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Abstract

Chest. 2006;130(4_MeetingAbstracts):292S-b-293S. doi:10.1378/chest.130.4_MeetingAbstracts.292S-b
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INTRODUCTION: Congenital unilateral absence of a pulmonary artery (UAPA) is a rare abnormality, commonly accompanied by cardiovascular anomalies. It may occasionally occur as an isolated finding. UAPA on the right side is reported more commonly than the left side. Usually detected in childhood, most common presenting symptoms are recurrent pulmonary infections, dyspnea or exercise limitation, chest pain, pleural effusion, and hemoptysis. Some patients may be asymptomatic and the diagnosis may be missed or delayed. We report a case with UAPA diagnosed in a 43 year-old female.

CASE PRESENTATION: A 43-year-old Jamaican woman with a presumed diagnosis of asthma since childhood, recurrent episodes of chest infection, especially in winter months, presented with worsening dyspnea on exertion of three months duration. She had experienced similar symptoms three years ago. The patient is a nonsmoker and has two children. The rest of the medical history was non-contributory. Due to persistent symptoms, the patient was sent to emergency department for evaluation. The physical examination demonstrated an obese patient in mild respiratory distress, with normal vital signs. Her SaO2 at rest on room air was 95%. Auscultation of the respiratory system revealed mildly decreased breath sounds on the right side. Routine laboratory values including B-type natriuretic peptide and D-dimer were normal. The alveolar arterial O2 gradient (A-a gradient) was normal. Chest X-ray demonstrated mild cardiomegaly, reduced lung volume on the right side with mediastinal shift. The ventilation perfusion scan showed complete absence of perfusion to the entire right lung. Anticoagulation therapy was commenced and contrast-enhanced CT of the chest was performed. The latter was negative for pulmonary embolism (PE), but showed unilateral absence of right pulmonary artery with hypoplasia of right lung.

DISCUSSIONS: Our patient was diagnosed with asthma since childhood although she demonstrated little improvement with inhaled bronchodilators. She also suffered from recurrent chest infections all her life which were labeled as “recurrent bronchitis”. During her last hospitalization, she was started on anticoagulation based on the interpretation of the ventilation perfusion scan (V/Q mismatch). The diagnosis of PE was not corroborated by other laboratory data including A-a gradient and D-dimers. This confusing imaging and laboratory data, in the light of a relatively stable patient lead to the performance of chest CT. UAPA is a very rare congenital anomaly. Most often, it is diagnosed in childhood and young adults. The presenting symptoms are non-specific. Both these factors may result in a missed or delayed diagnosis in adults. Often, the absence of unilateral pulmonary artery in adults is missed on CT scans primarily because of the rarity with which it is encountered in this patient population.

CONCLUSION: Although UAPA is rare disease, this diagnosis should be considered by the clinician while evaluating patients with recurrent respiratory infections, dyspnea or exercise limitations. Radiologists should suspect this entity when a chest radiograph shows small hemithorax, especially when the V/Q scan shows unilateral complete absence of perfusion and relatively preserved ventilation in an otherwise stable patient. Confirming the diagnosis may be achieved with CT chest, MRI, echocardiogram or with the more invasive pulmonary angiogram.

DISCLOSURE: Ayman Bishay, None.

Abstract: Case Reports

Chest. 2006;130(4_MeetingAbstracts):286S. doi:10.1378/chest.130.4_MeetingAbstracts.286S-a
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INTRODUCTION: Amyloidosis is a group of disorders characterized by deposition of protein conglomerates in extracellular tissue. The respiratory system may be involved either locally or as part of a systemic process. Tracheobronchial, laryngeal, or parotid gland involvement are rare types of localized amyloidosis. To our knowledge, we report the first case of a patient with combined tracheobronchial, laryngeal, and parotid gland amyloidosis.

CASE PRESENTATION: A 72-year-old woman presented to an outside facility with a few days of dry mouth, sore throat, and cough. She was treated for aspiration pneumonia and discharged two days later. The next day, the patient went to see her otolaryngologist who has been following her for chronic bilateral parotid gland enlargement. A parotid biopsy in 2000 was reported to show nonspecific chronic mild inflammation. Her past medical history was significant for coronary artery disease, and hypertension. Due to the presence of nasal and oral cavity blood, the patient underwent flexible nasopharyngoscopy which revealed a swollen and bleeding larynx and nasopharynx. Biopsies of the false vocal folds and parotid glands were performed. Post-extubation, the patient had a sudden drop in oxygen saturation and was reintubated with difficulty. The patient continued to have hemoptysis. Bronchoscopy revealed blood-filled airways, very friable mucosa, and a nodular irregular area in the distal trachea that was biopsied. All off the biopsies (parotid gland, false vocal folds, and trachea) were found to contain amyloid deposits by Congo red staining. Serum and urine electrophoresis did not show a gammopathy. An echocardiogram showed no evidence of cardiac amyloidosis. A tracheostomy was performed and she was discharged home 4 weeks after admission in a stable condition.

DISCUSSIONS: Pulmonary amyloid may be a manifestation of systemic disease or a local disease confined to the lungs. Diffuse interstitial involvement is the most common pattern seen in systemic disease. Local disease may occur in the airways (tracheobronchial) or the parenchyma (amyloidomas). Tracheobronchial amyloidosis is uncommon with only few hundred cases reported. It typically presents after the fifth decade with dyspnea, cough, and occasionally hemoptysis. It may lead to progressive airway obstruction, infectious complications, and respiratory failure. Laryngeal amyloidosis occurs most commonly in the supraglottic region. It is often asymptomatic, but can present with hoarseness, stridor, or a sensation of fullness. Fatal hemorrhage has been reported. Localized parotid gland involvement is extremely rare. The first case was described in 1998. It may cause the sicca syndrome and is often misdiagnosed as Sjogren's syndrome. The gold standard histological characteristic of amyloidosis is the unique green birefringence seen when Congo red stained tissue is viewed under a polarizing microscope. Management options for airway disease include surgical or bronchoscopic debridement, balloon dilation, Nd:YAG laser therapy, stent placement, and most recently radiation therapy.

CONCLUSION: Amyloidosis may involve all levels of the respiratory tract. We believe that this is the first case in which tracheobronchial, laryngeal, and parotid amyloidosis were found in a single patient. Early recognition is important as interventions effectively treat or delay serious complications associated with airway obstruction and hemorrhage. Differentiating local from systemic amyloidosis is also critical as the latter is associated with potentially treatable systemic diseases and carries a worse prognosis.

DISCLOSURE: Wael Berjaoui, None.

Chest. 2006;130(4_MeetingAbstracts):286S. doi:10.1378/chest.130.4_MeetingAbstracts.286S-b
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INTRODUCTION: Tracheal obstruction can have many underlying causes. Commonly encountered causes are neoplastic and benign stenoses. In adults, rare cases of tracheal obstruction are caused from external compression from vascular structures. This case demonstrates airway compromise caused by a combination of an innominate artery aneurysm and severe kyphoscoliosis.

CASE PRESENTATION: An 86 year old woman was admitted to the hospital for “shortness of breath.” Her medical history was remarkable for severe kyphoscoliosis and hypertension, and she was gainfully employed in a full time job as a cashier. She had been hospitalized several times over the last year with symptoms of feeling as though she were choking, chest tightness, wheezing, and intermittent stridor. Her symptoms were relieved with stretching/straightening her neck and leaning her head back, as well as with the use of nebulizers and humidified air. The patient reported that she never had any problems with her breathing until the last year, and she was a life long non-smoker. She was transferred to our hospital after a CT scan of the neck and chest was performed which revealed her trachea to be significantly compromised anteriorly by an innominate artery aneurysm and posteriorly by the anterior portion of her cervical spine. Reconstructed tracheal images revealed a tracheal diameter of 11.5mm at the narrowest point; on bronschoscopic exam, the diameter at maximum was 11.5mm however there was intermittent near complete obstruction of the trachea with pulsation of the innominate artery. A multidisciplinary team including interventional pulmonology, thoracic surgery, vascular surgery, and otolaryngology was involved in the case. The team concluded that endovascular stenting of the innominate artery aneurysm would not have alleviated the pressure in the innominate artery and therefore not alleviate the tracheal compression. Tracheostomy was felt risky; secondary to her severe cervical kyphoscoliosis the tip of the trach tube would lay at the level of the arterial aneurysm with increased theoretical risk of tracheo-arterial fistula. Cardiothoracic surgery reccommended open aneurysmal repair. The patient opted first for non-surgical attempts to alleviate her symptoms. Airway management included a series of rigid bronchoscopies. The first procedure entailed balloon dilation of the stenosed area to 15mm, followed by placement of a Dumon (Bryan Corp, Woburn MA) 16mm x 50mm stent - placed 3cm above the carina. Difficulties with cough ensued, with migration of the stent. The stent was repositioned back to its original location during the second rigid bronchoscopy. Further problems with cough, stent migration, and mucous clearance ensued. To achieve less stent migration, the decision was made to change from a Dumon stent to an Ultraflex (Boston Scientific, Natick MA) 20mm x 40mm covered metallic stent. The patient improved symptomatically and was able to return home with minimal respiratory limitation.

DISCUSSIONS: Innominate artery aneurysms causing tracheal stenosis have been reported in the medical literature. The epidemiology of these aneurysms ranges from Marfan's Syndrome, vasculitidies including Takayusu's arteritis, infectious causes such as leutic aneurysms, iatrogenically from line placements, and from hypertension. Treatment options include treating underlying diseases such as controlling hypertension, surgical resection, and now a successful case of endotracheal stenting relieving obstruction.

CONCLUSION: Innominate artery aneurysms in adulthood are rare. Management when causing tracheal compromise should take a multidisciplinary approach as medical, surgical and interventional pulmonology options are available. From our literature search this is the first tracheal obstruction case secondary to innominate artery aneurysm treated successfully with endotracheal stenting.

DISCLOSURE: Catherine Grossman, None.

Chest. 2006;130(4_MeetingAbstracts):286S-c-287S. doi:10.1378/chest.130.4_MeetingAbstracts.286S-c
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INTRODUCTION: The development of a tracheoesophageal fistula in a chronic ventilator patient is a rare occurrence. It can be associated with gastric distension and prior studies have suggested the use of a “breathing bag” sign and gastric air analysis to further suggest the diagnosis and prompt further invasive workup.

CASE PRESENTATION: A 58-year-old veteran with a past medical history of non-Hodgkins lymphoma and epilepsy was admitted to the ICU after an elective right upper and middle lobectomy for a pleomorphic carcinoma. The patients post operative course was complicated by gram negative pneumonia with sepsis and ARDS. The patient also had prolonged neutrapenia. Due to significant lung damage related to fibroproliferative ARDS and critical illness polyneuropathy the patient underwent tracheostomy and PEG placement early in his hospitalization. The patients recovery was further impeded by multiple hospital acquired infections. Approximately 3 months post tracheostomy the patient developed a global ileus which was thought at the time to be due to electrolyte abnormalities, narcotics, and critical illness. Obstruction was ruled out and TPN was initiated. After 2 weeks of continued failed attempts to restart enteral feeding a new finding of massive gastric dilation and the patients PEG drainage bag inflating like a balloon was noted. The patient was placed on 100% oxygen and a new PEG bag was placed which inflated in a few minutes. A syringe was used to draw air from the bag which was then run on an ABG machine with a resulting pO2 of 688mmHg. This then prompted an EGD and bronchoscopy which demonstrated a tracheoesophageal fistula at the point of contact with the cuff and posterior tracheal wall. A soft foam trach was then placed beyond the fistula which resulted in resolution of the ileus and restoration of enteral feedings. Three weeks later the patient died from a MRSA pneumonia as the family did not wish to pursue further aggressive treatments at that point in time.

DISCUSSIONS: Tracheoesophageal fistula is a known but rare complication of tracheostomy occurring in less than 1% of cases. Previous authors have suggested several different clues to help clinicians think of this diagnosis. One suggestion has been to observe for the phasic respiratory variation of a bag hooked to a patients NG tube. In this case our patient developed a similar finding where a PEG drainage bag inflated with respiration. Additionally, there has been the suggestion to use gastric air analysis to confirm the diagnosis. In our patient by using 100% oxygen a large gradient was created to confirm the diagnosis and suggest further testing.

CONCLUSION: The clinical techniques of gastric air analysis and observation for the “breathing bag” sign are both useful non-invasive measures to evaluate for tracheoesophageal fistula. It is also a rare cause of global ileus and gastric distention that should be considered in the differential diagnosis of a tracheostomy patient.

DISCLOSURE: David Hasselbacher, None.

Chest. 2006;130(4_MeetingAbstracts):287S. doi:10.1378/chest.130.4_MeetingAbstracts.287S-a
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INTRODUCTION: Upper airway injury can be propagated by intubation and tracheostomy, leading to a vicious cycle of ventilator dependence or, depending upon the location of obstruction, to death. We report a case of upper airway injury causing ventilatory failure that was healed with bypass using a trans-tracheal catheter and high-flow humidified tracheal insufflation.

CASE PRESENTATION: A 62-y-o woman was accepted in transfer because of upper airway lesions felt to require laser ablation. She had been intubated and extubated 4 times over 6 weeks. Bronchoscopy had shown necrotic granular tissue in the upper trachea with partial airway obstruction (Figure 1). The patient was intubated upon arrival. Bronchoscopy through the ETT without pull-back demonstrated granulation tissue (seen through the tube) around the sub-glottic and carinal areas. The upper tracheal process was felt to be the reason for recurrent respiratory failure. The area of inflammation/stenosis was low for a traditional tracheostomy tube. The patient was re-bronchoscoped, a pull-back was done to define the distal end of the upper tracheal process, and a trans-tracheal catheter (Trans-Tracheal Systems, Denver, Colorado) was placed into the trachea under direct bronchoscopic guidance. The skin insertion site was just above the sternal notch, and the entry site was about 4 cm distal to the vocal cords, much lower than a traditional trans-tracheal approach (Figure 2). Two days after insertion of the trans-tracheal catheter, the catheter was connected to high-flow humidified heliox 70/30 at 15 liters and 39o using a Vapotherm system ( ) and the patient was extubated. She was stable upon extubation; a blood gas demonstrated a pH of 7.39 with a pCO2 of 41 and a pO2 of 74. The patient remained stable off mechanical ventilation. After several days, the trans-tracheal gas mixture was changed to standard oxygen/nitrogen without clinical deterioration. Three bronchoscopies were performed with argon tissue ablation of upper airway tissue. Complete healing of the upper airway lesions was documented. The patient was verbal with a stable respiratory status for 3 weeks, when she was intubated again, this time for congestive heart failure. The trachea was normal at the time of intubation.

DISCUSSIONS: This case has several features which we believe to be relatively unique. First, tracheal stenosis can be a serious management problem. Very high lesions can be bypassed with tracheostomy and low tracheal lesions can be stented, but “upper mid” tracheal stenosis is a serious and potentially fatal process because neither approach may be viable. Persistent endotracheal intubation may contribute to ongoing injury and is not a viable long-term solution. We are unaware of any case in which a small trans-tracheal catheter and tracheal insufflation have been used to overcome these obstacles. Our approach not only succeeded in bypassing the stenosis, it also caused minimal new trauma (no propogation of injury) due to the gasses delivered. Warm, 100% humidified gasses may have been one factor in healing, as this bathed the injured tissues in a “physiologically neutral” gas mixture.The use of a very small distal trans-tracheal catheter gave full access to the trachea with a bronchoscope and allowed tissue ablation procedures in the upper trachea. This would have been impossible with an indwelling endotracheal tube and difficult to impossible with a tracheostomy tube.

CONCLUSION: Tracheal insufflation with warm, highly humidified gasses can be delivered below the traditional tracheostomy access site and may allow healing of upper airway injuries and may also permit upper airway interventions.

DISCLOSURE: Wissam Abouzgheib, None.

Chest. 2006;130(4_MeetingAbstracts):287S-b-288S. doi:10.1378/chest.130.4_MeetingAbstracts.287S-b
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INTRODUCTION: Gastrobronchial fistula (GBF) is a rare and potentially life threatening complication following esophagectomy. A high index of suspicion and accurate selection of diagnostic studies is critical to the early diagnosis and treatment of GBF. We describe a case of gastrobronchial fistula following esophagectomy for adenocarcinoma of the esophagus.

CASE PRESENTATION: A 69-year-old man presented with a 2-month history of worsening dyspnea, productive cough of green sputum, and a 100-pound weight loss. He underwent several months of neo-adjuvant chemo-radiation for adenocarcinoma of the esophagus before proceeding to an Ivor Lewis esophagectomy. He was discharged without complication on the tenth post-operative day. He was admitted to an outside hospital for treatment of right lower lobe pneumonia on three occasions. Upon the third admission, he was transferred to our facility for further evaluation of his recurrent pneumonia. On presentation, he had copious sputum production of approximately 2 liters per day. The volume increased following meals but he denied expectoration of food particles. He denied hemoptysis or dysphagia. He did report early satiety. On physical exam, he was cachectic, afebrile, and required 5-liters by nasal canula to correct his hypoxia. His lips were noticeably chapped and swollen. Pulmonary exam demonstrated decreased tactile frematus and egophony in the right base. The remainder of his physical exam was unremarkable. Laboratory studies only revealed anemia, metabolic alkalosis, and hypoalbuminemia. Admission chest radiograph displayed dense consolidation in the right lower lobe. The differential included chronic aspiration from severe reflux following his pull-up procedure versus a fistula communicating between the bronchial tree and gastrointestinal (GI) tract. A barium swallow and upper GI series revealed extraluminal contrast in the right posterolateral mediastinum as well as contrast in both lower lobes. However, no fistulous tract could be identified; and he was noted to aspirate contrast during the study. A CT of the chest demonstrated a contrast enhanced pouch just distal and lateral to the anastomotic site with apparent communication to the right lower lobe. He then underwent esophagogastroduodenoscopy (EGD). A 4 cm defect in the right posterolateral gastric wall just distal to the anastomotic site was identified. Lung parenchyma and several bronchi were visualized through the defect. He proceeded to surgical repair with a prolonged recovery, and was discharged to a rehab facility.

DISCUSSIONS: Gastrobronchial fistula is most commonly associated with prior esophageal or gastric surgery[1]. Pulmonary sepsis resulting from GBF carries a high mortality, and a clinical suspicion for GBF is warranted in post-esophagectomy patients presenting with pneumonia. Patients with GBF present with a history of productive cough and may expectorate food particles. Other findings may include hemoptysis, dyspnea, and malnutrition from recurrent pulmonary infections if the condition goes undiagnosed. Interestingly, our patient presented with severely chapped and swollen lips implying a caustic component of his sputum. This is not described in the literature as a presenting symptom of GBF. Barium swallow with upper GI series is the diagnostic tool of choice, but in our case did not reveal a fistulous tract. Additional diagnostic studies include CT scans with oral contrast, measuring the pH of bronchial secretions, and methylene blue dye test[1]. Bronchoscopy and EGD may be useful depending on the size and location of the fistula, but unlike our case are typically non-diagnostic[1]. Surgical repair is the treatment of choice with close attention to post-op nutritional support.

CONCLUSION: Gastrobronchial fistula is a known but rare complication following esophagectomy for carcinoma of the esophagus. A high clinical suspicion and diagnostic studies to evaluate for GBF are necessary in post-esophagectomy patients presenting with pneumonia in order to expedite definitive treatment.

DISCLOSURE: Heath Latham, None.

Chest. 2006;130(4_MeetingAbstracts):288S. doi:10.1378/chest.130.4_MeetingAbstracts.288S-a
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INTRODUCTION: Pseudomonas infection is a risk factor for allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis(CF), possibly related to increased release of aspergillus fumigatus antigens. The role of pseudomonas in the course of ABPA in non-CF patients is unknown. We present a case of severe ABPA, cavitary pseudomonas pneumonia, and hemodynamically significant hemoptysis requiring lobectomy.

CASE PRESENTATION: A 52-year-old lifetime nonsmoking man presented emergently with hemoptysis. He had a vague history of childhood asthma, and a ten year history of “COPD” characterized by chronic cough and purulent sputum, partially responsive to antibiotics and brief courses of corticosteroids. Evaluation one year ago showed a computerized chest tomogram with severe bronchiectasis, normal serum immunoglobulins, alpha-1 antitrypsin level (MM phenotype), and normal sweat chloride. ABPA was diagnosed with sputum culture positive for aspergillus fumigatus, aspergillus anti-IgE of 34.5kU/L (3699% reference), and a total IgE of 1260 kU/l. Oral prednisone and voriconazole improved symptoms, decreased IgE (140 kU/l), and increased FEV1 from 0.69 to 1.2 liters. Sputum cultures during exacerbations showed nocardia asteroides, methicillin sensitive staphylococcus aureus, and stenotrophomonas, each specifically treated. Two months before admission, while continuing on voriconazole and prednisone, the patient developed a new thick-walled left upper lobe cavitary lesion. Bronchoalveolar lavage showed a pan-sensitive pseudomonas aeruginosa treated with six weeks of intravenous antibacterial therapy. One week after discontinuing antipseudomonal therapy he developed low grade fever and new bright red hemoptysis which resolved with embolization of both upper lobe bronchial arteries. Recurrent hemoptysis three weeks later(one week before admission) prompted a repeat embolization focused on neovascularization surrounding the left cavity. On presentation he was in mild respiratory distress, tachycardic, coughing up bright red blood with a 4 gm/dl drop in hemoglobin. Intubation with a double lumen ET tube demonstrated persistent left sided airway hemorrhage; at one week he underwent successful left upper lobectomy. Pathologic diagnosis was characteristic of ABPA with no evidence of invasive fungal infection: an 8x7x5 cm cavity containing necrotic fragments and hemorrhage, lined by granulation tissue with a thick fibrous capsule. The parenchyma demonstrated extensive obstructive pneumonia, bronchiectasis, bronchopneumonia and emphysema, and fungal elements within the bronchial lumen surrounded by acute and chronic inflammation. Acid fast and auramine rhodamine mycobacterial stains were negative.

DISCUSSIONS: Although early treatment with oral corticosteroid therapy was delayed in this patient due to an atypical presentation and years of erratic uncoordinated treatment associated with a disadvantageous social situation, he improved markedly on ABPA therapy. The intrapulmonary cavity and associated florid inflammation lesion prompted an initial radiological suspicion of invasive aspergillosis, but surgical pathology showed only ABPA. We speculate that the interaction of pseudomonas and aspergillus observed in CF patients may also occur in non CF patients.

CONCLUSION: ABPA can be missed in patients presenting as adult chronic bronchitis. Massive hemoptysis can occur and lobectomy is lifesaving in this unusual setting. Prolonged antipseudomonal therapy may be required in patients with ABPA similar to CF patients, and suggest the possibility that an interaction between the pseudomonas infections and aspergillus enhanced the inflammatory response, mimicking invasive aspergillus infections. While antibiotic treatment for 6 weeks was considered sufficient for cavitary pneumonia, we now would consider prolonged antibacterial treatment as for a lung abscess.

DISCLOSURE: Marina Dolina, None.

Chest. 2006;130(4_MeetingAbstracts):288S-b-289S. doi:10.1378/chest.130.4_MeetingAbstracts.288S-b
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INTRODUCTION: Fibrosing mediastinitis (FM) is a condition of excessive fibrous tissue deposition leading to compression or constriction of mediastinal structures, most commonly stemming from mycobacterial or fungal infections. Tracheobronchial amyloidosis (TBA) is an extremely rare condition associated with the interposition of amyloid protein in the walls of the tracheobronchial tree. The following is a case of endobronchial amyloidosis in a patient with prior history of FM, leading to concentric airway narrowing.

CASE PRESENTATION: A 34yo woman presented with progressive dyspnea, wheezing and blood streaked sputum over 1 year. Past medical history was significant for asthma. Twelve years ago, she had a biopsy of a mediastinal mass incidentally found on chest radiograph. The biopsy was consistent with FM, thought most likely from histoplasmosis. Initially, no treatment was instituted, and the patient remained in normal heath until three years ago when symptoms of airway obstruction worsened, leading to prolonged treatment with oral corticosteroids and bronchodilators. Although initial pulmonary function testing was significant only for airflow limitation (FEV1 65% predicted), recent testing showed combined obstruction and restriction with preservation of gas transfer (DLCO 101.4% of predicted). CT imaging demonstrated a large conglomeration of lymph nodes with calcifications which encased and narrowed the right main-stem bronchus, and the right middle lobe bronchus was nearly occluded. Fiberoptic bronchoscopy was undertaken with the consideration of airway stenting. Under direct visualization, the right mainstem bronchus was concentrically narrowed with hyperemic, friable mucosa. An endobronchial biopsy from the proximal right side of the main carina showed submucosal deposition of an eosinophilic, acellular material and Congo-Red stain was focally positive. Amyloid A stain was positive, consistent with a diagnosis of TBA. Retrospective analysis of the specimens obtained 12 years prior including Congo-Red staining was void of amyloid protein. A diagnosis of FM with secondary TBA was made.

DISCUSSIONS: Both FM and TBA are quite rare. In the literature, we were unable to find an association between the two entities. Scant qualitative descriptions of the airway mucosa in FM have been made. Manali (2003) reviewed 3 cases FM with airway descriptions analogous to ours. Moreover, patients with TBA have presenting symptoms consistent with our patient (Berk 2002) and similar bronchoscopic airway descriptions. The relationship between TBA and FM in our patient is unclear. An initial insult, possibly histoplasmosis, led to the development of FM. Potentially, slow progression of fibrotic airway invasion with concomitant inflammation may have led to deposition of amyloid A protein. Functionally, the two processes in concert led to a profound narrowing of the airways with mucosal disruption manifested by symptoms of airflow limitation and bleeding. Treatment options for both FM and TBA are quite limited. Steroids have been tried with variable results. For central airway constriction, airway stenting with endobronchial debridement or laser resection is possible, although the diffuse nature of the infiltrative process may limit its utility. Nevertheless, the prognosis for both disorders remains dismal. Recently, several case reports of external beam radiation as a treatment modality for TBA have been published with promising early outcomes.

CONCLUSION: We believe this case is a unique description of the mucosa in a patient with FM. Furthermore, the presence of amyloid protein from biopsies has not been described. As there is heterogeneity in patient responses to FM, with only a subset of patients developing symptoms of hemoptysis along with airflow limitation, it begs the question of whether secondary amyloidosis is an under recognized epiphenomenon in patients with airway infiltration of fibrous material.

DISCLOSURE: Jeffrey Hoag, None.

Chest. 2006;130(4_MeetingAbstracts):289S. doi:10.1378/chest.130.4_MeetingAbstracts.289S-a
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INTRODUCTION: Spontaneous pneumothoraces can complicate COPD patients, often accompanied by a persistent air leak due to bronchopleural fistulas (BPF). The presence of BPF are rare but represents a challenging management. Bronchoscopic fistula closure has been reported as an acquiescent alternative to surgical interventions and its associated complications. In this case, we report our experience with closing a BPF by bronchoscopy using fibrin sealant in a patient with multiples comorbidities and high risk for surgical management.

CASE PRESENTATION: An 82 y/o male chronic smoker with Coronary Artery Disease and severe bullous emphysema, who for the last four months presented four episodes of recurrent left side spontaneous pneumothorax treated with chest thoracostomies. In his last admission he also suffered a subendocardial myocardial infarct, being discharged with ambulatory follow-up. One week later at the chest clinic, he was found again with a large left side pneumothorax. A new attempt with chest tube drainage was tried, but the patient persisted with an air leak due to a large BPF. Considering all the associated morbidities in this patient, we decided to attempt closure of the BPF with fibrin sealant. As described elsewhere, a Swan-Ganz (SG) catheter was introduced via bronchoscope. Upon distal balloon inflation of the SG catheter at the lung segments, wedging it in the apicoposterior segment of the left upper lobe ceased the bubbling in the water chamber seal, implying that this was the site where the fistula was present. Both subsegments were closed with Tisseel (fibrin and thrombin glue). 2 cc of fibrin sealant (Tisseel) were instilled through the SG cath distal tip. The balloon was left inflated for two minutes and then was deflated. The same procedure was performed on apical segment. Upon completion, bubbling in the water seal chamber stopped completely. Two days later chest tube was removed. Follow up chest radiograph showed resolution of pneumothorax. The patient was discharged home 4 days after the procedure. He is being follow in our clinics and no further clinical signs of pneumothorax recurrence have been reported since.

DISCUSSIONS: Spontaneous and recurrent pneumothoraces are serious, life threatening complications in COPD patients. The mortality range from 5 to 17% in the majority of series. BPF's are a rare complication in these patients but when present they are difficult to treat. If spontaneous sealing fails to occurs within the first 72 hours, surgical closure of the air leak together with pleurodesis or parietal pleurectomy may be indicated. Options for surgical repair include open-window drainage, thoracoplasty, omentopexy and intrathoracic muscle transposition. For patients with multiple comorbidities or that are too sick for surgical intervention there are reports of endoscopic closure of postoperative BPF with favorable results. Nonsurgical techniques for BPF closure include methacrylate, tissue glue, fibrin glue, gelfoam, tetracycline, autologous blood patch, lead plugs and balloon catheters.In this patient chest tube and talc pleurodesis therapy was ineffective. Due to the patient's multiple comorbidities both Cardiology and Thoracic surgery elected to manage the patient conservatively. Following case reports of successful closure of postresection BPF with minimal side effects with the use of fibrin sealant we decided for this method. We treated our patient through a flexible bronchoscope using a SG catheter in order to inflate its balloon and use the lumens. The fibrin glue was injected by high pressure followed immediately by thrombin through other lumen. A fibrin clot formed over the fistula, sealing the leak. It is believe that the fibrin glue is eventually reabsorbed, preventing tissue reaction to for foreign body.

CONCLUSION: Closure of BPF using fibrin glue is a safe and promising alternative to avoid surgical interventions and associated complications in patients with multiples comorbidities and high surgical risk.

DISCLOSURE: Brenda Loubriel, None.

Chest. 2006;130(4_MeetingAbstracts):289S. doi:10.1378/chest.130.4_MeetingAbstracts.289S-b
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INTRODUCTION: Tracheobronchitis caused by ulcerative colitis has been rarely documented in literature. The case reported here is unique because of similar pathologic findings observed on colonic and tracheal biopsy specimens and because of the original images observed on computed tomography (CT), magnetic resonance imaging (MRI) and endobronchial ultrasonography (EBUS).

CASE PRESENTATION: A 49-year-old woman presented with a one-year history of dyspnea on exertion and hoarseness. The patient had a total colectomy for ulcerative colitis 17 years prior to our evaluation, with a strong family history of collagen vascular disease. Her mother and sister both had rheumatoid arthritis. Physical examination revealed stridor during both inspiration and expiration but it was louder during inspiration. Flow volume loop showed flattening of both inspiratory and expiratory curves, which is consistent with fixed intrathoracic obstruction. CT and MRI showed diffuse narrowing of the trachea and thickening of the mucosal tissue. Bronchoscopy revealed edematous, hypervascular and floppy vocal cords as well as diffuse friable and hemorrhagic tracheobronchial mucosa. EBUS revealed circumferential thickening of the mucosa and intact tracheobronchial cartilaginous structures. The pathological findings of the trachea showed severe inflammatory cell concentration in the sub mucosal tissue, similar to the findings found on colonic biopsy. Symptoms improved after one week of treatment with corticosteroids and Cyclosporine.

DISCUSSIONS: There are some other differential diagnoses of diffuse tracheobronchial narrowing.•Wegener granulomatosis also causes the obstruction of the central airway. Wegener granulomatosis is characterized by the systemic granulomatous inflammation and necrotizing angiitis. Our paitent had no symptom of necrotizing angiitis such as renal or nervous system disorders. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative and no parenchymal lung disease was seen. Amyloidosis can be excluded on the point of clinical view because it is also a systemic disease. EBUS images•helped because mucosa was thickened circumferentially and the cartilage was normal while in relapsing polychondritis (RP) the cartilage is destroyed and the posterior membrane is normal. Rhinoscleroma or tuberculosis can cause laryngotracheitis, but the patient had no risk factor or symptoms for these infections. In our case, the patient has a medical history of ulcerative colitis. It is rare that tracheobroncitis is involved in ulcerative colitis. To us knowledge, only 12 cases have been reported previously. All reported patients had bowel disease and at least 75% patients had some respiratory symptoms such as cough, stridor or dyspnea. After comparing the tracheal apecimen with the lesected colon we noted similarities showing inliltration of the inflammatory cells around the grandular systems from pathology. Microabscesses were seen on both tracheal and colonic biopsy tissues. We started the treatment by oral prednisolone and cyclosporine. The symptoms and MRI findings exhibited improvement.

CONCLUSION: We report a unique case of ulcerative colitis with tracheobronchitis in which we found similar pathologic findings on tracheal and colonic mucosal biopsies. EBUS images helped differentiate from other diseases that cause diffuse tracheobronchial narrowing.

DISCLOSURE: Miho Nakamura, None.

Chest. 2006;130(4_MeetingAbstracts):290S. doi:10.1378/chest.130.4_MeetingAbstracts.290S-a
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INTRODUCTION: Traumatic bronchial disruption is a frequent result of high impact chest injury. Patients are usually not suitable candidates for bronchial repair at the time of their initial presentation, and require aggressive pulmonary hygiene. We describe a case in which a patient with severe distal left mainstem laceration undergoes repair with subsequent restoration of ventilation to the left lower lobe following several weeks of mechanical ventilation.

CASE PRESENTATION: This patient was a 41 year old male who sustained a fall from approximately 40 feet. He presented with multiple injuries, including a fracture of the left first rib and subcutaneous emphysema on his chest radiograph indicating a possible underlying bronchial disruption which was confirmed by bronchoscopy. The laceration was seen extending from the distal left mainstem bronchus at the bifurcation of the upper and lower lobes. An endobronchial blocker was inflated at the laceration site to tamponade bleeding and the patient underwent aggressive resuscitation. He also underwent thoracostomy for a right-sided hemorrhagic effusion. It was uncertain if he would survive his injuries, and a tracheostomy was placed. He made a full neurological recovery. Three weeks following his initial injury, he returned to the operating room for surgical repair of his left mainstem bronchial disruption. He had extensive granulation tissue with complete atelectasis of his left lower lobe. The bronchus was opened with blunt dissection and the use of endobronchial balloons and was closed with interrupted sutures. Post-operatively, his course was complicated by recurrent mucus plugging distal to the repair, and the regrowth of granulation tissue. He was unable to be liberated from mechanical ventilation. An interventional pulmonologist was consulted. The patient subsequently underwent rigid bronchoscopy with electrocautery to remove endobronchial granulation tissue and placement of a silicone-coated stent (Polyflex, Boston Scientific). The patient was then extubated and decannulated. Approximately eight weeks later, he presented with increased cough. His stent had migrated to the trachea. It was removed en bloc with biopsy forceps via fiberoptic bronchoscopy without complication. The patient is now ambulatory and fully recovered.

DISCUSSIONS: Patients with chest trauma and endobronchial disruption are usually managed with pneumonectomy and/or lobectomy at initial presentation. The mortality rate is approximately 43.7%, with the majority of deaths occurring within 72 hours of the initial trauma [1]. This results in a marked decrease in reserve pulmonary function, which leaves these patients at high risk for pulmonary compromise and ventilator dependence in the future. Our patient is unique, as his bronchial repair did not occur at the time of injury, but was delayed. This may lead to increased mortality if there is a lung laceration with significant intrapleural bleeding [1]. Following surgical repair, our patient developed persistent atelectasis and remained ventilator dependent. Finally, the patient was given a coated metallic stent. Most frequently, metallic stents are used which are nonremovable [2]. The benefit of these stents is that they have a lower incidence of migration, better tensile strength and patency. The disadvantage is that they have a higher risk of erosion and perforation. Silicone stents are safer, but more likely to migrate.

CONCLUSION: We conclude that from our experience, endobronchial repair with surgical closure and the use of a silicone-coated stent, may be considered as an alternative to pneumonectomy in patients with severe endobronchial disruption resulting from chest trauma.

DISCLOSURE: Jeffrey Kim, None.

Chest. 2006;130(4_MeetingAbstracts):290S. doi:10.1378/chest.130.4_MeetingAbstracts.290S-b
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INTRODUCTION: Endobronchial telangiectasias are a rare cause of hemoptysis. They have been reported in association with scleroderma, CREST and hereditary hemorrhagic telangiectasia (HHT). We present a case of a patient with hemoptysis and bronchial telangiectasias successfully treatment with electrocautery.

CASE PRESENTATION: A 67-year-old male presented with two month of persistent hemoptysis. He had no history of fevers, chills, weight loss, or tuberculosis exposure. He had no history of recurrent epistaxis, mucocutaneous bleeding, or melena, and no family history of hereditary hemorrhagic telangiectasia. He had no history of Raynaud's syndrome or dysphagia.His past medical history included smoking, alcoholic liver cirrhosis, remote nephrectomy for living donor transplantation, chronic renal insufficiency, gout, right carotid stenosis. His medications included spironolactone, pantoprazole, aspirin, codeine and citalopram.Physical examination revealed normal vitals signs and pulse oximetry. Cardio-pulmonary examination was normal. Abdominal ascites was present. No pulmonary or hepatic bruits were present. Palmar telangiectasias were present, but oral mucosal telangiectasias were not. Calcinosis or sclerodactyly was absent. Chest radiographs and computed tomography scanning of the thorax were unremarkable. Flexible bronchoscopy was performed under conscious sedation and demonstrated two areas of telangiectasias. The first was at the right upper lobe carina and covered an area ∼1 cm2 [Figure 1]. The second smaller telangiectasia was seen in a left lower lobe basilar segment. Hot biopsy forceps with soft electrocoagulation at 60 watts were used to simultaneously biopsy and coagulate the lesions (Forceps: FD-6C-1, Olympus America, Melville, NY; Electrosurgical generator: ICD350, ERBE USA Inc., Marietta, GA). No bleeding was noted. Pathology was consistent with telangiectasias [Figure 2]. Further investigations revealed a normal arterial blood gas, serum C3 and C4, rheumatoid factor. Antinuclear antibodies were positive at 1/2560 dilution with a nucleolar pattern, no anticentromere antibodies were seen. Testing for Jo-1, RNP, Scl-70, Sm, SS-A/Ro, SS-B/La, Ribo-P and chromatin was negative. On follow-up six months later the patient had no recurrence of hemoptysis.

DISCUSSIONS: Endobronchial telangiectasias causing hemoptysis have been reported in the setting of hereditary hemorrhagic telangiectasia (HHT), scleroderma, and CREST syndrome. Isolated bronchial telangiectasias have been reported in one case.Previously reported management of symptomatic endobronchial telangiectasias has been conservative, with no reports of endobronchial electrocoagulation for treatment of these lesions. Our experience, as well as published data on the utility of endobronchial electrocoagulation in bleeding endobronchial lesions , lead us to consider this modality for this patient. The use of endobronchial electrocoagulation is appealing due to its simplicity, low cost and low side effect profile. Specifically, the risk of airway perforation is very low and less likely than with laser therapy. The multifocal nature of the lesions would also make surgical resection problematic. The use of a hot biopsy forceps allows simultaneous coagulation of the lesion and sample collection for histopathological examination. This allows examination for an underlying malignant lesion as the cause of hemoptysis and vascular proliferation.

CONCLUSION: This patient had endobronchial telangiectasias with clinical and serological features of CREST. Longterm control of endobronchial bleeding was obtained by endobronchial electrocoagulation of the lesions.

DISCLOSURE: Andrea Loewen, None.

Chest. 2006;130(4_MeetingAbstracts):291S. doi:10.1378/chest.130.4_MeetingAbstracts.291S-a
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INTRODUCTION: Bronchopleural Fistula (BPF) is an uncommon but dreaded complication of several pulmonary conditions. Scientific evidence on the method of closure of BPF is scant, with therapeutic options ranging from conservative management to aggressive surgical management. We offer a novel method of closure not previously described in the literature.

CASE PRESENTATION: A 45-year-old female was evaluated by thoracic surgery for closure of an esophago-pulmonary-bronchial fistula into the right lung. Past medical history includes left upper lobectomy eleven years prior for bilateral upper lobe cavitary disease complicated by chronic coccidioides immitis infection. No complications were noted until one year prior to admission when the patient began coughing during meals and when lying flat. Work-up revealed an esophageal fistula 23 cm from the incisors, measuring 4 mm in length and 3 mm in diameter, communicating with an 11 cm cavity in the right lung apex and into the bronchial tree. During surgery, the esophageal fistula was closed, and the right upper lobe was found to be considerable shrunken and nonfunctional, and was removed. The bronchial fistula was stapled shut. Post-operative bronchoscopic surveillance demonstrated adequate closure of the BPF on the right, but revealed a large fistula at the left upper lobe stump, which measured 10 mm in diameter. Upon entering the pleural cavity thru the BPF, a large collection of dark brown purulent material was found, of which 250 cc was aspirated. Cultures revealed Psuedomonas Aeruginosa. The patient had a complicated post-op course requiring tracheostomy and admission to the ventilator weaning unit. A draining chest tube was placed in the left chest. However, in part due to persistent air leak through the chest tube due to the BPF, the decision was made to attempt to close the BPF using an Amplatzer Septal Occluder Device. Bronchoscopy was undertaken with the presence of an interventional cardiologist who was skilled in the use of the Amplatzer. An esophageal dilator balloon was used to measure the diameter of the BPF at 10 mm. The Amplatzer device, with a waist of 10 mm, was then introduced into the BPF and deployed. Examination revealed complete occlusion of the BPF, with the proximal portion of device situated so that the orifice of the lingular bronchus was occluded. Repeat bronchoscopy after 48 hours revealed no migration of the Amplatzer. The patient survived to discharge without complication.

DISCUSSIONS: This is the first report in the literature of the closure of a BPF using an Amplatzer Septal Occluder Device. The Amplatzer is a self-expanding double disk made from nitinol wire mesh. The disks are joined together by a connecting mesh tube, which acts to stent the defect. Polyester patches are sewn within the disks and central stent, which serve to occlude blood flow through the device. The waist portion also serves to self-center the device during deployment. The waist size varies from 4 to 40 mm. The Amplatzer device has traditionally been employed by interventional cardiologists to close atrial septal defects. BPF is a relatively rare complication of several pulmonary conditions that remains extremely difficult to treat. The incidence after lung resection has been reported from 1.5 to 28%. Reported treatment options are numerous, and include surgical closure, bronchoscopic application of sealant solutions (including ethanol, polyethylene glycol, cyanoacrylate glue, and fibrin glue), intrabronchial antibiotic injection, and calf bone occlusion.

CONCLUSION: This case demonstrates the first recorded use of the Amplatzer Septal Occluder Device to close a BPF. This technique may be applied to other similar patients in the future.

DISCLOSURE: Jason Golbin, None.

Chest. 2006;130(4_MeetingAbstracts):291S. doi:10.1378/chest.130.4_MeetingAbstracts.291S-b
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INTRODUCTION: Spontaneous ventricular arrhythmias have been noted to be predictive of sudden death in patients with hypertrophic cardiomyopathy (HCM). Nonsustained ventricular tachycardia occurs in 19-28% of patients with HCM, but spontaneous monomorphic ventricular tachycardia with reproducible initiation with programmed ventricular stimulation is rare. Left ventricular aneurysm without coronary artery disease is a rare phenomenon. The underlying disease is varied but the incidence of HCM in the dilated phase is high. Clinically stable ventricular tachycardia (VT) in these patients is rare: a relation with the presence of midventricular obstruction and apical aneurysm has been proposed.

CASE PRESENTATION: A 50 year old white male with a history of HCM presented about 10 years ago with syncopal VT and class II-III dyspnea. The transthoracic echocardiogram showed asymmetric hypertrophy of the left ventricular septum and a discrete apical aneurysm distal to mid ventricular obstruction; cardiac catheterization showed no coronary disease but visualized discrete apical aneurysm (Fig1). A permanent dual chamber pacemaker (DDD) with short atrioventricular delay and an implantable cardioverter defibrillator (ICD) were implanted at that time.Both patients'symptoms and ventricular arrhythmias were initially controlled with combined antiarrhythmic therapy and pacemaker programming in addition to several antitachypacing (ATP) therapies by the ICD for 10 years. However, the patient experienced recurrence of his VT which became incessant inspite of optimizing his antiarrhythmic medications and reprogramming the ICD. He continued to have frequent ATP therapies and defibrillator cardioversions.An electrophysiological study was performed in attempt to map and ablate the VT. The mapping showed a large but discrete segment of absent or very low voltage electrograms at the apical aneurysm site. Sustained monomorphic VT was consistently induced with program stimulation. Reentry mechanism was confirmed by entrainment criteria. Radiofrequency ablation was unsuccessful. Thereafter the patient was referred for surgery where a large discrete fibrous and trabeculated apical aneurysm harbouring small thrombi was found. Aneurysmectomy with subendocardial resection was performed. Following the surgical procedure all antiarrhythmic therapies were discontinued. The patient had no recurrence of VT and no ICD discharges during two years follow up. He was on metoprolol 25 mg twice a day orally.

DISCUSSIONS: HCM of midventricular type is a rare entity. Although non-sustained VT is common in patients with HCM, sustained monomorphic VT is rare. The major cause of mortality in HCM is sudden death. Clinical sustained monomorphic VT in patients with HCM is uncommon but may be underestimated because of early degeneration into ventricular fibrillation that has been well documented in these patients during electrophysiologic study. Alfonso et al found only two patients with clinical monomorphic sustained VT among 51 consecutive patients with HCM. In both patients, they described echocardiographic and ventriculographic evidence of apical aneurysm, with angiographically normal coronary arteries.

CONCLUSION: Treatment of VT in patients with HCM associated with aneurysm may not be successful with radiofrequency ablation because of inability to isolate completely the scar tissue. In only one case report of HCM with aneurysm was radiofrequency ablation successful in terminating the tachycardia but recurrence is common. Surgical resection of the aneurysm is well tolerated and successful in eliminating VT in the patient so it should be considered as the effective treatment in such rare cases of HCM.

DISCLOSURE: Rabih Touma, None.

Chest. 2006;130(4_MeetingAbstracts):291S-c-292S. doi:10.1378/chest.130.4_MeetingAbstracts.291S-c
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INTRODUCTION: Fibrosing mediastinitis is a rare disorder characterized by invasive proliferation of fibrotic tissue and dense collagen deposition within the mediastinum. In the US it most commonly occurs in areas endemic for histoplasmosis (prevalence 1:100.000) [1-2]. Clinical symptoms reflect compression and obstruction of mediastinal structures. Fibrosing mediastinitis affects predominantly young adults and is associated with substantial morbidity and mortality [1]. Herein we report a case of rapidly developing non-calcified fibrosing mediastinitis associated with histoplasmosis resulting in pulmonary venous obstruction.

CASE PRESENTATION: A 27-year-old woman presented with an 8 months history of dyspnea, cough and wheezing. Outside chest radiography revealed a right middle lobe infiltrate refractory to levofloxacin therapy. Chest CT showed bilateral ground glass infiltrates predominantly involving the right lower lobe, a small right pleural effusion, and extensive hilar and mediastinal lymphadenopathy.(FIG.1A,B) Mediastinoscopy demonstrated reactive lymphadenopathy. It was complicated by transient post-procedural hypotension. Transthoracic echocardiography revealed pulmonary hypertension (right ventricular systolic pressure 69 mmHg), with right ventricular dysfunction, dilatation and hypertrophy. Chest CT scan with pulmonary embolism protocol showed no pulmonary emboli. Subsequently, paroxysmal nocturnal dyspnea, orthopnea and peripheral edema developed. She was a never smoker from Wisconsin, without occupational exposures, HIV risk factors or history of substance abuse. Examination showed respiratory distress, blood pressure 118/70 mmHg, heart rate 115 beats/min, 84% oxygen saturation on room air, elevated jugular venous pressure, crackles in the right lung base, accentuated P2, 2/6 systolic murmur and 1+ pitting lower extremity edema bilaterally. The patient was hospitalized and bronchoscopy with transbronchial needle aspiration of subcarinal lymphadenopathy showed edematous mucosa but was otherwise non-diagnostic. Hypoxemia and hypotension during and following the bronchoscopy required ICU admission, mechanical ventilation and vasopressor support. Chest radiograph revealed pulmonary edema. Histoplasmosis titer by complement fixation was 1:8. Hemodynamic assessment disclosed: pulmonary artery pressure of 89/45 mmHg, wedge pressure of 38 mmHg, and cardiac index of 1.3 L/min/m2. Epoprostenol, nitric oxide and dobutamine were carefully administered, but ineffective. Transesophageal echocardiography and chest CT with contrast showed obstruction of the right inferior and left superior pulmonary veins, severe stenosis of the right superior and a patent left inferior pulmonary vein.(FIG.1C-E) Only the 90% stenosed right superior pulmonary vein was accessible to balloon angioplasty. Successful recanalization via right heart catheterization resulted in initial hemodynamic improvement but the patient continued to require vasopressors and died on the ninth hospital day. Autopsy confirmed severe pulmonary edema with venous infarcts and pulmonary venous obstruction caused by dense fibrosis consistent with fibrosing mediastinitis. Old necrotic granulomas with fungi consistent with Histoplasma (evaluated with silver stain) were also identified. (FIG.2A-C).

DISCUSSIONS: In the US, most cases of fibrosing mediastinitis are attributed to histoplasmosis and considered to represent late complications in susceptible individuals[1,2]. In the absence of a tissue diagnosis, Histoplasmosis associated fibrosing mediastinitis is clinically diagnosed in patients presenting with slowly progressive invasion and/or compression of mediastinal structures by localized, almost universally calcified mediastinal mass lesions[1,2]. Diffuse non-calcified mediastinal infiltration is typically encountered in the less common idiopathic form of fibrosing mediastinitis which is associated with retroperitoneal fibrosis, orbital pseudotumor, Riedel's thyroiditis and methysergide therapy[2]. Our case illustrates that Histoplasmosis associated fibrosing mediastinitis may present as rapidly progressive diffuse infiltration of the mediastinum compromising vital structures even in the absence of radiographic calcifications and convincing serologic evidence of Histoplasmosis. In the absence of effective medical therapy, percutaneous and surgical interventions to relieve mechanical obstructions remain the most beneficial interventions[2].

CONCLUSION: Current clinical criteria used to separate fibrosing mediastinitis associated with Histoplasmosis from idiopathic variants do not reliably distinguish between these entities.

DISCLOSURE: Tobias Peikert, None.

Chest. 2006;130(4_MeetingAbstracts):292S. doi:10.1378/chest.130.4_MeetingAbstracts.292S-a
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INTRODUCTION: Systemic lupus erythematosus is a systemic autoimmune disease that affects numerous organ systems and can affect any part of heart. Few case reports have demonstrated a beneficial effect of intravenous immunoglobulin (IVIG) therapy in lupus myocarditis. We present a case of severe lupus myocarditis treated with IVIG under hemodynamic monitoring.

CASE PRESENTATION: A 32-year-old woman presented with a chief complaint of worsening arthralgia and myalgia over the preceding 2 months. She had presented to her primary care physician 6 months earlier with complaints arthralgia and she was found to have a positive anti-nuclear antibody (ANA). A 2-D echocardiogram showed normal ejection fraction (EF) and trace mitral regurgitation (MR). Physical examination on admission was significant for discoid rash over the bridge of her nose and a grade III/VI holosystolic murmur loudest over the apex, radiating to axilla. Laboratory results revealed pancytopenia, positive ANA and anti-DNA titers, rhabdomyolysis, liver and renal dysfunction.She was treated with pulse dose steroids, cyclophosphamide and oral hydroxychloroquin for lupus flare-up. By day ten there was marked improvement in laboratory tests. On 18th hospital day she was intubated for pulmonary edema following a blood transfusion. Physical examination at that time revealed S3 and S4 gallops, bilateral crackles and 2 + peripheral edema. A portable CXR showed pulmonary edema with bilateral pleural effusion and 2-D echocardiogram showed global hypokinesis (EF 20%), 3+ MR and a small-moderate size pericardial effusion. A Swan-Ganz catheter (SGC) inserted the same day revealed: pulmonary capillary wedge pressure (PCWP) = 27 mm Hg, cardiac output (CO) = 2.76 L/min, systemic vascular resistance (SVR) = 1894 dyne sec/cm5. Milrinone infusion with IV furosemide was started and CO increased to 3.5 L/min. The following day milrinone was switched to dobutamine and nesiritide and CO remained at 3.68 L/min. On day 20 the patient was also started on IVIG. On day 21 the hemodynamics while on same doses of dobutamine and nesiritide were: PCWP = 19 mm Hg, CO = 5.19 L/min, SVR = 1264 dyne sec/cm5. A repeat echocardiogram on day 22 also showed an increase in stroke volume and CO without any change in left ventricle dimensions (Table 1). Over the next few days nesiritide, IVIG and dobutamine were stopped and on day 26 CO was 5.34 L/min (Figure 1). On day 29 the patient became febrile and septic secondary to a urinary tract infection requiring pressor support. Her condition deteriorated further and she died of a cardiac arrest a week later.

DISCUSSIONS: To our knowledge this is the first case report of IVIG use for lupus myocarditis that showed improvement in cardiac function within 48 hours, both by SGC and echocardiogram. Myocarditis in SLE may be related to an immunological phenomenon although accelerated coronary artery disease, hypertension, anemia, valvular disease may also contribute towards systolic myocardial dysfunction.IVIG has been used to treat different clinical manifestations of SLE with an overall success rate between 33-100%. Overall, an increase in C3, C4, and total complement hemolytic activity and a fall in anti-ds DNA antibody levels can be expected with IVIG therapy.

CONCLUSION: Our case shows the modest hemodynamic response to dobutamine, milrinone and nesiritide in a patient with lupus myocarditis and cardiogenic shock. The introduction of IVIG therapy coincided with significant and sustained hemodynamic recovery. Whether this represented the natural clinical history in this patient or was directly related to the introduction of IVIG therapy cannot be proven. However the dramatic temporal association certainly suggests the beneficial role that IVIG played in our patient. Additional case series would provide important information on the utility and timing of this therapy.

DISCLOSURE: Ather Anis, None.

Chest. 2006;130(4_MeetingAbstracts):293S. doi:10.1378/chest.130.4_MeetingAbstracts.293S-a
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INTRODUCTION: Pulmonary hypertension is a serious and potentially devastating disease of the pulmonary vasculature with diverse etiologies and pathogenesis. Schistosomiasis is a rare cause.

CASE PRESENTATION: A 50-year old male presented with a one year history of increasing abdominal girth and progressive shortness of breath. He reported a productive cough with occasional hemoptysis. His past history was significant for schistosomiasis in 1969 while in Yemen, for which he received treatment. He had moved to the United States approximately ten years prior to his presentation. His physical exam revealed room air oxygen saturation of 85%, jugular venous distension, loud P2, holosystolic murmur at apex and right parasternal heave. He had ascites and bilateral pitting edema of his extremities. Laboratory work revealed a normal complete blood count, basic metabolic panel and coagulation profile. Liver function showed only mild elevation in alkaline phosphatase. Hepatitis serologies and alpha fetoprotein were negative. Urine analysis was unremarkable and negative for red blood cells. Electrocardiograph revealed right ventricular hypertrophy and chest radiograph showed moderate cardiomegaly with prominent pulmonary arteries. Inspection bronchoscopy for evaluation of hemoptysis was unrevealing. His pulmonary function test, ventilation-perfusion scan and serologies for collagen vascular diseases were negative. A fast-ELISA test for schistosomiasis was non-reactive, stool studies and rectal biopsy were negative for parasites. Abdominal ultrasound showed periportal fibrosis. Echocardiogram showed a dilated right atrium and ventricle, severe tricuspid valve regurgitation with normal left ventricular systolic function. Cardiac catheterization confirmed severe pulmonary hypertension with mean pulmonary artery pressure of 66 mmHg, pulmonary vascular resistance 21 WU and cardiac index 2.1 L/min/m2 without pulmonary vascular reactivity to adenosine. Patient was started on home oxygen, aldactone, digoxin, lasix and oral pulmonary vasodilator therapy with endothelin receptor blocker,Bosentan.

DISCUSSIONS: Schistosomiasis is the third leading endemic parasitic disease in the world. Infesting more than 300 million individuals, these blood flukes live in the perivisceral veins. The eggs are shed in the urine and feces of infected individuals and the fresh water snail acts as an intermediate host. Pulmonary lesions are attributed to 3 species: S. haematobium, S. mansoni and S. japonicum. Acutely, transient chest radiographic abnormalities and nonspecific influenza-like symptoms can occur, including cough. Limited data suggests that cardiopulmonary schistosomiasis is seen most often in S. mansoni infections. In less than 5% of cases the eggs migrate through the pulmonary vessel wall and trigger a granulomatous response in the pulmonary vasculature. The end result of cumulative injuries to the pulmonary vasculature is the development of obliterative arteritis leading to fibrosis, pulmonary hypertension and subsequently cor-pulmonale. Diagnosis of cardiopulmonary schistosomiasis depends on the detection of viable schistosomal ova in stool , urine or rectal biopsy and serolgies along with evidence of characteristic hepatic fibrosis and pulmonary hypertension. Although treatment with praziquantel can effectively eradicate schistosomal infections with minimal toxicity, cardiopulmonary manifestations are not likely to be reversible given the chronic fibrotic tissue changes that are present. Our patient presented with right heart failure secondary to severe pulmonary arterial hypertension. With his history of residence in an endemic region, prior history of schistosomiasis and pathognomonic liver ultrasound findings with periportal fibrosis, chronic pulmonary schistosomiasis was diagnosed. In the absence of evidence for active infection specific treatment with praziquantel was not initiated. On treatment for heart failure and oral pulmonary vasodilator therapy, he demonstrated complete resolution of facial puffiness, ascites and pedal edema within a few days and a significant improvement in his exercise tolerance from a six minute walk distance of 335 meters to 550 meters over the next year.

CONCLUSION: To our knowledge, this is the first case report demonstrating the effectiveness of bosentan in treating a case of severe pulmonary hypertension secondary to schistosomiasis.

DISCLOSURE: Naricha Chirakalwasan, None.

Chest. 2006;130(4_MeetingAbstracts):293S-b-294S. doi:10.1378/chest.130.4_MeetingAbstracts.293S-b
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INTRODUCTION: ACCP guidelines do not routinely recommend lung biopsy for evaluation of pulmonary artery hypertension (PAH). We report a case of PAH in whom lung biopsy provided an unexpected diagnosis.

CASE PRESENTATION: A 66-year-old female presented with exertional dyspnea, dry cough, orthopnea and leg swelling for six weeks. Her past medical history was significant for sarcoidosis, quiescent for thirty years. Physical examination revealed hypoxia on room air, bilateral crackles, jugular venous distension and pedal edema. Echocardiogram showed normal left ventricular function and pulmonary artery systolic pressure of 80 mmHg, and on cardiac catheterization, the mean pulmonary artery pressure was 42 mmHg, with normal capillary wedge pressure and cardiac output. CT chest revealed mild, bibasilar interstial lung disease (ILD) without evidence of pulmonary embolism. V/Q and gallium scans of the lung, and 4-channel sleep study were non-diagnostic. Pulmonary function study showed a moderate restrictive ventilatory defect. Extensive rheumatological work up was negative, except for an elevated ESR (60 mm/hr) and a positive ANA (1:320) with nucleolar pattern. Patient continued to detoriate with worsening respiratory failure, necessitating an open lung biopsy, which showed histopathologic features of tumor embolism (figures1, 2) and PAH. Immunohistochemical staining was suggestive of a primary site in pancreas, kidney or upper gastrointestinal tract. Despite further work-up, the primary tumor was not found. Her post-op course was complicated by progressive cor pulmonale and respiratory failure. She died 2 weeks later despite maximal supportive therapy.

DISCUSSIONS: Pulmonary tumor embolism is a rare, but recognized, cause of PAH. However, this possibility is often not entertained. Pulmonary tumor embolism is characterized by occlusion of small pulmonary arteries, arterioles and alveolar septal capillaries by aggregates of tumor cells and platelet-fibrin thrombosis. Autopsy series of cancer patients has demonstrated pulmonary tumor embolism in up to 26% of patients, but the diagnosis is made ante-mortem in only 6% of autopsy-proven cases. Intravascular tumor emboli cause serious pulmonary disease in <1% of all cancer patients. PAH ensues when tumor emboli occlude >65% of pulmonary vasculature. Pulmonary tumor emboli are mostly associated with adenocarcinomas of breast, lung, stomach and colon, hepatoma, choriocarcinoma and renal cell carcinoma. Tumor cells enter the systemic circulation by invading small veins or releasing fragments into the tumor neovasculature. Pulmonary angiography is not sensitive for tumor emboli as they are <1 mm in diameter. Cytological examination of blood aspirated from a wedged pulmonary artery catheter has high sensitivity for diagnosis. Therapy is directed at the primary tumor and occasionally, this may result in resolution of the tumor emboli. Although, the official ACCP guidelines do not recommend lung biopsy as part of the evaluation of PAH due to increased morbidity and mortality, our case illustrates the possible role for early lung biopsy in cases of PAH of uncertain etiology. In our patient, the lung biopsy was done to evaluate ILD, because the degree of PAH was disproportionate to the pulmonary infiltrates. Early diagnosis of pulmonary tumor embolism would minimize the inadvertent use of unhelpful therapies, and may potentially improve survival if the primary tumor is treatable. Given the increasing popularity of costly designer treatments such as anti-endothelin antibodies, there exists an economic imperative to avoid use of these drugs in patients with little hope of response, such as those with tumor emboli. An alternative to lung biopsy may be to mandate cytological sampling from the pulmonary artery in idiopathic PAH. A right heart catheterization is commonly performed in the evaluation of PAH, and the cytology yield for tumor emboli is high.

CONCLUSION: Pulmonary tumor embolism should be included in the differential diagnosis of PAH. Lung biopsy may have a potential role in the evaluation of PAH of unknown etiology.

DISCLOSURE: Aditya Dubey, None.

Chest. 2006;130(4_MeetingAbstracts):294S. doi:10.1378/chest.130.4_MeetingAbstracts.294S-a
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INTRODUCTION: Acquired methemoglobinemia is a rare and potentially lethal complication of various oxidative chemicals, including aniline dyes, nitrites, antibiotics (dapsone) and topical anesthetics such as benzocaine. In most patients symptoms are mild and resolve without specific therapy, however, intravenous methylene blue may be required with more severe involvement. In cases of continued exposure to an oxidizing agent or enzyme deficiency, standard treatment may be ineffective and rarely, hyperbaric oxygen treatment or red-cell exchange transfusion can be efficacious. We present the case of an adult who was successfully treated with a red cell exchange transfusion.

CASE PRESENTATION: A 53-year-old female of Irish decent with diffuse infiltrates on chest radiograph developed hypoxemia requiring endotracheal intubation. In preparation for intubation the patient was given 20%-benzocaine as a topical anesthetic, as well as viscous lidocaine to the posterior pharynx. Shortly after intubation the patient developed cyanosis. Oxygen saturation by pulse-oximetry (Sp02) was 86% despite ventilation with 100% oxygen. An arterial blood gas was drawn and sent for multiple-wavelength co-oximetry. The results showed an arterial oxygen saturation of 40.8% despite a partial pressure of oxygen (PaO2) of 352. The methemoglobin level was 51.8%.The patient was given two doses of intravenous methylene blue without improvement. The patient became hypotensive and was transfused with type-specific packed red blood cells. Hyperbaric oxygen therapy was considered, however the patient was too unstable for transfer to a facility with this capability. She remained hypotensive with methemoglobin levels above 45%. Ascorbic acid (1000mg) was given and emergent red-cell exchange transfusion was initiated, with 93% of the circulating red-cell volume exchanged over four hours resulting in resolution of methemoglobinemia. The patient was successfully liberated from mechanical ventilation and subsequently had a full recovery. Glucose-6-phosphate dehydrogenase (G6PD) and cytochrome b5 reductase levels will be assessed 3 months after discharge.

DISCUSSIONS: Methemoglobin is an altered state of hemoglobin in which the ferrous irons of the heme molecule are oxidized to the ferric state rendering it unavailable for oxygen binding resulting in a functional anemia. Once formed, methemoglobin can be reduced enzymatically via either an adenine dinucleotide (NADH)-dependent reaction catalyzed by cytochrome b5 reductase or an alternative pathway utilizing the nicotine adenine dinucleotide phosphate (NADPH)-dependent methemoglobin reductase system. The former is the primary physiologic pathway. Additionally, cellular antioxidants such as ascorbic acid and glutathione can directly reduce methemoglobin non-enzymatically. Acquired methemoglobinemia is most commonly secondary to drugs or exposure to exogenous agents, which can accelerate the formation of methemoglobin. Pulse oximetry is inaccurate in monitoring oxygen saturation in the presence of methemoglobin and multiple-wavelength co-oximetry is imperative in establishing the diagnosis. Intravenous methylene blue is the treatment of choice for symptomatic patients. Response is usually immediate and the dose may be repeated within an hour, however this is usually unnecessary. For patients failing to respond to standard treatment, hyperbaric oxygen, blood or exchange transfusion is indicated. Exchange transfusion is more widely and rapidly available compared to hyperbaric oxygen. Exchange transfusion involves replacement of the patient's red cells with donor cells and has been used in the treatment of various hemoglobinopathies. Case reports on its use in methemoglobinemia are few and indications are based on anecdotal reports.

CONCLUSION: This case highlights several important issues in the diagnosis and treatment of methemoglobinemia. Recognition of a discrepancy between PaO2 and SpO2 followed by prompt diagnosis by the use of co-oximetry is essential. Furthermore, if a patient fails to respond to standard therapy, the use of exchange transfusion should be strongly considered.

DISCLOSURE: Michael Pritchett, None.

Chest. 2006;130(4_MeetingAbstracts):294S-b-295S. doi:10.1378/chest.130.4_MeetingAbstracts.294S-b
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INTRODUCTION: Fulminant liver failure often requires transplant for successful treatment. Causes include viral, toxins, metabolic, autoimmune, and vascular causes. We report a rare, but potentially treatable case of fulminant liver failure, due to a vascular cause.

CASE PRESENTATION: A 59-year-old female with end stage renal disease was admitted with shortness of breath, mild chest and back pain that had progressed over 24 hours. She was afebrile with a blood pressure of 200/104. A non-contrast CT of the chest abdomen & pelvis revealed atherosclerotic changes of the aorta. Laboratory values, including liver panel were normal, except for an elevated creatinine. She treated with antihypertensives, dialyzed and given several percocets for pain control. Her blood pressure stabilized and her dyspnea and pain improved. Three days after admission, she developed acute mental status changes, temp. of 103.1F, and a WBC of 16.5. She was started on empiric antibiotics. Fever work up was negative. Then, she developed right upper quadrant abdominal pain. A repeat liver panel revealed the following values: AST 2711, ALT 4855 and an alkaline phosphatase of 183. The patient was transferred to a tertiary care center for further management. On arrival, she had stable vital signs with equal blood pressures and pulses bilateral upper extremities. Cardiovascular exam was significant for equal pulses with a regular rhythm. Her lungs revealed rales at bilateral bases. Abdominal exam revealed moderate right upper quadrant tenderness, but was otherwise normal with guaiac negative stool. Neurologically, her mental status waxed and waned, intermittently answering questions. She also had asterixis, and was otherwise non-focal. Her laboratory values revealed a WBC of 14.5, PT of 25.3 seconds, PTT of 34.2 seconds, total bilirubin 2.2 mg/dL, AST 6789 iu/L, ALT 7482 iu/L, alkaline phosphatase 299 iu/L, with a lactate of 7.8. Her pH was 7.23. She was started on intravenous acetylcysteine, continued on empiric antiobiotics. A CT of her chest, abdomen, and pelvis which showed a Type III dissection arising distal to the left subclavian artery, proximal false lumen partially thrombosed with prominent flow in false channel in distal thoracic and proximal abdominal aorta with narrowing in true lumen. A liver ultrasound showed an enlarged heterogeneous liver with patent portal veins and left hepatic artery. Patency was not not confirmed in right hepatic artery.Vascular surgery was consulted. The patient's mental status deteriorated, along with her ability to protect her airway and she developed respiratory distress. We discussed further management options with her family, who decided that the patient would not want invasive interventions, and the goals of care were changed to comfort. The patient expired within 8 hours of arrival to the hospital.

DISCUSSIONS: Aortic Dissection has a 15-25% in hospital mortality and 33% have a branch artery occlusion. (1) However, our case is different from other published cases, in that she developed fulminant liver failure from decreased flow due to that flow from the false channel compressed the true lumen, along with the branches to the celiac trunk and superior mesenteric artery. However, our patient is unique due to the fact that she only showed signs of liver failure without mesenteric ischemia.

CONCLUSION: It is important to recognize that a potentially treatable cause of fulminant liver failure, without mesenteric ischemia can be related to a descending aortic dissection. This is a potential treatable cause of liver failure, by either surgical or by endovascular stent placement. (2).

DISCLOSURE: Howard Saft, None.

Chest. 2006;130(4_MeetingAbstracts):295S. doi:10.1378/chest.130.4_MeetingAbstracts.295S-a
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INTRODUCTION: Lemierre's syndrome, an oropharyngeal infection complicated by internal jugular vein thrombosis and metastatic abscesses in lung, is most commonly caused by the anaerobe Fusobacterium necrophorum. No cases, to date, have been found in current literature which document community- acquired Methicillin-resistant Staphylococcus Aureus (CA- MRSA) manifesting as Lemierre's Syndrome.

CASE PRESENTATION: A 37 year old male with history of known IVDA presents with a swollen neck for three days. The patient reported pulling out an ingrown hair on his chin with tweezers one week prior to presentation at which time the patient applied a hot pack to the area, and took some leftover amoxicillin 800mg. The patient then complained of fever, chills, drooling, and dysphonia. A CT scan of the neck demonstrated multiple punctate collections of air within the posterior oropharynx with enlarged right submandibular lymph nodes. The patient's neck swelling became worse and his airway became compromised, necessitating intubation and later a tracheostomy tube was placed. Cultures from I & D of the pharyngeal abscesses isolated CA-MRSA. The patient was then started on vancomycin and remained on ventilator for 2 weeks. A 2D transthoracic Echo was done showing no vegetation on any heart valves and CT of chest showed multiple abscesses in the lung. The patient slowly improved with vancomycin, was extubated and made a full recovery.

DISCUSSIONS: CA-MRSA is becoming more and prevalent in the critical care setting. This is the first documented case of Lemierre's syndrome caused by CA-MRSA that has been found. Lemierre's syndrome is usually caused by the anaerobe Fusobacterium. This presentation of Lemierre's syndrome illustrates the prevalence and variability of CA-MRSA infections.

CONCLUSION: Lemierre's syndrome has never before been documented as being caused by CA-MRSA. As CA-MRSA becomes more prevalent, we may see more unusual presentations of the organism.

DISCLOSURE: Richard Lovy, None.

Chest. 2006;130(4_MeetingAbstracts):295S. doi:10.1378/chest.130.4_MeetingAbstracts.295S-b
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INTRODUCTION: Pulmonary Embolism (PE) affects 0.5%-1 per 1000 people in the general population and the commonest cause of death among hospital inpatients. Intraoperative PE are relatively uncommon, but may occur with specific surgeries such as long bone fractures and tumor surgeries. Clinical presentation is usually sudden with cardiovascular collapse and death. In acute massive PE, 50% of the patient will die within 15 minutes and only 33 % will survive over 2 hours.

CASE PRESENTATION: A 44-year-old Male patient presented with a right ankle fracture. He was scheduled for open reduction and internal fixation. Past medical history was negative. He smoked, drank alcohol and used cocaine. He is 110 kg, height of 190 cm. Patient underwent general anesthesia for the surgery. He was placed on mechanical ventilation to maintain end tidal carbon dioxide tension (ETCO2) between 30 to 35 mmHg. Vital signs remained stable until 60 minutes after induction, following positioning in the left lateral decubitus postion, it was noted that the ETCO2 was 17 mmHg. Pulse oximeter saturation (SPO2) ranging from 95 to 100%. Vital signs remained stable. An immediate search for the cause was undertaken. Auscultation of the chest showed vesicular breath sounds. The breathing circuit did not reveal any leaks or disconnects. Bronchoscopy also showed the endotracheal tube to be in proper position. A possible diagnosis of pulmonary embolism was made and an ABG was sent for analysis. ABG showed respiratory acidosis with a pH of 7.21, pCO2 of 76, with an ETCO2 of 17. The surgeon was notified and surgery was expedited. Patient was kept intubated. Spiral chest CT which showed a pulmonary embolus involving the right main pulmonary artery.The patient was transferred to the ICU where he was started on enoxaparin 1 mg per kg q 12 hours. He also had an IVC filter placed. He made a slow but gradually recovery and was discharged home 2 weeks later.

DISCUSSIONS: As early as 1856, Rudolf Virchow defined vascular thrombosis and its triad –alteration of vessels, alteration of blood elements, and alteration of blood flow are the leading causes of venous thrombosis with subsequent pulmonary embolism.Risks factors include age > 40, varices in the lower limbs, previous DVT, major surgery, neoplasia, obesity, previous MI, CHF, use of estrogen, sepsis. The only risk factor that this patient had is age over 40 and obesity.PE may present in the awake patient with respiratory distress, hypoxia, hemoptysis, pleuritic pain, pleural effusion or shock. In anesthetized patient, initial presentation is usually cardiovascular collapse, however, in our patient, the presentation was decreased ETCO2 with minimal changes in vital signs except for slight reduction in oxygen saturation. Intraoperative decrease in ETCO2 is usually due to hyperventilation, bronchospasm, partial circuit disconnect or decreased cardiac output. Diagnosis can be verified by arterial blood gases (ABG) which showed metabolic acidosis with widened CO2 gradient secondary to massive increase in dead space. The gold standard for the definitive diagnosis of PE is the CT angiogram. Spiral CT may also show the embolus. Other test include duplex ultrasound of the lower extremities which if positive is highly suggestive of PE. D-dimer can be elevated as well.

CONCLUSION: Intraoperative pulmonary embolism if not massive maybe difficult to diagnose like in this case where the only positive finding was a decrease in ETCO2 with increase in PCO2 ETCO2 gradient. ABG should be one of the first line investigations to be performed anytime there is a low ETCO2 after other causes of decrease ETCO2 are ruled out.

DISCLOSURE: Adejare Windokun, None.

Chest. 2006;130(4_MeetingAbstracts):295S-c-296S. doi:10.1378/chest.130.4_MeetingAbstracts.295S-c
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INTRODUCTION: Stroke following sclerotherapy appears to be a rare complication–only two case reports have been published [1,2]. This report describes a well documented case of ischemic stroke following sclerotherapy for esophageal varices. Clinicians should be aware of this possibility when evaluating patients with neurologic disturbances after sclerotherapy.

CASE PRESENTATION: A 50-year old man with alcoholic and Hepatitis C cirrhosis was transferred to our medical center because of recurrent esophageal variceal bleeding. Over the preceding month, he had three episodes of variceal bleeding—controlled each time by endoscopic placement of esophageal variceal bands. On the day of transfer to our hospital, he presented to his local emergency department with a fourth episode of hematemesis. He was intubated for airway protection. A subclavian central venous catheter was placed uneventfully. He was resuscitated with blood products and crystalloid. Emergent endoscopy revealed bleeding grade IV esophageal varices. Because of his recent banding procedures, the gastroenterologist performed intralesional sclerotherapy with sodium morrhuate to hemostatic effect. The patient was then transferred to our hospital for consideration of transvenous intrahepatic portocaval shunt. Upon arrival at our hospital, the patient was hemodynamically stable. He had abdominal distention and dull percussion suggesting abdominal ascites. Initial neurologic exam was non-focal. Arterial blood gas on FiO2 1.0 was: pH 7.33, PaCO2 39, and PaO2 255. A chest radiograph showed clear lungs. Repeat endoscopy showed adherent clot, prior banding, sclerotherapy injection sites, and portal gastropathy without acute bleeding. No interventions were performed. The following day, the patient was observed to have no spontaneous movement of his left side. Motor responses of his left arm and leg were absent. Noncontrast head CT revealed a right hemispheric infarct involving both anterior cerebral artery and middle cerebral artery territories. MRI confirmed several acute infarcts in different vascular distributions, suggesting an embolic etiology. Transcranial doppler ultrasound (TCD) of the cerebral vessels showed hyperdynamic flow, and intravenous injection of agitated saline showed multiple bubbles in the middle cerebral arteries. Doppler ultrasound of his internal carotid arteries and lower extremities were unremarkable. Transthoracic echocardiogram with agitated saline contrast failed to demonstrate an intracardiac shunt. After aggressive rehabilitation, the patient was discharged home but continues to have residual left-sided weakness.

DISCUSSIONS: Neurologic complications following sclerotherapy are rare, but at least two cases of stroke following sclerotherapy have been reported. Both of those patients received injections with polydocanol. Our patient suffered a stroke within 36 hours of sclerotherapy with sodium morrhuate. The presence of cerebrovascular bubbles on the agitated saline portion of his TCD suggests he had an anatomic shunt that permitted paradoxical emboli. The fact that no intracardiac shunt could be identified suggests he may have anatomic intrapulmonary shunts, possibly due to type II hepatopulmonary syndrome. This possibility is further supported by the large alveolar arterial oxygen difference while FiO2 was 1.0.

CONCLUSION: This report describes a well-documented case of stroke following sclerotherapy. Thorough evaluation failed to identify another potential cause of stroke, and both vascular imaging and blood gas data suggested he had anatomic intrapulmonary shunt allowing paradoxical embolization of either sclerosant material, sclerosant-induced thrombus, or sclerotherapy-associated intravariceal air. Although rare, this complication can be devastating. Some authors have suggested pre-sclerotherapy screening for intracardiac shunts to identify at-risk patients [2], but in an emergent situation this may not be feasible. Stroke should be considered in patients with neurologic disturbances following sclerotherapy. Optimal ways to screen for patients at risk and to prevent this complication remain unknown.

DISCLOSURE: Carrie Chun, None.

Chest. 2006;130(4_MeetingAbstracts):296S. doi:10.1378/chest.130.4_MeetingAbstracts.296S-a
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INTRODUCTION: Acquired hemophilia A is a rare cause of post-operative bleeding. We present a case of acquired hemophilia A associated with acute pancreatitis.

CASE PRESENTATION: A 52 year old previously healthy man was transferred to our hospital for management of complications related to acute pancreatitis. He initially presented to an outside hospital with a diagnosis of acute pancreatitis which resulted in a short, uncomplicated hospitalization. He returned three weeks later complaining of abdominal pain, nausea, vomiting, fevers, and twenty pound weight loss. He was transferred after an abdominal CT demonstrated extensive peripancreatic abscesses and partial pancreatic necrosis. The patient continued to deteriorate after initial treatment with antibiotics and percutaneous drainage of the peripancreatic abscesses. Exploratory laparotomy was performed. During surgery the patient became profoundly coagulopathic and hemodynamically unstable. A damage control closure was performed and he was brought to the SICU for resuscitation. The patient received over fifty units of packed red cells, platelets, crypoprecipitate, fresh frozen plasma, and recombinant human factor VIIa (rFVIIa) during his initial resuscitation. Because of continued bleeding he was returned to the OR for further exploration. No distinct bleeding source was identified and he was returned to the SICU. He remained in the SICU for two weeks due to recurrent bleeding. Tagged RBC scan demonstrated a proximal duodenal source. Endoscopy identified no active bleeding, but clotted blood at the duodenal papilla was suggestive of hemosuccus pancreaticus. During these subsequent bleeding episodes his partial thromboplastin time (PTT) was persistently elevated at 88 seconds with INR minimally elevated at 1.37. Mixing studies did not correct the PTT, suggesting a factor inhibitor. Individual factor activity testing was notable for 1% factor VIII activity. Bethesda assay demonstrated 16.5 BU/mL. The patient had no personal or family history of bleeding disorders. He was diagnosed with acquired hemophilia secondary to factor VIII inhibitor. The patient was initially treated with prednisone and received additional rFVIIa for bleeding episodes. He was later treated with cyclophosphamide and rituximab for persistent PTT elevation. He improved and was discharged hospital day 56. Factor VIII activity and PTT normalized by 1 month after discharge.

DISCUSSIONS: Acquired hemophilia is a rare condition with an estimated incidence of 0.2 to 1 cases/million persons/year. It has an associated mortality rate of 7.9 to 22%.1 Associated conditions include pregnancy, malignancies, autoimmune disorders, and allergic drug reactions, though many cases remain idiopathic. To the best of our knowledge this is the first reported case associated with acute pancreatitis.The diagnosis of acquired hemophilia can be difficult, due to its rarity and due to different bleeding patterns compared to congenital hemophilias. Hemarthrosis is rare, whereas severe bleeding is a frequent presentation of acquired hemophilia.1 Laboratory evaluation demonstrates a prolonged aPTT that doesn't correct with mixing. Low factor VIII levels without history of bleeding problems makes the diagnosis more likely.The treatment of acquired hemophilia A involves treatment of the associated condition (e.g. malignancy, allergy), treatment of bleeding episodes, temporary reduction of the inhibitor titer, and eradication of the autoantibody. In patients like ours with a high inhibitor titer (>5 BU/mL) treatment of bleeding includes the use of rFVIIa or porcine factor VIII concentrates. High inhibitor levels can be reduced with plasmapheresis or immunoadsorption. Immunosuppressants are used to eliminate the autoantibody.1.

CONCLUSION: Coagulopathies are common in critically ill post-operative patients. In certain cases, however, further workup of a persistent coagulopathy may reveal other causes such as an acquired hemophilia.

DISCLOSURE: Jon Fuerstenberg, None.

Chest. 2006;130(4_MeetingAbstracts):296S-b-297S. doi:10.1378/chest.130.4_MeetingAbstracts.296S-b
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INTRODUCTION: αInvasive fungal infections represent a major complication of organ transplantation. Zygomycoses have a devastating clinical course and portend a poor prognosis with high mortality. Prior case reports and case series have demonstrated a favorable outcome following a combination of medical and surgical treatment for infections with rhizopus species. Infection with Absidia corymbifera in transplant recipients however, has been associated with a fatal outcome. We describe a case of Absidia corymbifera brain abscess in a lung transplant recipient who was successfully treated with a combination of medical and surgical treatment.

CASE PRESENTATION: A 21-year-old Caucasian male three months status post bilateral lung transplantation for end stage lung disease secondary to cystic fibrosis presented with right-sided temporal headache with retro-orbital pain, nausea, and photophobia. Immunosuppression on admission included tacrolimus, prednisone, and azothioprine. He was afebrile, vital signs were normal, and pulse oximetry was 100% on room air. Exam revealed right eye photophobia with blepharospasm, no meningismus or focal neurological deficit. MRI of brain revealed a 2.5cm right parietal ring enhancing cystic lesion with leftward midline shift and pansinusitis (Figure 1). Sinus aspiration and culture revealed Pseudomonas aeruginosa. Therapy was initiated with intravenous piperacillin/tazobactam, ciprofloxacin, oral voriconazole, and dexamethasone. Four days later, his headaches recurred. Neurologic exam was non focal and MRI of the brain did not reveal any significant change. Brain biopsy and aspiration of the lesion revealed “septate” hyphae suggestive of Aspergillus, however zygomycetes could not be ruled out and lipid complex amphotericin was initiated. His headaches persisted and he developed mental status changes two weeks into therapy with amphotericin. Repeat MRI of the brain revealed progression and he subsequently was taken to the operating room for a right parieto-occipital craniotomy with drainage of abscess and occipital lobectomy. Surgical specimens revealed fungal elements consistent with Absidia corymbifera (Figure 2). He was subsequently treated with a 10 week course of liposomal amphotericin after which he had fully recovered with mild residual deficits.

DISCUSSIONS: Invasive fungal infections represent a major complication of organ transplantation. The incidence of non-Aspergillus mold infections in transplant recipients has increased over the past decade with a reported incidence of 1-9%, 60 days after transplantation and the mortality has remained high. Risk factors include high dose corticosteroids, multiple or recent rejection episodes, hyperglycemia, poor transplant function, leucopenia, and older age.Zygomycoses usually have a devastating clinical course with a 56% overall mortality. Successful therapy involves co-ordinated combination of surgical removal of devitalized tissue and intravenous amphotericin B 1. Absidia corymbifera infections have been reported to involve cutaneous, rhino-cerebral, pulmonary and gastro-intestinal sites and are known to be voriconazole resistant. It is an uncommon cause of brain abscess. Although several cases of absidiomycosis have been described in literature, a vast majority of them have been fatal except for “cure” reported with pulmonary infection in a non-immunocompromised host 2. Outcome with rhino-cerebral infection has invariably been fatal. This is the first case reported of Absidia corymbifera brain abscess in a lung transplant host which was successfully treated with a combination of medical and surgical therapy.

CONCLUSION: There should be a high level of suspicion for potential zygomyces infections in immunocompromised hosts. Early diagnosis and surgical debridement along with amphotericin are requisite for a successful outcome.

DISCLOSURE: Nehal Bhatt, None.

Chest. 2006;130(4_MeetingAbstracts):297S. doi:10.1378/chest.130.6.1924
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INTRODUCTION: Granulomatous Pneumocystis carinii pneumonia is a rare subset of PCP in non-HIV patients.We present a rare case of granulomatous PCP,diagnosed on lung biopsy and did well with treatment.

CASE PRESENTATION: A 63-year-old woman who underwent an autologus bone marrow transplant 4 months back for multiple myeloma was admitted to the hospital for cough, rhinorrhea and a new right upper lobe pulmonary nodule. She denied other symptoms. She gave a past medical history of parainfluenza pneumonia, coagulase negative staphylococcus bactremia and hypertension. She had a 30 pack year smoking history but quit smoking five years back. Her medications were Valtrex, Triamterene, Leucovorin, Oxypamidronate and multivitaminsOn examination: Vital signs were normal and pulse oximetry was 98%.Her physical examination was normal. Her laboratory findings were all normal including CBC, Basic chemistry and lactate dehydrogenase.Blood culture and sputum culture were all negative. Chest radiograph and CT scan (Figure 1) showed a 1.5 X 1.1cm right upper lobe noduleShe was empirically started on antibiotics. Flexible bronchoscopy was performed. Broncho-alveolar lavage (BAL) demonstrated only alveolar macrophages and microscopic examination with appropriate stains and cultures for microbes were negative including Pneumocystis carinii pneumonia (PCP) direct fluorescence antibody (DFA) test. She underwent video-assisted thoracoscopic surgery (VATS) and lung biopsy. Histopathology showed several granulomas with central necrosis (fig. 2A and 2B).The AFB stains were negative. What is your diagnosis?.

DISCUSSIONS: Diagnosis: Granulomatous Pneumocystis carinii pneumoniaPneumocystis carinii pneumonia (renamed Pneumocystis jiroveci pneumonia) is a life-threatening opportunistic infection in immunosuppressed persons. PCP is increasingly recognized in HIV seronegative immunosuppressed transplant recipients and in patients undergoing treatment for hematological malignancies, collagen-vascular disorders and Wegener's granulomatosis with corticosteroids and /or chemotherapeutic agents (1). Long course corticosteroid treatment by in itself increases the risk of infection.Granulomatous Pneumocystis carinii pneumonia is a rare subset of PCP. In HIV infected patients with PCP the incidence of this subset is 5 % and lesser in non-HIV patients. Bondoc and White found only 3 cases of granulomatous PCP among all cases of PCP in HIV seronegative patients with malignancy, at a large tertiary cancer center over a 12-year period (1). These authors and others have pointed out several features of granulomatous PCP that make it difficult to diagnose. The onset of illness was insidious in these cases with mild cough or dyspnea with variable fever. Nodular or reticulonodular infiltrates were seen on chest radiography and the serum lactate dehydrogenase (LDH) values were normal or only slightly raised. Bronchoscopy, broncho-alveolar lavage (BAL) and transbronchial biopsy were nondiagnostic and open lung biopsy was necessary for diagnosis.The histological appearance of granulomatous PCP varies from an ill-defined granulomatous pneumonia to a well-formed necrotizing granuloma containing PCP. The typical intra-alveolar eosinophilic frothy exudate associated with PCP is absent.PCP is readily stained with Gomori methenamine-silver (GMS) and immunohistochemical stains. However appropriate histochemical stains and microbiological cultures are needed to rule out tuberculosis and fungal infections especially Histoplasma capsulatum, which typically shows a granulomatous pattern. Pneumocystis carinii and Histoplasma capsulatum organisms are similar in size, shape and staining characteristics. Treatment of granulomatous PCP is same as the usual form of PCP (1). Trimethoprim–sulfamethoxazole (TMP-SMZ) is very effective in both treating and preventing pneumocystis pneumonia.Our patient had adverse reactions to TMP-SMZ previously, so she was treated with Atovaquone , continued on prophylaxis and did well without any PCP recurrence in two months follow up.

CONCLUSION: In conclusion, awareness of various features of granulomatous PCP, as exemplified in our case, is important for early diagnosis and successful treatment of this potentially lethal infection.

DISCLOSURE: Manish Joshi, None.

Chest. 2006;130(4_MeetingAbstracts):297S-b-298S. doi:10.1378/chest.130.4_MeetingAbstracts.297S-b
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INTRODUCTION: Pulmonary involvement in Plasmodium falciparum malaria is frequently observed. It is associated with most malaria-associated deaths. The WHO (World Health Organization) recommends exchange transfusion (ET) in addition to standard pharmacologic therapy of quinidine or artemisinin derivatives in patients with severe malaria., especially those patients who develop coma, renal failure, or adult respiratory distress syndrome (ARDS) and is recommended regardless of the level of parasitemia, even less than 10%. However there are number of reports that question this modality of treatment. We present a case of 60-year-old male who presents to the hospital with complaints of fever, chills and shortness of breath. He was admitted and diagnosed with P. falciparum malaria, developed acute respiratory distress syndrome requiring mechanical ventilation. After intensive supportive care, treatment with intravenous quinidine and ET, the patient was eventually discharged.

CASE PRESENTATION: This is a 60-year-old male with past medical history of chronic obstructive pulmonary disease and malaria and recently arrived from Senegal, who was admitted to the hospital with complaints of shortness of breath, fever and chills for five days. He was diagnosed with P. falciparum malaria and was started on quinine and doxycycline orally. On the third hospital day he developed increased shortness of breath and increased oxygen requirements. A chest roentgenogram revealed diffuse bilateral infiltrates consistent with ARDS. He was transferred to the intensive care unit and intubated. In consultation with the infectious diseases service, oral quinine and doxycycline was discontinued and intravenous quinidine was substituted. His parasite load was 7.7%. Exchange transfusion was initiated for his ARDS and severe malaria. He improved dramatically, was extubated in 7 days and transferred out of the intensive care unit and discharged home after 12 days.

DISCUSSIONS: P. Falciparum is known to cause severe malaria and poses the greatest threat of death because it is often drug resistant, invades red cells of all ages, and the only one of the plasmodia species that produces microvascular disease. Our patient had a history of malaria and had recently traveled from an endemic area. He was diagnosed with P. falciparum infection with an initial parasite load of 7.7% and developed ARDS. The WHO recommend exchange transfusion for parasitemia greater than 10 % or for lower parasite loads in cases with evidence of organ dysfunction [1]. Severe malaria is considered if the patient has jaundice, oliguria, altered consciousness, severe anemia and/or pulmonary edema. Theoretically, ET for severe malaria has 3 beneficial effects: (1) rapid reduction of parasitemia, [2] improvement of the red blood cell structure and morphology thus decreasing vascular obstruction and endothelial cell injury by the parasitized cells and (3) removal of pro-inflammatory cytokines. The true utility of this therapy is unclear as a number of studies question its role in severe malaria. However no randomized controlled trials have been conducted to analysis its efficacy [2]. Our experience in this patient supports the use of exchange transfusion in severe malaria patients with ARDS.

CONCLUSION: In our patient with severe malaria and ARDS due to P. falciparum, exchange transfusion was successfully used in conjunction with intravenous quinidine and should be considered in other patients who present similarly.

DISCLOSURE: Sanjeev Kumar, None.

Chest. 2006;130(4_MeetingAbstracts):298S. doi:10.1378/chest.130.4_MeetingAbstracts.298S-a
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INTRODUCTION: Introduction: Rhinoscleroma is an important cause of nontraumatic subglottic stenosis. It is caused by the organism Klebsiella rhinoscleromatis, which is endemic in Central America, and has an affinity for the mucosa of the upper respiratory tract. Overcrowding and lower socioeconomic status are important epidemiological factors in its transmission. The disease often begins with rhinitis and progresses over years to a destructive fibrotic phase. Treatment requires a prolonged course of antibiotic.

CASE PRESENTATION: A twenty-year-old Hispanic female, who was a lifelong nonsmoker, presented with one-year history of progressive shortness of breath. Symptoms began shortly after emigrating from Mexico, with chronic rhinitis, which progressed over the subsequent year to exertional dyspnea and stridor. Flow volume loop was suggestive of fixed airway obstruction, CT scan of the neck and chest showed narrowing of the subglottic area to three centimeters secondary to circumferential thickening of the subglottic area. The CT of the chest was otherwise unremarkable. Flexible bronchoscopy showed thickened, erythematous mucosa from the level of the posterior pharynx to the subglottic area. Rigid bronchoscopy with dilatation was performed with debridement and biopsy of the subglottic tissue. Biopsy was negative for granulomatous tissue, with areas of chronic and focally acute inflammation, cultures grew Klebsiella rhinoscleromatis. The patient was subsequently lost to follow up.

DISCUSSIONS: This case illustrates the importance of recognizing rhinoscleroma as a cause of subglottic stenosis. The disease most commonly affects the nasal passages, initially leading to an exudative phase with nasal congestion, edema and suppurative necrosis. The next phase is the proliferative or granulomatous phase characterized by reddish nodules. Biopsies of the affected areas in this phase may reveal a characteristic finding in pathologic specimens known as Miculikz cells, which are vacuolated histiocytes containing the organism. The disease then progresses to the final stage, characterized by fibrosis. Granulomatous inflammation may lead to destruction of bone and cartilage in the upper respiratory tract. Curative treatment is often challenging, ciprofloxacin has been shown to have the greatest in vitro activity against Klebsiella rhinoscleromatis. Treatment should be continued for 6 months to one year. Treatment in the fibrotic stage is often supportive, and may include tracheal dilatation, laser excision, and tracheostomy.

CONCLUSION: Rhinoscleroma is an important cause of nontraumatic subglottic stenosis, and should be considered in individuals who have emigrated from endemic areas. As the number of individuals from endemic areas is increasing in the United States, this disease may be seen with increasing frequency. Clinical suspicion and early, prolonged treatment are required to prevent progression to the fibrotic stage.

DISCLOSURE: Pradeep Ramachandran, None.

Chest. 2006;130(4_MeetingAbstracts):298S-b-299S. doi:10.1378/chest.130.4_MeetingAbstracts.298S-b
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INTRODUCTION: Strongyloidiasis is an intestinal nematode infection caused by Strongyloides stercoralis. The prevalence of S. stercoralis is higher in HTLV-1 positive patients. Enhanced proliferation and decreased clearance is well documented in immunocompromised hosts. This hyperinfective state is associated with invasion of the gastrointestinal and respiratory system and may result in widespread dissemination into other body organs.

CASE PRESENTATION: We present a case of a 35-year-old Jamaican with a history of HTLV-1, who initially presented with severe abdominal pain, vomiting, twenty-pound weight loss and iron deficiency anemia. Endoscopy with biopsy revealed intestinal flattening with Strongyloides parasites (Figure 1). He was treated for his intestinal strongyloidiasis with ivermectin for two days. Three years later, he presented with a two-month history of fever, cough, shortness of breath, approximately ten-pound weight loss and generalized lymphadenopathy. Lymph node biopsy revealed adult T-cell lymphoma. The chest radiograph and CT scan revealed multiple parenchymal infiltrates. Bronchosopy with bronchoalveolar lavage revealed larvae of Strongyloidoses stercolaris (Figure 2). He was given six courses of hyper-CVAD regimen for Adult T-cell Leukemia/lymphoma (ATLL), as well as a two-day course of ivermectin and a ten-day course of albendazole 400mg twice daily for Strongyloidiasis. The patient was discharged after clinical improvement. Within a month after discharge, the patient was re-admitted with a history of increasing dyspnea at rest. While on broad-spectrum antibiotics and anti-parasitic therapies, the patient developed worsening hypoxemic respiratory failure and gram negative septicemia with Vancomycin-resistant enterococcus faecium. Despite aggressive treatment he expired due to multisystem organ failure.

DISCUSSIONS: Disseminated Strongyloidiasis is a significant contributor to the morbidity and mortality in immunocompromised patients. The mortality rate has been documented up to 86%. HTLV-1 and Strongyloidiasis are well-documented co-morbidities. Patients co-infected with HTLV-1 had higher levels of interferon-gamma and interleukin (IL) -10 and lower levels of IL-4, IL-5 and IgE than patients without Strongyloidiasis without HTLV-1. This was indicative of a relative switch from Th2 to Th1 response, which is important in the control of helminthic infection. This may contribute to severity of the disease and failure to respond to standard therapy. Eosinophils are thought to play an important part in protecting the host from fulminant infection. Our patient also had a poor eosinophil response.Gram-negative sepsis is a major cause of mortality in these patients. The organism is thought to play a part in direct spread of enteral organisms during its migration. Due to the overall immunocompromised state, standard therapy with albendazole and ivermectin for hyperinfection may not have been sufficient to eradicate the organisms. Further testing of the stool or special staining of the biopsy tissue should have been performed to ensure complete eradication of the organism. In cases where worm eradication is impossible or re-infection can not be confirmed, repeat treatment with thiabendazole, albendazole or ivermectin is indicated. The treatment duration may need to be extended based on clinical resolution. Short monthly courses of antihelminthic therapy have been shown to decrease the worm load and thus, prevent recurrent systemic disease.

CONCLUSION: In summary, conventional treatment duration's for S. stercoralis, predicated on normal host immunity, may not be sufficient to eradicate the organism in immunocompromised hosts.

DISCLOSURE: Agnieszka Petersen, None.

Chest. 2006;130(4_MeetingAbstracts):299S. doi:10.1378/chest.130.4_MeetingAbstracts.299S-a
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INTRODUCTION: Babesiosis is a tick-borne disease caused by parasites of the genus Babesia resulting in an intraerythrocytic infection that ranges from an asymptomatic state to a life-threatening illness. Human disease in the United States is most commonly due to B. microti. Transmission usually occurs via a bite from the Ixodid tick. We present a rare case of transfusion-associated babesiosis.

CASE PRESENTATION: A 57-year-old male was brought to our facility with complaints of chest and abdominal pain for 3-4 days. He also complained of passing melenic stools and having dark urine. His past medical history was significant for hepatitis C, cirrhosis, hypertension, and coronary artery disease. Six weeks earlier, the patient had been hospitalized with an upper GI bleed at which time he received several units of packed red blood cells. Upper endoscopy revealed the presence of a gastric ulcer. Current review of systems was remarkable for fever, chills, dyspnea, anorexia, and fatigue. He denied any travel outside of Texas within the past five years. Exam was notable for mild to moderate distress, jaundice, tachycardia, and diffuse abdominal tenderness without rebound or guarding. Laboratory studies revealed a white count of 6.5 with left shift, hemoglobin of 8, platelets of 12, INR of 2.7, LDH of 5539, total bilirubin of 8.7, and unconjugated bilirubin of 4.2. The patient was started on proton pump inhibitor therapy and transfusion of blood products was initiated. Given the elevation of bilirubin and the likelihood of hemolysis, the peripheral smear was reviewed. The presence of intraerythrocytic parasites was noted (see image). Based on the appearance of the parasites, the patient was started on multi-drug treatment for babesiosis. Despite aggressive therapy, his condition continued to deteriorate and he expired. Serologic testing confirmed the diagnosis of babesiosis postmortem. Transfusion-related transmission was confirmed after contact was made with the CDC. Of the three donors from whom the patient had received blood products on prior admission, one had positive serology for B. microti. Although this donor was not from an endemic region, he did have frequent travel to the northeast where babesiosis more frequently occurs.

DISCUSSIONS: Babesiosis is a disease that is mostly found in the United States and Europe. Despite its rarity, the incidence appears to be increasing in part due to expansion of endemic regions and migration of animal as well as human hosts. Of major concern is the effect that this entity has on the blood supply. Those most likely to be affected are immunocompromised hosts, asplenic individuals, and the elderly. In the case of our patient, he appeared to be more susceptible to fulminant infection because of his cirrhotic state. The keys to management remain early recognition and implementation of appropriate therapy. In most individuals infected with B. microti, clinical findings are mild. Severe manifestations are usually only observed with infection of high-risk individuals.

CONCLUSION: Babesiosis is a rare disease that is growing in incidence due to several factors including increased recognition and better diagnostic measures. It remains the most important transfusion-related tick-borne disease. More judicious use of transfusion is advisable in order to limit the possibility of infections. Better preventive strategies need to be implemented to protect both our blood supply and high-risk transfusion recipients.

DISCLOSURE: Rajesh Babu, None.

Chest. 2006;130(4_MeetingAbstracts):299S. doi:10.1378/chest.130.4_MeetingAbstracts.299S-b
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INTRODUCTION: Sarcoidosis is a poorly understood granulomatous disease that involves the lung and intrathoracic lymph nodes in more than 90% of patients.We report a unique case of a patient with sarcoidosis who presented with features suggestive of pulmonary embolism.

CASE PRESENTATION: A 68-year-old lady with history of hypertension and sarcoidosis presented with complaint of shortness of breath, chest pain and palpitation. Chest pain was localized to the left side with no radiation and there was no nausea, vomiting, diaphoresis, gastrointestinal or genitourinary complaints. On exam, she was tachycardic and tachypneic with blood pressure 185/102. Lungs were clear and remaining examination was unremarkable. Laboratory results reveal troponin of 0.24, blood gas- pH 7.47, pCO2 33.7mmHg,pO2 66.6mmHg and O2 sat 95.2% on rooom air.CBC and chemistry were normal. Chest xray(CXR) revealed bilateral hilar fullness with no infiltrates.EKG showed sinus tachycardia @110/min,T wave inversion in leads 3, V1 to V4, Q waves in lead 3 and an S wave in lead 1. A diagnosis of pulmonary embolism was entertained to rule out myocardial ischemia. Patient was started on anticoagulation and ventilation-perfusion(V/Q) scan and serial cardiac enzymes ordered.V/Q scan revealed normal ventilation with a large perfusion defect in the left lung field and small defect in the right lower lobe(high probabilty for Pulmonary Embolism). Echocardiography showed right ventricular dilatation, Left ventricular hypertrophy and ejection fraction of 55%. Lower extremity venous doppler was negative. Chest CT scan revealed enlarged bilateral hilar and mediastinal adenopathies encasing and compressing the left pulmonary artery(PA)with cut of flow and minimal compression of the right PA. V/Q mismatch now considered to be due to extrinsic compression of the left main pulmonary artery by adenopathies secondary to sarcoidosis.Bronchoscopy was negative for malignancy and all cultures negative. Anticoagulation was discontinued and steroid therapy commenced. Her functional capacity improved after about 2 weeks and repeat CT chest was said to have shown reduced lymphadenopathies.

DISCUSSIONS: The clinical presentation of sarcoidosis are widely varied.The histologic hallmark of sarcoidosis is the noncaseating(nonnecrotizing) granuloma composed of epitheloid cells and multinucleated giant cells. Special staining for acid fast bacilli and fungi should be performed to exclude infectious etiologies.Mechanisms of vascular involvement in sarcoidosis include (a)external compression by enlarged lymph nodes (b)granuloma surrounding the vessel walls and (c)granulomatous infiltration of the vessel wall.Compression of the pulmonary arteries by adenopathy is rare and may result in decreased pulmonary artery perfusion mimiking pulmonary artery embolism. The extent of compression will vary proportionately with the degree of adenopathy. Granulomata tends to surround small blood vessels, causing the loss of lung alveolocapillary transfer surface and decreased diffusing capacity.Granulomatous infiltration of the vessel walls may occur, and there may be intimal involvement, vascular thrombosis, and even vessel wall destruction.The extent of granulomatous vascular involvement was related to that of parenchymal granuloma.Chest CT scan demonstrating extrinsic compression of both PA by adenopathies spared this patient a long and unnecessary anticoagulation therapy.

CONCLUSION: Physicians should keep in mind that lung ventilation-perfusion mismatch in Sarcoid is not always due to thromboembolic disease and may be secondary to external compression by lymphadenopathies and in those cases such patients would respond to treatment of Sarcoid rather than anticoagulation.

DISCLOSURE: Adekunle Adekola, None.

Chest. 2006;130(4_MeetingAbstracts):300S. doi:10.1378/chest.130.4_MeetingAbstracts.300S-a
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INTRODUCTION: Alveolar hemorrhage leading to respiratory failure is uncommon. Various etiologies have been reported, including systemic lupus erythematous (SLE), which generally presents as pulmonary-renal syndrome. We report a case of SLE with massive hemoptysis and acute renal failure(ARF), successfully treated with plasmapheresis.

CASE PRESENTATION: 65-years-old woman with history of hypertension and positive lupus anticoagulant presented with massive hemoptysis and respiratory failure. Patient denied chest pain, fever, recent upper respiratory symptoms, gastrointestinal or urinary problems. No toxic habits or travel history. She has two children with no history of abortions.On examination patient was in respiratory distress and hypoxemic with a SaO2 90% on 100% non rebreather mask. She was afebrile with diffuse crepitations on the right lung. Rest of examination was normal including blood pressure. Laboratory results showed hemolytic anemia (Hb 18 g/dl- baseline was 34 five months ago) ARF (creatinine 7.2 mg/dl -baseline 1.1 five months ago). Chest roentgenogram and computed chest tomogram revealed diffuse bilateral infiltrates predominantly on the right side. Perfusion scan and echocardiogram were normal.Serology was positive for ANA, Ds DNA. Antineutrophil cytoplasmic antibodies (cANCA), anti-glomerular basement membrane (anti-GBM) antibodies and cardiolipin antibodies were negative. The patient was intubated and fiberoptic bronchoscopy showed diffuse oozing of blood from all right lung segments. Hemosiderin-laden macrophages were seen in the BAL. A diagnosis of SLE with diffuse alveolar hemorrhage and ARF was entertained and patient was given pulse steroids and cyclophosphamide with some improvement in infiltrates and renal insufficiency and resolution of hemoptysis. Five days later she had recurrent hemoptysis with new left lung infiltrates and hypoxemia despite the use of continuous systemic corticosteroids. Three cycles of plasmapheresis were given with resolution of hemoptysis and infiltrates. Patient was successfully extubated. Kidney biopsy was not performed due to the critical state of the patient.Subsequently she had a prolonged hospital course complicated with nosocomial pneumonia, sepsis and multiorgan failure and finally she expired after three months of hospitalization.

DISCUSSIONS: Diffuse alveolar hemorrhage leading to respiratory failure is uncommon. Various etiologies have been reported, including infections, inhaled toxins, coagulation disorders, catastrophic antiphospholipid syndrome, Goodpasture's syndrome, microscopic polyangiitis and Wegener's granulomatosis, as well as various types of collagenosis, such as scleroderma and SLE.Pulmonary-renal syndrome is characterized by the occurrence of both alveolar hemorrhage and glomerular-nephritis and is frequently associated with the presence of antineutrophil cytoplasmic antibodies or anti-GBM antibodies. The type of injury, (alveolar, glomerular or both) determines the evolution and prognosis of the syndrome.A diagnosis of alveolar hemorrhage is made with the findings of hemoptysis, new alveolar infiltrate, anemia and the presence of blood or hemosiderin-laden macrophages in BAL like in our patient. In SLE patients, diffuse alveolar hemorrhage is uncommon, occurring in only 2% of cases, and is often associated with mortality rates between 70-90%. Recurrent hemoptysis is seen in 10% of the cases. Concomitant renal involvement is also observed. Histological studies have shown that, in about 70% of lung biopsies, there are little inflammatory activity and a predominance of hemorrhagic characteristics, whereas in the remaining 30%, a neutrophilic capillaritis or diffuse alveolar damage is seen. Pulse steroids followed by high dose steroids with cyclophosphamide have been shown to control pulmonary hemorrhage. In case series, plasmapheresis had been used for recurrent hemoptysis although no survival benefit has been shown. Tacrolimus and immunoglobulin can be tried in refractory hemoptysis.

CONCLUSION: Diffuse alveolar hemorrhage is a life-threatening manifestation of SLE. An active diagnostic workup and aggressive immunosuppressive treatment are the cornerstones of the management. Early detection and active treatment of secondary infections are obligatory. Plasmapheresis needs to be considered in cases with refractory hemoptysis.

DISCLOSURE: Praveen Rudraraju, None.

Chest. 2006;130(4_MeetingAbstracts):300S. doi:10.1378/chest.130.4_MeetingAbstracts.300S-b
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INTRODUCTION: Amiodarone induced pulmonary toxicity is a well described phenomenon in patients receiving amiodarone chronically for tachydysrythmias. In the ICU, amiodarone is a common first-line therapy for patients with the onset of atrial and ventricular tachydysrythmias. Acute amiodarone pulmonary toxicity (APT) is a rare form of diffuse alveolar damage that may occur within days of initiating therapy with amiodarone to ventilated patients receiving high inspired fraction of oxygen (FiO2). We present a case of acute APT in our medical intensive care unit (ICU).

CASE PRESENTATION: A 58-year-old female with alcohol dependence presented at an outside hospital intoxicated with ataxia and confusion. After five days of detoxification, she remained confused and ataxic and was transferred to our institution for further evaluation. She was admitted to a neurology ward and diagnosed with Wernicke encephalopathy. On hospital day number eight she developed fever, hypoxia, and right lower lobe infiltrates. She was diagnosed with hospital acquired pneumonia and started on appropriate antibiotics. Intermittently, she developed atrial fibrillation (AF) with rapid ventricular response (RVR) which readily responded to intravenous rate controlling agents (metoprolol and diltiazem). On hospital day number 13, she had another episode of AF with RVR during which she had altered mental status and aspirated. She was transferred to the ICU where she was intubated and placed on mechanical ventilation. Her AF was initially rate controlled with additional doses of rate controlling agents and ultimately the addition of 720 mg of amiodarone loaded IV over 19 hours followed by 200 mg orally daily. During this time she developed a clinical picture consistent with acute respiratory distress syndrome (ARDS). Her diffuse alveolar infiltrates initially improved, but 6 days later, 4 days after completion of IV amiodarone, her chest x-ray showed increased peripheral and basilar alveolar densities in a “reverse pulmonary edema pattern,” and her oxygen requirement increased. Non-contrasted CT scan was consistent with APT showing dense infiltrates. Amiodarone was stopped and she was started on high dose corticosteroids (methylprednisolone 125mg every 6 hours). Lung infiltrates improved over the next week such that her chest x-ray resembled imaging done prior to the acute worsening attributed to APT.

DISCUSSIONS: Although APT is well described in surgical ICU patients who have undergone cardiothoracic surgery, only three other cases of APT have been described in patients with ARDS.1 Unfortunately, its pathophysiology is poorly understood. Leading theories include adaptive-immune-mediated hypersensitivity and direct drug induced phospholipidosis causing alveolar cell damage.2 There is little evidence on how to best diagnosis this entity or on how to treat it. Diagnostic strategies in this patient population include use of bronchial-alveolar lavage, computerized tomography, gallium-67 imaging, and lung biopsy. Interestingly, the CT findings in this patient were similar to those found in chronic APT, a finding not previously described in this patient population. Advocates for pulse dose steroids draw from literature supporting steroid use in APT caused by chronic amiodarone use.

CONCLUSION: APT should be suspected in ventilated patients receiving amiodarone who have sudden, otherwise unexplained worsening of their oxygen requirements associated with increased peripheral alveolar consolidations, especially if densities on CT are similar to that of bone. Although this is a rare disorder, its prevalence is likely underestimated due to poor recognition. A national database on ICU patients receiving amiodarone has been called for.2.

DISCLOSURE: Jeremy Pamplin, None.

Chest. 2006;130(4_MeetingAbstracts):300S-c-301S. doi:10.1378/chest.130.4_MeetingAbstracts.300S-c
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INTRODUCTION: Acute fibrinous and organizing pneumonia (AFOP) has been recently identified as an unusual variant of acute lung injury. The typical features of AFOP are an acute or subacute onset of illness, bilateral basilar infiltrate and histologic findings of intra-alveolar fibrin in the form of fibrin “balls”. All the previously reported seventeen patients with AFOP were identified by a retrospective review of case material at the Armed Forces Institute of Pathology. We report a patient who had received a heart transplant and developed AFOP while on corticosteroids and other immunosuppressants. To the best of our knowledge, this is the first prospectively diagnosed patient with AFOP.

CASE PRESENTATION: A 70 year old white male was referred to the pulmonary clinic for evaluation of a recent increase in shortness of breath on exertion with wheezing but no other Sympotms. He had received a cardiac transplant eight years ago for ischemic cardiomyopathy and had been receiving cyclosporine and prednisone until two months ago when prednisone was discontinued. In addition, he had twenty pack-year history of smoking (with abstinence since cardiac transplant), mild COPD, and a prior positive PPD skin test for which he received INH for six months.On physical exam, the patient had bilateral wheezing and tachycardia. Pulmonary function testing revealed mild worsening of his baseline airway obstruction with significant improvement post bronchodilator. The chest x-ray was normal, but CT scan of the chest showed central bronchiectasis. The patient's airway obstruction responded both clinically and spirometrically to a tapering dose of prednisone for six weeks. First relapse: when the prednisone dose was decreased to 10 mg po qd, the patient developed productive cough, fever, chills and a left lower lung infiltrate on chest X-ray. Bronchoscopy was performed, but the bronchalveolar lavage, the microbiology and cytology brushes, and transbronchial biopsy were non-diagnostic. He was treated with a two-week course of Levofloxacin and prednisone was again increased to 40 mg po qd. The patient improved clinically and radiographically within one month. Second relapse: two months later, he again developed severe dyspnea, fever, and productive cough. Chest x-ray showed a new infiltrate in the left lower lung. The patient was admitted to the hospital and treated with intravenous methylprednisolone and broad spectrum antibiotics. Bronchoscopy with bronchial wash and microbiology and cytology brushes was non-diagnostic once more. The patient's respiratory status deteriorated with worsening dyspnea, hypoxemia, and progressive bilateral interstitial markings. Multiple surgical biopsies from the left upper and the left lower lobes showed acute fibrinous and organizing pneumonia with prominent intra-alveolar fibrin balls.The patient showed significant improvement with a maintenance dose of prednisone 15 mg po qd. Follow up: six months after the hospital discharge he was stable and clinical and physiological functions had returned to baseline.

DISCUSSIONS: Our patient is the first reported patient with complete clinical description of his pulmonary status before developing the disease as well as during follow-up and recovery. He presented at age 70, which was within the range reported by Travis (33-78, average 62 years). When added to Travis' data, race continues to be non-contributory with 4 hispanics, 1 black, and now 11 whites affected (2 cases had unknown race)(1).Our patient is interesting in that he has history of obstructive airway disease, which may represent a risk factor for future development of AFOP under certain triggering factors.

CONCLUSION: Acute fibrinous and organizing pneumonia is likely underreported and under diagnosed. Identification of precipitating factors, clinical course, and factors affecting prognosis and treatment still need to be established.

DISCLOSURE: Mohammad Jarbou, None.

Chest. 2006;130(4_MeetingAbstracts):301S. doi:10.1378/chest.130.4_MeetingAbstracts.301S-a
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INTRODUCTION: Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis characterized by bone marrow histiocytes and lymphocytes, and long bone sclerosis sparing the epiphysis. Nonosseus disease occurs in the brain, orbit and vessels with typical pulmonary involvement manifesting as diffuse interstitial disease and pleural thickening.

CASE PRESENTATION: A 60-year-old previously healthy man presented in 2002 with intermittent right body spasticity and dysarthria. Brain magnetic resonance imaging (MRI) demonstrated abnormal signals in the pons and midbrain, and enhancing right temporal lesions. Electroencephalogram was negative for seizure activity. No diagnosis was established and there was no improvement after a brief trial of steroids. Chest computed tomography only revealed small noncalcified densities in the left lung (5 mm) and right middle lobe (RML) (3 mm). Positron emission tomography(PET) uptake was positive in the brain lesions only. Bronchoscopy with RML transbronchial biopsies were non-diagnostic, revealing reactive foreign body giant cells. Repeat brain MRIs demonstrated waxing and waning variable regression of the brain lesions. The patient was lost to followup, and re-presented in September 2005 with progressive dyspnea, left-sided weakness, spasticity, and diplopia. His exam was now significant for proptosis, cranial nerve palsies, left hemiparesis, and ataxia. Imaging revealed bilateral pleural effusions and a 7.0 x 4.5 cm soft tissue mass, with infiltration to the RML and right lower lobe (RLL), and a left lung soft tissue lesion. Sclerotic bone lesions were seen in the sternum, vertebral bodies, and humeri. PET scan uptake was present in the pulmonary lesions and brain. Rheumatological studies, HIV testing, and studies for coccidiomycosis, cryptococcus, and histoplasma were negative. Thoracentesis yielded clear, exudative, lymphocytic predominant fluid negative for malignancy and pathogens. Bronchoscopy suggested a stenotic RML orifice. Transbronchial biopsies revealed non-specific inflammation, and poorly defined histiocytes. Cultures were negative. Thorascopic biopsy demonstrated a hard mass in the RML and RLL and thickened pleura. RML biopsies demonstrated chronic fibrosis and foamy histiocytes, which stained positive for CD68. A bone marrow biopsy revealed histiocytes staining positive for CD68 and negative for s-100. Only reactive astrocytes and mild gliosis were seen on temporal lobe brain biopsy. Skeletal surveys showed bilateral long bone sclerosis with sparing of the epiphyses.

DISCUSSIONS: The constellation of findings in this patient was most consistent with Erdheim-Chester Disease (ECD).The differential diagnoses under consideration included necrotizing sarcoid granulomatosis, lymphomatoid granulomatosis, malignancy, and lymphoma. Sarcoidosis was not supported by multiple biopsies, and marrow findings were not representative of lymphoma. The waxing and waning nature of the lesions were inconsistent with malignancy or metastasis. Langerhan's Histiocytosis, Behcet's and Hashimoto's were considered. Rheumatologic studies and biopsies were not supportive of vasculitis. No infection was identified despite numerous cultures of multiple tissues. The diagnosis of ECD was supported by the bone marrow findings of histiocyte clusters positive for CD68 and negative for S-100. ECD was also supported by bilateral long bone sclerosis with eepiphyseal sparing. Other histiocytoses such as eosinophilic granuloma present with lytic bone lesions. Pulmonary nodules and masses in ECD have not been well described, although retroperitoneal soft tissue masses due to fat infiltration by histiocytes have been reported. Pleural thickening and pleural effusions have been described in ECD.

CONCLUSION: ECD should be considered in the differential diagnosis of a patient presenting with pulmonary, neurological, and osseus manifestions of unclear etiology. This rare entity has variable presentations, and requires exclusion of other malignant, infectious, and inflammatory processes. Interstitial lung disease and pleural involvement are most commonly reported, but pulmonary involvement can also include mass lesions.

DISCLOSURE: Joanne Bando, None.

Chest. 2006;130(4_MeetingAbstracts):301S-b-302S. doi:10.1378/chest.130.4.1143
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INTRODUCTION: Pulmonary Langerhans cell histiocytosis [LCH] is a smoking-related interstitial lung disease characterized by development of bronchiolocentric lesions containing histiocytic cells called Langerhans cells. Treatment options for patients with pulmonary LCH are limited. We report a unique case of Langerhans cell histiocytosis with prominent lung, bony and lymph node involvement that responded to treatment with systemic 2-chlorodeoxyadenosine [2-CDA] treatment.

CASE PRESENTATION: The patient, a 66-year-old never smoker, initially presented at another facility with 1-2 months of shoulder pain and a left supraclavicular mass. Empiric antibiotic treatment for presumed infection did not improve symptoms or resolve the supraclavicular mass. Subsequently, a CT of the chest, abdomen and pelvis was performed and demonstrated cervical lymphadenopathy, several pulmonary nodules [the largest measuring 7 cm in the right lower lobe], and a 2 cm subcarinal lymph node. A deep cervical lymph node biopsy showed diffuse infiltration by S100 and CD1A positive Langerhans cells with areas of necrosis. Due to the presence of bilateral pulmonary nodules and subcarinal adenopathy, a bronchoscopy with BAL and biopsies was performed. Bronchoscopic biopsy of one of the nodular lesions demonstrated Langerhans cell histiocytosis. An MRI was obtained because there was a history of back pain. This demonstrated lesions involving multiple vertebral bodies. Due to multiple organ involvement from LCH with lung, bone, and lymph node disease, the patient was treated with 4 cycles of 2-CDA (5mg/m2). Following treatment, the patient reported resolution of back pain, and CT chest demonstrated near complete resolution of the lung lesions.

DISCUSSIONS: : Langerhans cell histiocytosis is characterized by proliferation and infiltration of organs by specific types of dendritic cells called Langerhans cells. Pulmonary LCH occurs predominantly in young adult smokers. Recent studies show that more than 90% of patients with pulmonary LCH are either current or former smokers. Patients may be asymptomatic at presentation. Radiologic findings vary depending on the stage of disease when imaging is obtained. Early in disease the predominant HRCT finding is the presence of small nodules, some of which demonstrate cavitation. In later stages of disease, cystic changes may be found and as the disease progresses to an advanced stage, the cystic changes predominate. The chest CT in pulmonary LCH rarely demonstrates pulmonary nodules without associated cavitation or cystic change, as observed in our patient. Treatment of LCH is primarily targeted toward smoking cessation, which frequently leads to stabilization. Although the overwhelming majority of patients with pulmonary LCH are cigarette smokers, it is important to recall that smoking is not an absolute requirement for development of disease. Indeed, most published series imply that approximately 5-10% of patients have no prior history of either personal or second-hand cigarette smoke exposure, as is the case in our patient. The decision to treat this patient was based on the presence of bony disease in the spine, rather than the lung involvement. Since the patient was a non-smoker, and had multiple organ involvement, we treated with systemic therapy. Corticosteroids have been used in the management, but there is limited data to support their efficacy. The antimetabolite 2-CDA was chosen because reports in the literature demonstrate its ability to induce complete or partial remission of disease in adult patients with refractory LCH.

CONCLUSION: This case is unique because it demonstrates a unique radiologic presentation of pulmonary LCH and documents near complete resolution of pulmonary lesions with 2-CDA therapy. The patient tolerated treatment with 2-CDA well without side effects. Treatment with 2-CDA should be considered as a therapeutic option in patients with progressive pulmonary LCH. Whether this agent has therapeutic activity in smokers with progressive pulmonary LCH remains to be demonstrated by prospective studies.

DISCLOSURE: Michelle Aerni, None.

Chest. 2006;130(4_MeetingAbstracts):302S. doi:10.1378/chest.130.4_MeetingAbstracts.302S-a
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INTRODUCTION: Fibrosarcoma involving the pleura is extremely rare. I present a case of a young woman presenting with a pleural mass of spindle cell origin, found to be a fibrosarcoma.

CASE PRESENTATION: A 33-year-old Mongolian woman, with no known medical problems, presented with a 4 month history of right-sided chest pain and progressive shortness of breath. A computed tomographic scan of the chest revealed a large well-circumrscribed mass arising from the posterior aspect of the right lower lobe with an associated large pleural effusion (see figure1). Pleural fluid showed a lymphocytic exudate, with negative cytology. Thoracotomy revealed a 5cm mass that “popped out” of the lung and was free-floating in the pleural space. The resected mass had a fleshy tan appearance with areas of hemorrhage and necrosis. Microscopically, the tumor was composed of densely packed, regualr spindle cells showing an overlapping “herring-bone” pattern, with a moderate mitotic rate and foci of necrosis see (figure2). Immune stains showed the following results: CD31, CD34, calretinin and cytokeratins, were all negative. These results were consistent with a grade 2 fibrosarcoma of the pleura. Following surgery, the patient was discharged home with follow up with an oncologist.

DISCUSSIONS: Following radiographical analysis, the mass can be characterized as pleural based, of soft-tissue density, with an area of heterogeneous attenuation in the pleural effusion possibly consistent with blood. This pleural-based tumor may be benign or malignant. Benign tumors include benign fibrous tumors, lipomatous tumors, or multicystic mesotheliomas. Benign tumors with malignant potential include pleural thymomas and desmoid tumors. Based on the gross appearance of the resected tumors, specifically the areas of necrosis and hemorrhage, the most likely diagnosis is a primary malignant pleural neoplasm. These include localized malignant mesotheliomas, malignant solitary fibrous tumors, synovial sarcomas, and hemangiopericytomas. Based on the initial histological examination, spindle cells were identified. The differential diagnosis of a spindle cell neoplasm is rather large. In order to differentiate these, immunohistochemistry is performed. In our patient, based on immune stains, we excluded various spindle cell neoplasms such as mesothelioma of all types, synovial sarcoma, solitary fibrous tissue and hemangiopericytoma. The “herring-bone” pattern, whereby the bundle of spindle cells intersect at right angles, confirmed the diagnosis of fibrosarcoma. The grade of the fibrosarcoma tumor appears to depend on the number of mitotic figures as well as the presence of necrosis or hemorrhage. Grade 2 fibrosarcomas, as was in this case, are intermediate between grade 1 and grade 3. Grade 1 tumors have slightly larger nuclei than fibroblasts that characterize fibromatosis, and no areas of hemorrhage or necrosis. Grade 3, on the other hand, have closely packed cells with numerous mitotic figures, marked chromatin abnormalities and frequent necrosis.

CONCLUSION: This case highlights that although pleural tumors are not commonly seen, the differential diagnosis is extremely wide. Fibrosarcoma appears to be a diagnosis of exclusion based on immunohistochemistry as well as the classic “herring-bone” patttern. Pleural fibrosarcoma is very unusual, and our knowledge regarding its behaviour, prognosis and management is very limited. Nevertheless, reports have shown that prognosis appears to be related to the size and grade of the tumor.

DISCLOSURE: Serene Shashaa, None.

Chest. 2006;130(4_MeetingAbstracts):302S-b-303S. doi:10.1378/chest.130.4_MeetingAbstracts.302S-b
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INTRODUCTION: Bilothorax is a rare complication occuring after trauma, invasive procedures applied to abdomen. This case report of bilothorax was occured after an operation of pyloric stenosis since there were no fistula or rupture of diaphragm.

CASE PRESENTATION: 35-year-old man was admitted to the hospital with the complaints of nausea, vomiting, weightloss. He had a diagnosis pyloric stenosis in his gastrointestinal endoscopy. He had underwent on surgical intervention under general anaesthesia and a hemigastrectomy with gastrojejunostomy was performed. The complaints of dyspnoea and left chest pain were appeared on the postoperative 4th day. Physical examination revealed the loss of breath sounds at the lower field of left hemithorax. Chest roentgenogram showed a pleurisy at the left lower zone and thoracentesis revealed bilothorax (Table 1-Figure 1). On his computerized tomography, a left sided pleural effusion and the atelectasis of left lobar segments with calsification were detected. Abdominal exploration revealed there was no diaphragmatic defect or fistula, the same effusion in the abdominal space and a leakage from duedonal stump were detected. It had been drained about 2000 ml of effusion, the leakage from the duedonal stump was primarily repaired. Thereafter abdominal drain were inserted. After this procedure therapeutic thoracentesis applied if it was necessary. Postoperative 25th days, there was no fistula or defect on the diaphragmatic surface through fistulography. The chest X ray showed a minimal density on the left lower zone on the 32 th day.

DISCUSSIONS: In the English literature, the bilothorax was resulted usually from a fistula due to a trauma or related with a surgical intervention, diaphragmatic defect, another disease of the bile duct. Also it could be occured by the transmission of abdominal effusion from the diaphragmatic pores to the thorax. We are thinking that the reason of the bilothorax in our patient could be the latter. A significant contributing factor may be that he had a previous pyloric stenosis and gastric surgery, and they had been a faciliating effect of the passage of the bile. He had a left sided pleural effusion, and there was a few case reports of the left bilothorax in the literature related with thoracobilier fistula after trauma or invasive procedures. In the case report of Rowes, there was a perforation of the afferent loop of an old gastrojejunostomy (1 ).

CONCLUSION: In summary, the bilothorax is not a common complication of upper abdominal surgery. In this case, we should be carefully evaluated the reason, since it may be an important complication of the surgical procedure.

DISCLOSURE: Canan Hasanoglu, None.

Chest. 2006;130(4_MeetingAbstracts):303S. doi:10.1378/chest.130.4_MeetingAbstracts.303S-a
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INTRODUCTION: Lymphangiomatosis is a rare disorder characterized by multiple neoplasms and abnormal development of the lymphatic system. The thorax and neck are the most common sites involved, and disseminated disease can lead to a particularly agressive variant.

CASE PRESENTATION: A previously healthy 18-month-old male presented with a one week history of progressive dyspnea, with no history of fever, cough, or trauma. Chest imaging demonstrated a massive left pleural effusion (Figure 1). Thoracoscopy and pleural fluid analysis revealed chylothorax, and an ill-defined cystic lesion noted on the pleural surface of the left upper lobe. Biopsy of the pleural lesion was unremarkable. Despite continuous total parenteral nutrition, massive loss of chyle required repeat thoracoscopic exploration, which confirmed significant chyle leak after administration of a cream meal. The post-operative period was complicated by the development of empyema requiring antibiotic treatment and thoracoscopic debridement and drainage of loculated fluid. Following thoracic duct ligation, no further pleural fluid accumulation occurred. Evaluation for the underlying etiology of chylothorax was negative including a skeletal survey, abdominal CT, and several lung/pleural biopsy samples. The patient was discharged home in good condition. Five months later, he was rehospitalized due to significant respiratory distress with hypoxemia and left chest wall soft tissue swelling following an acute febrile illness. Chest imaging demonstrated bilateral pleural effusions, edema of the left chest wall with extension into subcutaneous tissue, and lytic bone lesions of the left humerus and clavicle. Thoracoscopic exam of the left pleural space revealed small effusion and edematous visceral pleura with dense adhesions. Partial pleural debridement, incisional biopsy of the chest wall soft tissue, and bone biopsies were performed. The procedure was complicated by unexpected cardiac arrest and death due to refractory shock. Autopsy findings showed lymphatic vascular malformations within the interlobular septa and subpleural regions of the upper and lower lobes of the left lung, the deep soft tissues of the neck and left chest wall, and involvement of the periosteum of the left clavicle and proximal humerus consistent with lymphangiomatosis. The morphology and immunostaining were consistent with the diagnosis (Figure 2).

DISCUSSIONS: Lymphangiomatosis is characterized by widespread and progressive abnormality of the lymphatic system. It typically presents in late childhood, but can occur from birth up to 80 years. Lesions can develop in any tissue, but lungs, mediastinum, pleura, chest wall, and spleen are most frequently involved, and bone lesions are common. In a recent literature review of 52 cases with thoracic lymphangiomatosis, chylothorax was the most common presentation (49%) followed by bone lesions (39%). The coexistence of lytic bone lesions and chylothorax serves as an important diagnostic clue, however, histologic examination is required for definitive diagnosis. Lymphangiogram, and recently, whole body lymphoscintigraphy can be used for visualization of the lymphatic vasculature. MRI is more sensitive than bone scan, and can reveal unsuspected bony lesions as well as the extension of soft tissue lesions. The prognosis of disseminated lymphangiomatosis is often poor, and depends on the extent of the disease. Involvement of lung or other visceral organs portends a particularly aggressive course. As seen in the case, infections can abrubtly worsen the pace of the disease. Surgical options are limited to the drainage of pleural and pericardial fluid, pleurectomy, chemical pleuredesis, pleuro- peritoneal drainage, and ligation of the thoracic duct. Systemic chemotherapy with interferon alfa, radiotherapy, ocreotide for neonatal chylothorax, and complete resection of chest wall have been reported successful in isolated cases.

CONCLUSION: The diagnosis and management of lymphangiomatosis is challenging. Diagnosis require a high index of suspicion, and should be considered in cases of unexplained chylothorax.

DISCLOSURE: Gulnur Com, None.

Chest. 2006;130(4_MeetingAbstracts):303S-b-304S. doi:10.1378/chest.130.4_MeetingAbstracts.303S-b
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INTRODUCTION: Pleural effusions can develop with airway obstruction due to atelectasis, postobstructive pneumonia, and/ or malignant pleural studding. Even in the presence of an endobronchial malignancy a significant percentage of these effusions have nonmalignant cytology. This poses a staging dilemma due to the poor sensitivity of thoracentesis. This dilemma leads to lack of confidence in staging, need for further procedures and delays in therapy.Thoracentesis with pleural manometry is used to diagnose trapped lung, safely relieve symptoms, and predict outcome of pleurodesis for malignant effusions.(1,2) The effect of endobronchial intervention on pleural pressures has not been studied. By defining the physiologic character of malignant and paramalignant effusions in this setting we hope to gain information that may solve the dilemma described above. This is the report of our first patient in a prospective series.

CASE PRESENTATION: Our patient is a 52-year-old female with Stage IIIB NSCLC originating in the LUL. Three months after chemotherapy and radiation she complained of worsening dyspnea. Her exam was significant for decreased breath sounds throughout the left side. A CT scan of the chest showed worsening LUL collapse and a moderate effusion. The patient was scheduled for thoracentesis and bronchoscopy with possible intervention. Prior to bronchoscopy, the thoracentesis was performed. Ultrasound guided placement of an 18g angiocath yielded straw colored thin fluid. Pleural pressure at exhalation was found to be -7.5 cm H2O by fluid column and -6 cm H2O by urodynamics monitor (Medtronics, Shoreview, MN) before drainage. The fluid column and monitor had been zeroed together before the procedure. Pleural pressures were reassessed after every 100-200ml removed by syringe pump. After drainage of 200ml the pressure, by both methods, was -9 cm H2O. After 400ml of drainage the pressure was -9 by fluid column and -10 by monitor. After 600ml of drainage the pressure was -16cm H2O by both methods. At this point the drainage slowed and the patient was prepared for bronchoscopy. More than 20ml of fluid remained in the pleural space to ensure accurate readings.On bronchoscopic exam the LUL was occluded, as previously described. The most significant interval change was a new significant stenosis of the lingual. Bronchography was done to further define the anatomy. Then a guidewire was used to feed a 6-7-8mm balloon into the superior subsegment. Flouroscopic images revealed an apple core stricture in the proximal subsegmental bronchus. Dilation to 7mm was successful at opening the bronchus. After bronchoscopy, the transducer, fluid column and catheter were leveled. And the fluid column and the pressure transducer were zeroed A measurement of the pleural pressure revealed a dramatic improvement. Pleural pressure was -5 cm H2O by both methods. Cytology was negative for malignancy in the transudative effusion.

DISCUSSIONS: Endobronchial obstruction and effusions are common complications of lung cancer. In the setting of endobronchial obstruction manometry may prove to be helpful in diagnosis of reversible trapped lung physiology, predicting effusion recurrence or assessing the benefit of an interventional procedure. By directly measuring pleural pressures before and after an intervention we were able to differentiate between a diagnosis of trapped lung and a reversible process. In our patient there was a dramatic change in pleural pressure after intervention by both methods of manometry.

CONCLUSION: Endobronchial obstruction may dramatically effect pleural pressures. We plan to further validate this novel approach to manometry and conduct a series to assess the effects of endobronchial intervention on pleural pressures.

DISCLOSURE: Franklin McGuire, None.

Chest. 2006;130(4_MeetingAbstracts):304S. doi:10.1378/chest.130.4_MeetingAbstracts.304S-a
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INTRODUCTION: Chylothorax is an unusual manifestation of tuberculous disease. Very few cases have been reported in the literature. Here we describe a case of tuberculous chylothorax which was diagnosed on mediastinal biopsy.

CASE PRESENTATION: 29-year-old female patient 4 weeks postpartum referred to us with nonresolving left-sided pleural effusion. Initially she presented to her family physician in her last trimester with a 6 weeks history of evening pyrexia, dry cough, anorexia and undocumented weight loss. Her investigations showed raised ESR and bilateral pleural effusion more on left side. Pleural fluid analysis showed lymphocytic exudate. She was started on anti tuberculosis treatment ( 4 drugs regimen, isoniazid, rifampin, ethambutol and pyrazinamide).In our clinic her physical examination was normal except the findings of left side pleural fluid. Keeping in mind of a young postpartum female with history of fever, cough and weight loss, with CXR evidence of bilateral pleural effusion differential diagnosis of Postpartum cardiomyopathy, Connective tissue disorder, Paradoxical increase in Effusion after starting of anti tuberculous treatment, multidrug resistant tuberculosis and Malignant pleural effusion as ? lymphoma were considered.ANA profile was done which was negative and Echocardiogram was also normal. We repeated pleural fluid analysis and inserted chest tube. The gross appearance of the fluid was milky white. The differential of the fluid was WBC = 100/cumm, polymorphs = 2 % ,Lymphocytes = 96 %, Proteins = 6.14mg/dl, ( serum proteins 7.47 ) LDH = 200 u/L, ( serum LDH 321) Triglycerides = 752mg/dl, cholesterol = 58 mg/dl, glucose = 111 mg/dl. Pleural fluid cytology did not reveal any malignant cells. Pleural biopsy result showed non specific inflammation.CT scan of the chest showed multiple enlarged lymph nodes seen in right Paratracheal, Pretracheal, lateral aortic, Anterior mediastinal, aorto pulmonary window, carinal and subcarinal region. Also seen in bilateral prevertebral and paraaotic region.For mediastinal lymph node biopsy thoracic surgeon was involved, who did thracotomy and mediastinal biopsy. Per operatively thoracic duct leakage was seen at multiple sites. Thoracic duct was ligated. Mediastinal biopsy revealed Chronic Granulomatous inflammation with caseation. Patient was continued on antituberculous therapy and currently improving clinically as well as radiologically.

DISCUSSIONS: Chylothorax is characterized by milky white or tubid appearing pleural fluid due to high lipid content consisting of triglycerides that enter the pleural space as chyle mostly from disruption of the thoracic duct. Non traumatic (72%) causes include most commonly lymphoma (about 50% of all chylothoraces). Other causes include Sarcoidosis, lymphagiomyomatosis, Multiple myeloma, Flariasis and tuberculosis. Anecdotal cases of chylothorax due to Mycobacterium tuberculosis have been reported in the literature. In our patient Lymphoma was a major differential, that's why we did mediastinal lymph node biopsy which came out to be chronic granulomatous inflammation with caseation, which is very rare.

CONCLUSION: We have to think of tuberculosis as a possible etiology of Chylothorax in developing countries where tuberculosis is endemic.

DISCLOSURE: Fayyaz Baig, None.

Chest. 2006;130(4_MeetingAbstracts):304S. doi:10.1378/chest.130.4_MeetingAbstracts.304S-b
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INTRODUCTION: Enlarging pulmonary nodules can be benign or malignant in nature. A rare cause of benign enlarging pulmonary nodules is thoracic splenosis. We describe a case of thoracic splenosis following thoraco-abdominal trauma diagnosed by Tc99m sulfur colloid scan.

CASE PRESENTATION: A 35-year-old woman with a 20 pack year smoking history presented for evaluation of abnormal chest radiograph. Nine years earlier, she was involved in a motor vehicle accident resulting in thoraco-abdominal trauma complicated with bilateral pneumothoraces and splenic trauma. At that time, she required bilateral chest tubes and partial splenectomy. Six year later, a CT of the chest was performed to evaluate persistent back pain just inferior to the left scapula. Imaging revealed two left lower lobe pleural-based nodules –with the larger measuring 2.3 by 1.5 cm –thought to result from posttraumatic pleural scarring. No further evaluation was advised. Four weeks prior to her current visit a chest radiograph, part of a pulmonary evaluation prior to abdominal surgery, revealed two discrete nodules in the left lower lung field. She denied any respiratory symptoms, weight loss or night sweats. Follow-up CT scan demonstrated multiple pleural based left lung nodules. The largest one measured 2.7 by 1.7. The spleen, which was located in the left upper quadrant of the abdomen, measured only 2.4 by 2.1 cm. Given the history of splenic trauma, the patient underwent a Tc99m sulfur colloid scan. As expected, Tc99m uptake was seen at the location of the spleen. In addition, Tc99m uptake was also present in the left lower lung field, corresponding to the 2.7 by 1.7 cm nodule on CT. The latter finding was considered diagnostic for thoracic splenosis.

DISCUSSIONS: Fewer than 40 cases of thoracic splenosis have been reported in the literature. The average duration between injury and diagnosis is 19 years –range 9 to 32 years. Thoracic splenosis results from autotransplantation of splenic tissue in the thoracic cavity following concurrent splenic trauma and disruption of the diaphragm. These implants can be diagnosed by tissue biopsy or, non-invasively, with radioisotope scanning, using either Tc99m sulfur colloid or labeled, heat-denatured red cells. Intrathoracic splenosis is a benign process, which is usually asymptomatic. The nodules can grow slowly. Morphologic characteristics on CT are non specific and alternative diagnoses, such as lymphoma and metastatic disease, need to be considered. Concerns for malignancy may lead to unnecessary biopsy procedures. And such removal of intrathoracic splenic tissue –in a patient without a functioning abdominal spleen –may result in asplenia with the associated increased risk for infections. The presence of Tc99m sulfur colloid activity in the left chest of this patient represented splenic tissue and rendered the need for further diagnostic evaluation unnecessary. Diagnosed 9 years following splenic injury, this case represents a relatively early diagnosis of thoracic splenosis.

CONCLUSION: In a patient with a history of abdominal and thoracic trauma, finding pleural based nodules may represent thoracic splenosis. This diagnosis can be confirmed noninvasively using a radionucleotide scan, such as Tc 99m sulfur colloid scan.

DISCLOSURE: Omar Hussain, None.

Chest. 2006;130(4_MeetingAbstracts):304S-c-305S. doi:10.1378/chest.130.4_MeetingAbstracts.304S-c
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INTRODUCTION: The prevalence of pulmonary hypertension in end-stage renal disease (ESRD) patients receiving hemodialysis (HD) has been documented to be 29%-52% [Ref 1] by echocardiography. With the rising burden of patients with ESRD, pulmonary hypertension in this cohort will be a significant problem for the health-care providers. Different mechanisms have been attributed to the pathogenesis of pulmonary hypertension in patients with ESRD on HD. Alterations in endothelium-dependent vasodilators have been proposed as one of the mechanisms causing pulmonary hypertension [Ref 2]. We present a case of pulmonary hypertension in ESRD on HD that had significant clinical and hemodynamic improvement with dual endothelin-receptor antagonist, bosentan.

CASE PRESENTATION: A 32-year-old African-American female was referred to our pulmonary hypertension clinic for WHO functional class III dyspnea and systolic pulmonary artery pressure of 110 mmHg on echocardiogram. At presentation she had dyspnea on exertion after walking one block that was limiting her activities of daily living. Her past medical history was significant for systemic hypertension, ESRD on HD for 15 years, status post failed renal transplant twice, and multiple arteriovenous fistulae. On examination she had elevated JVP, loud P2, right ventricular heave, palpable liver 6 cms below the right costal margin, and dependent edema up to the mid-shins. Her initial work-up for pulmonary hypertension included V/Q scan, liver function tests and autoimmune panel. She underwent a right heart catheterization, and was started on bosentan. She had marked improvement in activities of daily living on bosentan therapy to the point where she was able to go back to work. After a year and a half of treatment her WHO functional class improved from III to I. We repeated the right heart catheterization, which showed that the RA pressure had declined from 21 to 3 mmHg, mean PA pressure from 52 to 24 mmHg, pulmonary vascular resistance from 9.1 to 3.3 Wood units, and pulmonary capillary wedge pressure from 20 to 7 mmHg. The cardiac index improved from 2.4 to 3.4 L/min/m<sup>2</sup>. There was no significant change in her systemic blood pressure with bosentan therapy.

DISCUSSIONS: Various contributing factors are involved in the pathogenesis of pulmonary hypertension in patients with ESRD on HD. Volume status, diastolic dysfunction, pulmonary vascular disease, and high cardiac output from a left-to-right shunt across the arteriovenous fistula, can all contribute to pulmonary hypertension either singly or in combination. Delineating these 4 etiologies can be difficult without invasive hemodynamics. Our patient had evidence of diastolic dysfunction and fluid overload at initial catheterization as suggested by the elevated wedge pressure. However, the fall in pulmonary vascular resistance by 64% with marked improvment in cardiac index is strongly suggestive of pulmonary vascular disease as the primary underlying etiology for her pulmonary hypertension.

CONCLUSION: The reversal of pulmonary hypertension with dual endothelin-receptor antagonist in our patient supports the hypothesis that in patients with ESRD on HD, there is an alteration in endothelium-mediated vasodilators causing vasoconstriction and pulmonary hypertension. The pharmacokinetics and safety of bosentan in ESRD has already been demonstrated. This is the first reported case of improvement of pulmonary hypertension associated with ESRD on bosentan therapy.

DISCLOSURE: Tabarak Qureshi, None.

Chest. 2006;130(4_MeetingAbstracts):305S. doi:10.1378/chest.128.6_suppl.618S
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INTRODUCTION: For patients with a moderate-to-large ventricular septal defect (VSD), surgical repair is recommended in infancy or childhood to prevent pulmonary hypertension (PH) and subsequent right-to-left shunting (Eisenmenger Syndrome, ES). Historically, adults with an unrepaired VSD and ES have a poor prognosis as the operative mortality and risk of post-operative PH prevents surgical correction. We describe an adult with ES who was treated pre-operatively with epoprostenol and ultimately underwent successful surgical repair of his VSD as well as aortic valve replacement (AVR).

CASE PRESENTATION: A 44-year-old man with shortness of breath, hypoxemia, and VSD was referred to our hospital for consideration for heart transplantation. The VSD had been diagnosed during infancy, but had never been surgically corrected. Throughout childhood the patient had been asymptomatic. One year prior to presentation he had been diagnosed with bronchiolitis obliterans-organizing pneumonia (BOOP). He was treated with steroids with improvement in his symptoms, however he continued to require supplemental oxygen. After four months of steroid therapy the patient began to notice worsening dyspnea and hypoxemia in the absence of radiographic changes. At this point he was referred to our institution. At presentation the patient was New York Heart Association (NYHA) functional class 3. An echocardiogram revealed severe mitral regurgitation, severe aortic regurgitation, a dilated left ventricle, a dilated right ventricle with normal systolic function, and a mid-septal muscular 10 mm VSD. Right heart catheterization (RHC) revealed a pulmonary artery pressure (PAP) of 107/36 mm Hg (mean 64) and a wedge pressure of 33 mm Hg. The calculated pulmonary vascular resistance (PVR) was 6.05 U/m2. After inhaled nitric oxide (NO) the PAP decreased to 78/26 mm Hg (mean 43). Examination of oxygen saturations on room air revealed significant hypoxemia as well as right-to-left shunting. In an effort to decrease the PVR and therefore right-to-left shunting, the patient was started on epoprostenol. He noted an almost immediate improvement in symptoms. Repeat RHC one month later revealed a decrease in the PVR to 1.55 U/m2 and reversal of right-to-left shunting. Four months later he underwent cardiac surgery for VSD repair, mitral valve repair and AVR. Post-operatively he temporarily required inhaled NO for elevated PAPs. After the inhaled NO was stopped, sildenafil was started. One month after surgery the patient was NYHA class 1 and no longer required supplemental oxygen.

DISCUSSIONS: In this case we describe the use of epoprostenol in a patient with ES prior to surgical correction of a VSD. The use of prostacyclins in patients with PH associated with congenital heart disease (CHD) has been previously described. To our knowledge, however, only one case report has been published describing the use of prostacyclin in a patient with severe PH prior to surgical repair of CHD. In that report, the patient received epoprostenol for four years prior to the diagnosis of an atrial septal defect (ASD). After repair of the ASD, the patient was weaned off epoprostenol over the next six months. In contrast, our experience suggests a shorter duration of epoprostenol pre-operatively may be sufficient to allow surgical repair of CHD with ES, and post-operatively epoprostenol may be unnecessary.

CONCLUSION: In adult patients with uncorrected CHD and ES, short-term pulmonary vasodilator therapy prior to surgery may allow a subset of patients to undergo surgical repair of CHD who would otherwise not be surgical candidates.

DISCLOSURE: Sarah Schmidt, None.

Chest. 2006;130(4_MeetingAbstracts):305S-b-306S. doi:10.1378/chest.130.4_MeetingAbstracts.305S-b
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INTRODUCTION: The etiology of pulmonary arterial hypertension (PAH) is undefined. Serotonin is thought to have a role in the pathogenesis of PAH. We report the development of PAH in a patient with an islet-cell tumor of the pancreas and elevated levels of 5-hydroxyindoleacetic acid (5-HIAA), the major metabolite of serotonin.

CASE PRESENTATION: The patient is a 39-year-old male, who had presented with palpitations eight years back. An elevated alkaline phosphatase prompted an abdominal ultrasound that demonstrated a 12x20 cm pancreatic mass, encasing the superior mesenteric artery and vein. Biopsies were suggestive of an islet cell tumor. Immunochemical stains were positive for synaptophysin and chromogranin, with focal areas staining for glucagon and insulin. An octreotride scan was intensely positive. Serial CT scans over the next six years showed stable tumor size so no chemotherapy was initiated. His only symptoms consisted of facial flushing after drinking red wine and occasional diarrhea. In early 2004, he noted increasing dyspnea on exertion (WHO functional class 3). During the next three months, he had two episodes of syncope. An echocardiogram showed a normal left ventricle and a pulmonary artery systolic pressure of 81 mmHg. Right heart catheterization confirmed PAH with a pulmonary artery pressure (PAP) of 73/24 mmHg, a mean PAP of 48 mmHg and a wedge pressure of 15 mmHg. Cardiac output was 4.89 L/min and pulmonary vascular resistance was 540 dyne*sec/cm5. He did not have a response to nitric oxide. His six-minute walk time was 354 meters, with desaturation to 76 percent on room air. 5-HIAA level in the urine was elevated at 10.9 mg/24 hours (normal < 6.0). The patient was placed on oxygen and warfarin. Bosentan (125 mg twice daily) was added for treatment of PAH in combination with fluoxetine (20 mg once daily). He reported a significant improvement in his symptoms (WHO class 2) and no longer destaturated with walking.

DISCUSSIONS: Neuroendocrine cells are present throughout the body and secrete serotonin. In the lungs these cells also secrete other vasoactive peptides in response to hypoxia and hypercarbia. These cells have been found to proliferate in patients with PAH. Serotonin plays a role in the pathogenesis of PAH probably via its potent vasoconstrictor properties and induction of smooth muscle hyperplasia. On a molecular level, polymorphisms in serotonin transporter gene (SERT) may effect expression of bone morphogenetic protein receptor-2 (BMPR-2), the gene whose mutation is associated with familial PAH. Patients with PAH have been found to have high serotonin levels due to problems with processing serotonin. Current research has shown that treatment with serotonin-selective reuptake-inhibitors (SSRI) may be associated with improved outcomes in patients with PAH. Our patient had an elevated serotonin level due to a pancreatic neuroendocrine tumor. Chronic stimulation to his pulmonary vasculature probably led to the development of PAH. He showed significant clinical improvement with the combination of an endothelin-receptor blocker (bosentan) and an SSRI.

CONCLUSION: Patients with increased serotonin levels are at risk for the development of PAH. The role for combination therapy with endothelin-receptor blockers and SSRIs in the treatment of PAH should be explored further.

DISCLOSURE: Paul Strachan, None.

Chest. 2006;130(4_MeetingAbstracts):306S. doi:10.1378/chest.130.4_MeetingAbstracts.306S-a
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INTRODUCTION: Hydatid disease, or Echinococcosis, most commonly affects the liver, although echinococcal cysts are found in the lungs in 25% of cases. Pulmonary hypertension (PH) from echinococcal embolization to the pulmonary vasculature is quite rare, however, usually occurring with cardiac involvement. We report a patient with echinococcal embolic PH without cardiac involvement treated with targeted pulmonary vascular therapy and resection of the hepatic cyst.

CASE PRESENTATION: A 37-year-old Egyptian male was diagnosed with a single hepatic echinococcal cyst 7 years prior to our evaluation. He was treated with albendazole and praziquantel with intermittent compliance. Four years later, CT scanning of the chest revealed bilateral cavitating lung cysts. A thoracoscopic biopsy revealed an echinococcal cyst and pulmonary vascular changes consistent with PH. He was referred to our center. A right upper quadrant ultrasound revealed a 15 cm hydatid cyst in the right lobe of the liver. A Ventilation/Perfusion scan revealed multiple bilateral segmental perfusion defects and normal ventilation. No cysts were seen in the heart or thoracic vena cava on magnetic resonance angiography. A transthoracic echocardiogram showed a normal left ventricle, moderate right ventricular dilation and an RV systolic pressure of 81mmHg. During a 6 minute walk test (6MWT) the patient ambulated 1050 ft. A right heart catheterization showed a right atrial pressure of 9 mmHg, a pulmonary artery pressure of 106/37 mmHg (mean PA 66 mmHg), and a cardiac index of 2.2 L/min/m2; no wedge pressure was measured. He was treated with furosemide, albendazole, bosentan, and intravenous epoprostenol with rapid clinical and hemodynamic improvement, and the patient underwent resection of the hepatic cyst. The cyst encased the right hepatic vein and the cavity was open to the lumen of the inferior vena cava (IVC). The cyst and part of the IVC were excised. He was discharged one month later with the addition of sildenafil, and epoprostenol was discontinued. By 11 months, he reported a return of his exercise tolerance to preoperative baseline (6MWT distance = 1305 ft) on sildenafil, bosentan and albendazole, and he had returned to work by one year after surgery.

DISCUSSIONS: E. granulosus is the most common form of the human tapeworm Echinococcus. Endemic to the Middle East, this parasite has a lifecycle which involves a definitive host (usually a wild dog) which passes eggs in its feces, and an intermediate host (usually a grazing animal) which ingests the eggs. Humans are “accidental” intermediate hosts. Eggs typically encyst in the liver, but may travel to the lungs and other organs. Severe PH in echinococcosis is rare and is usually attributable to rupture of a cardiac cyst with embolization to the lungs. Our patient had no cardiac cysts; the intraoperative findings suggest that chronic seeding of cyst contents into the IVC with embolization to the pulmonary circulation had occurred. Our patient not only had histologic changes consistent with pulmonary arterial hypertension (PAH), but also responded to PAH therapy, demonstrating the potential importance of a small vessel arteriopathy in this disease. Case reports of acute and chronic echinococcal pulmonary emboli with PH describe a poor prognosis, with death occurring within months. In this case, peri-operative treatment with bosentan and epoprostenol permitted the safe resection of the hepatic cyst, eliminating a possible source for further embolization. Continued treatment of his PH has led to functional improvement for more than one year.

CONCLUSION: While the prognosis for PH in the setting of echinococcosis is poor, targeted therapy for PH may be clinically effective for this rare condition.

DISCLOSURE: William Bulman, Grant monies (from industry related sources) Research support from Actelion Pharmaceuticals and Pfizer, Inc.

Chest. 2006;130(4_MeetingAbstracts):306S-b-307S. doi:10.1378/chest.130.4_MeetingAbstracts.306S-b
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INTRODUCTION: Practice guidelines recommend that patients with idiopathic pulmonary arterial hypertension (IPAH) undergo vasodilator testing to determine potential responders to calcium channel blockers (CCBs). Patients who fail adequate doses of CCBs may benefit from other therapies believed to have broader efficacy.

CASE PRESENTATION: KS is a 37 year-old athletic woman who was without medical problems until age 31 when she developed chest pain and pre-syncope during a 4th of July potato sack race. She was diagnosed with IPAH and treated empirically with diltiazem CR, 300 mg daily with slight improvement in symptoms. Two years later she underwent catheterization, was noted to have a markedly elevated pulmonary arterial (PA) pressure, was challenged with epoprostenol and found to be reactive. Her diltiazem was increased to 420 mg daily, but because there was no improvement in her symptoms, the endothelin-receptor blocker bosentan, 125 mg twice daily was added to the regimen. At age 36, with no improvement in her symptoms, she was referred to our center to evaluate her for intravenous epoprostenol therapy. Her assessment at that time included a treadmill test by Naughton-Balke protocol where she walked for 6 minutes and 14 seconds; (4.40 METS). Cardiac catheterization done on the diltiazem and bosentan demonstrated a PA pressure of 85/40 mmHg with a mean of 56mmHg and a pulmonary vascular resistance (PVR) of 9.78 Units. Upon administration of adenosine at 50 mcg/kg/min, the PA pressure decreased to 45/20 mmHg with a mean of 26 mmHg and the PVR to 2.71 Units. Based upon this vasodilator response, bosentan and diltiazem were discontinued and amlodipine 20 mg daily was begun. One year later, KS had a marked improvement in symptoms with a treadmill test response of 14 minutes and 16seconds, (8.40 METS). Cardiac catheterization now demonstrated: PA pressure of 38/15 with a mean of 25 mmHg and a PVR of 3.98 Units. She was maintained on her high dose CCBs and continues to do well.

DISCUSSIONS: This case re-illustrates the importance of vasodilator testing in patients with IPAH, and the fact that in patients who respond, effective therapy requires treatment with high-dose CCBs, as opposed to conventional dose CCBs. Moreover, this case refutes the notion that endothelin-receptor blockers have broader efficacy than the CCBs, and suggests their mechanism of action is distinctly different than the vasodilator actions of the CCBs or epoprostenol.

CONCLUSION: High-dose CCBs remain first-line therapy for vasoreactive patients with IPAH. Endothelin-receptor blockers are not an adequate substitute for the CCBs in these patients.

DISCLOSURE: William Borden, None.

Chest. 2006;130(4_MeetingAbstracts):307S. doi:10.1378/chest.130.4_MeetingAbstracts.307S-a
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INTRODUCTION: Pulmonary venoocclusive disease (PVOD) is a rare disorder characterized by extensive and diffuse occlusion of the pulmonary veins by fibrous tissue that frequently results in a rapid progression to death. Etiology is uncertain, but factors including genetics, infection, thrombosis, autoimmune disease and exposures to toxins, such as chemotherapy, have been implicated in its development. Prognosis is generally poor with mortality near 100% at two years. Treatment with vasodilators has been advocated but may be complicated by the risk of worsening pulmonary edema. We report a case of PVOD treated successfully at one year with sildenafil monotherapy.

CASE PRESENTATION: A 53-year-old female with metastatic melanoma presented to the National Cancer Institute for an investigational therapy with tumor infiltrating lymphocytes (TIL). Treatment included high dose IL-2 followed by cyclophosphamide, fludarabine, total body irradiation, TIL cell infusion and CD-34+ stem cell infusion. The patient tolerated the treatment well and was discharged home. Four months later, the patient presented with 1 week of progressive exertional dyspnea. She had mild tachypnea but her physical exam was otherwise normal. A CXR, V/Q scan, HRCT scan of the chest, echocardiogram and a BNP level were normal. The 6 minute walk distance was 144 meters. On day 3 of the hospitalization, she became hypoxic at rest requiring oxygen. An echocardiogram was repeated and revealed an elevated pulmonary artery pressure (PAP) (tricuspid regurgitant (TR) jet of 3.5 m/s) and a normal left ventricle. A right heart catheterization was performed showing an elevated mean PAP and pulmonary vascular resistance that improved after 25 mg of sildenafil was administered. A lung biopsy was performed and was consistent with PVOD. Sildenafil therapy was continued at 25 mg three times daily and within two weeks supplemental oxygen was tapered and the patient had rapid and dramatic resolution of her resting dyspnea. Repeat echocardiogram after 3 months showed a TR jet of 2.8 m/s. One year later, the patient remained well on sildenafil 50 mg three times daily and had dyspnea and chest tightness only with exertion. The six minute walk was increased to 318 meters and the TR jet was reduced to 2.6 m/s. A right heart catheterization revealed exercise induced hemodynamic worsening associated with symptoms of chest tightness. Sildenafil was increased to 75 mg three times daily. Headaches were the only side effect of treatment and were well controlled with oral analgesics.

DISCUSSIONS: Treatment of PVOD presents a unique challenge because vasodilator therapy may result in arterial vasodilation without venous vasodilation increasing transcapillary hydrostatic pressure resulting in the formation of pulmonary edema. Optimal treatment is uncertain and mortality remains unacceptably high. In a recent randomized trial, sildenafil, a specific inhibitor of phosphodiesterase type 5, was shown to decrease PAP and increase 6 minute walk distance in patients with idiopathic pulmonary arterial hypertension (PAH) and PAH associated with connective tissue disease. It has been proposed as a treatment for other forms of pulmonary hypertension but has only been used in the treatment of PVOD in one report as an adjunct to intravenous epoprostenol. We present a patient with biopsy proven PVOD who demonstrated persistent and significant hemodynamic and symptomatic improvement at one year during treatment with sildenafil.

CONCLUSION: Our experience suggests that sildenafil may have unique properties which are useful in the treatment of PVOD. The role of sildenafil in the treatment of PVOD should be evaluated in a clinical trial.

DISCLOSURE: Christopher Barnett, None.

Chest. 2006;130(4_MeetingAbstracts):307S-b-308S. doi:10.1378/chest.130.4_MeetingAbstracts.307S-b
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INTRODUCTION: Primary ciliary dyskinesia (PCD) is characterized by sino-pulmonary disease and caused by abnormal ciliary structure and function. It is autosomal recessive, genetically heterogeneous with a prevalence of 1/12-17,000 (1). We report a case of PCD with a radiographic presentation of anterior mediastinal and left upper quadrant masses.

CASE PRESENTATION: A 41-yr-old gentleman presented with a recent diagnosis of bronchiectasis. He had recurrent pulmonary infections since childhood and recently expectorated 40mls of sputum daily. He had neonatal respiratory compromise (1), recurrent otitis media requiring grommet placements since childhood, and recurrent sinusitis requiring surgery at age 38. He had no children despite unprotected intercourse. He recently underwent a left-sided appendectomy with laparotomy findings of a left sided cecum and absence of colon in the left paracolic gutter. He was in excellent physical shape related to frequent aerobic and weight training exercise.Physical exam revealed a well nourished physically fit 90kg male with bilateral tympanic scarring with persistent grommet in the left ear. His naso-pharynx appeared moist and red. Pulmonary auscultation revealed bibasal crackles without wheeze.Spirometry identified normal FEV1 (102% predicted), FVC (96%) and FEF25-75% (140%) . Sputum culture identified smooth Pseudomonas aeruginosa. Chest x-ray revealed bilateral lower lobe bronchiectasis with a smooth large anterior mediastinal mass (Figure 1 (a)) and displacement of the stomach medially with soft tissue fullness in the left upper quadrant (Figure 1 (s)). Contrast-enhanced CT with high resolution slices confirmed right middle and bilateral lower lobe mild central bronchiectasis. Dilated ascending aorta and bilateral superior vena cavas accounted for the anterior mediastinal mass on chest x-ray. Multiple left upper quadrant splenules accounted for the left upper quadrant mass (Figure 2 (s)). Inferior vena cava interruption with hemiazygous continuation was also identified.The diagnosis of PCD was confirmed by low nasal nitric oxide (48 nl/min, normal= 376+/-124 nl/min (1)) and nasal ciliary biopsy and electron microscopy of ciliary ultrastructure revealing absent outer dynein arms. Genotype DNAI1 mutations was negative (1).

DISCUSSIONS: Although this patient was diagnosed late with PCD, he displayed many classic features including neonatal respiratory compromise, otitis media, sino-pulmonary disease, bibasal bronchiectasis and infertility (1). His preserved pulmonary function was in part related to airway clearance associated with frequent exercise. Estimates suggest approximately 20,000 cases of PCD in the USA, and many are undiagnosed. Situs inversus totalis (SI) occurs in ∼50% of PCD patients (Kartagener's syndrome) which helps with accurate diagnosis (1). There are a few prior reports of PCD with situs ambiguus (heterotaxy), organ laterality defects other than situs inversus totalis. There are a number of subtypes of situs ambiguus including abdominal situs inversus (visceral heterotaxy), isolated dextrocardia and left (polysplenia syndrome) and right (asplenia syndrome) disorders of isomerism sequence.Features of the polysplenia syndrome include multiple splenules, bilateral bilobed lungs, and duplicate superior vena cavas (2). A debilitating association is congenital heart disease (90-100%); however the severity of phenotype varies (2).To determine the incidence of situs ambiguus and associated anatomic anomalies in a population of PCD patients, we investigated 326 well-characterized PCD patients from four international centers and identified an incidence of 6% (unpublished data). 50% of these patients displayed features of the polysplenia syndrome.

CONCLUSION: A case of PCD with features of the polysplenia is presented. Screening for PCD should be considered in patients with anatomical features of heterotaxy with concomitant sinopulmonary disease.

DISCLOSURE: Marcus Kennedy, None.

Chest. 2006;130(4_MeetingAbstracts):308S. doi:10.1378/chest.130.4_MeetingAbstracts.308S-a
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INTRODUCTION: Mounier-Kuhn syndrome is a rare disorder characterized by abnormal dilatation of the trachea and main bronchi, sometimes associated with tracheal diverticulosis, bronchiectasis, and recurrent lower respiratory tract infection.

CASE PRESENTATION: A 62-year-old female with a history of hypertension and type 2 diabetes presents with dyspnea on exertion for two months duration accompanied with a chronic cough productive of yellowish sputum for several months. She received several courses of antibiotics by her primary medical doctor with some improvement of her symptoms.She denied any history of fever, chills, hemoptysis, chest pain, tuberculosis, smoking, allergies, or familiar lung disease. Medications include nifedipine and glipizide. She is a married retired secretary, with two children. She denied alcohol and illicit drugs.Patient looked healthy, well developed, not in acute distress, afebrile, and examination was remarkable only for loud bilateral basilar crackles. Her BMP and CBC were normal. Her chest x-ray on lateral view showed enlarged trachea, and chest CT scan was significant for tracheobronchomegaly (trachea > 3.5 cm, and main bronchi >2cm in diameter), with bronchiectasis. A pulmonary function test revealed a mild obstructive ventilatory defect and decreased DLCO. Patient showed no evidence of secondary tracheobronchomegaly. Her symptoms appear related to bronchiectasis secondary to Mounier-Kuhn syndrome. Patient was placed on antibiotics, and pulmonary rehabilitation recomended.

DISCUSSIONS: The etiology of Mounier-Kuhn syndrome (Described in 1932), is unknown. A congenital defect or atrophy of the elastic and smooth muscle tissue of the trachea and main bronchi have been suggested. The familial form has been described as a possible recessive inherited disorder, and the acquired form as a complication of pulmonary fibrosis in adults or mechanical ventilation in preterm neonates. Secondary tracheobronchomegaly has been described in association with Ehlers-Danlos syndrome, Marfan syndrome, cutis laxa, and light chain deposition disease.Patients may have few or no symptoms. Excessive sputum production with occasional hemoptysis may occur and patients may develop dyspnea with respiratory failure as the lungs become progressively damaged. Chest radiograph may show increased caliber of the airways. Tracheomegaly is characterized by enlarged trachea (>3cm), right main bronchus (>2cm)or left main bronchus (>1.8cm) on chest CT. Tracheal diverticulosis is seen in 1/3 of patients. Asymptomatic patients require no specific therapy. Smoking cessation recommended. The management of symptomatic patients consists of intensive and appropriate antibiotic therapy and postural drainage. Tracheal stenting has been shown to be useful in advanced cases.

CONCLUSION: Mounier-Kunh syndrome should be consider in a patient with recurrent respiratory infections and chronic sputum production. A careful radiographic analysis of the central airways is obligatory.

DISCLOSURE: O. Ozir, None.

Chest. 2006;130(4_MeetingAbstracts):308S. doi:10.1378/chest.130.3.890
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INTRODUCTION: Tracheoesophageal fistula (TE fistula) is a well-known complication of penetrating traumas. TE fistulas also have been described as rare complications of prolonged endotracheal intubations and malignancy. We present a case of TE fistula formation after blunt chest trauma.

CASE PRESENTATION: A 24-year-old restrained passenger sustained blunt thoracic trauma in a high-speed motor vehicle collision. He was initially alert and oriented, but his mental status rapidly declined and he was intubated, uneventfully, at the scene. Physical examination upon arrival to the hospital revealed abrasions and contusions on his anterior chest wall in the distribution of a seat belt. Initial radiographs revealed bilateral pneumothoracies, and sternal, clavicular, and rib fractures. A CT angiogram diagnosed an intimal tear of the aorta at the takeoff of the brachiocephalic artery. Bilateral thoracostomy tubes were placed. The dissection was managed by strict blood pressure control. The patient became febrile on the fourth day of hospitalization. A chest radiograph demonstrated new left lower lobe opacity. Bronchoscopy was done for evaluation of ventilator-associated pneumonia. Thorough airway evaluation at that time showed no airway abnormalities. Broad-spectrum antibiotics were started and his fevers abated. On the eighth day of hospitalization the patient again developed high fevers along with hypotension and hypoxemia. Physical exam showed a distended abdomen and gurgling inspiratory sounds in his chest suspicious for an air leak around the cuff of his endotracheal tube. He also had a new and persistent air leak in his left chest tube. Chest radiography showed the endotracheal tube to be in good position and a left pneumothorax. Gastric contents were suctioned from the patient's endotracheal tube. Emergent bronchoscopy revealed a one-centimeter tear at the posterior wall of the proximal left main bronchus. Chest CT confirmed the TE fistula. The patient underwent emergent thoracotomy and a pleural flap was used to repair the TE fistula. The flap failed seven days later and the patient developed another left pneumothorax. An esophageal stent was placed and the fistula was allowed to heal. The patient stabilized and the remainder of his recovery was uneventful.

DISCUSSIONS: TE fistula is rare following blunt thoracic trauma. In this case, it appeared to be a late complication as signs of a TE fistula did not appear until the eighth day of hospitalization, and the patient underwent bronchoscopy early in his course with no evidence of airway injury at that time.We speculate that the initial trauma caused a partial submucosal tear of the bronchus that was not evident at the time of the first bronchoscopy, and it subsequently eroded through to form the TE fistula. Alternatively, a contained esophageal perforation and localized abscess may have eroded into the posterior wall of the bronchus. The gurgling sound suggestive of an air leak around his endotracheal tube was likely air coming from the bronchus into the esophagus and escaping through the mouth.

CONCLUSION: Although rare, TE fistula can occur following blunt thoracic trauma. Early recognition is important for guiding definitive management. In our case, the initial clue of escaping inspired air was mistakenly attributed to endotracheal tube cuff failure. The correct diagnosis was confirmed after recognition of persistent pleural air leak and aspiration of gastric contents from the airway. Trauma and pulmonary and critical care physicians should be aware of TE fistula as a possible complication of blunt trauma and should be familiar with its presenting features.

DISCLOSURE: Ann Chen, None.

Chest. 2006;130(4_MeetingAbstracts):308S-c-309S. doi:10.1378/chest.130.4_MeetingAbstracts.308S-c
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INTRODUCTION: Lobar torsion after pulmonary resection is a rare complication. The reported incidence is 0.089% among 7887 pulmonary resections (2). Although torsion has been reported previously in the setting of open lobectomy, large series of thoracoscopic lobectomy have not reported it (1).

CASE PRESENTATION: A 60-year-old smoker female sustained a traumatic ankle fracture. An open reduction and internal fixation was planned and as part of her preoperative evaluation, she required a chest radiograph. She was found to have an incidental right upper lobe lung nodule. Further work-up proved the lesion to be a non-small cell lung carcinoma. A positron emission tomogram did not reveal mediastinal or distant metastasis and she underwent an uneventful vats (video-assisted thoracoscopic surgery) right upper lobectomy. On post-operative day 3, the patient had a low-grade fever, tachycardia, and continued to require supplemental oxygen. The chest radiograph and the chest CT scan both showed opacification of the middle lobe. Flexible bronchoscopy demonstrated narrowing of the middle lobe orifice and distortion of the bronchus intermedius. The patient was re-explored thoracoscopically and the diagnosis of right middle lobe torsion was confirmed. The middle lobe appeared hepatized and non-viable. A thoracoscopic middle lobectomy was carried out uneventfully. The patient was discharged on the morning of post-operative day 8.

DISCUSSIONS: Prompt recognition of lobar torsion after pulmonary resection is a diagnostic challenge. The Hallmark of making the diagnosis is a chest radiograph showing opacification of the involved lobe without apparent volume loss. CT scan and flexible bronchoscopy are the diagnostic tests of choice. Although torsion has been reported previously in the setting of open lobectomy, large series of thoracoscopic lobectomy have not reported it. The literature on post-lobectomy lobar torsion was reviewed, and to our knowledge, this case represents the first report of a minimally invasive management of lobar torsion following pulmonary resection.

CONCLUSION: Lobar torsion complicating pulmonary resection is a surgical emergency. Prompt diagnosis and re-exploration of the chest are essential componenets of successful management. Although an open approach remains the gold standard, a minimally invasive technique is a feasible alternative.

DISCLOSURE: Jon Wee, None.

Chest. 2006;130(4_MeetingAbstracts):309S. doi:10.1378/chest.130.4_MeetingAbstracts.309S-a
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INTRODUCTION: Kayexalate is a powdered suspension of sodium polystyrene sulphonate frequently used for the management of hyperkalemia. The aspiration of this material with associated pneumonitis or bronchitis as been reported in the pediatric and adult population.We report a case where kayexalate particles with giant cell reaction were found at postmortem examination in the lung tissue.

CASE PRESENTATION: A 58-year-old man with history of hypertension, diabetes, Parkinson's dementia, gastro esophageal reflux (GERD) and chronic respiratory failure on mechanical ventilator was admitted for respiratory distress. One year prior to the current admission he developed Klebsiella pneumonia with respiratory failure,subsequently he had three admissions for recurrent pulmonary infiltrates that were treated as nosocomial pneumonias.At this time, the patient was in ARDS.The examination revealed a comatose cachectic male, with normal vital signs and extensive bilateral rhonchi and crackles on lung examination. The CXR and CT scan showed extensive bilateral alveolar infiltrates with air bronchograms and no evidence of pulmonary emboli. Echocardiogram was normal. Laboratory revealed potassium of 5.8 mEq/L and normal WBC count and renal function. Broad spectrum antibiotics and kayexalate (sodium polystyrene sulphonate) were started through gastrostomy. The patient's clinical condition continued to deteriorate and he expired 72 hours after admission. As per review of all prior medical records, this was the first time the patient received kayexalate. Postmortem examination of the lungs revealed acute bronchopneumonia with foci of organizing pneumonia. In addition all lobes showed polygonal plate-like foreign particles.

DISCUSSIONS: Sodium and calcium polystyrene sulphonate are cation exchange resins given orally or by retention enema for the treatment of hyperkalemia. Sodium polystyrene sulphonate (Kayexalate) uniquely stain strongly by a direct Schiff's reagent procedure without any preoxidation and by the Ziehl-Neelsen method.They have the virtually pathognomonic feature of direct Schiff positivity with a characteristic basophilic, amorphous foreign material on histologic sections(1).The identity of this material can be confirmed by Fourier transform infrared microspectrophotometry(2) The possible aspiration of different organic materials might sometime skew the histology.When present,vegetable particles, cotton fibers (such as contaminants from surgical dressings), and amyloid all stain strongly, but they do not have the parallel laminations of kayexalate. The association between the use of this resins and lung disease trace back to 1975 with a case report of aspiration pneumonia due to the administration of calcium resonium in an elderly debilitated patient(1).Pulmonary complications reported with kayexalate aspiration are bronchitis, bronchopneumonia and pneumonitis.Our patient had findings consistent with acute bronchopneumonia with foci of organization and kayexalate particles. Probably his underlying pulmonary condition was bronchiolitis obliterans with organizing pneumonia either primary or infectious related and the kayexalate particles were an incidental finding. The medical literature has suggested that kayexalate is an inert particle but in our case there was evidence of giant cell reaction around the kayexalate particle.

CONCLUSION: Sodium polystyrene sulphonate is commonly given orally for the treatment of hyperkalemia. Pulmonary complications reported with kayexalate aspiration are bronchitis, bronchopneumonia and pneumonitis. We report the incidental postmortem finding of aspirated kayexalate with evidence of giant cell reaction around the kayexalate particle which is unique in its presentation.It is unclear if the aspiration precipitated his demise. This case together with other cases reporting pulmonary complications illustrates the potential complication of oral administration of kayexalate in patients with high risk for aspiration.We suggest that care should be taken to avoid aspiration and a high level of suspicion to monitor for pulmonary complications.

DISCLOSURE: Anant Dalvi, None.

Chest. 2006;130(4_MeetingAbstracts):309S-b-310S. doi:10.1378/chest.130.4_MeetingAbstracts.309S-b
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INTRODUCTION: Primary tracheal adenoid cystic carcinoma (ACC) is a relatively rare salivary gland-type neoplasm originating in the bronchial gland. The tumor can go undiagnosed for months to years because of its nonspecific presenting symptoms, slow-growth, and silent nature. We describe a case of respiratory failure secondary to ACC presenting with near complete obstruction of bilateral main stem bronchi misdiagnosed as severe asthma exacerbation.

CASE PRESENTATION: 51-year-old Korean female was admitted for severe asthma exacerbation. She was diagnosed with asthma 2 years prior. No prior history of tobacco use. Upon admission, her temperature was 100.4 F, blood pressure was 135/60 mmHg, heart rate was 120 beats/min, respirations were 32 breaths/min, and pulse oximetry was 94% on room air. She was moderately uncomfortable using accessory muscles for breathing. Lung auscultation revealed diffuse wheezes. Laboratory examination including complete blood count and comprehensive panel were unremarkable. Arterial blood gas revealed pH 7.49, PCO2 34, PO2 157, HCO3 26 on 2 liters by nasal canula. A chest X-ray showed hyper-expansion of the lungs. Spirometry showed a severe obstructive ventilatory defect. The patient became increasingly tachypneic, short of breath, and developed respiratory failure requiring endotracheal intubation and mechanical ventilation within 24 hours of admission. Despite maximal medical treatment for asthma and appropriate ventilator management, her condition continued to decline. She developed worsening hypercarbia, elevated peak pressures of 80 cm H2O, and bilateral pneumothoraces. Bilateral chest tubes were placed. A CT scan reviewed in addition to pneumothoraces, small polypoid intraluminal mass with narrowing at the level of the carina and bilateral main stem bronchi. Bronchoscopy reviewed white-yellow lobulated intraluminal masses obstructing the tracheal lumen at the level of the carina, extending into the left and right main stem bronchi with resultant marked narrowing of both bronchi. Biopsies of the mass disclosed adenoid cystic carcinoma. The patient was transferred to another facility and underwent laser resection, stent placement of the trachea, bilateral main stem bronchi, and successful extubation. She subsequently received radiation therapy.

DISCUSSIONS: Primary adenoid cystic carcinoma of the lung accounts for about 0.2% of primary lung cancers. It is considered a low-grade malignancy due to its slow growth and relatively long clinical course, with a tendency for local recurrence and late metastasis. Symptoms are nonspecific and stem from chronic bronchial obstruction or irritation, averaging 2 years in duration prior to diagnosis. Patients with ACC of the trachea are significantly younger at presentation compared to those with squamous cell carcinoma (SCC) with an average age of 44 years. Unlike SCC, adenoid cystic carcinoma is more common in non-smokers. There seems to be a predilection for women. Presenting symptoms include dyspnea, chronic cough, hoarseness, stridor/wheezing, hemoptysis, and difficulty clearing secretions. Symptoms are often misdiagnosed as asthma, or in smokers, as chronic obstructive pulmonary disease (COPD). Even with detailed history and physical exam, visualization of the airways is essential in diagnosis. CT scan and/or bronchoscopy should be considered in those who fail optimal presumed obstructive airway disease treatment. Surgical resection is the preferred and first-line treatment, followed by postoperative radiotherapy. Complete tumor resection is essential since recurrence is high when there is residual tumor. In one study, the 5-year survival rate is 91% in the resected group and 40% in the non-resected group. ACC is not responsive to chemotherapy.

CONCLUSION: Tracheal adenoid cystic carcinoma can easily be misdiagnosed due to its nonspecific presentation and slow clinical course. When a patient presents with nonspecific respiratory symptoms including dyspnea, cough, wheezing, a tracheal primary tumor should be in the differential diagnosis, particularly in whom optimal treatment for presumed asthma or COPD fails.

DISCLOSURE: Michelle Cao, None.

Chest. 2006;130(4_MeetingAbstracts):310S. doi:10.1378/chest.130.4_MeetingAbstracts.310S-a
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INTRODUCTION: Unusual locations of glomus tumors, including the mediastinum and trachea, have been reported and can be mistaken with carcinoid tumors.

CASE PRESENTATION: A 48-year-old white male, nonsmoker, presented with a three month history of mild hemoptysis, cough, and a left forearm subcutaneous mass. Past medical history was remarkable for a tracheal carcinoid tumor which was resected 17 years ago. Physical examination revealed a left forearm soft tissue mass of about 2 x 2 cm which was nontender and without overlying skin changes. The remainder of the exam was normal. Serum chemistries and CBC were within normal limits. Chest CT demonstrated a polypoid lesion in the distal trachea and precarinal region. Bronchoscopy showed a polypoid mass extending from the anterior tracheal wall just above the main carina resulting in 80% occlusion of the right mainstem bronchus take-off and 70% occlusion of the left mainstem bronchus take-off (figure 1). The tracheal mass was resected with electrocautery using a snare and blunt probe achieving 100% patency. Pathology was initially read as showing a carcinoid tumor. Biopsy of the forearm mass revealed a tumor composed of nests of large, eosinophilic cells deposited in a collagenous and myxoid matrix with immunohistochemistry positive for muscle specific actin and smooth muscle actin and negative for cytokeratin AE1/3, chromogranin and synaptophysin. This was consistent with a glomus tumor. Subsequent mmunohistochemical staining of the tracheal tumor demonstrated similar findings and was felt to also be a glomus tumor. At bronchoscopy three months later there was abnormal mucosa that was treated with electrocautery followed by radiotherapy. Six months later there was no evidence of endotracheal recurrence (figure 2).

DISCUSSIONS: Glomus tumors are benign neoplasms derived of glomus cells that resemble the modified smooth muscle cells of the normal glomus body. Glomus tumors typically involve the skin of the extremities, with subungual region of the finger being the most common site. This tumor is extremely rare in the mediastinum and lung. Within the respiratory tract, the trachea is the most frequent site of involvement, with only 16 cases reported in the literature. All patients had tumor arising from the posterior membranous wall of the trachea, except in our case where the tumor was arising from the anterior wall. None of the tumors were metastatic although our patient had a concomitant glomus tumor in his left forearm. Histologically, glomus tumors consist of medium sized cells with round, regular nuclei and eosinophilic cytoplasm with nested pattern that surround vascular channels. Glomus tumors are uniformly positive for vimentin, smooth muscle actin and variable for desmin. They are negative for cytokeratin, chromogranin and synaptophysin. One major differential diagnosis of a glomus tumor is a carcinoid tumor. Carcinoid tumors have a coarsely granular or salt and pepper chromatin, do not have a prominent vascular channels, and are positive for cytokeratin and neuroendocrine markers such as chromogranin and synaptophysin. Most peripheral and visceral glomus tumors are histologically and clinically benign, however atypical types do exist and fatal cases have been reported. The recommended treatment for glomus tumor of the trachea is sleeve resection with primary reconstruction which is curative and requires no adjuvant treatment. Bronchoscopic laser resection with adjuvant radiotherapy has been used with good results. In our patient, we utilized electrocautery and radiotherapy with no evidence of recurrence so far.

CONCLUSION: Glomus tumors should be included in the differential diagnosis of tracheobronchial lesions and are often mistaken with carcinoid tumors. Bronchoscopic resection and adjuvant radiotherapy is a valid treatment option. To the best of our knowledge, this is the first patient reported with concomitant tracheal and subcutaneous glomus tumors.

DISCLOSURE: Sebastian Fernandez-Bussy, None.

Chest. 2006;130(4_MeetingAbstracts):310S-b-311S. doi:10.1378/chest.130.4_MeetingAbstracts.310S-b
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INTRODUCTION: Tracheal hamartomas are very rare. The diagnosis requires a high index of suspicion.

CASE PRESENTATION: A 40-year-old woman without significant past medical history presented to pulmonary clinic with decreased exercise tolerance, progressively worsening shortness of breath and wheezing for two years. She had a persistant cough productive of white phlegm and “chunky white flecks”. The wheezing had no diurnal variation and had responded to steroids but recurred. Symptoms resulted in multiple visits to emergency rooms and treatments for asthma exacerbations. Two years prior to presentation, pulmonary function tests (PFTs) and laryngoscopic exams were normal. Oxygenation was normal by pulse oximetry. Lung exam revealed normal breath sounds but her forced inspiratory and expiratory effort resulted in intractable coughing. Repeat PFT's now revealed severe obstruction. The flow/volume loop suggested a fixed intrathoracic airway obstruction. Bronchoscopy revealed a large mid-tracheal polypoid mass with a stalk obstructing appoximately 95% of the airway. Computed tomography (CT) confirmed near complete occulusion of the trachea with no other abnormalities. The mass was removed via rigid bronchoscopy. The patient reported complete resolution of symptoms and was discharged home. Histological examination revealed a lesion lined by unremarkable respiratory epithelium. The sub-epithelial region had myxoid stroma with mature adipose tissue and benign cartilaginous tissue. A diagnosis of benign polypoid hamartoma was made.

DISCUSSIONS: Hamartomas are the most common benign tumors of the lung. However, tracheal hamartomas are very rare. Their presence in the trachea can result in severe airway obstruction, often leading to prolonged misdiagnosis of asthma. They are typically composed of cartilage, fat, connective tissue, muscle and incorporated respiratory epithelium. The incidence of pulmonary hamartomas is 0.25% with only 10% of them occurring endobronchially. A review of the literature yields only a few reports of tracheal hamartomas. A 1998 review documented seven cases with tracheal hamartomas, the majority found in men. One series reviewed 27 tracheobronchial tumors over an 18 year period and found only one tracheal hamartoma. A study on argon plasma coagulation for tracheobronchial tumors found 11 patients over a 3 year period; however only one patient with a hamartoma. Little imagery is available to illustrate their obstructive nature. To our knowledge, only one case report shows a bronchofiberscopic image of a tracheal hamartoma. This case is unique in that it yields fiberoptic images of near complete occlusion of the trachea by a hamartoma which are complimented by CT images in a woman.

CONCLUSION: Tracheal hamartomas are benign tumors with potentially severe morbidity. In patients in whom wheezing or asthma does not respond to conventional therapy, one should entertain the diagnosis of tracheal lesions including hamartomas. The treatment for this mimicker of asthma is surgical and reminds us that “not all that wheezes is asthma”.

DISCLOSURE: Adan Mora, Jr., None.

Chest. 2006;130(4_MeetingAbstracts):311S. doi:10.1378/chest.130.4_MeetingAbstracts.311S-a
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INTRODUCTION: Endobronchial maligancy causing symptomatic bronchial obstruction is a common problem facing many pulmonary physicians. Most of the therapies offered patients involves palliation of symptoms. We present an unusal case of metastatic endobronchial melanoma causing symptomatic left main stem bronchus obstruction treated with photodynamic therapy (PDT).

CASE PRESENTATION: An 80-year-old male complained of dyspnea and dry cough associated with fatigue for one month. He had history of prostate cancer treated with radiation in 2003, and melanoma of his left ear excised in 2004. Two courses of antibiotics failed to relieve symptoms. On examination there was diminished breath sounds on the left side. A chest radiograph revealed a hilar infiltrate. Computed tommography (CT) scanning demonstrated a large spiculated hilar mass compressing the left upper lobe bronchus with loss of lung volume. Bronchoscopy revealed an obstructing lesion in the left mainstem bronchus (graphic 1). Pathology confirmed the presence of malignant melanoma. Prior to receiving chemotherapy he presented with productive cough and increasing dyspnea. Examination revealed markedly diminished breath sounds on the left side. Chest radiograph demonstrated a completely atelactatic left lung. PDT with argon laser to relieve endobronchial obstruction was initiated. The patient had a total of three PDT treatments and four salvage bronchoscopies to remove fragments of the endobronchial lesion. He had relief of his dyspnea following PDT. Repeat radiograph showed increased aeration in the left lung and bronchoscopy revealed a patent left mainstem bronchus (graphic 2). His symptoms improved and he was discharged home.

DISCUSSIONS: Endobronchial metastasis from nonbronchogenic primary solid tumors are a rare cause of bronchial obstruction. The more common nonbronchogenic tumors involved in endobronchial metastasis include breast, colorectal and renal cell carcinoma. To our knowledge there are only two cases of metastatic endobronchial melanoma treated with PDT reported in the literature.

CONCLUSION: Metastatic endobronchial melanoma is a rare cause of endobronchial obstruction. This case demonstrates that treatment with PDT is potentially safe and effective in alleviating symptoms associated with endobronchial obstruction due to metastatic melanoma.

DISCLOSURE: Ismael Martin, None.

Chest. 2006;130(4_MeetingAbstracts):311S. doi:10.1378/chest.130.4_MeetingAbstracts.311S-b
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INTRODUCTION: Endobronchial ultrasound allows visualization of smaller structures for needle aspiration biopsy. Moderate sedation is required and airway patency may be compromised. Patients with obstructive sleep apnea (OSA) experience airway obstruction during these procedures. In this case report, a laryngeal mask airway (LMA) was used to maintain airway patency during endobronchial ultrasound in a patient with morbid obesity and OSA.

CASE PRESENTATION: The patient is a 60-year-old male whose complaints of hematuria led to a right nephrectomy for renal cell carcinoma. Initial chest imaging demonstrated mediastinal adenopathy. Several months later, a repeat scan documented an increase in the right paratracheal nodes to greater than 2 centimeters. Positron emission tomography failed to show metabolic activity of the enlarged mediastinal nodes or metastatic lesions. He denied hemoptysis. Any prior surgical procedures were not complicated by airway problems. He is 68 inches tall and weighs 138.3 kilograms, with a body mass index of 46 kilogram per meter squared. Endobronchial ultrasound guided fine needle biopsy was planned. Because of his large tongue and concerns over the potential for airway compromise during sedation and instrumentation, an LMA was chosen to maintain a patent airway. The larger diameter ultrasound bronchoscope does not pass easily through an endotracheal tube, and patient breathing becomes labored. After aperture bar removal, this bronchoscope passes easily through the size 5 and 6 LMA. Routine monitors were applied. The airway was anesthetized with 120 mg nebulized lidocaine 4%, followed by 80 mg delivered by atomizer to the base of the tongue and posterior oropharynx to abolish any gag reflex. While awake, a size 5 LMA Unique™ was inserted into the oropharynx. During insertion, a finger directed the cuff tip caudad into the hypopharynx. Cuff inflation required 25 milliliters of air. Air exchange was unimpeded and phonation was possible. A bite block was placed over the airway tube and between the teeth. When supine, lidocaine was instilled into the LMA without a cough or gag response. Supplemental oxygen was provided. Conscious sedation by an experienced endoscopy nurse was initiated. A diagnostic bronchoscope was inserted easily to visualize the LMA in good position over the glottis. The glottic structures and lung exam were normal. The larger ultrasound bronchoscope passed easily through the size 5 LMA. Ultrasound of the distal trachea localized the right paratracheal lymph node for six ultrasound guided fine needle aspiration biopsies. The procedure concluded after 70 minutes. Drug totals were midazolam 5 milligrams, propofol 1,120 milligrams, and fentanyl 200 micrograms.The patient maintained spontaneous respirations and a patent airway throughout the procedure. He awakened rapidly and smoothly. Upon command, he opened his mouth to allow removal of the LMA.

DISCUSSIONS: In the past fifteen years, the LMA has gained popularity as an effective airway device for routine surgical procedures and management of difficult airways. Outside of the operating room, few clinicians have opportunities to experience the efficacy of an LMA. But, pulmonary and critical care physicians need to become familiar with the device and its applications to airway management for procedures and resuscitation. With time, the LMA may play a greater role in endoscopy, whether the airway evaluation portends a greater potential for airway compromise, or an unexpected response to sedatives renders a patient apneic.

CONCLUSION: The LMA is effective for airway management in many clinical scenarios. For endobronchial ultrasound, the larger diameter bronchoscope works best when placed through the mouth. In selected patients at risk for airway compromise, the LMA should be considered as an airway alternative for ease of use, bronchoscope maneuverability, patient comfort and safety.

DISCLOSURE: Perry Nystrom, None.

Chest. 2006;130(4_MeetingAbstracts):311S-c-312S. doi:10.1378/chest.130.4_MeetingAbstracts.311S-c
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INTRODUCTION: Endobronchial foreign bodies usually present with cough and complications of obstruction such as atelectasis or pneumonia. We present a case of hemoptysis for two years caused by a foreign body.

CASE PRESENTATION: A 66-year-old white male presented to his primary care physician with a chief complaint of hemoptysis. Over a two-year period, the hemoptysis was scant and intermittent. He was a non-smoker with a history of only hypertension treated with amlodipine 5 mg daily and gastroesophageal reflux treated with lansoprazole 30 mg daily. CT scans of the chest and paranasal sinuses were normal, as were spirometry, lung volumes and carbon monoxide diffusing capacity. Laboratory studies showed no abnormalities. The patient was treated with several courses of antibiotics with no improvement, and a laryngologic examination was negative. A flexible bronchoscopy revealed an obstruction that occluded about 50% of the lumen of the bronchus intermedius. There was mild edema of the area, and the proximal portion of the occlusion showed polypoid granulation tissue emanating from the medial aspect of the bronchus with a yellowish mucous-like concretion. Distal to it was a lobulated mass protruding from the lateral aspect of the airway. A biopsy of the abnormality revealed inflammation, granulation tissue and plant matter with bacteria. Bronchoalveolar lavage was negative. A subsequent rigid bronchoscopy with laser exfulguration was performed, and a protruding foreign body was revealed which was extracted with difficulty. Pathology identified it as a pistachio nut shell. The distal bronchus was patent with mild edema of the distal segments. Patient tolerated the procedure well, and had no more hemoptysis. He had no recollection of aspiration events at any time.

DISCUSSIONS: Most endobronchial foreign bodies occur in children, and present with cough and obstructive sequelae such as atelectasis or pneumonia. Hemoptysis is uncommon, and distinctly rare without these sequelae. In this case, the patient had only hemoptysis for two years, and repeat CT scans of the chest did not reveal the foreign body. Also, it illustrates that patients with this disorder need not present with the classic risk factors for aspiration, nor recall that the event happened.

CONCLUSION: Patients with unexplained hemoptysis should be evaluated with bronchoscopy.

DISCLOSURE: Kelvin Shiu, None.

Chest. 2006;130(4_MeetingAbstracts):312S. doi:10.1378/chest.130.4_MeetingAbstracts.312S-a
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INTRODUCTION: Foreign body aspiration is more common in children than adults.[1] In children, clinical symptoms and radiological findings have low diagnostic value.[2] If not diagnosed or treated in a timely manner, delayed diagnosis (>4 weeks) increases the incidence of complications, one of which is bronchiectasis.

CASE PRESENTATION: A 27-year-old man was seen in clinic to evaluate a persistent cough that started four years prior, after he was diagnosed with pneumonia. He noted episodes of productive sputum, hemoptysis and shortness of breath several times per month. He denied fever, chills, or generalized malaise. On extensive questioning, he remembered inhaling or swallowing a fake tooth that was part of his vampire costume 8 years ago. He was evaluated in the ER at that time with no diagnostic testing or follow-up. Past medical history was unremarkable. Patient did not take any medications. He had no history of tobacco, alcohol, or illicit drug use. He denied occupational, chemical, dust, asbestos, or exotic pet exposure. He had no significant travel history. In clinic, the patient was in no respiratory distress, afebrile with normal vital signs. His SpO2 was 97% on room air. His chest exam and spirometry were normal. PA and lateral chest x-ray showed consolidation in the posterior segment of the right lower lobe. Chest CT showed right lower lobe volume loss with variable bronchiectasis and hyperlucent material in a basilar segment. Flexible bronchoscopy revealed granulation tissue occluding the right lower lobe bronchus. No foreign body was visualized. Rigid bronchoscopy, performed that day, revealed the edge of a foreign body that was removed with a forceps. The foreign body was a “V-shaped” plastic tooth approximately 1.5 cm at its base. Following extraction of the foreign body, a moderate amount of non-obstructing granulation tissue was present in the lower lobe bronchus. The basilar segmental airway appeared relatively normal. Patient will be seen at one month for repeat bronchoscopy to assess healing of the airway. Further studies, eg repeat chest CT for evaluation of bronchiectasis, may be indicated pending his clinical course.

DISCUSSIONS: Foreign body aspiration is more common in children than adults. Delayed diagnosis (>4 weeks) significantly increases the incidence of complications, mainly pneumonia, broncho-esophageal fistula, and bronchiectasis[3,4,5,6] and is attributed to misdiagnosis or negligence.[3] In particular, bronchiectasis can develop 14 to 58 days after FB aspiration in animals.[7] Even though bronchiectasis in general is considered permanent and irreversible, cylindrical bronchiectasis can be considered reversible.[8] Though considered permanent and irreversible, case reports have showed that when the FB is removed, objective signs of bronchiectasis resolve.[5,9] More specifically, inorganic FB aspiration, once object is removed has shown no long-term affects.[6].

CONCLUSION: The immediate diagnosis of foreign body aspiration is important to decrease long-term complications.

DISCLOSURE: Antonio Salud II, None.

Chest. 2006;130(4_MeetingAbstracts):312S-b-313S. doi:10.1378/chest.130.4_MeetingAbstracts.312S-b
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INTRODUCTION: Vascular invasion of tumor is well described in Lung cancer. We wish to report a case of lung cancer which was extending intravascularly into the pulmonary artery masquerading as a pulmonary thrombus. Pulmonary endoarterial biopsy was obtained via a percutaneous transvenous catheter which confirmed the diagnosis of recurrent lung adenocarcinoma. To the best of our knowledge, this is first reported case of lung cancer diagnosed with this novel approach.

CASE PRESENTATION: A 76-year-old male with prior history of Non-small cell lung cancer (NSCLC) status post right middle and lower lobectomy and partial right upper lobe wedge resection presented with gradually progressive dyspnea and cough. Initial CT chest failed to reveal any new suspicious pulmonary or mediastinal lesions. Progressive symptoms warranted close follow up. A repeat CT chest 6 months later revealed mild fullness in the right hilar area, but a subsequent Positron Emission Tomography scan failed to reveal any abnormalities. After a period of another six months, CT chest revealed a decrease in caliber of right upper lobe and right main pulmonary arteries. MRI of the chest showed an intravascular mass suggestive of a thrombus. Follow up CT chest with contrast confirmed proximal extension of this lesion into the main Pulmonary artery completely occluding the right pulmonary artery raising the question of thrombus versus tumor (Figure 1). The lesion was not easily amenable to a surgical approach. Therefore, a pulmonary angiogram was performed and biopsy specimens were taken with the use of an endovascular biopsy catheter which confirmed the diagnosis of recurrent NSCLC (Figure 2).

DISCUSSIONS: Direct intravascular extension of bronchogenic carcinoma is not uncommon. Lung cancers unlike other visceral cancers are more prone to vascular invasion and it is thought to be related to the relatively low pulmonary arterial pressures which may render itself more susceptible to neoplastic penetration. Macroscopic vascular extension is relatively rare and has been reported to portend a poor outcome. In this case, the tumor recurred inferior to Right upper lobe Pulmonary artery and invaded the right pulmonary artery resulting in complete occlusion of the lumen mimicking a pulmonary thrombus. Yamaguchi et al, reported a case series of Lung carcinoma with polypoid intravascular invasion of main pulmonary artery, but the diagnosis could not be made pre-operatively 1. The location of tumor and patient's limited pulmonary reserve made surgical intervention a high-risk procedure. Therefore, a novel endovascular approach was adopted. Very few cases of this diagnostic approach have been reported in literature. Endovascular approaches have been used to diagnose lung cancer with superior vena caval invasion, right and left atrial tumours and pulmonary artery leiomyosarcoma. To the best of our knowledge, this is the first reported case of NSCLC with intravascular extension in to the pulmonary artery that was diagnosed with a pulmonary endoarterial biopsy. A high-risk surgical intervention was obviated by this procedure.

CONCLUSION: When neoplastic vascular invasion of the pulmonary artery or great veins is suspected, trans-vascular approach to biopsy should be considered. It appears to be safe and can provide diagnostic tissue while also assisting with staging of the patient.

DISCLOSURE: Todd Gienapp, None.

Chest. 2006;130(4_MeetingAbstracts):313S. doi:10.1378/chest.130.4_MeetingAbstracts.313S-a
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INTRODUCTION: Although various malignancies have been reported in Paraneoplastic encephalomyelitis, 80% of the cases are associated with bronchial cancer, typically small cell lung cancer.

CASE PRESENTATION: We present a 74-year-old lady admitted with complaints of shaking of the whole body, tremors and rolling of the eyes. She had a history of hypertension and chronic bronchitis. She was a chronic smoker for 40 years and had recently been tapering the cigarettes. . Her psychiatric medications were discontinued in the nursing home 9 days prior to this admission and she was started on Ativan. The clinical impression was withdrawal symptoms versus psychosis. In the hospital she was lethargic and agitated, very anxious and afebrile. The patient continued to have tremulous/dyskinetic movements of the jaw, eyes and upper extremities more than the lower extremities. She responded to verbal commands and responded to her name. A CT scan without contrast of brain showed changes related to atrophy and a chest x-ray was negative. She was also started on Ativan for dystonia and anxiety. A clinical suspicion of meningitis vs encephalitis was entertained and a lumbar puncture was done which showed a normal glucose, normal protein, a negative gram stain and mild lymphocytic pleocytosis. Fungal, TB and viral cultures were all negative. VDRL, Cryptococcal antigen and Lime titers were also negative. She was started on vancomycin, Ciprofloxacin and Acyclovir. A clinical impression was that of a partially treated meningitis or aseptic meningitis or encephalitis. A CT scan of chest with IV contrast showed bilateral emphysema and a 2 x 2 cm low attenuation lesion likely representing adenopathy vs prominent pulmonary artery shadow. Her condition kept on deteriorating and was only responsive to verbal commands and her name. Her prognosis was guarded and she died on Jan. 8, 2006. Autopsy showed small (1 cm) nodule of oat cell (small cell) carcinoma in the right upper lobe of the lung with metastasis to 3 hilar and tracheal lymph nodes. The primary site was smaller than the lymph node metastasis. Her lungs also showed extensive emphysema of both upper lobes of lungs. Her brain microscopically showed encephalomyelitis involving bilateral amygdala, basal ganglia and spinal cord compatible with Paraneoplastic encephalomyelitis syndrome.

DISCUSSIONS: Paraneoplastic Encephalomyelitis (PEM) describes patients with cancer who develop multifocal neurologic deficits and signs of inflammation involving multiple areas of the nervous system, resulting in a mixture of symptoms derived from limbic encephalitis, cerebellar degeneration, brainstem encephalitis, myelitis and autonomic dysfunction. The antineuronal antibodies more frequently encountered in PEM are anti-Hu, anti-CRMP5, anti-Zic, and less frequently anti-amphiphysin. All of these antibodies associate with SCLC and may co-occur in the same patient.75% of patients have MRI abnormalities and PEM rarely improves with treatment. Prompt treatment of the tumor might stabilize the disorderSymptoms can precede the diagnosis of cancer in some cases. Symptoms usually progress over the course of weeks to months causing severe neurologic disability as in this case. In fact neurologic impairment may be more debilitating than the associated cancer as in this case. No effective therapeutic approaches have been established. In this case chest x-rays did not reveal the lung tumor, hence the diagnosis of lung tumor with paraneoplastic syndrome was not made. Retrospectively, all the patient's symptoms can now be explained by this paraneoplastic encephalomyelitis syndrome.

CONCLUSION: PEM often heralds small cell carcinoma of lung. However, to make a definitive diagnosis of PEM, demonstration of presence of antineuronal antibodies, as well as absence of a viral infection is crucial.

DISCLOSURE: Muhammad Rehman, None.

Chest. 2006;130(4_MeetingAbstracts):313S. doi:10.1378/chest.130.4_MeetingAbstracts.313S-b
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INTRODUCTION: Non Tuberculous Mycobacteria (NTM) is ubiquitous to the environment, and its pulmonary manifestations can vary from colonization to hypersensitivity pneumonitis to advanced fibrocavitary disease. Many patients with NTM are immunosuppressed or have an underlying lung disease. The most common associated conditions include COPD, old TB/fungal disease, bronchiectasis, silicosis, cystic fibrosis, and AIDS.

CASE PRESENTATION: A 32 year-old man with no significant past medical history presented with 2 weeks of productive cough and right sided chest pain. He denied hemoptysis, fever, weight loss, night sweats, or shortness of breath. The patient worked as a machine operator. He denied recent travel, TB exposure, or having exotic pets at home. He smoked 1 1/2 packs of cigarettes per day for the past 16 years, and he rarely drank alcohol. Chest exam was normal and no other significant abnormalities were detected on physical exam. Chest X ray and CT scan revealed a 5 cm x 5 cm thick-walled cavity in the superior segment of the left lower lobe. The wall measured approximately 20 mm. Diffuse “tree-in-bud” appearance was present in the right middle lobe and the lingula. PPD and HIV test were negative. Sputum acid fast bacillus (AFB) smear was 3+ positive in 3 consecutive daily samples. AFB culture revealed Mycobacterium Avium Complex. Treatment with Ethambutol, Rifampin, and Azithromycin was initiated. Four weeks later, he complained of left sided headache. He had night sweats but was afebrile. He also complained of right sided flank pain without radiation or hematuria. Physical exam was otherwise negative. CT and MRI of the brain showed multiple lesions in the frontal and occipital lobe. Lumbar puncture revealed malignant cells. Bronchoscopy with transbronchial biopsies of the left lower lobe revealed adenocarcinoma.

DISCUSSIONS: Lung cancer by itself in a 32-year-old man is unusual, but this patient was diagnosed during treatment for cavitary NTM. There were many diagnostic pitfalls and management challenges that characterized this case. First, this patient had no apparent risk factors for cavitary MAC, but he did have a significant risk factor for lung cancer (16 pack year smoking). Second, the thickness of the cavitary wall was a sign that this may be more than simply mycobacterial infection. In a study of 65 solitary lung cavities, when the wall was over 15 mm in thickness, 92% of the lesions were malignant. (1).

CONCLUSION: This case illustrates that in a smoker with thick-walled cavity, cancer may coexist with active infection, and tissue biopsy may be required. Our case also emphasizes the importance of considering lung cancer in younger patients who smoke. The proportion of bronchogenic adenocarcinoma is higher in the young compared with all patients with lung cancer, and younger patients more frequently present with advanced disease at diagnosis, resulting in an extremely poor survival. (2).

DISCLOSURE: Razaq Badamosi, None.

Chest. 2006;130(4_MeetingAbstracts):313S-c-314S. doi:10.1378/chest.130.4_MeetingAbstracts.313S-c
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INTRODUCTION: Severe lobar venoarterial shunting in bronchioloalveolar cell carcinoma (BAC) has been infrequently reported [1,2]. A patient with BAC is presented with a severe right lower lobe venoarterial shunt corrected by pulmonary artery occlusion and effectively palliated with a right lower lobectomy.

CASE PRESENTATION: A 69-year-old Caucasian female with a four year history of advanced BAC was referred for severe refractory hypoxemia. She presented to the hospital with a resting oxygen saturation (SaO2) between 60 to 80%, despite high flow oxygen supplementation. She reported severe dyspnea, malaise, productive cough and an inability to ambulate 20 feet. Examination revealed a thin, alert, cyanotic and dyspneic woman. Vital signs were heart rate 102/min, blood pressure 97/62 mmHg, temperature 37 °C and respiratory rate 24/min. Lung examination revealed inspiratory crackles, egophony and dullness to percussion confined to the right middle and lower lobes. Cardiac examination revealed tachycardia with normal heart sounds and no murmurs. Peripheral pulses were normal. Acrocyanosis was present. The remainder of the physical examination was noncontributory. Complete blood count revealed a hemoglobin of 17.6 g/dL and hematocrit of 50%. Complete metabolic panel was normal. Resting arterial blood gas on 8 Lpm oxygen demonstrated a pH of 7.47, PaCO2 of 29 torr, PaO2 of 38 torr and SaO2 of 76%. Chest roentgenography and computed tomography demonstrated consolidation and air bronchograms within the right middle and lower lobes [Figure 1]. Ventilation-perfusion scan demonstrated low probability of pulmonary embolus and 49% differential perfusion to the right middle and lower lung zones [Figure 2]. Transthoracic echocardiography demonstrated normal left ventricular ejection fraction, a mildly dilated aortic root and no intracardiac shunt. Right heart catheterization and pulmonary angiography were performed. Mean pulmonary arterial and capillary occlusion pressures were within normal limits. Fick cardiac index was 4.1 L/min/m2, systemic vascular resistance was 1042 dyne/sec/cm3 and pulmonary vascular resistance was 78 dyne/sec/cm3. Mixed venous arterial saturation was 70%. SaO2 on supplemental oxygen was 77% but improved to 93% following occlusion of the right lower pulmonary artery. Pulmonary angiography demonstrated normal pulmonary arterial vasculature. Following a thorough preoperative evaluation and consent process, a right thoracotomy with resection of the right lower lobe was performed. Division of the right lower lobe pulmonary artery resulted in improvement in SaO2 from 78% to 92% on single lung ventilation. Following an unremarkable postoperative course, the patient was discharged to home requiring no supplemental oxygen. Four months following surgery, she remains off of oxygen and no longer describes activity-limiting dyspnea. Room air SaO2 with ambulation remains 93%.

DISCUSSIONS: Pulmonary resection for metastatic non-small cell carcinoma of the lung (stage IV) is relatively contraindicated. However, this patient experienced severe symptoms and hypoxemia related to a shunt through lung densely consolidated with tumor. Demonstration of reversibility of the venoarterial shunt by selective pulmonary artery occlusion and abrogation of her supplemental oxygen requirement was key in determining candidacy for palliative lobectomy.

CONCLUSION: Severe isolated lobar venoarterial shunting associated with BAC is an uncommon complication of disease that may result in debilitating sequelae. Though surgical intervention does not cure the underlying malignancy, in select cases, where symptomatic shunting can be localized and corrected, palliative resection may afford a significant improvement in quality of life.

DISCLOSURE: Peter Crossno, None.

Chest. 2006;130(4_MeetingAbstracts):314S. doi:10.1378/chest.130.4_MeetingAbstracts.314S-a
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INTRODUCTION: Pulmonary blastoma (PB) is a rare neoplasm with a high mortality rate. A subset of PB, biphasic pulmonary blastoma (BPB), is reported to comprise less then 0.25% of all primary non-small cell (NSC) lung tumors and has the highest fatality rate (1).We herein report a case BPB in a 39-year-old African-American (AA) female diagnosed incidentally on a chest x-ray, only thoracotomy and lung resection confirmed the diagnosis. There are less than approximately 200 total cases of PB and of those only 50 adult cases of BPB are described in the literature to date.

CASE PRESENTATION: A 39-year-old AA female with a past medical history significant of hypertension, asthma and a 20 pack year history of smoking tobacco, presented with non specific chest pain and a recent history of unspecified weight loss. She had no prior occupational exposures and history of recent travel. Her father had died of cirrhosis, and her mother was alive at 69 with a medical history of a cerebral vascular accident and coronary artery disease. An EKG and a chest x-ray were requested. The chest x-ray was remarkable for an 8cm X 6cm mass in the right upper lobe. A CT scan of her thorax confirmed the mass as 4.5cm X 9.5cm, bilobular, abutting the mediastinum, with narrowing and displacement of the right upper lobe bronchus. A fiberoptic bronchoscopy with transbronchial biopsy and washings was unmarkable for any abnormal pathology. A mediastinoscopy and biopsy of the lesion also did reveal any diagnosis. Following this she underwent an exploratory thoracotomy and a right upper lobectomy, which on pathology revealed BPB, with prominent stromal overgrowth showing features of a spindle cell carcinoma with anaplastic giant cells. She remains alive to date 7 years in her follow-up with no reoccurrence of the tumor. Our AA patient is one of the longest survival case in USA to date.

DISCUSSIONS: PB is a rare malignant neoplasm of the lung comprised of epithelial and mesenchymal elements that recapitulate the fetal lung at 10-16 weeks' gestation.Barrett and Barnard originally described the tumor in 1945 (2), with subsequent modifications to the terminology made by Barnard and later by Spencer. 75 % of all cases of PB occur in children of less than 5 years of age with a further 20% of cases occur in individuals less than 20 years of age. The exact histogenesis of PB continues to be debated but an association with heavy smoking has been described.Pathologically PB is classified into three types: (1) well differentiated fetal adenocarcinoma (WDFA) and (2) Pleuro-pulmonary blastoma (occurs predominantly in children and is the most common variant) and (3) BPB is composed of both epithelial and mesenchymal malignant cells. Biphasic histology and a size >5 cm in greatest dimension are considered to be very poor and dire prognostic indicators.

CONCLUSION: The prognosis of PB, regardless of histology, is poor. About 50% die with in 3-6 months after diagnosis. Francis and Jacobsen reported the overall 5-year survival to be 16%. To date the only curative treatment is surgical resection; both radiotherapy and chemotherapy have been attempted without any reproducible measure of success. We emphasis, when suspicion is high, surgery is not only the means of diagnosis but also cure.

DISCLOSURE: Amir Khan, None.

Chest. 2006;130(4_MeetingAbstracts):314S-b-315S. doi:10.1378/chest.130.4_MeetingAbstracts.314S-b
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INTRODUCTION: We present a case of myoepithelial carcinoma of the lung with brain metastases. To our knowledge only 7 reported cases have been previously reported.

CASE PRESENTATION: This 38-y-old man presented to the Regional Medical Center at Memphis with complaints of headache, visual changes, unstable gait for 6 weeks and a 15 pound weight loss over 3 months. The patient was a 30 pack-year smoker with a prior history of emphysema and cocaine use. A head CT revealed three ring enhancing masses in the left parietal- occipital, right temporal and cerebellar areas. Chest X-ray and CT studies revealed a LUL mass. A retrospective review of films taken 3 years ago similarly revealed a subtle LUL nodule that had not been recognized at that time. The patient underwent resection of the left parieto-occipital lesion and later biopsy of the LUL. The histology specimens from both lung and brain were identical and revealed a malignant spindle cell malignancy. Given that both the lung and brain lesions were immunoreative for glial fibrillary acidic protein(GFAP), multifocal gliosarcoma with lung metastasis was initially considered. However, further workup revealed coexpression of smooth muscle actin (SMA), S- 100 protein, and various cytokeratins, including CK7. This distinctive immunoprofile established the diagnosis of myoepithelial carcinoma of the lung with brain metastasis.

DISCUSSIONS: Primary lung carcinomas showing features of salivary gland-type neoplasms are rare. Most are mucoepidermoid carcinomas and adenoid cystic carcinomas. Myoepithelial carcinoma of the lung is extremely rare, and in the WHO classification is classified under 'Others' among carcinomas of the salivary gland. A literature search revealed only 7 reported cases. The fact that tumor cells can be positive for GFAP is an underappreciated pitfall and led to the initial consideration of gliosarcoma or glioblastoma. Nevertheless, an expanded study confirmed the diagnosis of metastatic myoepithelial carcinoma due to the coexpression of additional myoepithelial markers (SMA, S-100),as well as CK7, the form of cytokeratin most commonly expressed in the lung.

CONCLUSION: This Unusal case of myoepithelial carcinoma of the lung is the 8th reported case to our knowledge. Very litttle is known about the treatment of metastatic myoepithelial carcinoma of the lung. Hopefully with more case reports our understanding will Improve of this unusal carcinoma of the lung.

DISCLOSURE: Yasser Aleech, None.

Chest. 2006;130(4_MeetingAbstracts):315S. doi:10.1378/chest.130.4_MeetingAbstracts.315S-a
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INTRODUCTION: Post-extubation stridor and upper airway obstruction are among the complications of endotracheal intubation. Here I present a patient with severe hypothyroidism who has developed stridor after extubation and later found to have supraglottic myxedema.

CASE PRESENTATION: 59-year-old woman with no past medical history presented to the emergency department with complaints of weakness and body aches. On exam she was noted to have hypothermia, bradycardia, macroglossia, and non-pitting edema. Patient was admitted to the hospital with diagnosis of severe hypothyroidism. The next day she was transferred to the intensive care unit (ICU) due to confusion, and persistent hypothermia. Shortly after ICU admission patient had an episode of tonic-clonic seizure prompting endotracheal intubation for airway protection. After 2 days of thyroxine and hydrocortisone replacement, patient's confusion, hypothermia, and bradycardia had resolved. The patient was extubated and transferred to medical wards. The patient was transferred back to ICU the next day with recurrent mental status changes. She was found to have bilateral lung infiltrates, and hypoventilation on an arterial blood gas. This has prompted reintubation and initiation of mechanical ventilation and antibiotic treatment. After 6 days of mechanical ventilation and continued thyroxine replacement, patient was extubated for the second time. She immediately developed severe stridor and upper airway obstruction. She was intubated with great difficulty with a size 6 endotracheal tube. Later in the same day a tracheotomy was done. A bronchoscope was used to visualize upper airways and large amount of myxedematous material, presumably mucopolysaccharide deposition (Figure 1) was observed. Later in the course of the hospital stay, patient remained stable, completing her course of antibiotics and the process of liberation from mechanical ventilation. Patient was transferred to a rehabilitation facility for further care. A follow up bronchoscopy was done after 4 weeks of thyroxine replacement and showed near total resolution of the mucoploysaccharide depositions (Figure 2).

DISCUSSIONS: Hypothyroidism may have a multitude of effects on respiratory system including respiratory failure that can be caused by a reduction in central respiratory drive, respiratory muscle weakness and pleural effusions. In severe hypothyroidism mucopolysaccharides accumulate in the ground substance of the dermis and other tissues. This material, also known as myxedema, is responsible for the naming of the hypothermic stuporous state known in patients with severe, long-standing hypothyroidism as myxedema coma. The patients typically present with hypothermia, hypercapnia, hypotension and bradycardia. The deposition of the mucopolysaccharides into the tissues of the hypopharynx result in edema of the supraglottic structures. Resulting supraglottic myxedema may cause upper airway obstruction. Another cause of airway obstruction in hypothyroidism patients is tracheal compression with a large goiter. Our patient did not have an appreciable goiter. It is uncommon for hypothyroidism to result in upper airway obstruction at the level of the supraglottis or glottis. In fact we were only able to find two previous case reports in English language literature (1, 2). Severe supraglottic myxedema resulting in post-extubation stridor has not been previously reported.Treatment of myxedema coma entails aggressive supportive care, including endotracheal intubation and mechanical ventilation if necessary, as well as intravenous thyroxine replacement. Similar to our patient, resolution of supraglottic edema within 3 weeks of institution of treatment was reported in a recent case report (2).

CONCLUSION: Severe hypothyroidism can lead to upper airway obstruction and post-extubation stridor. The supraglottic myxedema is reversible within few weeks with the treatment of hypothyroidism.

DISCLOSURE: Aydin Uzunpinar, None.

Chest. 2006;130(4_MeetingAbstracts):315S-b-316S. doi:10.1378/chest.130.4_MeetingAbstracts.315S-b
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INTRODUCTION: Although mediastinal masses are not uncommon, progressive respiratory failure due to tumor induced phrenic nerve injury has not been well described. This case serves as a model in diagnosing bilateral diaphragmatic paralysis in the critical care setting.

CASE PRESENTATION: A 37-year-old woman with end stage renal disease developed progressive orthopnea over a two month period. She was found to have a large mediastinal mass on radiographs. A CT scan showed an extensive 7 x 9 centimeter mass occupying her mediastinum and surrounding the airways (see figure). She was admitted for mediastinoscopic biopsy and pathology revealed malignant lymphoma. During her recovery she developed respiratory distress and orthopnea. On day five post-op she required re-intubation and was transferred to the ICU. A bronchoscopic airway exam showed no extrinsic compression or endobronchial lesions. Again she was extubated, however rapidly developed accessory muscle use, displayed paradoxical respirations and became somnolent with significant hypercapnea. She was re-intubated and immediately recovered from the episode. The possibility of diaphragm paralysis was entertained. Esophageal and gastric pressure measurements were obtained using balloon tipped catheters while the patient remained intubated on a spontaneous mode of ventilation. Negative waveform deflections during inspiration in both the esophageal and gastric pressure monitor tracings resulted in a diaphragm pressure of zero by the equation (P diaphragm = P gastric - P esophageal). This finding was consistent with the diagnosis of bilateral diaphragm paralysis1. A nerve conduction study and electromyogram of the phrenic nerves confirmed bilateral phrenic neuropathy with denervation of the diaphragm.

DISCUSSIONS: The location of the phrenic nerves in the mediastinum leaves them susceptible to damage from masses, trauma, and iatrogenic injury. Neuropathy from compressive conditions has been described in the literature; hematomas forming after anticoagulation and thrombolytics, compression from left atrial enlargement, and intra-thoracic thyroid goiters. Diabetic neuropathy affecting the phrenic nerve has also been reported. Peripheral neuropathies have been associated with lymphomas. Pathogenesis of lymphoma-associated neuropathy has been elucidated and includes direct invasion and infiltration of nerve bundles by lymphoma cells, metabolic derangements, vascular impairment, infection, and immunologic mechanisms including paraneoplastic neuropathy. Bilateral phrenic neuropathy may present as dyspnea and tachypnea at rest, with the symptoms worsening in the supine position. Use of accessory muscles and paradoxical respirations are the primary clinical sign of bilateral phrenic neuropathy. Diagnostic evaluation includes chest radiography, fluoroscopy, pulmonary function testing, esophageal and gastric manometry, and EMG/nerve conduction studies.

CONCLUSION: Evaluations of diaphragm function in the critical care setting can be difficult, especially with mechanically ventilated patients. We used esophageal and gastric balloon catheters to evaluate pressure waveform tracings while the patient remained on the ventilator. Although an unfortunate situation, this case serves as a reminder to consider diaphragm dysfunction when formulating a differential diagnosis in acute or progressive respiratory failure. In cases of diaphragm dysfunction, knowledge of the defect prior to extubation can allow the critical care team to anticipate the needs of the patient and plan for successful weaning.

DISCLOSURE: Anthony Zachria, None.

Chest. 2006;130(4_MeetingAbstracts):316S. doi:10.1378/chest.130.4_MeetingAbstracts.316S-a
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INTRODUCTION: Mobile right heart thrombi (MRHT) have been observed in patients with severe pulmonary embolism (PE), and are associated with a poor prognosis. The incidence is unclear and no widely accepted guidelines exist regarding optimal therapy. We present a case of venous thromboembolism complicated by MRHT successfully treated with systemic thrombolysis.

CASE PRESENTATION: 48-year-old male presented with acute onset of dyspnea of 1 day duration, accompanied by chest pain, productive cough and chills. On admission he was afebrile, tachycardic 105 per minute, tachypneic, blood pressure was 98/60 mm Hg and pulse oximetry 93% on room air. Initial laboratory values included creatinine = 1.8mg/dl, leukocytosis and transaminatis. Chest X-Ray showed pulmonary artery enlargement. Ventilation–perfusion (V/Q) scan was obtained, with a preliminary report of low-probability for PE. Initial therapy consisted of 2.5 L of normal saline heparin and antibiotics with improvement of blood pressure and tachypnea. Overnight patient became hypotensive and cyanotic despite volume expansion, and transferred to MICU. Laboratory analysis revealed lactic acidosis, elevated brain natriuretic peptide level. Arterial blood gases revealed respiratory alkalosis and hypoxia. Transthoracic echocardiography (TTE) showed right atrial and ventricular dilatation with mobile clot in hepatic vein, inferior vena cava (IVC), right atrium and ventricle. Alteplase (10 mg IV bolus, 90 mg IV over the next 2 hours) was administrated, with the initial development of hypotension and chest pain, requiring norepinephrine. Symptoms resolved, repeated TTE after thrombolysis completion documented complete resolution of MRHT. Doppler ultrasound of lower extremities has shown bilateral, near-occlusive and mobile popliteal thrombosis. Temporary IVC filter was placed. V/Q scan interpretation was subsequently revised as high- probability for PE. Renal function normalized and patient was discharged home on warfarin.

DISCUSSIONS: The incidence of MRHT ranges between 3 and 23% with some data suggesting that transesophageal echocardiography is more sensitive than TTE. We are aware of no randomized, controlled trials of treatment of MRHT. Proposed treatment recommendations include surgery, thrombolysis and anticoagulation and are based on case series with no clear consensus (1). Analysis of a European multicenter PE registry showed no overall difference in mortality between these treatment modalities. However, subgroup analysis showed increased mortality in comparable groups of patients with and without MRHT treated with heparin alone (23.5% vs. 8% respectively)(2). These findings suggest that anticoagulation alone may not be sufficient treatment for patients with PE and MRHT. A large metanalysis(1) summarized 177 cases from 95 studies between 1966 and 2000. Mortality post thrombolysis was significantly lower than with surgery or anticoagulation alone (11.3% vs. 23.8% and 28.6% respectively). It is unknown if new MRHT caused late clinical deterioration in our patient or MRHT was present during the initial evaluation. The later case, if true, may argue for consideration of more aggressive therapy in hemodynamically stable patients with PE and documented MRHT.

CONCLUSION: MRHT seems to be a sign of a severe PE. TTE is a safe test that may be useful to establish diagnosis and guide a treatment in patients with suspected PE. It may be particularly helpful in the settings of initially non-conclusive studies when use of contrast–utilizing modalities is undesirable. MRHT can embolize both prior to and after the thrombolysis. Alteplase may be an effective and safe treatment option in this patient group.

DISCLOSURE: Mikhail Gabrilovich, None.

Chest. 2006;130(4_MeetingAbstracts):316S-b-317S. doi:10.1378/chest.130.1.307
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INTRODUCTION: Pericardial tamponade can be a life-threatening condition and has at its source multiple and varied causes. We report a case of purulent pericardial tamponade, caused by erosion of an infected hepatic cyst.

CASE PRESENTATION: A 69-year-old male with past medical history of hypertension, reflux disease, congestive heart failure, diverticulosis, chronic pancreatitis, polycystic kidney and liver disease, and end-stage renal disease, status post cadaveric renal transplant 5 years prior, presented to the emergency department with a history of 2 weeks of intermittent fever, chills, nausea and non-bilious vomiting. A previous admission, 8 months prior, had been for an infected liver cyst complicated with septicemia. In the emergency department, the patient became hypotensive, tachypneic and experienced worsening oxygen saturation. He was intubated for respiratory distress. A chest X-Ray showed an enlarged heart silhouette and left pleural effusion, while an echocardiogram showed right ventricular collapse and evidence of pericardial tamponade (Fig. 1). The EKG was normal. In the catheterization laboratory, 2 liters of purulent pericardial effusion was drained via pericardiocentesis. In order to achieve better drainage, the patient was, two days later, taken to the operating room for a pericardial window procedure and a left segmental liver resection. This liver segment contained the infected cyst that had eroded into the pericardium (Fig. 2). Both the fluid from the pericardium as well as the liver cyst showed microbiological evidence of Pseudomonas aeruginosa. The patient recovered from the hemodynamic and infectious standpoint and was transferred to the general surgical ward.

DISCUSSIONS: Pericardial tamponade can be classified into 4 subtypes as acute, subacute, regional and low pressure(1). On physical examination sinus tachycardia, increased jugular venous pressure, absence of an inspiratory decrease in jugular venous pressure (Kussmaul's sign), pulsus paradoxus (75%), and friction rub (30%) can all be appreciated. and may present with distant heart sounds (“quiet heart”), increased jugular venous pressure, and hypotension (Beck's triad). It can be diagnosed with transthoracic or transesophageal echocardiography, as well as computerized tomography (CT). CT imaging is not necessary if echocardiography is available. The echocardiogram can show diastolic collapse of the anterior right ventricular free wall, right atrial collapse, left atrial collapse (25% of patients) and very rarely left ventricular collapse. Inferior vena cava dilatation (i.e. no collapse on inspiration) and a “swinging heart” can be seen. Electrical alternans is a pathognomonic finding on the EKG.In the differential diagnosis the clinician should include acute coronary syndrome (ACS), aortic dissection, and congestive heart failure. ACS will have characteristic EKG findings, aortic dissection should not cause an increase in jugular venous pressure and neither is associated with pulsus paradoxus. Congestive heart failure can be differentiated by the use of echocardiography. Treatment can be achieved by catheter pericardiocentesis with echocardiographic guidance (class I recommendations by the European Society of Cardiology (2)), surgical pericardiectomy, which permits biopsies but exposes the patient to the risks of general anesthesia, and percutaneous balloon pericardiotomy, especially in patients with malignant effusions.In the published literature, most of the infectious causes of pericardial tamponade stem from hydatid cysts, either in the pericardium or from a nearby ruptured hepatic hydatid cyst. To our knowledge, this may be the first case of a non-hydatid infected hepatic cyst rupturing into the pericardium causing tamponade physiology. The treatment of pericardial tamponade from varied etiologies is similar and can lead to a favorable outcome.

CONCLUSION: Although rare, pericardial tamponade from a fluid-filled cystic structure as in this case can be caused by erosions of nearby structures. Timely diagnosis and treatment can result in favorable outcome.

DISCLOSURE: Andrew Miller, None.

Chest. 2006;130(4_MeetingAbstracts):317S. doi:10.1378/chest.130.5.1547
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INTRODUCTION: We present a case of a 26-year-old man who was involved in a mining accident. He was trapped for approximately 42 hours with carbon monoxide levels measured at 1300 parts per million within the mine shaft. The patient had a significant decrement in his cognitive function, and had reduced cardiac function. After 3 treatments with hyperbaric oxygen, the patient survived. He had gradual, but dramatic improvement in his cardiovascular and neurologic function.

CASE PRESENTATION: A 26-year-old male was involved in a mining accident caused by an explosion igniting methane gas. He was trapped in the mine for approximately 42 hours. Rescuers were delayed 12 hours after the explosion due to high concentrations of carbon monoxide and methane gas in the shaft. Air near where the miners were last known to be stationed contained 1,300 parts per million of carbon monoxide (400 parts per million is the maximum considered safe). Our patient was found alive, and was the only survivor found. He was noted to be in respiratory distress and had an altered sensorium. The patient was intubated, placed on mechanical ventilation and taken to a nearby hospital. Initial carboxyhemoglobin levels were inaccurate. The patient was stabilized and transferred to our institution for hyperbaric oxygen therapy. Carboxyhemoglobin levels at the previous tertiary care center were reported as 20%. Upon arrival to our institution, the patient was in multisystem organ failure. Echocardiography revealed an ejection fraction of 20% with global hypokinesis. Computerized tomography of the brain revealed multiple focal areas of hemorrhage within the subcortical white matter. The globus pallidus was spared. The patient received three treatments of hyperbaric oxygen therapy at 3 atmospheres for 30 minutes and 2.4 atmospheres for 60 minutes. He demonstrated significant improvement in all aspects of his care. His ejection fraction improved to 35% with normal wall motion. His neurological status improved significantly as he is currently mobile, is communicative and has his cognitive functions intact.

DISCUSSIONS: Carbon monoxide (CO) is a tasteless and odorless gas caused by incomplete combustion of carbon based compounds. In the US, 1000-2000 accidental deaths occur each year. CO binds oxygen at 240 times that of oxygen consequently decreasing oxygen carrying capacity of blood. The severity of carbon monoxide poisoning is a function of the duration of exposure and the concentration of the gas. The initial carboxyhemoglobin level correlates poorly with outcome. Mechanisms of acute carbon monoxide poisoning include hypoxia, reduced cellular oxygen metabolism, lipid perioxidation leading to oxidative injury, and damage to vascular endothelium. Persistent or delayed neurologic sequalae can ensue. It may also lead to myocardial injury leading to increased long term mortality. Standard treatment includes removal from exposure site, administration of supplemental oxygen, and general supportive care. The half life of carboxyhemoglobin is approximately 320 minutes which is decreased to 23 minutes with hyperbaric oxygen treatment. Furthermore, hyperbaric oxygen therapy has been shown to decrease morbidity and mortality. It has also been shown to decrease the delayed neurological sequalae associated with carbon monoxide poisoning. To our knowledge, this is the first reported case of a patient surviving long term exposure to lethal doses of carbon monoxide levels after hyperbaric oxygen therapy.

CONCLUSION: Carbon monoxide poisoning remains an important cause of accidental and intentional injury worldwide. This is the first case reported of a patient surviving prolonged periods of carbon monoxide exposure at lethal doses after treatment with hyperbaric oxygen. We recommend the use of hyperbaric oxygen therapy in patient with sustained and toxic levels of carbon monoxide that have neurologic and cardiac toxicity.

DISCLOSURE: Anil Singh, None.

Chest. 2006;130(4_MeetingAbstracts):317S. doi:10.1378/chest.130.4_MeetingAbstracts.317S-b
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INTRODUCTION: In 1991, Nierenberg and colleagues published a case report of a patient presenting with respiratory failure and cardiac arrest after intense exposure to mineral spirits (1). Mineral spirits are a heterogeneous mixture of hydrocarbons found in various commercial products including wood stains and cleaning solutions. We present a case of a patient with similar exposure history.

CASE PRESENTATION: The patient is a 54-year-old obese white male who was in good health. He had been applying a floor staining product, Minwax ™ in his basement when he became acutely dyspneic. He was found by emergency services in his backyard, unresponsive, with pink frothy sputum, but with adequate pulse and BP. In the ED, he required an emergency tracheostomy and ventilatory support. His past medical history was notable for a myocardial infarction two years ago. He was a non-smoker, did not use illicit drugs or have HIV risk factors. He worked as a printer where he is exposed to paper dusts but has not had any prior respiratory symptoms. He had no family history of respiratory disease. On examination his vital signs were stable. He sustained laceration and abrasion injuries to his forehead and nose. He had a normal chest wall configuration and scattered rales were heard on lung exam. The rest of his exam was unremarkable. His initial chest radiograph demonstrated diffuse alveolar infiltrates. A trauma CT series revealed no significant injuries but showed patchy bilateral alveolar infiltrates. His initial EKG showed a normal sinus rhythm and a left bundle branch block, age unknown. Initial laboratory data were unremarkable including normal cardiac enzymes and BNP. He was oxygenating and ventilating well on a tidal volume of 700 and a fiO2 of 60%. Because of his history of CAD, he was admitted to the coronary care unit to rule out MI. His cardiac work-up including cardiac enzymes and a 2Decho were unremarkable. The following day bronchoscopy was performed. BAL was not suggestive of acute eosinophilic pneumonia or hypersensitivity pneumonitis. His follow up CXR showed dramatic clearing of the infiltrates. He was liberated from the ventilator to trach collar within 4 days and quickly decannulated. He was discharged without any functional limitation.

DISCUSSIONS: An exposure history is very important when evaluating respiratory failure. We feel our patient suffered from acute lung injury (ALI) secondary to exposure to a product containing mineral spirits (Minwax ™). Mineral spirits are commonly found in commercial and industrial products like varnishes and sealants. They are petroleum distillate mixtures of hydrocarbons. There are reports of noncardiogenic pulmonary edema following inhalation of other common commercial compounds like phosgene and sulfuric acid(2). The pathogenic mechanism is likely that of ALI. Radiographically, ALI can appear as normal appearing lungs to diffuse alveolar filling. Clinically, it can progress to ARDS. Residual lung function can vary from normal to permanent respiratory dysfunction. Our patient presented with acute respiratory failure and pulmonary edema. He was ruled out for an MI. Given his inhalation history plus the negative cardiac work-up, ALI from mineral spirits was considered. Other less common but important pulmonary etiologies were ruled out like acute eosinophlic pneumonia and hypersensitivity pneumonitis. Our patient dramitically improved and was discharged with no functional limitations.

CONCLUSION: Exposure history is of the utmost importance. This case illustrates the development of acute lung injury secondary to mineral spirits, a common component of many commercial and industrial compounds.

DISCLOSURE: Francis Castiller, None.

Chest. 2006;130(4_MeetingAbstracts):318S. doi:10.1378/chest.130.4_MeetingAbstracts.318S-a
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INTRODUCTION: Benign metastasizing leiomyomatosis (BML) is a rare condition that usually affects women with a history of uterine leiomyomatosis who have undergone a hysterectomy. It is characterized by multiple, benign, slow growing, smooth-muscle tumors outside the uterus. The lungs are the most common site of metastasis.

CASE PRESENTATION: We report the case of a 41-year-old African American woman with a history of uterine arterial embolization (UAE) at age 38 for the treatment of uterine leiomyomatosis. The patient was referred to the pulmonary clinic after the incidental discovery of multiple right lower lobe nodules on an abdominal computed tomography (CT) scan ordered for the evaluation of abdominal pain and constipation. A dedicated chest CT showed small, bilateral pulmonary nodules from 2-13mm in diameter. On review of systems, the patient complained of a non-productive cough for about a year, which had been attributed to gastric reflux and nasal rhinitis. Otherwise, she was asymptomatic. She had no other significant past medical history and no history of occupational exposures. She had lived in areas that were endemic for histoplasmosis and coccidiomyocosis, but urine histoplasmosis antigen and serum coccidiomyocosis titers were within normal limits. A complete blood count, liver function panel, and serum chemistries were also unremarkable. A whole body positron emission tomography scan demonstrated no increased fluorodeoxyglucose uptake in the lesions. The patient underwent a CT guided biopsy, which showed bundles of benign smooth muscle cells. The histopathology and immunohistochemical stains were consistent with metastasis of the patient's benign uterine leiomyoma. Surgical castration and hormonal therapy were recommended. However, the patient was relatively asymptomatic and has not yet consented to either. She has been closely followed for clinical or radiographic evidence of disease progression.

DISCUSSIONS: Benign Metastasizing Leiomyomatosis (BML) is a rare condition that is characterized by the presence of multiple, indolent, smooth muscle tumors in the lung. It is thought to be the result of hematogenous spread of a benign primary uterine leiomyoma. Because BML usually occurs months to years after hysterectomy, it is theorized that the tumor cells may enter the blood stream during the trauma of surgery and disseminate. Most patients with BML are minimally symptomatic, but patients can have significant chest pain, dyspnea, and cough. The tumors tend to be hormonally sensitive. There have been reports of successful treatment with surgical castration, progesterone, gonadotropin releasing hormone analogues, aromatase inhibitors, luteinizing hormone releasing hormone agonists, and selective estrogen receptor modulators. In recent years, uterine arterial embolization has gained popularity as a minimally-invasive option for the treatment of uterine leiomyomatosis. To our knowledge, this represents the first reported case of BML in a patient who has undergone UAE.

CONCLUSION: BML is a rare condition whereby benign uterine leiomyomas metastasize to sites outside the uterus. It usually occurs after hysterectomy. The tumors tend to be indolent and hormonally responsive. UAE has become a popular alternative to hysterectomy for the treatment of uterine leiomyomatosis. We report the first case of BML after UAE. Physicians should be aware that there is a potential relationship between UAE and BML. As such, the diagnosis of post-UAE BML should be considered in the appropriate clinical setting.

DISCLOSURE: Joshua Sill, None.

Chest. 2006;130(4_MeetingAbstracts):318S. doi:10.1378/chest.130.4_MeetingAbstracts.318S-b
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INTRODUCTION: Common causes of a solitary lung cavity are tuberculosis (TB), fungal infection, malignancy, and benign tumors such as hamartoma. Rare causes include bronchogenic cyst, sequestration, congenital lobar emphysema and congenital cystic adenomatoid malformation (CCAM). This is a case of placental transmogrification (PT) of the lung in a patient with newly discovered uterine cancer.

CASE PRESENTATION: 66-year-old woman was referred for an incidental solitary cavity found on radiographs. She had recently been diagnosed with Stage IB endometrial cancer by D&C after presenting with post menopausal bleeding. Staging CT chest/abdomen/pelvis showed a cavity in the RUL and multiple hepatic lesions suggestive of hemangiomas. The patient was asymptomatic. A work up to rule out extra uterine disease included: CT needle biopsy of a hepatic lesion which was benign, PET scan showed mild uptake of 2.1 in the rim of the cavity suggesting benign etiology, and bronchoscopy with washing and lavage done by thoracic surgery was negative for malignancy and infection. The cavity was believed to be benign so TAH/BSO was performed and she received six cycles of carboplatin and paclitaxel and intravaginal brachytherapy. Patient was then referred to the pulmonary service to follow up on the cavity. She had no history of pulmonary disease and no prior radiographic studies. She was a teacher who never smoked, rarely traveled, and lived in New York City. She denied any exposure to TB and had a negative PPD one year prior. She was taking no medications. Her physical exam was unremarkable. Repeat CT chest was done after completion of chemotherapy and compared to the initial staging CT chest 6 months prior showed stable RUL cavity. Repeat PPD test was negative. The lesion was observed over two years, during which time it slowly increased in size. After an unrevealing FNA of the cavity and unchanged PET scan, a wedge resection of the cavity was done. The pathology was pulmonary PT.

DISCUSSIONS: Pulmonary PT is a rare benign condition. The pathology of the lesion is a bulla filled with papillae similar in appearance to a placental villus and hence the name. The papillary core consists of vascular fibrous tissue lined by pneumocytes. It is described as an isolated entity or in association with other lung diseases such as emphysema, fibrochondromatous hamartomas, lipomatosis, and Swyer-James Syndrome. The etiology of PT is unknown. It may be a congenital malformation, a reaction to emphysema, an unrecognized hamartoma or due to vascular abnormalities. The radiographic presentation of PT is usually an air filled cyst or thin walled cavity which at times may be large enough to cause mediastinal shift or rupture to cause pneumothorax. It may also present as a fluid filled cyst, nodule or mass. FNA is usually non diagnostic. The diagnosis is made by surgical resection which is only required if the patient is symptomatic or there is concern that the lesion could be malignant.

CONCLUSION: PT is a rare benign cause for a solitary cystic lesion of the lung, and should be considered in the differential diagnosis. Most of the rare causes of cystic lung malformations mentioned above are benign, except for CCAM which carries the potential of malignant transformation. Therefore, observation is necessary, and surgery is only required if the lesion starts to increase in the size or the patient becomes symptomatic.

DISCLOSURE: Samer Homsi, None.

Chest. 2006;130(4_MeetingAbstracts):318S-c-319S. doi:10.1378/chest.130.4_MeetingAbstracts.318S-c
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INTRODUCTION: Primary tumors of the lung are rare in children and clinical manifestations can be nonspecific. We present the case of an adolescent female who was referred to a tertiary care medical center with persistent cough and left upper lobe density unresponsive to antibiotics. Her work up revealed an inflammatory pseudotumor which was treated successfully with steroid therapy.

CASE PRESENTATION: KB is a 17-year-old African-American female who presented with a three month history of persistent cough and an unresolving left upper lobe density. She had been treated with three courses of antibiotics unsuccessfully and was experiencing dyspnea on exertion, anorexia and weightloss. She had no history of fever and her chest was clear to auscultation but she was tachypneic. Review of systems, past and family history were unremarkable. She had no exposure to tuberculosis and was not engaged in high risk behavior. Her chest X-ray changes were stable. There was no evidence of oligemia of lung fields. She had a negative Mantoux test, negative sputum cultures, non-diagnostic angiotensin converting enzyme level, negative histoplasmosis titres, normal lactate dehydrogenase level and an elevated C- reactive protein (CRP) level. A chest computerized tomographic (CT) scan was performed which showed a granulomatous process involving the parenchyma of the left upper lobe extending into the mediastinum and encasing the left pulmonary artery. The lesion showed both fibrous and scattered small calcific elements. A ventilation and perfusion scan revealed compromised blood flow and ventilation to the left upper lobe of lung (Right/Left ratio of 93/07). A biopsy of the lesion showed fibroadipose tissue with chronic inflammatory infiltrate suggestive of inflammatory pseudotumor. The patient and family refused surgical treatment. She was thus treated with a three month course of oral cyclo-oxygenase-2 inhibitor (COX2I) anti-inflammatory agent with continued exertional dyspnea, cough and weightloss. The patient was then treated with a prolonged course of prednisolone over six months with involution of the tumor, decreasing CRP levels, improved left pulmonary arterial blood flow and symptomatic relief.

DISCUSSIONS: Inflammatory pseudotumor is believed to be the most common primary pulmonary childhood tumor, its etiology and pathogenesis remain obscure. Important differential diagnoses include granulomatous diseases such as tuberculosis, fungal infections and sarcoidosis as well as other neoplasms. The case highlights the importance of chest CT evaluation of suspicious lesions as it may better delineate the extent and nature of a lesion and in our case demonstrated unsuspected impressive pulmonary artery compression which had not been appreciated on Chest X-ray. While resection is usually curative, the close vascular relationship of the tumor and refusal for surgery were limitations to excision in our patient. Having reviewed non-surgical treatment options and their side-effects with the family, a decision to try COX2I was made, based on a case report; without success. Reports of use of steroid therapy for inflammatory pseudotumor are scarce and anecdotal however given the anatomical and treatment limitations in our patient with the progressive nature of the disease process this therapy was used successfully with significant anatomic and symptomatic improvement. Mild hypertension and Cushingism were the only significant side-effects of therapy.

CONCLUSION: Primary pulmonary tumors must be considered in the differential diagnosis of persistent respiratory symptoms in pediatric patients. Chest CT scan is useful for the evaluation of persistent lung lesions and should be resorted to without delay. While excision of inflammatory pseudotumor of lung is often definitive therapy, recurrences are known. Non-surgical treatment options are limited; steroids may be used when excision is not possible or limited in patients with inflammatory pseudotumor.

DISCLOSURE: Ameet Daftary, None.

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