuation of the inciting agent and occasionally, immunosuppressive therapy. Continuous intravenous epoprostenol therapy improves the survival, exercise tolerance, hemodynamics and quality of life of patients with pulmonary hypertension. EPO is a strong vasodilator and inhibitor of platelet aggregation. While EPO works partly through adenylate cyclase activation, its immunomodulatory actions are poorly understood. We present the first described case of leukocytoclastic vasculitis associated with epoprostenol therapy. The rash of LCV began and worsened with increasing doses of EPO despite concurrent immunosuppressive therapy. Complete resolution of LCV followed the withdrawal of epoprostenol.

CONCLUSION:  Epoprostenol should be included among agents implicated in the development of leukocytoclastic vasculitis.

DISCLOSURE:  C.W. Hargett, None.

Chest. 2004;126(4_MeetingAbstracts):959S. doi:10.1378/chest.126.4_MeetingAbstracts.959S

INTRODUCTION:  Spread of primary lung carcinoma to the heart by direct extension of metastasis is rare. Disruption of normal organ anatomy and physiology in this area requires extreme care during diagnostic and therapeutic interventions. We report a case of invasion of left atrium in primary lung cancer.

CASE PRESENTATION:  This patient is 60 years old AAM smoker with PMHx of rheumatoid arthritis and COPD presented for evaluation of a lung mass. His presenting complaints were dyspnea on exertion, NYHA class II progressing to class III and increasing sputum production over 6 months. He also c/o anorexia and weight loss of 70 lbs over last one year. He had CXR and CT scan, which showed a left hilar mass with possible extension into left atrium. Echocardiogram confirmed the invasion of left atrium. He underwent flexible bronchoscopy, which revealed a large fungating mass in left main bronchus within 1 1/2 cm of the carina. An electrocautery snare was used to remove part of the endobronchial mass, histopathologic review was consistent with non-small cell cancer. The patient was treated with carboplatin and taxol. At the time of this abstract he is doing well.

DISCUSSIONS:  Direct extension of primary lung cancers to heart is a rare feature. There are about 30 case reports of extension of cancer into left atrium. The types of cancer that invaded the left atrium included non-small and small cell cancer, fibrosarcoma and carcinoid tumor. The studies helpful to make diagnosis include transthoracic and transesophageal echocardiogram; CT scan; and MR imaging and angiography. The presenting features include systemic embolism, stroke, multiple metastasis, congestive heart failure. Prognosis is poor, if the patient does not respond to chemotherapy. There are published case reports of aggressive surgery including pneumonectomy with en bloc or partial removal of left atrium. Four patients are reported in case report by Kodama. Two of them underwent pneumonectomy and left atrial resection and cardiopulmonary bypass, one died seven months after surgery, the other one had longer survival but had recurrence. Two patients underwent ordinary lobectomy as there were no abnormal findings on hilar examination. However, lumen of pulmonary veins were filled with tumor tissue. One patient died next day because of massive embolism and second patient was discharged without complications but later developed distant metastasis because of dislodgement of tumor into aorta. Other case report by Olearchyk had 3 patients who underwent surgery, two of them underwent adjuvant chemotherapy. One of them died after nine months because of ruptured abdominal aneurysm, the second patient died three months later from complications of chemotherapy. One of them was alive after 2 years of surgery at the time of publishing the article.

CONCLUSION:  The involvement of heart by direct extension in primary lung cancer is rare and presents as challenge for diagnosis and treatment. Radical surgery with pneumonectomy and atrial resection is an option but has a high morbidity and mortality. Further studies comparing chemotherapy and or radiation therapy alone or as an adjuvant to surgery are needed.

DISCLOSURE:  A.S. Qureshi, None.

Chest. 2004;126(4_MeetingAbstracts):959S-a-960S. doi:10.1378/chest.126.4_MeetingAbstracts.959S-a

INTRODUCTION:  Pulmonary artery sarcoma is a rare tumor of the cardiovascular system. In many instances, the diagnosis is difficult and delayed. We present a case of a 68-year-old male who had a thrombus in the main pulmonary artery, and was later diagnosed with pulmonary artery sarcoma.

CASE PRESENTATION:  A 68-year-old Portuguese Male with no significant medical history was seen in the ED complaining of productive cough for 3 months, with associated intermittent fever, dyspnea, orthopnea, and weight loss. He was being treated with antibiotics and bronchodilators without improvement. The patient had a 40 pack-year smoking history, and he worked in a metal factory. On examination, his vital signs were stable, O2 sat 94%. Lung findings showed symmetrical air entry without rales, rhonchi or wheezes. He had no clubbing, but he had palpable inguinal and axillary lymph nodes. CXR on admission was unremarkable. His initial ABG was normal, FEV1 was 115% predicted. However, his V/Q scan showed total lack of perfusion of the left lung with small defect seen in the right apex (Fig 1). His Spiral CT showed thrombus in the main pulmonary artery extending into the left pulmonary artery, and extensive thrombus in the right pulmonary artery (Fig 2). Venous duplex of lower extremities showed negative for DVT, and the hypercoagulable work-up was also negative. His 2D echocardiogram showed moderately dilated right ventricle and pulmonary artery systolic pressure of 43mm/Hg. Patient was treated with continuous anticoagulation. Follow up chest CT 3 weeks later showed no improvement in the filling defect. Patient was sent for pulmonary thromboendarterectomy, and the surgical pathology showed presence of pulmonary artery sarcoma.

DISCUSSIONS:  Pulmonary artery sarcoma (PAS) is an unusual tumor, with approximately 150 cases reported in the literature since Mendelstamm reported the index case in 1923. More than half of the patients are over the age of 50. Clinical and radiological features mimic those of pulmonary thromboembolic disease, thus, almost half of the time, it is frequently misdiagnosed as pulmonary embolus. By convention, PAS are classified under the common Soft Tissue Tumors (STT) that arises from fat, fibrous tissue, skeletal muscle, neurovascular elements and smooth muscle tissue. The histopathological diagnosis in our patient was Leiomyosarcoma, and the immunohistochemical (IHC) stains were positive for vimentin, smooth muscle actin and muscle actin, conferring a definitive diagnosis of high-grade spindle cell sarcoma. Pulmonary Artery Sarcoma is thought to arise from the multipotential mesenchymal cells of the intima or bulbus cordis. It frequently occurs in the pulmonary trunk. In this case, the lesion arose from the dorsal surface of the pulmonary trunk then spread distally into the right and left pulmonary arteries producing a saddle embolus. Without surgical resection, median survival for patients with PAS is a dismal 1.5 months irrespective of chemotherapy use. Resection independent of adjuvant therapy confers a survival of 10 months to 3.5 years.

CONCLUSION:  Pulmonary Artery Sarcoma is unusual neoplasm, and they are frequently misdiagnosed as pulmonary embolism. It is important to consider PAS as a possibility when a persistent filling defect is present in the pulmonary artery, and there is no response to optimal anticoagulation treatment.

DISCLOSURE:  E.N. Elkady, None.

Chest. 2004;126(4_MeetingAbstracts):960S. doi:10.1378/chest.126.4_MeetingAbstracts.960S

INTRODUCTION:  Mediastinal masses are categorized as either anterior, middle, or posterior based on their location on the lateral chest roentgenogram. Mediastinal tumors show wide variability in histological classification and the frequencies of certain neoplasms differ in adults and children. Sarcomatoid Carcinoma (SCA) is a rare malignant tumor having a mixture of carcinoma and sarcoma containing differentiated mesenchymal elements. It may occur in such diverse locations as the uterus, breast, thyroid, lung, and upper gastrointestinal system. However, up to date a primary mediastinal SCA was only reported once in the literature(1).

CASE PRESENTATION:  A 44 year old African American man with a history of motor vihecle accident 3 years before “had a jaw fracture”, chronic smoking, and alcohol abuse, was admitted with a complain of pain between the scapulae bones, low grade fever, and 7 kg weight loss (all symptoms were for the last 3 months), the chest x-ray (postero-anterior and lateral) showed left side huge posterior mediastinal mass, chest CT showed a large lobulated mass measures 16 x 9 x 10 cm, occupies the posteromedial aspect of the left hemithorax in the paraspinal region extending form the level of the right hemidiaphragm to the clavicular head. A transthoracic core biopsy was done and biopsy showed highly pleomorphic malignant cells, containing spindle cells and huge multi-nucleated cells, mitosis were numerous, immunohistochemical staining was obtained and showed positivity for both carcinoma [Cytokeratin (CK) (CAM 5.2, and AE-1/3)] and Sarcoma [(SMA, Actin, and Vimentin)], and stain negative for Epithelial membrane antigen (EMA), CK 20, and CK-7 (which are markers for Carcinomas), and also negative for Desmin (which is marker for sarcoma). Head CT showed multiple brain lesions suggestive of brain metastasis. The patient was treated with chemotherapy and radiation of the brain lesions.

DISCUSSIONS:  Mediastinal tumors show wide variability in histological classification and the frequencies of certain neoplasms differ in adults and children. Mediastinal tumors have high probability of being malignant and 25–49% malignancy rates are reported(2). Due to this high malignancy rate, diagnostic steps must not be full of details and therapy must be started as soon as possible. The diagnostic approach in these patients mostly includes diagnostic mediastinoscopy with biopsy and thoracotomy, the former being more frequent(3). SCAs of are uncommon neoplasms to which several other historical designations have been given. These include “spindle cell carcinoma,” “carcinosarcoma,” “pleomorphic carcinoma,” and “carcinoma with pseudosarcomatous stroma.” Current concepts regarding this lesion suggest that it is a clonal proliferation which is basically epithelial in nature, but with the potential to undergo divergent differentiation and assume various mesenchymal phenotypes.

CONCLUSION:  In general carcinosarcoma being a mixed tumor can be localized in a wide variety of areas in the body and can originate from various organs including the lungs, organs of the gastrointestinal and the genitourinary systems. However, up to date a primary mediastinal carcinosarcoma has been reported once only in the literature. For all mediastinal tumors with a sarcomatoid pattern, especially a malignant fibrous histiocytoma pattern, extensive samples should be obtained and immunoperoxidase or ultrastructural studies done to identify epithelial differentiation.

DISCLOSURE:  M. Alakhras, None.

Chest. 2004;126(4_MeetingAbstracts):961S. doi:10.1378/chest.126.4_MeetingAbstracts.961S

INTRODUCTION:  Pulmonary intravascular bronchoalveolar tumor (IVBAT) also recognized as pulmonary epithelioid hemangioendothelioma, is a rare malignant vascular tumor of unknown etiology. IVBAT is a tumor of multicentric origin. The lungs are rarely involved, with only 50 cases of pulmonary IVBAT described in the literature. The prognosis is very unpredictable, with life expectancy ranging from 1 to 15 years. We report an unusual case of pulmonary IVBAT that recurred in the lung with metastasis to the mediastinum diagnosed by mediastinoscopy.

CASE PRESENTATION:  A 58-year-old woman presented with right lateral costal margin tenderness of 4 weeks duration that she first noticed while working in her yard. She denied other symptoms. In 1984, she presented with bilateral lung nodules for which a left lower lobe resection was performed. Histology revealed pulmonary IVBAT. She was not offered therapy. Serial chest radiographs demonstrated regression of the lung nodules. She never smoked. On examination, she was in minimal distress due to pain. Her vital signs were normal. She had no lymphadenopathy. She had digital clubbing and tenderness along the right costal margin. There was dullness to percussion in the right infrascapular and lower axillary region with inspiratory crackles. A radiograph and a computerized tomography of the chest revealed a well-circumscribed homogenous mass in the right upper-lobe, posterior segment with mediastinal lymphadenopathy. A positron emission tomography scan revealed intense fluoro-D-glucose uptake in the mass and in the mediastinal lymph nodes. The patient underwent mediastinoscopy which confirmed the histologic diagnosis of pulmonary IVBAT consistent with her previous disease.

DISCUSSIONS:  Pulmonary IVBAT was first described by Dail et al in 1975(1). The tumor was thought to be an aggressive form of bronchoalveolar carcinoma with a propensity to invade adjacent vasculature. Immunohistochemistry and electron microscopic studies demonstrated that IVBAT is of endothelial origin and the tumor was renamed epithelioid hemangioendothelioma. IVBAT is a rare vascular tumor of low-grade malignancy, although cases of more aggressive behavior have been reported. IVBAT is a tumor of multicentric origin that can arise from liver, bone, and soft tissues simultaneously or sequentially. However, lungs are rarely involved. IVBAT is often diagnosed incidentally, as patients are usually asymptomatic or have minor symptoms at the time of diagnosis. There is no single effective treatment. Reports show that some patients respond to chemotherapy with carboplatin plus etoposide while others demonstrate a partial response to interferon therapy(1,2). The prognosis is very unpredictable. Spontaneous regression of the tumor has described for up to 15 years after initial diagnosis without any treatment. Extensive spread to the intravascular, endobronchial or pleural regions as well as the presence of liver nodules and peripheral lymphadenopathy may predict a worse prognosis. The life expectancy may vary from 1 to 15 years. In this case, recurrence of lung IVBAT occurred after 20 asymptomatic years. Our patient had minor symptoms with an incidental diagnosis of lung nodules at both presentations. The current presentation appears to be more aggressive involving mediastinal lymphnodes along with the lung mass. Mediastinoscopy yielded the diagnosis, unlike previous case reports.

CONCLUSION:  Pulmonary IVBAT is thought to be a low grade malignant tumor. However, the prognosis is variable, and the predictability of recurrence is unknown. In our case, we find that the recurrence of this disease with aggressive presentation may occur after a prolonged period of remission.

DISCLOSURE:  T. Chen, None.

Chest. 2004;126(4_MeetingAbstracts):961S-a-962S. doi:10.1378/chest.126.4_MeetingAbstracts.961S-a

INTRODUCTION:  The role of surgical management in T4 non-small cell lung cancer (NSCLC) remains controversial. Treatment is usually palliative, yet a certain proportion of patients may benefit from curative treatment. We report on radical en bloc resection of a right upper lobe (RUL) NSCLC invading the 3rd and 4th thoracic vertebrae.

CASE PRESENTATION:  A 51-year-old white male smoker developed right shoulder and upper back pain. Initial conservative management failed to resolve the pain. Plain films revealed degenerative joint disease. The pain persisted. Computed tomography (CT) and magnetic resonance imaging showed a 5 x 5.1 cm RUL mass invading the right T3-T4 neural foramen and a portion of the T2 vertebral body. CT-guided biopsy revealed squamous cell carcinoma, and mediastinoscopy was negative for N2 disease. Clinical stage was T4N0M0 (Stage IIIB) NSCLC. After neoadjuvant therapy (Taxol/Cisplatin and 5000 cGy), a Neotec scan revealed no evidence of distant disease. Pulmonary function tests were normal. Repeat imaging studies showed no evidence of cord compression and partial tumor response. The patient was taken to the operating room where he underwent radical en bloc resection of the tumor, which included a right upper lobectomy, chest wall and three level vertebrectomy. Spinal integrity was achieved by anterior cage reconstruction followed by posterior rod stabilization. A complete resection was achieved histologically. Three years later, he is well, without radiologic or clinical evidence of recurrence.

DISCUSSIONS:  Surgery remains the ‘gold standard’ for early-stage lung cancer. Stage IIIB NSCLC is considered an inoperable disease; however, Stage IIIB represents a heterogeneous group of patients, including the subgroup of T4N0M0 patients. These patients may benefit from an aggressive surgical approach, oftentimes after neoadjuvant therapy. Several authors have described acceptable outcomes after surgical treatment. DeMeester, et al (1) reported 12 patients with T4N0M0 NSCLC with tumor adherent to the vertebral column who underwent preoperative radiotherapy and radical surgical excision with a 5-year Kaplan-Meier survival of 42%. Grunenwald et al (2) demonstrated a 14% predicted 5-year survival in 19 patients undergoing en bloc partial and total vertebrectomy for lung cancer invading the spine. Other authors (34567) have reported 5-year survival rates ranging from 10-28% in a heterogeneous group of T4 lung cancer patients. Operative morbidity and mortality have been acceptable in this high-risk group of patients. Factors contributing to long-term survival appear to include response to neoadjuvant therapy, ability to achieve complete histologic resection, and absence of N2 or N3 nodal disease.

CONCLUSION:  The role of surgery for Stage IIIB NSCLC remains controversial. Various authors have advocated surgical therapy for certain subsets of Stage IIIB NSCLC. We present a case of T4N0M0 NSCLC invading several levels of the thoracic vertebrae. Aggressive en bloc surgical resection and reconstruction after neoadjuvant therapy resulted in a negative margin resection and long-term survival.

DISCLOSURE:  E.D. Cox, None.

Chest. 2004;126(4_MeetingAbstracts):962S. doi:10.1378/chest.126.4_MeetingAbstracts.962S

INTRODUCTION:  We describe a case of glioblastoma multiforme (GBM) with pleuropulmonary metastasis in whom diagnosis was made with bronchoscopic tranbronchial needle aspiration.

CASE PRESENTATION:  A 63-year-old man referred for evaluation of pleural effusion and lung mass. Initial work up of dyspnea with CXR, CT scan and thoracentesis was done outside. The CT scan of the chest showed a mass and pleural effusion (Figure 1). The effusion was exudative with no malignant cells. CXR from last year was normal. Physical examination revealed findings suggestive of pleural effusion. GBM was diagnosed 18 months ago and treated with surgery and chemoradiation. Despite therapies, he had residual tumor that had remained stable. He has a smoking history of 10-pack year. The day after presentation to our hospital, he underwent bronchoscopy that revealed a distorted RUL bronchus with extrinsic compression of the distal trachea. Multiple transbronchial needle aspirates through the distal trachea were performed. Preliminary diagnosis of non-small cell was made on-site cytology evaluation. Three days later, a repeat thoracentesis was again negative for malignant cells. The smears of fine needle aspirates showed with ill-defined cells and cytological features overlapping with poorly differentiated non-small cell carcinoma of lung. Immunophenotyping on cellblock ruled out lung primary, paragangliomas and melanoma. Tumor cells were reactive for CD56 and positivite for glial fibrilary acidic protein (GFAP) confirming metastatic GBM (Figure 2). After the diagnosis, palliative treatment options were discussed with the patient. He decided not to proceed with any treatment and died in 6 weeks.

DISCUSSIONS:  Although GBM is a locally invasive tumor, distant metastasis is rare. Incidence of metastatic GBM is 0.44% (1). Various organ involvements have been described (2345678910111213). The low incidence attributed to absence of cerebral lymphatics, occlusion of draining venous channels and short survival. Common characteristic feature is prior debulking surgeries in most cases (4, 14). There have been more reports of metastatic cases with gliosarcoma, a variant of GBM (1, 2, 567) DNA studies suggested that systemic metastasis in glioblastoma may represent the emergence of neoplastic subclones (8). Ueda showed the similar patterns of gene expression in primary and metastatic tumors, suggesting the direct infiltration of tumor cells into extracranial blood vessels (3). Pleuropulmonary metastasis of GBM has been reported in 10 cases in the English literature (1,2, 567). Of 8 cases had gliosarcoma. The secondary metastatic GBM case (3) found to have lung metastasis on autopsy. Greif (2) published a case who developed pleural effusion and lung mass after surgery. They made the diagnosis by percutaneous core needle biopsy. Because of the location of the mass, we elected to perform bronchoscopy. Our initial clinical impression was primary lung cancer. Immunophenotyping studies confirmed diagnosis of metastatic GBM. This is the first case in whom lung metastasis diagnosed with needle aspitation. Because of the low number of cells obtained by needle aspirates and similarities of the glioblastoma tumor cells to poorly differentiated other tumors, preparation of cellblock for further immunophenotyping studies may improve the diagnosis of metastatic GBM.

CONCLUSION:  Pleuropulmonary metastasis of GBM can be made with transbronchial needle aspirates. Early diagnosis and palliative treatment may have impact on survival.

DISCLOSURE:  N. Gupta, None.

Chest. 2004;126(4_MeetingAbstracts):962S-a-963S. doi:10.1378/chest.126.4_MeetingAbstracts.962S-a

INTRODUCTION:  We report an unusual case of partial airway obstruction resulting from distant metastasis of a clear cell carcinoma of the kidney. This patient had no prior diagnosis of cancer before the tracheal mass was endoscopically resected using a holmium:YAG laser, and post-resection work-up revealed the primary kidney tumor.

CASE PRESENTATION:  A healthy 54-year-old man developed a persistent cough, progressive shortness of breath and paroxysmal nocturnal dyspnea. He was treated for new onset asthma, but his symptoms persisted. Despite a normal chest x-ray, chest computed tomography (CT) demonstrated a 15-20mm polypoid lesion in the distal trachea. Intra-operative flexible bronchoscopy revealed a white, firm lesion in the distal trachea with partial airway obstruction. The 1.5 x 1.0 cm mass was densely adherent to the tracheal wall, and was endoscopically excised using a holmium:YAG laser. His symptoms improved considerably. Pathologic diagnosis demonstrated metastatic renal clear cell carcinoma. Work-up demonstrated a 3.5 x 5.6 cm mass in the right kidney by CT. The patient had a left nephrectomy as a child secondary to chronic infections. Therefore, an attempt was made to spare as much normal kidney as possible, and he underwent a complex right partial nephrectomy. Pathology confirmed multifocal renal cell carcinoma, clear cell type, nuclear grade 2. Six weeks following resection, he developed rib and lung lesions. He was treated with high-dose Interleukin-2, and is being followed closely.

DISCUSSIONS:  Metastatic tumors to the tracheobronchial tree are highly unusual, and occur in 2% of patients with solid tumors [1]. Most patients with tracheobronchial metastases have a primary diagnosis of cancer prior to identifying the metastases. Tumors of the breast, colon, rectum, pancreas, kidney, thyroid, and skin have been shown to metastasize to the tracheobronchial tree [2,3]. These tumors are often diagnosed late in the course of the disease because of the delayed onset of specific symptoms. The large diameter of the trachea, relative to the bronchi, allows tracheal tumors to grow to large sizes before symptoms can become significant. In contrast to lung parenchymal metastases, the significantly lower number of reported tracheal metastases is thought to be secondary to a difference in blood supply. The lung parenchyma is supplied by the pulmonary artery, through which all systemic blood passes, while the trachea is supplied by the bronchial artery. The bronchial artery receives only a small portion of the systemic circulation. Thus, hematogenous spread is more likely to favor the lung parenchyma. Commonly, metastatic lesions to the tracheobronchial tree are located distal to the carina and cause atelectasis in the affected segmental lung field. Plain chest radiographs can often demonstrate these lesions, but CT is also helpful for making the diagnosis. Laser bronchoscopy and resection have become increasingly useful in excising the tumor and in obtaining a tissue diagnosis.

CONCLUSION:  There are few reported cases in the literature of metastatic tumors to the trachea, and none of these have involved finding the metastatic lesion in a patient without a prior diagnosis of cancer. Laser bronchoscopy is an effective and straightforward treatment method. Control of the primary tumor needs to be aggressively pursued while addressing the tracheobronchial metastasis.

DISCLOSURE:  G.H. Wheatley, None.

Chest. 2004;126(4_MeetingAbstracts):963S. doi:10.1378/chest.126.4_MeetingAbstracts.963S

INTRODUCTION:  Cryptogenic Adult Bronchiolitis is a rare clinicopathologic syndrome that represents a diagnostic challenge to clinicians. The pathologic findings are typical for cellular obliterative bronchiolitis, with acute and chronic inflammatory infiltrates in the bronchioles while the alveolar parenchyma is spared.[1] Cases are labeled cryptogenic when cultures and serology for infectious sources are negative and no etiology is elicited. The incidence and pathogenesis are unknown, though the disease tends to occur in middle-aged women who present with non-productive cough for several months.[1] Patients usually have a progressive course and there are no proven therapies. [2] Macrolide antibiotics have been shown to have dramatic effects on survival when used to treat patients with Diffuse Panbronchiolitis (DPB).[3] Given the current lack of effective therapy for Cryptogenic Adult Bronchiolitis, it seems reasonable to try long-term macrolide therapy in this unique patient population.

CASE PRESENTATION:  A 46 year-old woman was evaluated in October 2003 for chronic cough of three months duration, without any apparent precipitating event, that did not respond to conventional therapies, including cough suppressants, short course antibiotics (azithromycin for five days), reflux medications and asthma inhalers. She had no significant past medical history and was otherwise in good health. Her clinical exam was normal except for diffuse, end-inspiratory squeaks. Pulmonary Function Tests revealed a restrictive pattern (Fig. 1). Initial chest CT showed increased interstitial markings, peripheral bronchiectasis and a mosaic pattern seen only on expiratory views. Laboratory testing was significant for negative ANA and RF. A bronchoscopy with lavage was performed and all subsequent cultures were negative, including viral, legionella, mycoplasma and mycobacterial. The patient was empirically started on prednisone 40mg qd, but after one month of therapy, she denied any symptomatic improvement and her pulmonary function tests were unchanged (Fig. 1). In addition, she developed an elevated fasting glucose and the steroids were discontinued. The patient underwent a surgical lung biopsy that revealed a patchy bronchiolocentric inflammatory process with minimally involved alveolar parenchyma. Airway lumens were distorted and narrowed by lymphoplasmacytic and neutrophilic infiltrates. (Fig 2) The clinical and pathologic diagnoses were consistent with Cryptogenic Adult Bronchiolitis. The patient recovered quickly from surgery, but still complained of chronic cough and limited exercise tolerance. She was empirically started on Clarithromycin 250mg bid. After one month of treatment, she stated that her cough had diminished and her dyspnea was improved. Pulmonary function tests were repeated and demonstrated improvement in FEV1, FVC, FEF 25-75%, and TLC (Fig. 1).

DISCUSSIONS:  The cryptogenic presentation of bronchiolitis remains a diagnostic and therapeutic mystery. The pathologic findings, as well as the response to long-term macrolides in the case described, raises the suspicion that Cryptogenic Adult Bronchiolitis may have an unidentified infectious etiology or an undefined immune mechanism. The immunomodulatory activity of macrolides has been demonstrated in several pulmonary disorders, including Cystic fibrosis, Asthma, and DPB. DPB, which had a 26% five year survival rate in 1984 before the use of macrolides, now has a 10 year survival rate of 94%.[3] Most patients experience a clinical and physiologic improvement within the first month of treatment.

CONCLUSION:  We believe this case is the first report using long-term macrolide therapy in patients with Cryptogenic Adult Bronchiolitis. The mechanism of disease and therapy are unknown, but may be related to DPB given the similarity in response. Further studies are warranted.

DISCLOSURE:  B.D. Gelbman, None.

Chest. 2004;126(4_MeetingAbstracts):964S. doi:10.1378/chest.126.4_MeetingAbstracts.964S

INTRODUCTION:  Broncholithiasis is a rare condition characterized by the presence of calcified material within the lumen of the tracheobronchial tree. This material can have multiple origins, but by far the most common is as a result of previous fungal, mycobacterial, or actinomyces infection. We report a case of bilateral post-obstructive pneumonia and respiratory failure secondary to broncholithiasis in a patient with known bilateral cystic bronchiectasis and a history of tuberculosis.

CASE PRESENTATION:  A 51 year old male with a history of remote tuberculosis infection was transferred to this institution after being admitted to an outside hospital for pneumonia leading to respiratory failure. He was intubated and bronchoscopy demonstrated a “foreign body” in the superior segment of the right lower lobe with purulent material behind it. The patient was sent to this hospital for a repeat bronchoscopy. CT scan revealed calcified material within the airways of both superior segments of the lower lobes with associated post-obstructive infiltrates. Bronchoscopy was performed via the endotracheal tube with a flexible bronchoscope. Bilateral broncholiths within the superior segment airways were encountered. With close inspection, the broncholiths were slightly embedded in the airway wall. Using endoscopy forceps, the broncholiths were removed without complication (Graphic 1). The purulent secretions were suctioned and sent for culture. The patient had rapid resolution of his respiratory failure and was able to be extubated within 24 hours. On histologic examination, the presence of actinomyces was confirmed. The origin of his broncholithiasis could be related to both the remote tuberculosis and superimposed actinomyces infection. The patient was seen in followup approximately one month later and was doing well. He was without supplemental oxygen requirement and completed a 28 day course of antibiotic therapy for the actinomyces.

DISCUSSIONS:  Hemoptysis, infection, abscess formation, and respiratory failure have been reported as a direct consequence of broncholithiasis (2,3). This case demonstrates two of the complications of broncholithiasis (post obstructive pneumonia and respiratory failure) and the ability to resolve these complications using flexible rather than rigid bronchoscopy or surgery despite the fact that the broncholiths were partially embedded in the airway. Controversy remains throughout the surgical and medical communities on the definitive optimal management of broncholithiasis. The vast majority of broncholiths that are managed nonsurgically by extraction (broncholithectomy) are done so using rigid bronchoscopy (1). Surgical approaches including thoracotomy and lung resection have been recommended for many cases, but also carry the risks of major thoracic surgery. Retrospective studies have demonstrated that the incidence of massive hemorrhage (the most frequent concern among physicians) and other postoperative complications of broncholithectomy via bronchoscopy were low (1). However, the majority of these cases were performed with rigid bronchoscopy. We contend that partially embedded broncholiths can be safely removed using flexible bronchoscopy.

CONCLUSION:  Even broncholiths that are partially embedded in the airways are amenable to removal with a flexible bronchoscope and endoscopy forceps.

DISCLOSURE:  J.W. Toth, None.

Chest. 2004;126(4_MeetingAbstracts):964S-a-965S. doi:10.1378/chest.126.4_MeetingAbstracts.964S-a

INTRODUCTION:  Laryngeal tuberculosis (TB) is an uncommon and a highly virulent form of tuberculosis. We present a case of a patient with dyspnea, weight loss, and a newly diagnosed supraglottic mass.

CASE PRESENTATION:  A 39 year old male with no significant past medical history presented with dysphonia and dyspnea. A direct laryngoscopy one month prior to admission demonstrated a supraglottic mass involving the pre-epiglottic space, epiglottis and piriform sinus (Figure 1). Biopsy of the mass revealed granulomatous inflammation with necrosis and ulceration. Tissue stains for fungus and acid fast bacilli (AFB) were negative. The patient’s symptoms continued to worsen. He was subsequently admitted with complaints of progressive dyspnea, fatigue and a 20 pound weight loss. There is no history of hemoptysis or night sweats. Social history revealed that he has no known exposures to asbestos or tuberculosis, however, he did spend 90 days in jail over 12 months ago. A PPD placed two months prior at his primary care physician’s office was negative. The patient is employed as an insulator, working with fiberglass and using a mask approximately 50% of the time. Initial work up revealed significant arterial hypoxemia and a chest X-Ray demonstrated signs of apical bullous emphysema and bilateral micronodular infiltrates. A chest CT was performed which showed severe interstitial lung disease with a fine reticulonodular pattern in the upper lobes, bullous emphysematous changes, nodular calcifications and cavitary lesions. The largest cavitary lesion was located in the left upper lobe (Figure 2). No lymphadenopathy was noted. Initially the patient did not have any sputum production and was subsequently referred for bronchoscopy for diagnostic evaluation. A bronchoalveloar lavage (BAL) of the left upper lobe was performed. A protected brush specimen and forceps biopsies were obtained from the same area. The BAL gram smear was positive for AFB. A PPD placed in the hospital became positive.

DISCUSSIONS:  Upper airway structures can be infected with Mycobacterium tuberculosis. Structures such as the trachea, larynx, epiglottis and nasopharynx are exposed to organisms in the expectorated sputum of patients with active pulmonary cavitary lesions. Laryngeal TB is uncommon with relatively few cases reported in the literature. Occasionally this disease may be associated with pulmonary cavities. Hoarseness, cough and sore throat represent the most common symptoms of laryngeal TB. Pathology may reveal ulceration or necrotizing granuloma. This patient was started on quadruple therapy (rifampin, isoniazid, pyrazinamide, and ethambutol) and felt significant improvement within 48 hours.

CONCLUSION:  Laryngeal tuberculosis is a highly infectious yet treatable disease that responds well to therapy. The right clinical scenario and high index of suspicion allow for earlier diagnosis and subsequent treatment.

DISCLOSURE:  J.C. Reap, None.

Chest. 2004;126(4_MeetingAbstracts):965S. doi:10.1378/chest.126.4_MeetingAbstracts.965S

INTRODUCTION:  Tracheopathia osteoplastica (TPO) is a disease of the trachea that is not well recognized and under diagnosed. We present a case of TPO with the novel treatment of tracheal stent placement under flexible bronchoscopy.

CASE PRESENTATION:  A 65-year-old Caucasian male presented with persistent cough and progressive dyspnea over 10 months. He reported decreased exercise tolerance but denied orthopnea, chest pain, pleuritic pain, weight loss and other constitutional symptoms. He took multiple courses of antibiotics for intermittent fever and purulent sputum. Past medical history included rheumatic fever, asbestos pleural plaques, chronic bronchitis, and moderate copd after a 60 pack-year tobacco history. He had no family history of pulmonary disease. He has no known drug allergies. Medications included prednisone 5mg, guaifenex, albuterol and atrovent nebulizer therapy, and flovent. Physical examination revealed a healthy male. Blood pressure was 137/75, pulse of 87, respiratory rate of 24, and oxygen saturation of 97% on 2L nasal cannula. His lungs had coarse breath sounds with good air movement, no audible stridor, normal inspiratory to expiratory ratio. He had neither clubbing nor peripheral edema. The remainder of his exam was normal. Laboratory data noted normal blood work. Pulmonary function tests demonstrated an FEV1=1.8, FVC=4.0 with ratio of 46%, TLC=8.1 (128%), DLCO=14 (66%) and normal flow-volume loops. Chest CT revealed tracheomalacia. Sputum cultures were negative. However, flexible bronchoscopy revealed marked mid- to distal tracheal stenosis with nodules and cartilaginous tracheal ring hypertrophy, and fishmouthing of the right and left main ostia. Needle biopsies revealed cartilaginous hyperplasia.

DISCUSSIONS:  Differential diagnosis for tracheal stenosis includes malignancy (bronchogenic, laryngeal, esophageal and thyroid carcinomas, Hodgkin’s lymphoma), endotracheal intubation or tracheostomy removal, idiopathic laryngealtracheal stenosis, tuberculosis tracheobronchial stenosis, mucopolysaccharidoses, relapsing polychondritis, Sjogren’s syndrome, Wegener’s granulomatosis, tracheal amyloidosis, saber sheath trachea, and TPO. Pathologic results of cartilaginous hyperplasia established the diagnosis of TPO. TPO is a segmental, degenerative disorder of the tracheobronchial tree characterized by multiple submucosal cartilaginous and osseous nodules causing narrowing of the upper respiratory tract(1). TPO typically involves the first tracheal ring to the carina, but sparing the posterior wall. There is neither gender nor familial predominance. Etiology of TPO is unknown - theories range from elastic tissue neoplasia to chemical or mechanical irritation inciting cartilaginous and osseous metaplasia(2). As the disorder is chronic and often asymptomatic, estimates of incidence vary between 1/2000 bronchoscopies and 1/400 at autopsy(3). Symptoms include cough, dyspnea, chronic bronchitis, purulent sputum, hemoptysis and airway compromise(4). Abnormalities of pulmonary function tests depend on the degree of airway stenosis and can be misleadingly normal. Therefore, diagnosis is dependent on bronchoscopy and pathology. To date, no specific medical therapy is available, with the mainstay of treatment hydration, humidification, mucolytics, antibiotics and expectorants. Optimal management of airway compromise entails rigid bronchoscopy with dilation and removal of obstructing spurs. Use of laser therapy has shown mixed results(5,6). Complete segmental tracheal resection was reported as treatment in one case of massive TPO(7).

CONCLUSION:  We report the first case of TPO treated successfully by tracheal and bronchial stenting with flexible bronchoscopy. At recent follow-up, our patient’s dyspnea remains resolved.

DISCLOSURE:  D.K. Loo, None.

Topics: stent , trachea
Chest. 2004;126(4_MeetingAbstracts):965S-a-966S. doi:10.1378/chest.126.4_MeetingAbstracts.965S-a

INTRODUCTION:  Lithium is a medication that is associated with a multiplicity of potential adverse effects. We report the first case of lithium-induced stridor resulting in respiratory failure requiring mechanical ventilatory support.

CASE PRESENTATION:  The patient is a 58-year-old female with a prior medical history of depression for which she was treated with lithium for over 25 years. She was admitted on 3/31/04 after a suicide attempt when she overdosed on lithium. She was noted to have severe stridor and required immediate intubation and mechanical ventilatory support. During the intubation under visualization by direct laryngoscopy, the vocal cords were extremely edematous. Upon presentation to the Medical Intensive Care Unit, the patient was hemodynamically stable and was oxygenating well with a pulse oximetry of 100% with on FIO2 of 35%. She exhibited severe encephalopathy and was not arousable even to painful stimulation. The deep tendon reflexes, however, were hyper-reflexic. Laboratory data revealed an elevated lithium level of over 4 and renal failure with a BUN of 37 and a creatinine of 2.1. She underwent urgent hemodialysis twice within 24-hours of admission for a total of 7 hours of dialysis in order to bring down the lithium levels below 2. By the next day she was responsive to tactile stimulation but not verbal command. She continued to demonstrate hyperreflexia and the lithium levels ranged between 1.2 and 1.8. She underwent a spontaneous breathing trial using pressure support of 12 and on an FIO2 of 30% demonstrated adequate ventilation and oxygenation. She was therefore extubated but immediately developed severe stridor requiring re-intubation. She was treated with intravenous solumedrol 60 mg every 6 hours for the next three days. She again underwent a successful spontaneous breathing trial, and an air leak was demonstrated when the cuff was deflated. She was extubated, but once again developed severe stridor and required immediate re-intubation. Steroids were continued for another 72 hours. At that time she underwent a fiberoptic bronchoscopy, which revealed persistence of vocal cord edema and therefore she continued to require intubation and mechanical ventilatory support. After 13 days of steroid therapy, fiberoptic bronchoscopy revealed that the vocal cord edema has disappeared and she was successfully extubated.

DISCUSSIONS:  Lithium is a drug with a very narrow therapeutic index. Although it is the most effective therapy for patients with bipolar disorder, it must be monitored carefully to prevent the development of toxicity. The most common manifestations of lithium toxicity include neuromuscular irritability, diarrhea, tremulousness, encephalopathy, renal failure and nephrogenic diabetes incipidus. Our patient demonstrated renal and neurological manifestations of toxicity, but presented with a previously undescribed manifestation of lithium toxicity, namely stridor. Stridor was severe and prolonged and subsided only after multiple courses of dialysis to completely remove the lithium from the body and the use of a prolonged course of high dose intravenous steroids to reduce the inflammation of the vocal cords. It is difficult to know when it is safe to extubate patients who have developed stridor. There is conflicting data in the literature concerning the predictive value of deflating the endotracheal tube balloon and listening for an air leak. This case demonstrates the lack of utility for deflating the balloon and determining an air leak in that reliance on this test resulted in premature extubation and a difficult re-intubation. Fiberoptic endoscopy demonstrated resolution of the vocal cord edema and correctly predicted a successful weaning outcome.

CONCLUSION:  Vocal cord edema leading to severe stridor is an uncommon manifestation of lithium toxicity.

DISCLOSURE:  V. Baimeedi, None.

Chest. 2004;126(4_MeetingAbstracts):966S-967S. doi:10.1378/chest.126.4_MeetingAbstracts.966S

INTRODUCTION:  Human monocytic ehrlichiosis (HME) can be a life threatening illness and requires a high index of suspicion for early diagnosis. It is caused by Ehrlichia chaffeensis and principally transmitted by the Lone Star Tick (Amblyomma americanum). We report a case of severe acute HME complicated by septic shock, adult respiratory distress syndrome (ARDS), and disseminated intravascular coagulation (DIC) associated with trimethoprim-sulfamethoxazole (TMP-SMX) therapy.

CASE PRESENTATION:  A 19 year old white female with past medical history notable for frequent episodes of sinusitis was in her usual state of health until two weeks prior to admission when she developed rhinorrhea, headache, and fever. One week prior to admission she was diagnosed with sinusitis and treated with TMP-SMX. Symptoms continued to progress until one day prior to admission she developed nausea, vomiting, stiff neck, and malaise and was admitted to an outside facility. She attended college in Chapel Hill, NC and was on the national swim team, but presented in Florida on summer vacation. She had no known history of tick exposure. Pertinent exam findings included fever, tachycardia, decreased level of arousal, meningismus, diffuse muscle tenderness, hepatosplenomegaly, and the absence of rash. Laboratory evaluation was notable for pancytopenia with a left shift, mild azotemia, and elevated liver tests. A non-contrast head CT was unremarkable, and CSF examination revealed a monocytosis with elevated protein and a normal opening pressure. She was treated with vancomycin, ceftriaxone, doxycycline, and acyclovir and transferred to our facility. Review of prior CSF sample revealed monocytes with intraleukocytic morulae, diagnostic of HME (Figure 1). Bone marrow aspirates revealed similar findings (Figure 2). Titers for Ehrlichia chaffeensis were markedly elevated. Antibiotics were continued, however her course was complicated by ARDS, DIC, and multi-organ failure. She died despite maximal supportive efforts. Post-mortem examination revealed findings consistent with septic shock and DIC.

DISCUSSIONS:  The acute form of HME caries an overall mortality rate of 2-5%. Clinical features include a non-specific illness characterized by fever, malaise, myalgia, nausea, and vomiting. Lab abnormalities are prominent and include leukopenia, thrombocytopenia, elevated transaminases, and elevated lactate dehydrogenase (LDH). The differential diagnosis includes Rocky Mountain spotted fever, meningococcemia, viral illnesses (including acute mononucleosis and hepatitis A), hematological malignancy, and thrombotic thrombocytopenic purpura. Prompt diagnosis is critical, as delayed treatment with doxycycline or chloramphenicol results in increased morbidity and mortality (1). Treatment of HME with TMP-SMX has been reported to worsen the course the disease by an unknown mechanism, and was associated with ARDS and DIC in a case report (2). The association of TMP-SMX with ARDS has been previously reported with rickettsial infections and has also been shown to worsen Mediterranean spotted fever, illnesses caused by organisms closely related to ehrlichia species (3).

CONCLUSION:  Acute HME is a severe life threatening illness that mimics multiple other diseases. The diagnosis requires a high index of suspicion and treatment with doxycycline or chloramphenicol should be initiated early. Treatment with TMP-SMX appears to worsen outcomes due to an association with ARDS and DIC.

DISCLOSURE:  S.M. Rowe, None.

Chest. 2004;126(4_MeetingAbstracts):967S. doi:10.1378/chest.126.4_MeetingAbstracts.967S

INTRODUCTION:  Septic shock is one of the most common reasons that patients require intensive care unit (ICU) admission. We present a patient that had all the markings of septic shock, but a careful review of the data revealed a rare alternate diagnosis.

CASE PRESENTATION:  A 20-year-old female was admitted to the hospital with a complaint of right knee swelling, pain, and erythema. Her past medical history was significant for an occasional arthritic condition. The patient underwent aspiration of the right knee and was given vancomycin and ceftriaxone for suspected septic arthritis. Labs on admission were significant for a white blood cell (WBC) count of 19.4 x 109/L with 56% neutrophils and 32% bands, and C-reactive protein 196 mg/dl. Renal and liver function were essentially normal. All joint aspirate cultures and blood cultures were negative, and no crystals were observed. WBC count of the knee aspirate was 90,000 x 109/L (neutrophils 91%). Anti-nuclear antibody, double stranded DNA, rheumatoid factor, and HIV testing were all negative. The hospital course was marked by recurrent fevers, development of cervical and inguinal lymphadenopathy, increasing bandemia, anemia, elevation of liver function tests (LFTs), coagulopathy, and worsening renal failure. On the 11th day of hospitalization the patient had a generalized tonic clonic seizure. She became hypotensive, was transferred to the ICU and intubated. The physical exam upon arrival to the ICU showed cervical and inguinal lymphadenopathy. Her WBC count 6.9 x 109/L (bands 88%), hemoglobin 6.9 g/dl, platelets 115 x 109/L, internationalized normalized ratio 2.77, aspartate aminotransferase (AST) 2653 U/L, total bilirubin 2.9 mg/dl, direct bilirubin 1.7 mg/dl, albumin 1.8 g/dl, creatinine 3.5 mg/dl, CRP 24 mg/dl, and ferritin level 152,197 ng/ml. Peripheral blood smear showed no evidence of hemolysis. The initial ICU course was remarkable for hypotension requiring support with norepinephrine, continued mechanical ventilation, worsening liver and renal function, and institution of hemodialysis, and initiation of broad-spectrum antibiotic coverage. The history of a poorly categorized arthritis with the above presentation and laboratory data, combined with persistently negative cultures led to a diagnosis of juvenile idiopathic arthritis complicated by macrophage activation syndrome (MAS). This diagnosis was confirmed by bone marrow aspirate and biopsy that displayed macrophages actively phagocytosing hematopoietic cells (see figure). Methylprednisolone 1 gm IV daily and cyclosporine A 4 mg/kg/day were administered. By hospital day 20, the patient had been extubated. She no longer required blood products or hemodialysis; both her coagulopathy and LFTs improved and she was transferred to the general medical floor.

DISCUSSIONS:  MAS is a rare complication of childhood systemic inflammatory disorders, such as juvenile idiopathic arthritis (JIA), that can present acutely and dramatically. Clinical features include high fever, elevated serum liver enzymes, coagulopathy, pancytopenia, and lymphadenopathy. As in our patient, neurologic, renal, and pulmonary involvement has been reported. The patient may show a paradoxical improvement in the signs and symptoms of the underlying inflammatory disease at the onset of MAS. The pathognomonic feature is a bone marrow biopsy showing hematophagocytosing macrophages. The primary modality of treatment is high dose parenteral corticosteroids, but cyclosporine has been shown to be helpful in steroid resistant cases. Other agents such as intravenous immunoglobulins, cyclophosphamide, plasma exchange, and TNF-alpha inhibitors have had variable success.

CONCLUSION:  MAS is one of the many diseases that can present with a systemic inflammatory response that mirrors the signs of sepsis. This case emphasizes the importance of maintaining a healthy suspicion for other unusual disorders in the intensive care unit.

DISCLOSURE:  A.J. Blaivas, None.

Topics: sepsis , septicemia , shock
Chest. 2004;126(4_MeetingAbstracts):967S-a-968S. doi:10.1378/chest.126.4_MeetingAbstracts.967S-a

INTRODUCTION:  The maculopapular rash associated with multisystem organ failure in immunosuppressed patients is commonly caused by bacterial, fungal, and viral infections. Among the fungal organisms, Trichosporon cutaneum (TC) is an unusual, but fatal cause of disseminated infection in patients with hematologic malignancies. We present a case of disseminated trichosporonosis in a patient with acute leukemia following chemotherapy.

CASE PRESENTATION:  A 60-year old man with acute lymphoblastic leukemia (ALL) was transferred to the intensive care unit (ICU) with multisystem organ failure and multiple erythematous maculopapular lesions on the skin of the trunk and upper extremities. In the three days prior to ICU admission, he was treated with intravenous dexamethasone and cytarabine, and intrathecal methotrexate. ICU supportive care included mechanical ventilation, continuous renal replacement therapy, blood products and broad-spectrum antimicrobials. Within 24 hours of ICU admission the patient became neutropenic. Antifungal therapy (liposomal amphotericin) was initiated empirically on ICU day 4. Etoposide was administered on ICU day 5. Cultures of tracheal aspirates and blood samples collected on ICU day 3 were reported as positive for Aspergillus fumigatus and Candida tropicalis on ICU days 7 and 8, respectively. Caspofungin was then added. However, on ICU day 14, Caspofungin was discontinued and Voriconazole was started, when blood cultures obtained on ICU days 5-9 were reported as positive for TC. Subsequently, skin biopsies demonstrated purpura; however, the skin cultures were negative. GCSF was added on ICU day 14 and neutropenia was resolved by day 18. Despite ongoing double antifungal therapy, blood cultures obtained on ICU days 13 and 17, were still reported as positive for TC on ICU days 21 and 22. The patient remained in refractory shock with multisystem organ failure and expired on ICU day 23.

DISCUSSIONS:  TC may cause an unusual but fatal infection in immunocompromised patients, particularly those receiving chemotherapy for hematologic malignancies. The febrile maculopapular rash and multisystem organ failure should raise suspicion for this unusual pathogen. TC is ubiquitous in the environment and can present as superficial skin infection or as an invasive localized or disseminated infection. The cutaneous lesions appear as purpuric maculopapules and nodules of the trunk and extremities. The systemic infection may present as persistent fever, respiratory dysfunction with bilateral non-specific infiltrates, and renal and hepatic insufficiency or failure. Typical of many fungal species, there is a long lag time between specimen collection and positive culture report for TC. It takes several days for TC to grow as yeast on the BD Bactec Plus media. Final identification requires subculture on special fungal media. In our patient, it took 9 days to grow TC and all the cultures were reported as positive on the same day probably because of differences in fungal loads. In immunosuppressed patients, the mortality rate of disseminated trichosporonosis is as high as 80%. There is no current consensus on the selection of appropriate antifungal therapy, especially in neutropenic patients. In the pre-azole era, amphotericin and flucytosine were the favored agents. However, resistance to amphotericin has been reported and flucytosine is contraindicated in neutropenic patients. The Echinocandins (Caspofungin) are ineffective. In contrast, the Azoles (Voriconazole) alone or in combination with amphotericin appear to offer therapeutic promise. When clinically appropriate, neutropenia should be actively treated with granulocyte stimulating factors. Unfortunately, despite following the accepted treatment protocol, our patient expired. Earlier institution of empiric therapy by combining amphotericin and voriconazole may have contributed to a better outcome.

CONCLUSION:  Novel strategies for rapid diagnosis and treatment of disseminated trichosporonosis are necessary to identify and decrease the high fatality rate associated with this condition.

DISCLOSURE:  S. Gaeta, None.

Chest. 2004;126(4_MeetingAbstracts):968S-969S. doi:10.1378/chest.126.4_MeetingAbstracts.968S

INTRODUCTION:  Streptococcus pneumonia is the most common cause of community-acquired pneumonia. Local complications include pleural effusion, empyema and pericarditis. Endocarditis, septic arthritis and meningitis can occur secondary to bacteremia. In sever cases disseminated intravascular coagulation can happen with bleeding and thrombosis. Systemic peripheral gangrene (purpura fulminans) has been described as a rare complication.

CASE PRESENTATION:  23 years old white male presented to the emergency room with productive cough, nausea, vomiting, abdominal pain and abdominal distention. He has past medical history of acute lymphoblastic leukemia at age five treated with chemotherapy and radiation and he has been in remission since that time. He had inguinal hernia repair as a child. He was not taking any medication. He did not drink, smoke or use illicit drugs. On physical exam he was drowsy, His blood pressure 124/60 mmHg, pulse 130 beat per minute, respiratory rate was 26 breaths per minute and his pulse oximetry was 100% on room air. He had bilateral basal crackles on his lung exam. His abdomen exam was consistent with acute abdomen (distended, rigid, rebound tenderness). He had trace lower extremity edema. His labs were white cell count 2800/cmm with 85% neutrophills, hemoglobin 12.9 g/dL, platelets 37,000/cmm, sodium 137 mEq/L, potassium 4.3 mEq/L, chloride 104 mEql/L, bicarbonate 16 mEql/L, blood urea nitrogen 30 mg/dL, Creatinine 1.4 mg/dL, glucose 47 mg/dL, INR 1.9, PTT 29.3 second, PT 22.1 seconds and lactate 8.7 mmol/L. His arteril blood gas was PH 7.22, PCO2 26.9 mmHg and PO2 70.8 mmHg on room air. He was found to be hepatitis C positive. His chest x-Ray showed Left lower lobe infiltrate with small effusion. CT scan showed pneumonic consolidation of the left lower lobe, cirrhosis of the liver, splenomegaly, thickening of the ascending, descending colon walls and terminal ileum. He was given Intravenous fluids, wide spectrum antibiotics (to cover for community acquired pneumonia and gut ischemia), bicarbonate drip for his metabolic acidosis. Surgical consult was obtained. He was taken to the operating room where he underwent right hemicolectomy secondary to ischemia and necrosis. His blood cultures came back positive for streptococcus pneumonia on his second hospital day. He went to the operating room multiple times for anasomosis of the bowels and stepwise closure of his abdomen. He was extubated on his sixteenths hospital day. He did well for two weeks before he was intubated and placed on mechanical ventilation. Patient condition continued to deteriorate after that secondary to liver failure, kidney failure and vancomycin resistant enterococcus sepsis.He died after long hospital stay. The pathology form the right colon came back as tansmural acute inflammation and hemorrhagic necrosis with associated acute inflammation of vessels wall, thrombosis and numerous diplococci.

DISCUSSIONS:  In this case the patient was not hypotensive or on inotropic agent that may contribute to bowel ischemia. This leavs pneumococcal pneumonia bacteremia with invasion and thrombosis of blood vessels the cause of bowel ischemia.

CONCLUSION:  There have been few reports about symmetrical peripheral gangrene (purpura fulminans) as a complication of pneumococcal sepsis. I would like to add bowel ischemia as an unusual complication of pneumococcal pneumonia. Physicians shuold keep in mind that in cases of sever pneumococcal sepsis blood vessels invasion by pneumococcua and thrombosis of these vessels can lead to serious ischemic complications.

DISCLOSURE:  K.R. Al Khasawneh, None.

Chest. 2004;126(4_MeetingAbstracts):969S. doi:10.1378/chest.126.4_MeetingAbstracts.969S

INTRODUCTION:  ACS exists when Intra-abdominal Hypertension(IAH) is associated with clinically observed organ dysfunction. ACS adversely affects multiple organ systems. Normal Intraabdominal pressure(IAP) is atmospheric or subatmospheric. Subclincial organ effects progress to overt clinically manifest organ dysfunction beginning with IAH > 10 mm Hg. Pre-renal effects and Direct Renal Parencymal compression are the mechanism of renal failure. Direct ureteral compression is not implicated in renal dysfunction even with IAP∼40mm Hg.(5) Lovenox(LMWH) is infrequently reported (<1%) to cause spontaneous retroperitoneal bleeding and rarely caused ACS(3).

CASE PRESENTATION:  49 year old female with Refractory Stage IV Large B-cell Non-Hodgkin’s Lymphoma(NHL), DVT in left lower extremity 2 months ago and receiving Lovenox 80 mg SQ BID developed increasing abdominal pain with wall distension and hematoma over a day. The patient developed progressive hypotension, syncope and decreased urine output, progressing from oliguria to anuria. Bladder pressure measured in the supine position via the Foley’s cathether revealed a pressure of 24 mm Hg. Pertinent Physical : T 98, BP 90/50 on Levophed, RR 20, HR 115 Abdomen Anterior abdominal wall distension, Ecchymosis and tenderness over both the lower quadrants, hard to palpation Rectal Exam : Guaic Negative Brown Stools Ext : cool Labs : ( change over 24-48 hours) Hb : decreased from 10 to 6.7 Cr increased from 0.6 to 1.7 PT: 17 INR 1.6 PTT 31 Pan-cultures : Negative CT Abdomen and Pelvis : 09/16/03 : Large organizing hematoma 15 x 15 x 13 cm extending in the anterior abdomen upto the level of umbilicus. Compression of the bladder and ureters bilaterally with hydronephrosis 09/19/03 : Increase in size; Resolution of the previously seen bilateral hydronephrosis after ureteral stents. Day # 1 : Anuria despite aggressive crystalloid resuscitation. Cytoscopy and Retrograde Pyleogram reveals Bilateral Hydronephosis. Bilateral Ureteral Stenting performed. Day 2 & 3 : Oliguria Day 4 and 5 : UO increases by 1000 cc/day.

DISCUSSIONS:  Incremental reductions in GFR and renal plasma flow are observed with graded elevations in the IAP. Oliguria at IAP of 15 mm Hg progressing to Anuria at IAP > 30 mm Hg has been reported. Direct ureteral compression does not seem to play a significant role. In animal models, where IAH was induced by implating inflatable bags in the peritoneum, ureteral stents had no impact on renal function and excretory urograms have shown no obstruction(5). Neurogenic Bladder, Bladder Trauma or Compression from a pelvic hematoma may yield inaccurate bladder estimates of the IAP. The bladder pressure in this case may inaccurately overestimate the true IAP from direct bladder compression by the overlying pelvic hematoma. In such settings, other indirect, albeit less preferred methods of IAP measurement via the nasogastric tube may be more accurately reflective of the true IAP. Hydronephrosis has not been reported to occur alongwith ACS. This patient’s de novo hydronephrosis is attributable from direct compression of the bladder and both the ureters. Hydronephrosis can co-exist with ACS; and does not imply a cause-effect relation to the IAP. Unlike the animal models with uniform elevation of the IAP, the obstrutive uropathy here is caused by the predominant pelvic component of retroperitoneal bleeding.

CONCLUSION:  ACS can occur as a complication of abdominal bleeding with LMWH. Retroperitoneal bleeding with a predominant pelvic component can cause hydronephrosis and can co-exist with IAH/ACS. Ureteral stents in this subset of patients with ACS with hydronephrosis may improve the renal dysfunction.

DISCLOSURE:  S.J. Hansalia, None.

Chest. 2004;126(4_MeetingAbstracts):969S-a-970S. doi:10.1378/chest.126.4_MeetingAbstracts.969S-a

INTRODUCTION:  Lemierre’s syndrome (LS) is a rare disease characterized by septic thrombophlebitis of the internal jugular vein. LS usually starts with pharyngitis and is followed several days later by the development of fever, rigors, swelling and neck pain and in many cases evidence of other organ involvement. Mortality was 90% in Lemierre’s original series but is now much lower (7%) with effective antibiotic use.

CASE PRESENTATION:  A previously healthy 24 year old female with no significant past medical history presented to her primary physician with complaints of sore throat and fever. A throat culture was negative and she was diagnosis with a presumed viral infection. One week later she presented to the emergency department with significant shortness of breath. In the ED, she was in severe respiratory distress with a room air PaO2 of 44. She was intubated and transferred to the intensive care unit. Upon arrival at our ICU, the patient required 100% oxygen and high PEEP. Vital signs showed a temperature of 36.1, blood pressure of 100/38, and heart rate of 130. On physical exam, the neck showed no JVD, nuchal rigidity, swelling or adenopathy. There were crackles in both lung bases. Exam was otherwise unremarkable. The initial laboratory results were notable for an elevated white count of 15.8 with 49% bands, platelets of 31,000, and creatinine of 1.5. A chest x-ray showed bilateral alveolar infiltrates. The patient was initially treated for presumed severe community acquired pneumonia with Levoquin and vancomycin. Blood, urine and sputum cultures were obtained. Her clinical status deteriorated rapidly and she developed oliguric renal failure due to ATN, severe DIC, septic shock and possible TTP within 48 hours of hospitalization. She was treated with CVVH, plasmaphoresis and vasopressors. Her oxygenation continued to decline and she developed acute respiratory distress syndrome. A lung protective strategy was employed. On hospital day four, fusobacterium necrophorum was isolated from her admission blood cultures. This raised concern about Lemierre’s Syndrome so an ultrasound of her internal jugular veins was obtained. There was a clot in the right jugular vein, confirming the diagnosis of LS. A CT scan of her chest showed extensive septic emboli bilaterally. Clindamycin, Metronidazole and Lovenox were added to her regimen. Fortunately, her condition slowly improved and she was able to make a full recovery.

DISCUSSIONS:  This case highlights several aspects of LS. As in our case, LS usually affects previously healthy adolescents and young adults. The usual causative organism of LS is f. necrophorum, a gram negative anaerobe which is part of the normal oropharyngeal flora. F. necrophorum is somewhat slow growing so cultures may not turn positive until 5 to 8 days. Spread to other organs is common, with the lung (85%) and joints (26%) being the most frequently affected. The heart and GI tract are occasionally involved. CXR findings can include nodules, which may cavitate, and pleural effusions. Our patient showed extensive lung involvement. As was seen in our patient, the neck exam may be unrevealing. Diagnosis is based upon appropriate imaging studies, usually either CT or ultrasound of the neck, although MRI is also sometimes used. Metronidazole, clindamycin and penicillins with anaerobic activity are the mainstays of treatment. Duration of treatment is variable, but is generally between 2-6 weeks. The role of anticoagulation in LS is uncertain but some authors advocate its use. Surgical ligation of the IJV was utilized prior to the use of antibiotics, however, such surgery is now rarely required. Severe DIC and ARDS, as in our patient, are very rare.

CONCLUSION:  Lemierre’s disease generally affects healthy patient with significant morbidity resulting from the septic emboli.

DISCLOSURE:  G.L. Schumaker, None.

Chest. 2004;126(4_MeetingAbstracts):970S. doi:10.1378/chest.126.4_MeetingAbstracts.970S

INTRODUCTION:  The association between IgA nephropathy(IgAN) and pulmonary capillaritis is rare. We present a unique case where the IgAN had progressed to end-stage renal failure before developing diffuse alveolar hemorrhage(DAH) which was treated with plasma exchange, systemic steroids and cyclophosphamide. This highlights the varied clinical course of IgAN and successful outcome with aggressive immunosuppression.

CASE PRESENTATION:  A 20-year-old Chinese male presented with small volume fresh hemoptysis and progressive dyspnea for two weeks. There were no other symptoms or contact history with tuberculosis. His drug history was unremarkable. There was no improvement despite a prior course of oral antibiotics. He was hemodialysis dependent from IgAN that was diagnosed by immunofluoresence on renal biopsy three years earlier.On examination he was febrile, tachycardic and tachpneic. There were no skin lesions. Bilateral coarse crepitations were heard on ascultation.Chest radiograph showed extensive bilateral alveolar infiltrates.(figure 1) Hemoglobin was 6.9 grams per deciliter(baseline 8.7). The white-cell count was 8110 per cubic millimeter with 85% neutrophils. Coagulation profile and electrolytes were normal. PaO2 was 84 mmHg on 40% supplemental oxygen.Community acquired pneumonia was the initial diagnosis with a differential of DAH. Intravenous ceftazidime, erythromycin and cloxacillin were initiated. Bronchoscopy on day two identified fresh blood throughout the tracheobronchial tree. Bronchoalveolar lavage(BAL) from the middle lobe obtained progressively bloodier returns. He was too dyspneic to undergo transbronchial lung biopsy.Blood, sputum and BAL work-up for sepsis was negative. Abundant neutrophils and hemosiderin-laden macrophages were noted on BAL cytology. Serum anti-nuclear, anti-basement membrane and anti-neutrophil cytoplasmic antibodies were negative. Serum complement level(CH50) was 41(normal 60-153).Daily intravenous methylprednisolone at one gram and plasma exchange of 2.5 liters was initiated for the next three days. The hemoptysis rapidly resolved and he was weaned off oxygen. He was discharged on oral prednisolone at one milligram(mg) per kilogram per day.After declining open lung biopsy, a transbronchial lung biopsy revealed acute inflammation that was consistent with pulmonary capillaritis.(figure 2) Cyclophosphamide(50 mg/day) was added after a second hospitalization for frank hemoptysis. He has been in remission for a year.

DISCUSSIONS:  All previous reports indicate pulmonary involvement in IgAN either coincides with renal disease[1234] or precedes it.[5] Our patient had already progressed to renal failure and the immune pathology in the kidneys was quiescent. Subsequent development of an immune mediated capillaritis suggests that this is an independent manifestation of the same underlying disease process.The prognosis of pulmonary capillaitis in IgAN is poor with only three survivors[1,3,5] among seven previously reported cases.[1-5] Plasma exchange and azathioprine therapy is reported in one case, but it was unsuccessful.[2] All the other cases were managed with either steroids[1,345] or supportive care.[4] Ours is the first report of cyclophosphamide as adjunctive therapy to steroids in DAH related to IgAN.

CONCLUSION:  Although bleeding tendencies are common in uremic patients, the rare association between IgAN and pulmonary capillaritis must not be overlooked. Occurrence of DAH is independent of the stage of renal disease. Aggressive immunosuppression can result in a good outcome.

DISCLOSURE:  D. Anatham, None.

Chest. 2004;126(4_MeetingAbstracts):971S. doi:10.1378/chest.126.4_MeetingAbstracts.971S

INTRODUCTION:  Sirolimus (rapamycin) is a macrocyclic triene antibiotic produced by the actinomycete Streptomyces hygroscopicus. It has potent immunosuppressant properties, which works independent of the calcineurin pathways. It does not induce renal insufficiency and is generally used to prevent progression of renal insufficiency. Its use has been associated with infrequent cases of interstitial pneumonitis. We report a case of diffuse alveolar hemorrhage associated with its use.

CASE PRESENTATION:  51-year-old male presented to the ED with complaints of shortness of breath for 3 days. He was status post transplant for lupus nephritis and had developed cyclosporin toxicity and was started on sirolimus 6 weeks prior to presentation. At the time of presentation he was on cellcept, medrol, nifidipine, lexapro, bactrim and lasix. Physical exam revealed that he was afebrile however had significant, tachypnea and hypoxia. He also had decreased breath sounds at both bases and along with bilateral extensive crackles. Laboratory data showed a stable but elevated creatinine of 5.0 with normal platelets and coagulation profile. Based on laboratory data his systemic lupus erythematosis (SLE) was deemed to be not active. Chest radiography demonstrated bilateral extensive interstitial and alveolar infiltrate. He required intubation to undergo bronchoscopy, which showed evidence of diffuse alveolar hemorrhage (DAH). Broncho alveolar lavage (BAL) showed 76% neutrophils, 16% monocytes and 7% lymphocytes, lipid-laden macrophages and iron stain positive for hemosiderin. No organisms were seen or isolated. Sirolimus was stopped and he recovered completely within 15 days. It was not restarted and he continues to do well.

DISCUSSIONS:  Morelon et al (1) described a case series of 8 renal transplant patients who developed sirolimus induced interstitial lung disease. In their case series there was one patient with diffuse alveolar hemorrhage. They also noted a lymphocyte predominant on BAL. However our patient had a neutrophil predominant BAL. This may because our patient had been on the medicine for a shorter time or it may be an association in patients with DAH. Seethamaraju et al (3) described 4 lung transplant cases that developed interstitial pneumonitis while on sirolimus. All 12 cases and our patient were on Sirolimus because of calcineurin toxicity. To our knowledge there is only 1 reported cases of DAH/hemoptysis associated with the use of sirolimus. (1). The diagnosis of sirolimus toxicity is established on clinical grounds after excluding other infectious and non-infectious causes and on stopping or reducing medication.

CONCLUSION:  Sirolimus continues to be a very effective anti rejection drug however its use is associated with rare but significant risk of alveolar hemorrhage and interstitial lung disease which is reversible on withdrawing or decreasing the dose of the offending agent.

DISCLOSURE:  J.S. Brar, None.

Chest. 2004;126(4_MeetingAbstracts):971S. doi:10.1378/chest.126.4_MeetingAbstracts.971S-a

INTRODUCTION:  Pleurisy, coughing and/or dyspnea are often the first clues of lung involvement in SLE.

CASE PRESENTATION:  We present a 42 year old woman with a history of pelvic vein thrombosis and left sided pneumonia in the preceeding two years who was referred to our department for evaluation of progressive exertional dyspnea, unproductive cough, fatigue and migratory pleuritic chest pain. She also described having recurrent episodes of tender and swollen metacarpophalangeal and proximal joints. She was a smoker with a history of 50 packyears.The physical examination was unremarkable. Chest x-ray and high resolution computer tomography (HRCT) showed normal lung structure. Pulmonary function testing revealed a restrictive pattern (total lung capacity 75% pred), a significant reduction of the diffusing capacity for carbon monoxide (43% pred) and exertional hypoxemia with normal oxygen partial pressure at rest. Bronchoscopy was performed. Bronchioalveolar lavage showed a normal cell distribution and transbronchial biopsy showed changes compatible with chronic bronchitis, discrete alveolar septal thickening and interstitial fibrosis. Immunologic testing was positive for antinuclear antibodies (1:640) including those to double stranded DNA, suggesting the diagnosis of SLE. For further confirmation an open lung biopsy was performed. The histopathologic findings were similar to those of the transbronchial biopsy. Therapy with 75mg prednisone and 100mg azathioprine was started and tapered to 25mg prednisone and 50mg azathioprine after 12 weeks. Under therapy the patient reported a marked improvement of her exertional dyspnea and coughing. Lung function testing after 12 weeks showed normal lung volumes, the exertional hypoxemia has resolved.

DISCUSSIONS:  Our patient presented with a significant pulmonary functional impairment. HRCT is considered a sensitive technique for detecting pulmonary changes in SLE but was negative in the present case. The invasive diagnostic procedures did little to confirm our diagnosis as the histopathological findings were only discrete. Her history of fatigue, arthralgias and pleurisy has led us to suspect a systemic disease, but the major diagnostic clue were the immunologic abnormalities. In applying the criteria of the American Rheumatism Association the patient could be classified having SLE.

CONCLUSION:  In patients with SLE and lung involvement with significant impairment of their lung function, correlating changes in HRCT may be absent.

DISCLOSURE:  D. Kiss, None.

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