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Case Reports: Monday, November 1, 2010

Chest. 2010;138(4_MeetingAbstracts):1A. doi:10.1378/chest.10120
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INTRODUCTION: Catastrophic antiphospholipid syndrome (CAPS) is an infrequent potentially devastating syndrome that can involve every organ, including the lungs. Our patient with CAPS with multiple thrombosis and diffuse ground glass pulmonary opacities was refractory to conventional therapies. To our knowledge, this is the first report documenting the efficacy of rituximab in resolving pulmonary manifestations.

CASE PRESENTATION: A 38 year old man presented with a 4 year history of intermittent episodes of dyspnea, fatigue and thrombocytopenia. Our work up yielded restricted lung volumes with a severely decreased DLCO. A CT of the chest revealed bilateral areas of ground glass opacities. Serologies were positive only for lupus anticoagulant and anticardiolipin antibodies. Weeks later he developed a deep vein thrombus (DVT) and was anticoagulated with warfarin. Factor II activity was monitored with a goal of 5-35% activity. An open lung biopsy revealed plexiform pulmonary arteriopathy, pulmonary emboli, mild emphysematous changes and interstitial lymphoid hyperplasia. Despite achieving factor II activity goal, he developed a second DVT. At this point enoxaparin was initiated along with cyclophosphamide at 500mg/m2 monthly and prednisone 60mg daily. Two months later he was diagnosed with bilateral pulmonary emboli and was hospitalized. This was complicated by acute renal failure, digital ischemia, acute non-ST elevation myocardial infarction, transient ischemic attack (TIA) and significant hypoxia requiring 4 lpm oxygen. A CT chest revealed significant worsening of bilateral areas of ground glass opacity in a similar distribution compared to the original CT of the chest. A diagnosis of CAPS was made and emergent plasmapharesis along with parenteral methylprednisolone 2mg/kg was begun. Failing both first and second line therapies, rituximab was added to his steroid regimen. Over the following 10 days he was stabilized and discharged home. Fifteen days after the first dose of rituximab, a second dose was administered. One week after that, a repeat CT chest revealed complete resolution of the ground glass opacities. This was accompanied by a clinical resolution of digital ischemia, renal failure and TIAs. The prothrombin time and partial thromboplastin times also improved.

DISCUSSIONS: Proposed criteria to diagnose CAPS are as follows: 1. 3 or greater organ system involvement 2. Simultaneous (within 1 week) multiorgan involvement 3. Histopathologic confirmation of small vessel occlusion 4. Presence of antiphospholipid and/or anticardiolipin antibodies. 1 There is considerable heterogeneity in the presentation of CAPS. The most common precipitating event is infection (22%) followed by surgical procedures (10%) and difficulty managing anticoagulation (8%)2This case illustrates the pulmonary parenchymal and vascular manifestations of primary antiphospholipid syndrome and highlights the challenges in its management. Evidence to support the use of a third line agent such as rituximab in controlling the pulmonary manifestations is limited. After exhausting more conventional means, rituximab, an anti-CD20 monoclonal antibody, induced a remission in this life-threatening disease. Following the second dose, the patient continued to demonstrate a sustained improvement in his dyspnea and complete resolution of the pulmonary abnormalities. We believe that the rituximab was effective as a rescue therapy after failure of cyclophosphomide and prednisone. He is expected to get 5 more doses of rituximab with close clinical follow up.

CONCLUSION: Rituximab, an anti CD 20 antibody, proved to be an effective rescue therapy for our patient with catastrophic primary antiphospholipid antibody syndrome with pulmonary manifestations who was resistant to conventional therapy including cyclophosphomide.

DISCLOSURE: Anthony Loschner, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):2A. doi:10.1378/chest.9815
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INTRODUCTION: Aortic dissection is the most common and most lethal catastrophe involving the human aorta. The prognosis of untreated aortic dissection is dismal, with 25 percent mortality within 24 hours and 50 percent within 1 week. Lower extremity ischemia as one of the presenting manifestations of aortic dissection is seen in up to 26 percent of patients , but isolated ischemia of the lower extremity due to aortic dissection is rare and is described in only a few reports. This report describes a case of aortic dissection in which the main feature at presentation was acute right leg ischemia.

CASE PRESENTATION: A 25 year old African American gentleman with no known past medical history presented with vomiting and pain in right thigh for one day. As per patient he attended a party last night where he participated in a group dance for 15 minutes. Two hours later he was engaged in sexual activity, but had to stop because of sudden onset of severe pain in right thigh. Next morning he had three episodes of non-bloody projectile vomiting with red discoloration of urine. No complains of shortness of breath, chest pain, cough, headache, trauma, smoking or drug abuse. On physical examination patient was alert, awake and oriented, lying in bed in moderate distress. Vitals were within normal limits. Chest was clear to auscultation with bilateral vesicular breathing. Both heart sounds were regular with no added sound. Right thigh was tender on medial side. No discoloration or bruising was noticed. Pulses were equal in both limbs. Laboratory data showed severe Rhabdomyolysis (CPK 332674) and acute renal failure. CXR was normal except for prominent aortic knuckle. Initial assessment of Severe Rhabdomyolysis secondary to trauma/ischemia was made. Patient was admitted to medical floor. IV hydration was started with target output of 250 to 300ml/hr. Sodium bicarbonate IV was given to maintain urinary PH > 6.5. Twelve hours later, patient was still complaining of right thigh pain and tightness. Pulses in right lower extremity were feeble on palpation. Labs showed CPK of 683502 with worsening renal and liver functions. Echocardiogram was done to rule out Intra-cardiac thrombus, which revealed dissection of ascending aorta. Patient was immediately transferred to the nearest cardiothoracic centre where a computer tomography scan with intravenous contrast revealed ascending thoracic aorta Stanford Type A dissection, extending distally to external iliac artery. He underwent interposition graft repair of the aortic dissection. Later he was discharged home in a stable condition.

DISCUSSIONS: Incidence of pain free dissection varies between 5-15% and is more common in Marfan syndrome or other connective tissue disorders. Lower extremity symptoms accompanying dissecting hematomas of the aorta are well described but isolated lower extremity ischemia as the presenting syndrome of aortic dissection is rare and frequently misinterpreted so that the diagnosis of aortic dissection is delayed or missed, often with catastrophic results. . An acronym "ILEAD" had been coined standing for ischemia of the lower extremity due to AD . It is a rare presentation the incidence of which in one study was less than 10 out of 1751 cases of AD.

CONCLUSION: 1-Aortic dissection is the most common and life threatening catastrophe affecting aorta, which requires prompt recognition and treatment to avoid dismal outcomes.2-Isolated ischemia of the lower extremity due to aortic dissection is rare and is described in only a few reports. Aortic Dissection should be considered in differential diagnosis of patients presenting with isolated lower limb pain.

DISCLOSURE: Saurabh Parasramka, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: pain , thigh
Chest. 2010;138(4_MeetingAbstracts):3A. doi:10.1378/chest.10269
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INTRODUCTION: Pulmonary vein stenosis (PVS) following radiofrequency ablation (RFA) for symptomatic atrial fibrillation (AF) poses a diagnostic challenge for clinicians. Symptoms, signs and radiographic findings of PVS are nonspecific and can be attributed incorrectly to pneumonia, thromboembolism or interstitial lung disease. For this reason, identification of clues to the diagnosis of PVS is important. We report a case of PVS with pulmonary hypertension (PH) where the presence and location of PVS was suggested by the observation of regional loss of a V wave during the measurement of pulmonary artery wedge pressure. To the best of our knowledge, this is the first description of this sign of PVS or occlusion.

CASE PRESENTATION: A 65-year old male with a history of chronic AF resistant to anti-arrhythmic therapy and electrical cardioversion was referred to the PH clinic 3 years after mini maze and 2 years after RFA. He reported dyspnea on exertion and cough. Evaluation in the emergency department several weeks before resulted in a diagnosis of left lower lobe pneumonia which did not improve with antibiotics. PVS was suspected and magnetic resonance angiography was performed. The images showed obstruction of the left inferior pulmonary vein. A transesophageal echocardiogram showed mitral regurgitation. Measurements of left atrial pressure, recorded during attempted balloon angioplasty, showed a V-wave. Right sided heart catheterization assessed suspected PH. The right pulmonary artery wedge pressure (Pawp) pressure recording showed a V wave and the right Pawp was 22mmHg. The left Pawp wave form showed no V wave and the left Pawp was 35mmHg.

DISCUSSIONS: PVS after RFA presents with nonspecific symptoms, signs, and radiographic findings, and may be associated with PH. Pulmonary artery catheterization is essential to the assessment of PH. Others have described variation of Pawp between sites in the lungs as a clue to the diagnosis of pulmonary veno-occlusive disease, but the loss of a V wave on Pawp hemodynamic tracings has not been described before as a clue to the diagnosis and location of PVS.

CONCLUSION: Regional loss of V waves recorded during pulmonary artery wedge pressure measurements is a clue to the diagnosis and location of pulmonary vein stenosis.

DISCLOSURE: Mateo Porres Aguilar, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):4A. doi:10.1378/chest.11085
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INTRODUCTION: We present the first reported case of combined medical and interventional therapies for the treatment of PH associated with Takayasu's Arteritis (TA).

CASE PRESENTATION: A 49 year-old female presented with a three-year history of progressive shortness of breath, fatigue and exertional chest discomfort. She had TA for 18 years; although, the disease had been in clinical remission for 8 years; she was taking no medications. Computed tomography pulmonary angiogram demonstrated a 2.2 cm stenosis of right pulmonary artery (RPA) and focal stenosis of left pulmonary artery with distal aneurysmal dilatation. Right and left heart catheterization demonstrated severe pulmonary hypertension (PASP 150mmHg, PADP 41 mmHg, mean PA pressure 80 mmHg and pulmonary vascular resistance [PVR]) 1600 dyn s/cm5), and single vessel coronary artery disease. Due to the severity of the PH, epoprostenol was begun. Despite an increasing dose of epoprostenol during the subsequent 4 years, evidence of right heart dysfunction ensued. Repeat right heart catheterization demonstrated mild improvement in pulmonary artery pressure; but the PVR remained markedly elevated (1012 dyn sec/cm 5) and the cardiac index had decreased. A pulmonary arteriogram demonstrated that the 2.2 cm stenosis of the main RPA had a peak trans-stenotic pressure gradient of 92mm Hg (mean gradient 42mm Hg). The PH regimen was intensified by addition of sildenafil; but, she could not tolerate this and there were increased admissions for volume overload. Evidence of right heart dysfunction progressed despite increased epoprostenol and diuretics: she developed ascites, congestive hepatopathy, progressive renal dysfunction and was only able to walk 25 feet (WHO Functional Class IIIb-IV). At this point, the large stenosis of the RPA was successfully stented and the peak trans-stenotic gradient decreased to 35mm Hg. The increased blood flow to the chronically under-perfused right lung (estimated by Poiseuille's Law as 33 times greater), did result in transient pulmonary edema of the right lung. However, within one week, the diuretic dose was decreased; renal function, BNP and liver function had normalized, oxygenation improved, and she was able to walk >100 feet. At 13 month follow-up post-procedure: she can now walk >400ft (WHO Functional Class II); there are no overt signs of right heart failure; however, there has been no significant change in RVSP by echocardiogram.

DISCUSSIONS: In patients with TA, 45-80% have an abnormal lung scan and 30-74% an abnormal pulmonary angiography.(1) The natural history of pulmonary involvement in TA is unclear; however it does appear that many patients with pulmonary involvement develop PH. As in this patient, PH associated with TA can be severe and is associated with a poorer prognosis. The etiology of the PH is likely multi-factorial and not simply related to mechanical obstruction of the pulmonary vascular bed by stenoses due to the arteritis. In the current case, the patient initially improved with systemic prostanoid therapy (epoprostenol). Previous reports of medical therapy of PH associated with TA have had a much shorter follow-up period. Not unexpectedly, due to the extensive and severe RPA stenosis, the response to medical therapy in this patient was limited. Relieving the extensive obstruction to the right PA, although technically challenging, resulted in significant clinical improvement and a marked decrease in signs of right ventricular dysfunction.

CONCLUSION: Pulmonary arterial involvement in TA is not uncommon and results in significant morbidity. We report the first case of PH associated with TA successfully treated initially with long-term epoprostenol, and, when the effectiveness of medical therapy eventually waned, was complimented by endovascular stenting of the severe RPA stenosis.

DISCLOSURE: Praveen Govender, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):5A. doi:10.1378/chest.10543
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INTRODUCTION: Pulmonary Embolism (PE) can present with a variety of cardiac arrhythmias and conduction abnormalities. We describe a case of complete heart block secondary to a large saddle PE.

CASE PRESENTATION: A 77 year-old man presented to the emergency department with the complaint of acute onset dyspnea 2 days prior to arrival. In the emergency department, an electrocardiogram (ECG) revealed complete heart block (CHB) with an idioventricular rhythm (Right bundle branch morphology) of 55 beats per minute and an underlying sinus rhythm. He had no known previous cardiac problems, and had not experienced any dizziness or syncope. His past medical history was remarkable for hypertension, gastroesophageal reflux and dyslipidemia. A computed-tomography (CT) scan of his chest showed large saddle pulmonary embolism. Heparin infusion was initiated after an initial bolus dose. Within a few hours he began to have increasing dyspnea, respiratory distress, and worsening hypoxia. He was then given thrombolytic therapy after placement of an IVC filter. Repeat chest CT revealed lysis of the saddle PE but smaller thrombi present in more distal pulmonary vessels. The next morning, an ECG revealed CHB with an idioventricular rhythm, now of left bundle branch morphology. Two days after his presentation, ECG showed a sinus rhythm with 1st degree AV block, right bundle branch block (RBBB) and a left anterior fascicular block. Echocardiogram was performed and showed normal left ventricular size with ejection fraction of 68%, dilated right ventricle with a right ventricular systolic pressure was 53 mmHg. His symptoms improved and he was discharged in good condition. At a follow-up visit, he was noted to have continued tri-fascicular block, and underwent uncomplicated implantation of a dual-chamber pacing system.

DISCUSSIONS: Although electrocardiographic changes associated with pulmonary embolism are common, the presence of complete atrioventricular block is rare. It is well known that patients with acute pulmonary embolism may have findings of cor pulmonale including sinus tachycardia, new S1Q3 QRS pattern in lead I, and negatively directed T wave in lead III, and incomplete or complete RBBB. The mechanism by which RBBB occurs is thought to be related to right ventricular dilatation. Since the right bundle branch is relatively superficial in its path along the right ventricular side of the septum, it may be particularly sensitive to acute distension of the right ventricular cavity. In this case the mechanism by which the patient developed complete heart block is not clear. We believe he had pre-existing conduction system disease involving his AV node, His-Purkinje system, right bundle branch and left anterior fascicle, although no previous electrocardiograms had been performed. It is possible that the embolism triggered a hyperadrenergic state resulting in stimulation of the Bezold-Jarish reflex leading to an increase in the efferent vagal response, decrease in sympathetic tone, and subsequent complete atrioventricular block in the setting of an already diseased conduction system. Another plausible, though less likely, scenario is that the massive embolism triggered transient myocardial ischemia to the AV node resulting in transient complete heart block.

CONCLUSION: Our case suggests that complete heart block can be produced by pulmonary embolism and subsequent treatment for the PE may result in restoration of a more stable rhythm.

DISCLOSURE: Rita Mukerji, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):6A. doi:10.1378/chest.11036
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INTRODUCTION: Atrial Septal Defect (ASD) is the commonest adult congenital heart defect (CHD). 15 % of these patients will eventually develop pulmonary hypertension (PHTN) if left untreated. ASD closure is not recommended when PHTN is irreversible. We present a patient with ASD and irreversible PHTN who was treated with Epoprostenol. She underwent percutaneous closure of her ASD with an altered closure device to allow right to left shunting with exercise.

CASE PRESENTATION: 35 yr female presented with 1 year of worsening dyspnea, presyncope with activity and inability to climb one flight of stairs. Her past medical history is notable for 2 pregnancies. During her second she experienced a pulmonary embolism and was treated with anticoagulation for 6 months. On examination BP 102/70, HR 90/min and O2 Sat 99%. Lung fields were clear and cardiac exam revealed prominent second heart sound with fixed splitting of both components. She had mild clubbing, no edema. A TTE performed showed a dilated RV and ostium secundum ASD. Her workup for other causes of pulmonary hypertension was negative. Cardiac catheterization demonstrated severe PHTN. She was started on IV Epoprostenol and was titrated to 33ng/kg/min. At 6 months her 6 minute walk, functional class and hemodynamics improved. 2 yrs later she remained on Epoprostenol at 42 ng/kg/min. Her functional status and hemodynamics continued to improve with titration. Due to the desaturation with exercise it was felt that she had a right to left shunt with exercise. As a result she underwent partial closure with a fenestrated 34 mm Amplatzer Septal Occluder. A covered stent was placed through the ASD device and ballooned to 9mm to maintain a pop-off for potential right to left shunting. She is currently on Epoprostenol with NYHA II functional status.

DISCUSSIONS: Atrial Septal Defects account for 18.8 % of CHD in the United States. It is more common in women. Most patients reach adulthood; however if it is not surgically corrected the mean age of death is 37.5 ± 4.5 years. Dyspnea is the most common presenting symptom. Alternative presentations include CNS manifestations from paradoxical emboli, supraventricular arrhythmias or recurrent pulmonary infections. Evaluation is performed utilizing TTE with contrast, a TEE or cardiac MRI to better estimate the location, defect size and shunt. Cardiac catheterization is performed to establish hemodynamic parameters and evaluate for reversibility. ASD closure is selected in patients whose Qp/Qs ratio is > 1.5. Patients with irreversible PHTN have higher mortality with closure compared to medically managed patients. Irreversible PHTN is suggested by a Qp/Qs ratio of less than 1, PVR > 15 and/or a Rp:Rs <0.33. Desaturation with exercise is suggestive of a right to left shunt, one that occurs from necessity due to high PVR impairing right sided cardiac output. The use of selective pulmonary vasodilators improve survival in patients with PAH and have shown to be beneficial in patients with shunt related PHTN. These agents are vasodilators and may over time remodel the pulmonary vasculature. In this case we were able to demonstrate improved hemodynamics with Epoprostenol over time followed by a partial closure of the ASD. The partial closure was facilitated by the addition of the stent within the device itself, effectively allowing a right to left shunt when needed.

CONCLUSION: Congenital Heart Disease should be considered in the evaluation of dyspnea in a young adult. The management of ASD with associated PHTN is difficult. It is pertinent that a detailed hemodynamic assessment be undertaken. The current vasodilator therapies may have role in making inoperable candidates operable. Manipulation of the device allowing right to left shunting contributed to the optimal outcome in this case.

DISCLOSURE: Jarrod Bruce, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):7A. doi:10.1378/chest.10366
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INTRODUCTION: Trans Urethral Resection Syndrome is an important post operative complication of TURP posing therapeutic challenges due to wide Variety of presentation.

CASE PRESENTATION: A 60-year-old male was treated by the urology service for prostatic hypertrophy manifesting itself as urinary incontinence, urgency, and dribbling. Despite medical therapy the patient remained symptomatic. He was, therefore, scheduled for transurethral resection of the prostate (TURP). Preoperatively, the patient’s vital signs were normal. He was alert and oriented. His serum electrolytes and biochemical profile were normal. The patient underwent spinal anesthesia in preparation for the surgery. Intraoperatively the patient’s urinary bladder was irrigated with a 1.5 % glycine in sterile water solution but no intravenous fluids were administered. Approximately 45 minutes into the procedure the patient developed hypotension, nausea with vomiting, and confusion.The procedure was quickly terminated and the patient was transferred to the intensive care unit (ICU). There was an estimated blood loss of 200 cc during the procedure. His past history was significant for hypertension, hyperlipidemia, well-controlled insulin-dependent diabetes mellitus and diverticulosis. Upon arrival to the ICU the patient’s blood pressure was 82/46 mmHg, pulse 105 beats per minute, respiratory rate 16 breaths per minute. The patient was confused and was not oriented to person and time. His lung and cardiovascular examinations were normal. There were no focal or lateralizing neurological findings present. His initial serum glucose was normal but his serum sodium was 111 mEq/L. Conservative treatments with administration of intravenous normal saline at a rate of 200 cc/hour and discontinuation of his outpatient medications was initiated. The patient responded well to this modality of therapy. His mental status improved as his serum sodium returned to normal. Other causes of hyponatremia and hypotension such as SIADH, adrenal insufficiency, myocardial ischemia and hemolysis were ruled out.

DISCUSSIONS: TURP is one of the most frequently performed procedures in United States with 400,000 cases performed each year. This procedure is relatively safe and typically well tolerated. One serious complication, however, is transurethral resection syndrome (TURS).TURS occurs in 1.5-2% of patients undergoing TURP. TURS should be considered when the patient undergoing TURP becomes hypotensive and confused. Low serum sodium, in the absence of other disorders that cause hyponatrenia, confirms the diagnosis. The etiology of the hyponatremia is probably multifactorial .While our patient had a relatively benign clinical course, some patients suffer severe and life threatening complications. These complications include: permanent brain injury, myocardial infarction, and blindness. While TURS may be known to the practicing urologist, but internists and Intensivists may not be as well acquainted with this disorder.

CONCLUSION: We report this case to alert critical care physicians to this complication of a common urologic procedure.

DISCLOSURE: Abhijit Raval, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):8A. doi:10.1378/chest.10771
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INTRODUCTION: Cardiac tamponade due to sarcoidosis is rare. To our knowledge, there are only eleven case reports of sarcoid associated tamponade. The patients were 27-59 years old, nine were women and eight presented 2-60 months after being diagnosed with sarcoidosis. Only three patients had tamponade as the first presentation of their disease (1,2). We present a case of a young healthy woman who came to us in tamponade and was found to have sarcoidosis.

CASE PRESENTATION: A 30 year old African-American woman presented to the ER with severe dyspnea and tachycardia. Her symptoms were insidious and progressive over three months. The CXR showed an enlarged heart. 2-D echocardiogram revealed a large circumferential pericardial effusion with right-sided diastolic collapse. An emergent pericardial drain removed 800 cc of straw colored fluid with significant clinical improvement. Post procedure EF was 30%. An endomyocardial biopsy showed non-caseating granulomas suggesting sarcoidosis, mycobacterial or fungal infection. TB and fungal infection was ruled out by a non-reactive ppd, negative serum IGRA , negative AFB and fungal stain and cultures from the pericardial fluid, myocardial biopsy and sputum, negative PCR for TB from the pericardial fluid, negative serum galactomannan and beta-D-Glucan. A CT scan showed multiple perilymphatic lung nodules and hepatic and splenic lesions compatible with sarcoidosis. Prednisone was started and slowly tapered over 8 months. The patient had complete normalization of her cardiomyopathy, pericardial effusion and spleen and liver lesions. She is asymptomatic with only mild obstructive lung disease.

DISCUSSIONS: Only 5 percent of sarcoidosis patients have symptomatic cardiac disease, but in autopsy series 30 percent show cardiac involvement. Sudden cardiac death causes 25-65% of cardiac sarcoidosis related mortality and can be the first presentation. First-degree heart blocks and bundle branch blocks are common and can progress to complete heart block. Ventricular premature beats and ventricular tachycardia are seen in 22% . Granulomatous infiltration of the myocardium can cause systolic or diastolic heart failure and is responsible for 25 -75 percent deaths. Perfusion defects can be seen. Valve involvement can cause regurgitation defects. SVT's, aortic aneurysms, pericarditis and pericardial effusions are rare. The optimal diagnostic strategy for cardiac sarcoidosis is unclear due to variable presentation as well as the low sensitivity and specificity of diagnostic tests. These include endomyocardial biopsy, CXR, EKG, Holter monitoring, exercise stress test, echocardiography and nuclear imaging. Echocardiography may show hyperechogenicity in tissue with extensive granulomas or wall motion abnormalities not in the normal distribution of the coronary vessels. Myocardial radionuclide imaging may show perfusion defects that actually decrease in size with excercise. Nuclear imaging can be used to follow up patients. Steroids are the mainstay of treatment and decrease 5 year mortality from 90% to 25%. When steroids are weaned, relapse is common and is treated by restarting from high doses of prednisone. Alternative agents such as methotrexate are used in patients unresponsive to steroids. ICD/ pacemakers should be placed if indicated. Cardiac transplant can be done in advanced cases with good results. Recurrent sarcoidosis can occur in the transplant.

CONCLUSION: To our knowledge, our case is the fourth case of cardiac tamponade as initial presentation of sarcoidosis to be reported in literature. Sarcoidosis should be considered in any young or healthy patient with cardiac symptoms. Although most patient with cardiac sarcoidosis will die from it, early initiation of treatment leads to 89% 5-year survival (1). Since there is no established method for screening for sarcoidosis, physician awareness is of utmost importance.

DISCLOSURE: Basel Altaqi, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):9A. doi:10.1378/chest.10490
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INTRODUCTION: We report a case of recurrent diffuse alveolar hemorrhage(DAH) due to IgA capillaritis in a renal tranplant patient on chronic immunosuppressants and review the available literature.

CASE PRESENTATION: A 44 year old male with history of IgA nephropathy(IgAN) status post renal transplant on chronic immunosuppressants presented with a two day history of cough, without hemoptysis, chest tightness and fever after recent discharge from the hospital. On physical examination, the patient was non-toxic appearing. His temperature was 101F, BP 170/100 mm Hg, pulse 76 beats/minute, respirations 40 breaths/minute, and oxygen saturation 98% on 50% FIO2. Initial lung examination was significant for bilateral crackles. Laboratory was remarkable for WBC of 6.6/mm3, hemoglobin of 8.0 gm/dL and creatinine 4.23 mg/dL. BNP level 4700. Chest radiograph showed bilateral patchy airspace disease. Patient was transferred to the intensive care unit(ICU) for tachypnea and hypoxia. Therapy for health care associated pneumonia and opportunistic infections was begun. A fiberoptic bronchoscopy(FOB) showed a bronchoalveolar lavage(BAL) with 10,900 RBC's and 49% macrophages. Patient improved with antibiotics, steroids for possible opportunistic infection, and diuresis and was discharged home. Patient was readmitted to the ICU for tachypnea, hypoxia, fever, and bilateral infiltrates. He was intubated and FOB revealed 40,000 RBC's and 68% macrophages in BAL. A focal organizing pneumonia with intra-alveolar foamy macrophages was the histological diagnosis from transbronchial biopsy. Therefore, open lung biopsy was performed and showed immune complex mediated acute capillaritis diffuse alveolar hemorrhage and hemosiderin was verified on iron stains. Immunofluorescence was positive with C3 and IgA. Pulsed methylprednisolone was initiated and patient was discharged on high dose prednisone with improvement. cyclophosphamide was not prescribed due to renal disease. After tapering prednisone, patient was readmitted to the ICU with shortness of breath, fever, cough, and hemoptysis. Patient was intubated and FOB with serial aliquots showed a progressively bloody BAL Pulsed methylprenisolone, plasmapheresis and rituximab were initiated and patient was discharged. After the second dose of rituximab, the patient presented to the hospital one week later with fever, cough and tachypnea. The chest radiograph showed worsening bilateral infiltrates. Broad spectrum antibiotics were started and prednisone was continued. BAL cultures were negative. The transbronchial biopsy showed intra-alveolar hemosiderin laden macrophages and fibrin, consistent with alveolor hemohorrage. The patient was started on high dose steroids, cyclophosphamide, and hemodialysis(HD) with rapid improvement. Unfortunately, the patient had presented to the ICU with DAH five times in a six month time period despite continued treatment.

DISCUSSIONS: Pulmonary capillaritis presenting as DAH is a rare manifestation in patients with IgAN.1 Review of the literature reveals only ten patients reported with this disease. The patients range in age from fourteen to sixty-six. The largest case series is of three patients, two with asymptomatic IgAN and one on HD with DAH. This is the first report of IgA capillaritis causing DAH in a renal transplant patient on immunosuppression. Lai et al hypothesized that the immune complex mediated pulmonary injury was related to the systemic nature of IgAN.2.

CONCLUSION: We present the first reported case of recurrent DAH due to IgA capillaritis in a renal transplant patient on chronic immunosuppressants. It can be refractory to treatment with high doses of corticosteroids, plasmapheresis, and rituximab. This case represents the difficulty in diagnosis due to a broad differential, recalcitrance to standard treatment, difficulties due to underlying comorbidities, as well as an extremely rare etiology of DAH.

DISCLOSURE: Marcia Henderson, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):10A. doi:10.1378/chest.10858
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INTRODUCTION: Humidification of inspiratory gases is essential during mechanical ventilation. Heat and moisture exchangers are commonly used to aide humidification but malfunctions can occur. We report a case of ventilator circuit obstruction by a heat and moisture exchanger with a review of prior literature.

CASE PRESENTATION: A 24 year-old woman with a past medical history of asthma was admitted to the ICU in status asthmaticus. She was intubated in the emergency department. A heat and moisture exchanger (HME) was inline in the breathing circuit. Continuous nebulizer treatments were begun with albuterol 0.083% and ipratropium 0.02%. Her ventilator was set to assist volume control with a set rate of 16, tidal volume (TV) 400 mL with positive end-expiratory pressure 5 cm Water with FiO2 0.40. Her initial peak inspiratory pressures (PIP) varied between 25 and 40 cm Water. At 24 hours, the TVs were noted to drop to 150-200 mL with inspiratory pressure alarm limits reached reducing the maximal ventilator delivered TV. No leak was found in the ventilator circuit. Decreased bilateral breath sounds were noted on physical exam, vitals were stable and trachea was mid-line. The suction catheter passed easily, the patient was not biting the endotracheal tube, and there were no secretions in the patient circuit nor in the HME. The patient was disconnected and was easily ventilated with the bag-valve mask. It was noted that the ventilator was still sensing high-pressure. The HME was removed from the circuit and the ventilator immediately reported low pressures. The patient was reconnected to the ventilator and TVs of 400ml were restored. We believe continuous administration of albuterol and ipratropium in saturation of the HME leading to resistance to airflow. Our patient was extubated by the 6th hospital day and left the hospital on the 9th day. She remained well five months after admission.

DISCUSSIONS: Heat and moisture exchangers (HMEs) are passive devices that provide 60-80% of the necessary humidity by capturing exhaled moisture and allowing it to be used in subsequent breaths. HMEs are simple, cheap and require little maintenance. The central component of HMEs is hygroscopic material which readily absorbs moisture. When an HME possesses bacteria filtering properties it can be designated as a HMEF. Various critical occlusions of HMEs have been reported, primarily in the anesthesiology literature. Several of the reports describe defects or loose components in the HME itself. Prados reported a case of obstruction by a plastic plug in a defective HME that acted as one way ball valve that caused air trapping. Prasad reported a partial separation between two interconnecting parts of the plastic housing resulting in a significant leak. Casta reported acute airway obstruction due to a dislodged paper component of an HME. Several reports describe obstruction of the HMEF by contaminants causing pore occlusion of the filter membrane. Contaminants described include condensed water, hemoptysis, and proteinaceous fluid associated with pulmonary edema. McEwan and associates described a case of obstruction of a bacterial filter in the expiratory limb of a breathing circuit that resulted in bilateral tension pneumothorax. The filter was clogged with sputum, ciliary epithelium, and inflammatory cells. Several cases describe obstruction with nebulised medications: albuterol, ipratropium and acetylcysteine. Walton and associates described two cases of obstruction using nebulized albuterol with a breathing circuit where both filters were coated with a powdery white film. Nebulization of normal saline, the diluent for albuterol, did not reproduce this obstructive coating.

CONCLUSION: Obstruction of the heat and moisture exchanger should be considered in the differential diagnosis of airway obstruction and insufficient tidal volumes. Heat and moisture exchangers should be removed prior to the administration of aerosol or metered dose inhaler treatments.

DISCLOSURE: Jeffrey Williams, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):11A. doi:10.1378/chest.10555
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INTRODUCTION: Acute respiratory failure in an immunocompromised patient carries a wide differential. We present an unusual cause of acute respiratory failure in a patient with HIV.

CASE PRESENTATION: 48 Y male with h/o HIV since 1988 presented to the emergency department with sudden onset of shortness of breath and cough of few weeks. His past medical history was significant for renal transplant, atrial fibrillation and hypertension. Home medications included antiretrovirals, low dose prednisone, mycophenolate and tacrolimus. He was noted to be tachycardic, tachypneic and hypoxic (HR 160/ min, BP 138/104 mm, respiratory rate of 50/min, oxygen saturation of 75% on room air) and was intubated for acute respiratory failure. His physical exam was significant for coarse crackles, irregular heart rhythm and lower extremity pedal edema. Significant laboratory values included a hemoglobin of 10.7g/dL , white count of 15.9, CD4 count of 662 and HIV RNA copies <48 copies/ml. Urine toxicology screen was negative for recreational drugs. CXR showed new diffuse bilateral hazy opacities. CT chest confirmed bilateral ground glass opacities. He was pan cultured and started empirically on Vancomycin and cefepime. Bronchoalveolar lavage grew CMV. Occasional macrophages with crystalline structures were also noted. Thoracoscopic lung biopsy was performed showed intense exudation within alveoli of macrophages laden with birefrengent needle-shaped crystalline structures, consistent with talc material. There was no evidence of vasculitis, granulomas or viral cytopathic changes. Stains and cultures for AFB and fungus were negative. Although BAL grew CMV, serological viral loads were low and there was no evidence of cytomegalic inclusion bodies in the surgical lung biopsy. CMV pneumonitis was therefore not felt to be the cause of his respiratory failure, and antivirals were not started. He improved over the course of several days with supportive management, and was successfully extubated. He subsequently reported a history of avid use of talc powder. He also reported a recent change in daily routine of directly inhaling talc powder via an aerosolizing device to help him relax before sleeping.

DISCUSSIONS: Talc is a mineral composed of magnesium silicates. Pulmonary involvement has been described in the form of talcosilicosis, talcoasbestosis, talcosis caused by inhalation of talc, and inadvertent intravenous administration (1).Radiological features described include diffuse ground glass appearance, reticulonodular and fibrotic changes. Late complications include cor pulmonale and pulmonary arterial hypertension. Most frequent pathologic change is diffuse fibrosis containing macrophages and multinucleate giant cells with absorbed particles. These appear as birefringent particles when viewed with polarized light. In talcosis secondary to IV drug use, there is a predominant perivascular deposition of talc. In inhalational talcosis, talc deposits predominantly in and around terminal and respiratory bronchioles. Treatment is largely supportive, with a few case reports suggesting benefit from steroids.

CONCLUSION: Pulmonary talcosis has been reported as a result of chronic occupational exposure, recreational IV drug abuse and iatrogenic exposure secondary to pleurodesis. Acute respiratory failure due to talc has been described in patients after talc pleurodesis and IV drug abuse (2). Our HIV patient developed acute respiratory failure as a result of recreational inhalation of talc powder, which makes this case fairly unique in literature. It is important to consider pulmonary talcosis in the differential diagnosis of acute diffuse pneumonitis in a patient with relevant exposures.

DISCLOSURE: Awungjia Leke-Tambo, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):12A. doi:10.1378/chest.10841
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INTRODUCTION: Pseudoaneurysm of the cystic artery is a rare complication following laparoscopic cholecystectomy. We report a case of pseudoaneurysm of the cystic artery and hemobilia as a late complication of this operation.

CASE PRESENTATION: A 33-year-old African American male presented to the emergency room with nausea and vomiting blood clots that started the same afternoon. He had a medical history of sickle cell trait, rheumatoid arthritis, choledocholithiasis and had an ERCP and laparoscopic cholecystectomy 2 months prior to admission. His hemoglobin was 5.9g/dl on arrival. He had significant right upper quadrant abdominal pain. He was admitted to the medical ICU and the gastroenterology service was consulted. An initial EGD did not reveal any source of bleed. A few hours after the EGD he vomited more than 1 liter bright red blood. He was intubated for airway protection and placed on mechanical ventilation. An emergent EGD again, did not reveal the source of bleed in the stomach. The duodenal was successfully intubated and careful inspection of the duodenum did not show any obvious lesions in both, the first and second portions of the duodenum. The major papilla was visualised and there was no overlying clot on it. Bile was seen draining draining through the major papilla. Emergent superior mesenteric, inferior mesenteric and celiac axis arteriograms were performed by interventional radiology that showed gross extravasation at the region of the stump of the cystic artery. This was successfully embolised and a subsequent angiogram showed no more extravasation. During his admission stay, he required 10 units of packed red cells. After embolisation, there no more episodes of bleeding and he was discharged home.

DISCUSSIONS: Cystic artery pseudoaneurysm which developed following a cholecystectomy and resulting in upper gastrointestinal bleeding is a rare entity, with only four cases described in the literature [1]. The symptoms may appear in the early postoperative period or as late as 120 days after surgery. Among the possible causes are the excessive use of electrocautery during the dissection at the infundibulum of the gallbladder, causing thermal injury to the vascular wall, and erosion of the inner wall of the cystic artery caused by contact with the tip of the metal clip used to occlude the cystic duct [2]. The classical triad of upper gastrointestinal bleeding, pain in the right upper quadrant and obstructive jaundice described by Quincke is present in about a third of the patients. Even though our patient demonstrated gross extravastion of contrast, the presence of a dilated cystic artery stump on angiogram following cholecystectomy is an “ominous sign”, even in the absence of active extravasation of contrast.

CONCLUSION: GI bleed constitutes a significant proportion of MICU admissions. Intensivists should be aware that cystic artery bleeds can present as a massive GI bleed. It should definitely be considered in the differential diagnosis of upper GI bleed in a patient with previous cholecystectomy, more so if EGD fails to locate the bleeding source. Emergent angiography and embolization can life-saving.

DISCLOSURE: Somnath Ghosh, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):13A. doi:10.1378/chest.10723
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INTRODUCTION: Percutaneous vertebroplasty and Kyphoplasty are procedures done for compression fractures. Polymethylmethacrylate cement mixed with opacifying agent is injected under radiological guidance as a liquid and it quickly hardens and stabilizes the collapsed vertebra. Some degree of local leakage of cement during the procedure is very common. We report a case of a cement pulmonary embolism following kyphoplasty procedure.

CASE PRESENTATION: 62-yr-old female with L2 compression fracture was admitted for Kyphoplasty.Polymethylmethacryate was injected under fluoroscopic guidance. Extravasation of the cement was noted. CT scan of Lumbar Spine was performed which confirmed extravasation of cement reaching border of Inferior vena cava.Subsequent CT of the chest showed cement in right upper and middle lobe pulmonary arteries.Physical exam was unremarkable.Patient remained hemodynamically stable. She had no complains of chest pain, shortness of breath, hemoptysis etc. and ABG showed no hypoxia.Patient was subsequently started on therapeutic enoxaparin. She was discharged home later without anticoagulation.

DISCUSSIONS: Complications due to kyphoplasty and vertebroplasty are low and include infection, hypotension and leakage of cement outside the vertebral body. Occasionally, polymethylmethacrylate extravasates outside the vertebral body and extends into epidural and paraspinal veins, extending into inferior vena cava and may result in pulmonary embolism. Factors determining risk of pulmonary embolism are insufficient polymerization of cement, needle position with respect to vertebral vein and overfill of vertebral body. Most leaks are asymptomatic. In patients with clinical evidence of pulmonary embolism, management mainly consists of anticoagulation because there is risk of thrombus formation on the cement embolus.

CONCLUSION: Physicians should be aware of possibility of cement pulmonary embolism. It could potentially be fatal. Few cases have been reported.It is recommended that this procedure should be done under fluoroscopic/ CT guidance. As soon as leakage into perivertebral veins is seen, procedure should be discontinued.

DISCLOSURE: Aleksander Shalshin, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: lung , embolism , kyphoplasty
Chest. 2010;138(4_MeetingAbstracts):14A. doi:10.1378/chest.9966
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INTRODUCTION: Several studies have attributed the occurrence of acute myopathy in intensive care patients to the combination of corticosteroids (CS) and neuromuscular junction blocking agents (NMBA) or other sedatives. We present a case of spontaniously breathing patient who developed acute respiratory failure secondary to administration of systemic CS .

CASE PRESENTATION: A 43 year old female patient with chronic obstructive pulmonary disease (COPD) presented with a two weeks history of worsening dyspnea. Physical exam was notable for decreased breath sounds, wheezing and prolonged expiratory phase. Room air arterial blood gas was consistent with a mild respiratory acidosis (PH 7.36, PCO2 46, PO2 58, HCO3 25) and chest roentogram revealed hyperinflation but no focal consolidation. She was initiated on aerolized bronchodilator therapy and placed on 60 mg of intravenous Methylprednisolone every 6 hours for exacerbation of her obstructive airway disease. Initial improvement was noticeable and she was subsequently transferred to a rehabilitation unit while being maintained on CS therapy. Clinical course during rehabilitation was protracted and complicated by progressive peripheral muscle and motor weakness and deteriorating gas exchange on supplemental O2 (PH 7.27, PCO2 103, PO2 99, HCO3 45). Physical examination was not suggestive of presence of acute bronchospasm or airway obstruction and chest radiograph showed no acute disease; nevertheless she progressed to severe acute hypercapnic respiratory failure requiring endotracheal intubation and invasive mechanical ventilation. Neuromuscular disease, paraneoplastic syndrome and CS myopathy were considered as potential causes for her illness. Creatine phosphokinase and Aldolase were increased whereas total body imaging with computed tomography failed to reveal an occult mass or neoplasm. Electromyography indicated normal motor and sensory nerve conduction velocities but reduced motor nerve response amplitude. A Left deltoid muscle biopsy revealed type IIb fibers atrophy with sparing of the type I and IIa consisting with a diagnosis if acute CS induced myopathy. Consequent discontinuation of systemic CS was associated with gradual and rapid improvement in neuromuscular function, decline in myopathic enzymes and liberation from mechanical ventilation. A short period of physical rehabilitation followed prior to discharge home.

DISCUSSIONS: Most illustrated cases in the intensive care unit of CS induced myopathy are difficult to wean mechanically ventilated patients who were receiving CS along with NMBA.1 Concurrent administration of CS with the anesthetic Propofol was also more recently linked to a similar pathology in a small case series. Two distinct types of CS myopathy exist, acute and chronic.2 The chronic form occurs after prolonged use of corticosteroids and has a more insidious course. The acute form is less common, is associated with muscle lysis and evolves rapidly while the patient is receiving moderate to high-dose CS such as occurred in our patient. To our knowledge, acute respiratory failure due to steroid myopathy has not been previously described. A unique aspect presented by this case is that our patient presented with a mild COPD exacerbation and did not require initially neither invasive nor non invasive mechanical ventilation; however after administration of CS, she developed acute myopathy that resulted in respiratory failure and need for mechanical ventilation.

CONCLUSION: Corticosteroids are frequently administered in patients with exacerbation of COPD but treatment can be associated with deleterious effects. Acute steroid-induced myopathy should be suspected in patients receiving CS as early recognition and discontinuation of the offending agent are rapidly associated with return of muscle strength and resolution of the clinical syndrome.

DISCLOSURE: Mazen Zouwayhed, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):15A. doi:10.1378/chest.10725
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INTRODUCTION: We report the case of a patient with hypereosinophilic syndrome (HES) who experienced initial clinical improvement on mepolizumbab. Development of recurrent pneumonias and squamous cell carcinomas of the skin while receiving mepolizumab led to discontinuation of the drug.

CASE PRESENTATION: A 46 year old man with a history of asthma presented with worsening dyspnea and wheezing in October 2004. Medical history included atopic dermatitis and eosinophilic gastroenteritis. A complete blood count demonstrated 4500 eosinophils. Chest CT scan showed no significant infiltrates or bronchiectasis. He was initiated on systemic steroids. ANCA studies were negative and a bone marrow biopsy showed no malignancy. Attempts to decrease prednisone led to worsening asthma and increasing eosinophilia. In September 2005, he was enrolled in a trial of mepolizumab for treatment of HES. After receiving three monthly doses, eosinophil count decreased to zero and asthma symptoms improved. Prednisone was discontinued. By 2007, eosinophil counts rose to 3000 and he suffered multiple asthma exacerbations requiring prednisone. In 2008, he resumed mepolizumab, administered for compassionate use. Asthma symptoms improved, eosinophil counts decreased to < 100 and prednisone was discontinued. In December 2008, he developed left lower lobe pneumonia. Two months later, he developed right upper lobe pneumonia. Sputum grew Haemophilus influenza. The following month, he again developed fever, cough and pneumonia with growth of Streptococcus pneumoniae. He had 3 additional episodes of pneumonia in 2009, all which resolved with antibiotics. Subsequently, multiple sputum cultures grew mycobacterium avium. Throughout 2009, he also developed eight squamous cell carcinomas of the skin. Because of concerns of recurrent infection and skin cancer, mepolizumab was discontinued.

DISCUSSIONS: Hypereosinophilic syndrome (HES) is a rare group of disorders characterized by overproduction of eosinophils, end-organ eosinophilic infiltration and injury with no identifiable cause. Forty percent of patients with HES develop pulmonary disease. The primary goal of treatment of HES is to reduce eosinophil-mediated end-organ damage. For most patients, high dose corticosteroids remain first line therapy. Second line agents include interferon-alpha, hydroxyurea and vincristine. IL-5 is the major cytokine responsible for eosinophil maturation and function. Mepolizumab, an anti-IL-5 antibody, is currently only available in clinical trials or for compassionate use. In a recent study, patients with steroid-controlled HES were randomized to receive placebo or mepolizumab. Compared to placebo, significantly more patients receiving mepolizumab achieved a reduction in prednisone dose to < 10mg/day, with almost 50% becoming corticosteroid-free. In another study, mepolizumbab reduced exacerbations and eosinophil levels in patients with refractory eosinophilic asthma. Questions that remain regarding the use of mepolizumbab include long-term safety, patient selection and duration of effect after discontinuation. An open-label extension study is ongoing to address these issues. In the original study, there were no differences in the rates of pneumonia between treatment and placebo groups. With regard to malignancy, one patient in the extension study developed angioimmunoblastic lymphoma which was thought to be related to mepolizumab. The patient described in this case discontinued mepolizumab because of the temporal correlation between starting the drug and the occurrence of frequent pneumonias and skin squamous cell carcinomas. Whether these events were related to treatment is unknown. Long-term follow-up from ongoing trials and reporting by physicians caring for patients on mepolizumab may help to answer these questions.

CONCLUSION: Mepolizumab has shown promising results in clinical trials in the treatment of HES. Compassionate use should be considered in patients who are refractory to standard treatment. However, further follow-up studies are required to determine its long-term safety.

DISCLOSURE: Cyrus Shariat, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):16A. doi:10.1378/chest.10932
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INTRODUCTION: Diagnosing neurosarcoidosis can be challenging without a CNS biopsy. However, treatment with immunosuppressive agents maybe be given in the right clinical context. Some of these agents may lead to viral reactivation ,and ultimately to lymphoproliferative disease.

CASE PRESENTATION: We present the case of a 45 year old Caucasian woman, diagnosed with pulmonary sarcoidosis in 2005 by mediastinal lymph node biopsy, who presented with new onset generalized tonic-clonic seizure. Following her initial diagnosis of sarcoidosis, she was treated with prednisone. In the year preceding her seizure, she was tapered off prednisone because of marked weight gain and treated with methotrexate 20 mg per week for persistent pulmonary symptoms. Computed tomography scans of the chest during the year prior to admission,while on methotrexate,showed worsening parenchymal disease. As part of her seizure evaluation, an MRI with contrast of the brain was performed and revealed three enhancing brain lesions .She was started on prednisone and Azathioprine for presumed neuro-sarcoidosis, Levetiracetam for seizure prophylaxis and trimethoprim-sulfamethoxazole for pneumocystis prophylaxis. Despite treatment, one month later she presented to the hospital again with a second episode of generalized tonic clonic seizure. She also experienced three instances of auras in her right visual field which had been treated with increased doses of Levetiracetam.She had no other medical problems and the rest of her history was unremarkable. Physical exam revealed a healthy middle aged woman with normal vital signs. Her physical examination, including eye and pulmonary exam was unremarkable.Routine laboratory studies were unremarkable. Repeat brain MRI showed an increase in the size of all 3 lesions.She ultimately underwent a brain biopsy of the left occipital mass.This showed hypercellular and dense lymphoid tissue infiltration ;comprised of small lymphocytes, admixed larger atypical lymphoid cells and few plasma cells; completely replacing normal brain parenchyma, consistent with Immunodeficiency associated(Epstein Barr virus associated) diffuse large B-cell lymphoma ,most likely secondary to Methotrexate therapy. PCR assay for heavy chain rearrangement was negative. EBER in situ for Epstein Barr virus was positive.She was rapidly tapered off the prednisone and Azathioprine and begun on Rituximab.

DISCUSSIONS: To our knowledge this is the first case of CNS EBV associated lymphoproliferative disorder developing while on Methotrexate therapy for sarcoidosis. One other EBV associated lymphoproliferative disorder reported in a sarcoidosis patient involved the lower gastrointestinal tract.Clinically apparent nervous system involvement occurs in about 5-15% of sarcoidosis patients. Neurosarcoidosis can occur in isolation without other organ systems involved. It can present acutely or as an insidious chronic illness. The diagnosis of neurosarcoidosis is definite when the clinical presentation is compatible with sarcoidosis and histopathology findings support direct nervous system involvement. Probable neurosarcoidosis can be diagnosed when the clinical and radiographic features are consistent with neurosarcoidosis and evidence exists of non caseating granulomas in another affected organ. We initially made a diagnosis of probable neurosarcoidosis given the previous histologic confirmation of sarcoidosis in the thorax, worsening lung disease and compatible radiographic findings. However, when disease progressed other causes were investigated . EBV associated lymphoproliferative disease has been reported in association with treatment of rheumatoid arthritis, dermatomyositis, polymyositis and psoriasis with low dose and high dose methotrexate. MTX induces reactivation of EBV from latent infection in host cells and leads to the release of infectious virions. Patients with rheumatoid arthritis or polymyositis who were treated with an MTX-containing regimen had statistically significantly higher EBV loads than patients on immunosuppressive regimens that did not include MTX. MTX also impairs the immune response to EBV.

CONCLUSION: Our case report should heighten awareness that EBV associated lymphoproliferative disease may occur during methotrexate treatment for sarcoidosis.

DISCLOSURE: Krithika Ramachandran, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):17A. doi:10.1378/chest.10897
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INTRODUCTION: Inhalational injury is a known cause of primary bronchiolitis and a number of substances including chlorine, sulfur dioxide, and phosgene have been identified as inciting agents. A small cohort of US Army soldiers were diagnosed with biopsy proven bronchiolitis after service in Iraq. All had presented with duty-limiting dyspnea and many were exposed to the sulfur dioxide fires at the Mishraq Sulfur Mine near Mosul in June 2003.(1) A portion of this same group of soldiers reported no inhalational exposures despite similar symptoms. Thus, there appears to be other unknown inhalants producing similar pathophysiological manifestations as the sulfur. We report a case of an Army soldier who has developed limiting bronchiolitis after serving in Iraq during 2005 and 2009 despite the absence of exposure to sulfur dioxide fires.

CASE PRESENTATION: A 56-year-old non smoking male presented to his primary care physician with a complaint of dyspnea on exertion of one year's duration. He carries a history of obstructive sleep apnea with nightly non-invasive ventilation (NIV), post-traumatic stress disorder, peripheral neuropathy and post-vaccine encephalitis. He served as a surgeon for 1 month in Balad (2005) as well as 3 months in Tikrit (2009). For his dyspnea, he underwent an initial cardiac evaluation which was normal. His subsequent pulmonary evaluation was notable for an adequate 6 minute walk test, and mild gas exchange abnormality on his pulmonary function tests. A computed tomography (CT) scan demonstrated subpleural, posteriorly located right lower lobe airspace disease. Subsequent VATS lung biopsy revealed mild, chronic inflammation of the small airways with bronchiolectasia and mild peribronchial fibrosis. Physical examination was notable only for a body mass index of 40, with no physical manifestations of hypoxemia. Repeat pulmonary function testing demonstrated a mild restrictive ventilatory defect with a mild gas exchange abnormality. Cardiopulmonary exercise testing revealed normal functional capabilities with a mildly reduced VO2max. The patient was instructed to lose weight and required no systemic therapy.

DISCUSSIONS: Morbid obesity has been previously reported to be associated with reductions in expiratory reserve volumes as well as DLCO. It can also account for our patient’s suboptimal cardiopulmonary exercise test. However, the documented bronchiolitis and CT findings would be consistent with airway injury not explained by his obesity. Inhalation of a variety of toxic substances has the potential to cause bronchiolitis and an injury pattern similar to the one exhibited here. Furthermore, a similar pattern of findings were exhibited by the soldiers deployed from Fort Campbell to Iraq, specifically those without sulfur dioxide exposure. It is not clear what particular agent may be responsible for these findings, but given the variety of exposures associated with the intense combat environment within Iraq, including its desert location, burn pits, burning human waste, weapon operation and diesel exhaust, any number of possibilities could be implicated.(2) Given the large number of troops deployed during the Iraq War, the potential exists for a growing patient population with a similar presentation and duty-limiting complications.

CONCLUSION: Bronchiolitis should be on the differential diagnosis for soldiers serving in Iraq presenting with similar symptoms and imaging. Further research and insight will standardize an approach to diagnosis, treatment and potentially preventative measures.

DISCLOSURE: William Bender, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: bronchiolitis , iraq
Chest. 2010;138(4_MeetingAbstracts):18A. doi:10.1378/chest.10067
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INTRODUCTION: Primary pulmonary lymphomas (PPL) are rare and are seldom considered in the initial differential diagnosis when a patient presents with a clinical picture of "non-resolving pneumonia". Even so, the majority of PPL are B-cell derived neoplasms. We report an unusual case of non-HIV related PPL of natural killer/T-cell origin presenting as non-resolving pneumonia.

CASE PRESENTATION: A 31 year old nonsmoking Egyptian male without significant past medical history presented to the hospital with shortness of breath, cough and low-grade fever of several weeks duration. He was diagnosed with community acquired pneumonia based on chest x-ray findings of bilateral alveolar infiltrates. Despite treatment with antibiotics, he clinically declined, and developed acute respiratory failure requiring mechanical ventilation. Systemic corticosteroids were added and antibiotics were broadened which improved his clinical status. He was then successfully extubated and discharged from the hospital after a few weeks. HIV test done during the hospitalization was negative. Over the next few weeks, he had recurrence of symptoms upon corticosteroid taper and was re-admitted to the hospital for progressive respiratory insufficiency. A chest CT scan revealed bilateral diffuse patchy lung parenchymal consolidation with air bronchograms without significant mediastinal or hilar lymphadenopathy. He failed to improve on empiric regimen of systemic corticosteroids and broad spectrum antibiotics. A fiber-optic bronchoscopy was non-diagnostic, following which a video-assisted thoracoscopic lung biopsy was performed. The surgical lung specimen showed atypical lymphocytic interstitial infiltrates. The immunohistochemical stains confirmed the diagnosis of NK/T-cell lymphoma. A total body PET imaging study showed abnormal uptake only in the lung fields with no hypermetabolic lymphadenopathy elsewhere in the body. A bone marrow biopsy was negative for lymphoma. The patient was treated with chemotherapy for a few months which resulted in radiographic resolution of lung infiltrates and successfully underwent an allogeneic hematopoietic stem cell transplant. Unfortunately, this was complicated by gastrointestinal graft-versus-host disease, cytomegalovirus (CMV) pneumonitis, and refractory thrombocytopenia. He expired from an intracranial hemorrhage approximately one year after initial presentation.

DISCUSSIONS: PPL are rare and represent only 0.5-1% of all primary lung malignancies and less than 1% of all cases of Non-Hodgkin's lymphoma. The diagnostic criteria of PPL usually include: 1) involvement of the lung without evidence of mediastinal adenopathy 2) no previous diagnosis of extrathoracic lymphoma 3) no evidence of extrathoracic lymphoma or lymphatic leukemia at the time of diagnosis of PPL or for three months thereafter. PPL are typically of B-cell linage and thought to arise from the bronchial mucosal associated lymphoid tissue (BALT). Very few cases of PPL of T-cell origin have been reported in the literature with NK/T-cell lymphoma being rarer still. Such cases often exhibit aggressive metastatic potential that may lead to a rapid clinical decline. Our patient presented clinically as "non-resolving pneumonia" with radiographs illustrating diffuse lung parenchymal consolidation. The absence of lymphadenopathy and negative bone marrow specimens support a primary pulmonary origin in this case. Following chemotherapy, this patient's clinical status improved and the pulmonary infiltrates resolved; unfortunately he expired from complications of hematopoietic stem cell transplantation.

CONCLUSION: The differential diagnosis of lung parenchymal consolidation presenting as "non-resolving" pneumonia is vast. However, in reporting this case, we bring awareness that NK/T-cell lymphoma may present as a primary pulmonary tumor. Early suspicion, biopsy and appropriate chemotherapy regimens are necessary for this otherwise aggressive lymphoma.

DISCLOSURE: Brian Davis, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):19A. doi:10.1378/chest.10441
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INTRODUCTION: Lung tumors with the histological appearance of meningioma are very rare. We describe a case of a woman who presented with right lower lobe mass which was diagnosed as meningioma.

CASE PRESENTATION: A 67 year old woman presented to outpatient clinic for right lower lobe mass lesion. The patient went to emergency room with intermittent episodes of right sided chest pain lasting for two to three days prior to the clinic visit. She described pain as dull in nature and mainly located over lower right side of her chest wall. Her chest X-ray at that time reveled the presence of right lower lobe opacity During the clinic visit she denied fever, chiils, further episodes of chest pain, dyspnea at rest or upon exertion, cough, PND, orthopnea and trauma to chest. She had a history of meningioma followed by complete resection five years ago. She denied prior history of cigarette smoking, alcohol or drug abuse and recent travel. On physical examination, the patient appeared comfortable. Her vital signs were: T 37.3, HR 84, RR 18, BP 134/82 and SpO2 96% on room air. Chest examination was unremarkable. Cardiac examination showed a soft S1 and a blowing holosystolic murmur best heard at the apex with radiation into the axilla. Rest of the examination was within normal limits. Initial laboratory parameters were witnin normal limits. Chest X-ray showed a mass lesion in the anterior segment of right lower lobe. Chest CT with IV contrast revealed a non calcified and round 4.4 x 3.2 cm mass with smooth margins in the superior segment of right lower lobe. The PET/CT scan was suggestive of FDG positive mass lesion corresponding to CT chest images with a SUV of 6.9. The patient underwent right lower lobectomy and mediastional lymph node sampling. Light microscopy showed sharply demarcated nests of tumor cells located in the interstitial space. They were often located around small veins but an association with bronchioles or arteries could not be shown. The cells were arranged in whorls, bands, and small clusters. The findings were suggestive of a meningioma.

DISCUSSIONS: Pulmonary meningioma (PM) is an extremely rare tumor. Since the first case was described by Erlandson in 1981, about 30 cases have been reported. Only two of these cases showed malignant characteristics. As is the case in central nervous system meningiomas, PM may grow very slowly without being responsible for any clinical symptoms. Thus, PM presents most often as an asymptomatic solitary pulmonary nodule or mass lesion. Only two cases of persisting cough that motivated specific investigations have been reported. The PM can be primary or metastatic. As in our case and others previously described, it may be difficult to differentiate a primary PM from isolated metastasis by clinical and radiographic criteria; this is especially true in patients with a known primary intracranial tumor. Moreover, it has recently been reported that PM can be responsible for false-positive PET scan with an increased FDG uptake. The treatment of PM is to perform a complete resection, which has been possible in all reported cases. The previous cases also suggest that there is no risk of recurrence if the resection is complete.

CONCLUSION: We describe a rare case of pulmonary meningioma. The differentiation between primary versus secondary lung meningioma represents a challenge for the clinician.

DISCLOSURE: Chirag Pandya, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: right lung
Chest. 2010;138(4_MeetingAbstracts):20A. doi:10.1378/chest.10866
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INTRODUCTION: Bronchioloalveolar cell carcinoma (BAC) presents in a variety of different radiological patterns. We report an unusual radiological presentation of BAC with multifocal bilateral cavitating lesions.

CASE PRESENTATION: A 55 year old Caucasian male with 15 pack-year history of smoking and otherwise no significant past medical history, was admitted for treatment of pneumonia. He was symptomatic for productive cough, shortness of breath and chest radiograph showed a right lower lobe infiltrate. A chest computed tomography (CT) showed mixed areas of gas and fluid throughout an opacified right lower lobe, and a loculated right pleural effusion. Bronchoscopy with lavage was negative for bacterial, fungal, and mycobacterial organisms. Due to lack of clinical or radiological improvement, the patient underwent right lower lobectomy with decortication and was found to have organizing pneumonia with fibrosis, and foci of mucinous type BAC. Surveillance chest CT scans over the next 2 years revealed progressive bilateral cavitating nodules. Repeat bronchoscopy was negative. Positron emission tomography (PET)-CT scan did not show significant uptake in the lesions. The patient underwent a thoracoscopic wedge resection of the left upper lobe cavitatory lesion. There were multiple microscopic nodules of BAC. Epidermal growth factor receptor (EGFR) status was negative and patient was evaluated for initiation of cytotoxic chemotherapy.

DISCUSSIONS: Bronchioloalveolar cell carcinomas account for about 4% of all non small cell lung cancers. They exhibit a lepidic growth pattern (growth along intact alveolar septae), aerogenous and lymphatic spread, but without vascular invasion. BAC differs from other lung cancers as there is a female preponderance (53%), and a significant number of lifelong nonsmokers(37%) are affected. Clinical symptoms include cough, shortness of breath, and hemoptysis. Bronchorrhea (>100ml/24hrs) and intrapulmonary shunting due to replacement of alveoli with tumor can be life threatening. Often, patients are asymptomatic with incidentally detected radiological abnormalities (1)Radiologically, three patterns are reported: (a) solitary pulmonary nodules or mass, (b) consolidative changes resembling pneumonia, or (c) multifocal nodules or masses. The solitary nodule is the most frequent radiographic presentation(56%) and can be a ground glass opacity or more solid with ill defined margins. Intratumoral radiolucencies in the form of air bronchograms, pseudocavitation, heterogenous attenuation and pleural tags are often present. Diffuse consolidative changes are present in 30% of the patients making it difficult to differentiate from bacterial pneumonia. Multifocal disease is seen in the form of groundglass opacities, solid nodules or masses and occurs frequently with the mucinous subtype. Cavitatory forms of BAC are exceedingly rare. The CT angiogram sign described in BAC as visible vessels with contrast through an area of tumor, can be seen in benign consolidative conditions.(2) Surgical resection is the therapy of choice in early stage tumor. In patients with EGFR mutations and advanced disease, chemotherapy with EGFR inhibitors has shown improved outcomes. Radiation therapy and outcomes are similar to other non small cell lung cancers.The prognosis of patients with surgically resected solitary nodules is good with five year survivals in the 100% range with low rates of recurrence. The multifocal and consolidative forms have a poorer prognosis.

CONCLUSION: In the absence of infectious or inflammatory etiologies, BAC and other lung cancers should be considered in the differential diagnosis of cavitatory lung disease.

DISCLOSURE: Charbel Maskiny, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):21A. doi:10.1378/chest.10721
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INTRODUCTION: Oct3/4 (POU5F1), a transcription factor essential for the maintenance of totipotency in embryonic stem cells, is expressed in germ cell tumors and has been labeled as the marker for primordial germ cells. Its interaction with the caudal-related homeobox gene (Cdx2) is responsible for the differentiation and maintenance of trophectoderm at the initial stages of development. Oct3/4 along with a transcriptional cofactor expressed in embryonic stem cells (Sox-2,Ch 3) are known to form a regulatory complex needed for the pluripotency of primitive embryonic stem cells. Extragonadal germ cell tumors are rare and it is even rarer to have the primary tumor located in the lung. We report a case of an Oct3/4 positive, Cdx2 negative germ cell tumor in the lung with no evidence of gonadal lesion. We also report the findings from in-situ hybridization studies that were done to analyze the chromosomal pattern of the tumor cells. We believe that this is the first report of such unique findings.

CASE PRESENTATION: A 48-year-old woman, chronic smoker, was admitted to our hospital with a dry cough and occasional hemoptysis for 3 months. On admission, the CT scan chest demonstrated three large soft tissue masses, the largest one being 12 x 10 cm. CT scan of the abdomen and pelvis was negative for involvement of any abdominal or pelvic organs. Pertinent labs included an alpha-fetoprotein (AFP) level of 254ng/ml, bHCG < 2 MIU/ml, alkaline phosphatase level of 131 units with intestinal isoenzyme level of 0%. The patient then underwent a left thoracotomy and left lobectomy for tissue diagnosis. Histochemical studies performed on resected specimen showed focal positivity for cytokeratin, alpha-fetoprotein, epithelial membrane antigen, and HCG. Placental alkaline phosphatase (PLAP), c-kit gene product (CD 117) , thyroid transcription factor-1 (TTF-1), Cdx-2, cytokeratin (CK) 7 and 20 were all negative. It was focally positive for Oct3/4 and strongly positive for the monoclonal antibody Ki-67. Cytogenetic analysis was performed using fluorescent in-situ hybridization technique. The most consistent result was the presence of Chromososme 3 polysomy seen in all the cells studied. The patient was put on Cisplatn and Etoposide and 6 month follow up showed increase in tumor size following which a second surgery was performed.

DISCUSSIONS: It appears that the tumor arose from primordial germ cells or embryonic stem cells because the tumor cells were positive, by immunostaining, for Oct3/4 and negative for CD34. Pelosi and colleagues in their reported case of a pure yolk sac tumor of the lung showed that the tumor had diffuse Cdx2 immunoreactivity [1]. Our case was completely negative for Cdx2 but showed diffuse Oct3/4 immunostaining. We hypothesize that the more differentiated form of this tumor may have some Cdx2 staining while Oct3/4 expression predominates for tumors that are more undifferentiated. Whether or not the degree of differentiation affects the response to chemotherapy is subject to further testing but studies have shown that Oct 3/4 expression confers resistance to platinum-based chemotherapy and more tumor invasion in bladder carcinoma. We also found, in our study of the cells, by FISH technique, consistent chromosome 3 polysomy. Whether this association is casual is to be evaluated by further studies.

CONCLUSION: We hypothesize that Oct3/4 is the marker for poorly differentiated germ cell tumors in the lung and confers resistance to Cisplatinum based chemotherapy regime. Also chromosome 3 polysomy may be playing a role through Sox-2 and Oct3/4 interaction in the development of the tumor.

DISCLOSURE: Jagmeet Singh, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):22A. doi:10.1378/chest.10736
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INTRODUCTION: Obstructive sleep apnea (OSA) is a common, yet serious, medical condition that may result from an anatomically narrowed upper airway lumen. Lymphoma is a rare cause of tonsillar hypertrophy most often effecting the pediatric population. We report, for the first time in the literature to our knowledge, the case of an adult with undiagnosed large B-cell lymphoma and bilateral tonsillar hypertrophy who presented because of signs and symptoms of obstructive sleep apnea.

CASE PRESENTATION: A thin 39-year-old man presented to his primary care physician (PCP) for evaluation of disruptive snoring. Several months earlier he had experienced tender, swollen cervical lymph nodes on the right which had resolved spontaneously. Aside from a globus sensation when he swallowed, he had no other complaints. The patient was referred to the ear, nose and throat (ENT) clinic where asymmetric bilateral tonsillar hypertrophy without associated cervical lymphadenopathy was detected. Although he denied excessive daytime sleepiness (Epworth Sleepiness Scale = 2), he complained of severe snoring (Snoring Severity Scale = 8) and had a history of hypertension. He was subsequently referred for polysomnography which showed repeated airway obstruction (apnea/hypopnea index = 57.4) associated with severe oxygen desaturations (low SpO2 = 71%). Given the apparent anatomic cause for his symptoms, the patient elected to undergo surgical modification of the upper airway which included uvulopalatopharyngoplasty (UPPP) with tonsillectomy and coblation-assisted turbinate reduction. The excised tonsils were asymmetrically enlarged (right, 4.5 x 4.5 x 2.5 cm; left, 4.5 x 2.5 x 1 cm) and pathology demonstrated large B-cell lymphoma effecting both tonsils. He was subsequently treated with CHOP chemotherapy.

DISCUSSIONS: OSA is a common disorder effecting overweight/obese adults. Non-obese adults with obstructive sleep apnea should be carefully assessed for evidence of an anatomic limitation of the upper airway lumen that might result from various craniofacial anomalies, macroglossia and adenoid tonsillar hypertrophy. Tonsillar hypertrophy is a common remedial risk factor for OSA in the pediatric population, but may also play a role in the pathophysiology of sleep-related upper airway obstruction in adults1. The most common causes of asymmetric tonsils are unequal scarring from prior infection and benign lymphoid hyperplasia. An asymmetric appearance of the tonsils may also result from unequal depth of the surrounding tonsillar fossa. Despite the uncertainties, routine tonsillectomy with pathologic evaluation is the standard of care2 and recommendation of the American Academy of Otolaryngology for children with asymmetric tonsillar hypertrophy. No such recommendations exist for adults and individuals with asymmetric tonsils should be carefully questioned for symptoms or signs of malignancy including pain, ulceration, cervical adenopathy, and constitutional symptoms.

CONCLUSION: Patient’s with signs and symptoms of obstructive sleep apnea should be carefully examined for evidence of anatomic abnormalities/obstruction of the upper airway including pharyngeal tonsil enlargement. Asymmetric tonsillar hypertrophy is most often benign; however underlying malignancy should always be considered.

DISCLOSURE: Jose Gomez, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):23A. doi:10.1378/chest.9936
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INTRODUCTION: Primary cardiac tumors are a rare entity and only one-fourth of these tumors, usually sarcomas, exhibit malignant characteristics. These tumors can metastasize to the lungs, mediastinum, thoracic lymph nodes, and vertebral column. We report a case of a woman with a history of pre-B ALL who presents with an RV mass, pericardial mass, and multiple pulmonary nodules. Biopsy of all three masses reveal metastatic spindle cell type, high grade rhabdomyosarcoma.

CASE PRESENTATION: A 75 year old Caucasian female presented to the Emergency Department with a 2 week history of dyspnea on exertion and an abnormal EKG. She had a history of pre-B ALL treated with chemotherapy 1 year prior to presentation. A chest radiograph was performed, showing a small to moderate right pleural effusion and multiple irregular patchy opacifications in both lung fields. At the time of presentation, a ventilation perfusion scan was performed showing several bilateral moderate mismatches. A left and right heart catheterization showed an ejection fraction of 70% and an abnormal contour at the apex of the right ventricle. Subsequently, the patient had a CT Angiogram which revealed multiple pulmonary nodules ranging from 5 mm to 2 cm, a left pericardial mass measuring 2.6 cm, and an RV mass measuring 2.9 x 6 x 5.6 cm. For further diagnostic evaluation, the patient underwent CT guided biopsy of a lung nodule, thoracentesis, and RV mass biopsy. Cytologic examination of all samples were performed as well as special staining. The final diagnosis was determined to be rhabdomyosarcoma, spindle cell type, high grade. Pt was started on vincristine and ironotecan with documented shrinking of her pulmonary nodules. However, the patient had persistent diarrhea and neutropenia requiring a change in her chemotherapy to vinscristine, actinomycin-D, and cyclophosphamide (VAC). Five months after her initial diagnosis the patient had worsening of her heart failure and a new onset pneumonia. She went into respiratory failure and succumbed to her disease.

DISCUSSIONS: Metastatic sarcoma is 30 times more common than primary cardiac tumors. In adults, one quarter of primary cardiac tumors are malignant. Rhabdomyosarcomas are extremely uncommon. This subtype is associated with an infiltrative growth pattern and an aggressive clinical course. Most patients with cardiac involvement present with progressive dyspnea and unexplained heart failure as well as precordial pain and pericardial effusions. Cardiac findings are determined by the extent of invasion and the location of the tumor. For initial screening, transthoracic echocardiography can be performed. MRI or CT can show the degree of tumor infiltration. Final diagnosis should include a tissue biopsy of the primary tumor or an accessible metastasis. Although possible in less than 50% of patients, complete excision of the tumor allows patients a better overall prognosis. Anthracycline plus ifosfamide based chemotherapy has shown benefit in some case series. If the tumor is primarily intramural, arrhythmias, conduction abnormalities, and sudden death can occur. This tumor usually leads to a rapid downhill course over weeks to months due to hemodynamic compromise, local invasion, or distant metastasis. Orthotopic heart transplantation may be an option for those patients who have unresectable primary tumors without distant metastasis.

CONCLUSION: Primary cardiac rhabdomyosarcoma is a very rare tumor with a poor prognosis usually because it is diagnosed late in the disease course. Echocardiography can be used as a first diagnostic step. Surgery and chemotherapy does alter the disease course, but survival rates continue to be dismal.

DISCLOSURE: Jessica Freyer, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):24A. doi:10.1378/chest.10029
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INTRODUCTION: Pleurodesis is the symphysis between the visceral and parietal pleural surfaces. Compared with other agents for chemical pleurodesis, talc may be the most effective with the least recurrence of effusion(1). The safety of intrapleural application of talc has been debated with known cases of respiratory failure and acute respiratory distress syndrome(1). We present a unique case of a patient who developed a stable pleural mass following this treatment, eventually causing symptoms 17 years after talc pleurodesis. A PET/CT revealed intense hypermetabolic uptake suggesting malignancy. A core biopsy demonstrated an intense inflammatory reaction with macrophage ingestion of a polarizable foreign body, talc.

CASE PRESENTATION: A 37 year old female with a history of extensive tobacco abuse presented to our clinic as a referral for evaluation of a left pleural mass. The patient had a distant history of recurrent left pneumothoraces requiring chest thoracostomy tubes with concurrent large upper lobe bullae. She underwent a left upper lobe bullectomy and talc pleuriodesis 17 years prior to her presentation. She was followed with serial CT scans of the chest because of calcified left pleural nodularity. Her chief complaints at her intital visit to our clinic were left chest pain for the prior several years and dyspnea on exertion. Because of a questionable change in the morphology of the pleural mass, a PET/CT was ordered. The imaging demonstrated intense hypermetabolic uptake with a maximum SUV of 13.8 in mulptiple soft tissue densities along the left pleura including the major fissure, concerning for malignancy. The radiographic appearance suggested localized fibrous mesothelioma. A CT guided core biopsy of the left pleural lesion was performed and demonstrated fibrosis and an extensive foreign body reaction. There was diffuse polarizable material with intense macrophage ingestion. There was no evidence of malignancy.

DISCUSSIONS: Talc pleurodesis is a widely utilized and successful prodecure in the management of recurrent pneumothoraces or recurrent and refractory pleural effusions. The most common adverse events occurring after talc pleurodesis are fever, dyspnea, pain, and gastrointestinal symptoms. Less common side effects include arrhythmia, empyema, talc dissemination and respiratory failure secondary to ARDS. Talc is a sclerosing agent which induces an intense, but intentional inflammatory response resulting in rapid adhesion of the parietal and visceral pleura. In the literature, only scarce information is available on the inflammatory mechanism. It is theorized that sclerosing agents such as talc induce an influx of polymorphonuclear neutrophils followed by an accumulation of mononuclear cells. Several chemokines have been implicated including interleukin-8 (IL-8) and monocyte chemotactic protein (MCP-1)(2). Our patient has a cellular response suggesting acute inflammation, to a foreign body which has resided in her pleural space for almost two decades.

CONCLUSION: In summary, we present a unique case of a 37 year-old patient with chronic chest pain, a hypermetabolic calcified pleural mass which is proven to be an intense inflammatory reaction to talc pleurodesis 17 years prior. Ongoing symptomatic acute inflammation to very remote pleural talc administration has not previously been desribed in the literature.

DISCLOSURE: Anwar Haque, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):25A. doi:10.1378/chest.10767
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INTRODUCTION: We report six observations of pneumothorax ex-vacuo or “trapped lung” associated with hepatic hydrothorax in patients with end-stage liver disease (ESLD). In all cases, the diagnosis of “trapped lung” was made based on the presence of a pneumothorax or hydropneumothorax after evacuation of a long-standing hepatic hydrothorax and failure of the lung to re-expand after chest tube placement. While pneumothorax ex-vacuo is a known phenomenon in the setting of malignant effusions, to our knowledge, it has never been described in association with hepatic hydrothoraces. In one of our observed cases, the lung remarkably expanded without intervention after orthotopic liver transplantation (OLT).

CASE PRESENTATION: A 24-year-old Hispanic male with hepatitis C contracted at birth subsequently developed ESLD complicated by variceal bleeding, portal hypertension, ascites, and a persistent right-sided hepatic hydrothorax requiring frequent thoracenteses. Given his refractory effusions, an indwelling Pleur-x catheter was placed at an outside institution 8 months prior to his OLT for self-drainage of the effusion at home. The Pleur-x catheter was removed 1 month after its placement, and he was managed with diuretics and therapeutic thoracenteses as needed. He subsequently underwent an uncomplicated OLT, and a pre-operative chest x-ray revealed a large, chronic, right-sided pleural effusion. He was successfully extubated after OLT, and thus, no intervention was performed on the effusion. However, on post-operative day 5, the patient developed respiratory distress with new, left-sided infiltrates consistent with pneumonia. He was placed on antibiotics, and a right-sided therapeutic thoracentesis was performed with removal of 1.3 liters of fluid. Post-thoracentesis, he had a large hydropneumothorax suspicious for a pneumothorax ex-vacuo. A small-bore chest tube was inserted and placed on suction without any additional re-expansion of the lung or any change in his respiratory status. The chest tube was removed 2 days later, and a subsequent chest CT confirmed the presence of a large, right hydropneumothorax with underlying visceral and parietal pleural thickening/fibrosis. This caused restriction and constriction of the underlying right lung, i.e. “trapped lung”. His respiratory status fortunately improved with antibiotic treatment of his left-sided pneumonia. During the hospitalization, the thoracic surgery service was consulted for possible video-assisted thoracoscopic surgery (VATS), but the decision was made to defer the surgery until his immunosuppression was tapered. He was discharged home on post-operative day 20 without the need for supplemental oxygen. Repeat chest x-rays at 1 month showed improvement in the hydropnemothorax and at 6 months showed near-complete re-expansion of his right lung without any surgical intervention. Five other cases of pneumothorax ex-vacuo or “trapped lung” have been seen at our institution.

DISCUSSIONS: We describe the first case series of pneumothorax ex-vacuo or “trapped lung” occurring in the setting of hepatic hydrothorax. Previously, this phenomenon has only been reported in the setting of malignant pleural effusions, which are associated with a poor prognosis. Similar to experiences seen with pneumothorax ex-vacuo in malignant effusions, chest tube placement in this setting does not result in lung re-expansion. Surgical intervention can carry significant mortality and morbidity in patients with ESLD due to their underlying coagulopathy, and the ideal management remains undefined. As described above, one patient remarkably had spontaneous resolution of his “trapped lung” after liver transplantation.

CONCLUSION: To our knowledge, this is the first case series reported of the development of pneumothorax ex-vacuo in association with hepatic hydrothorax. Awareness of this phenomenon in ESLD patients with recurrent hepatic hydrothoraces and the various options for its management (including observation) are important for clinicians to be cognizant of.

DISCLOSURE: Yan Kim, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):26A. doi:10.1378/chest.10670
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INTRODUCTION: Hemopneumothorax is frequently associated with trauma and can be a complication of invasive procedures. Resolution involves evacuating the chest cavity and correcting the underlying problem. We report a case of a hemopneumothorax as a result of a screw protruding into the pleural space following spinal cord decompression and corpectomy with placement of a titanium and bone graft vertebral cage.

CASE PRESENTATION: A 59 year old male with history of lung adenocarcinoma diagnosed six months prior presented to the hospital with severe non-radiating mid-back pain for seven days. The patient also complained of tingling in his lower extremities. He had no difficulty with urination or bowel movements and could ambulate, but with pain. At diagnosis his cancer Stage IIIB and he was treated with chemotherapy and radiation. Physical exam revealed normal vital signs and normal cardiovascular and respiratory exam. He had strong pulses in his lower extremities bilaterally. Neurological exam revealed decreased sensation in his lower extremities and hyperreflexia at his knees. MRI of the thoracic spine was performed and revealed a metastatic lesion at the T5 vertebral body with cord compression. The patient was given high dose steroids, and underwent palliative cord decompression with anterior cage placement to maintain spine integrity. For three days following his procedure he was noted to have an increasing large right pleural effusion and thoracentesis revealed bloody fluid with a hematocrit greater than 55% of his serum, consistent with a hemothorax. Follow up thorax CT scan showed that one of the pedicular screws used for the cage placement pierced through the parietal pleura and protruded 1.5cm into the chest cavity. A chest tube was placed and 1500 ml of blood was immediately evacuated from his right lung. Surgical intervention to cut the protruding screw and repair the bleeding vessel was recommended, however, the patient refused. After 2 days there was no further drainage from the chest tube with resolution of the effusion on chest x-ray, therefore the tube was removed. The patient passed away two months later.

DISCUSSIONS: Corpectomy involves removing vertebral bodies and creating a vertebral cage made from bone graft and titanium to stabilize the spine. This procedure is associated with pulmonary complications in up to 5% of cases, typically atelectasis and less common, hemopneumothorax. The etiology of the bleeding may include inadvertent piercing of vessels or lacerations of lung parenchyma or diaphragm by the screws, and tearing of intercostal artery sutures. Hemopneumothorax may occur immediately intraoperatively or may be delayed and occur days after the operation. This complication normally necessitates returning to the OR to cut the protruding screw tip, ligate the leaking artery, and repair lacerations. Further, blood must be removed from the chest cavity to give the patient symptomatic relief and to prevent lung entrapment. The present case report demonstrates treatment of a large hemopneumothorax likely caused by piercing of a vessel from a protruding screw with only tube thoracotomy and no further surgical intervention. We speculate the removal of blood in the pleural space caused the lung to re-inflate and tamponade the leaking vessel.

CONCLUSION: A complication from spinal surgery requiring screw placement can lead to significant bleeding into the pleural space causing patients to become very symptomatic, and if left untreated can lead to trapped lung and death. Treatment involves immediate drainage of the space and normally, surgical intervention to repair the vessel. This case demonstrates a non-surgical correction of the damaged vessel by the re-inflation of the lung and tamponade of the damaged vessel.

DISCLOSURE: Luca Paoletti, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):27A. doi:10.1378/chest.11086
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INTRODUCTION: Synovial Sarcomas are soft tissue tumors which are usually found in the extremities. However, they have been described in unusual locations, with mesenchymal cell origins. Most pleural synovial sarcomas are metastatic and ‘primary’ pleural synovial sarcomas are rare .They are frequently misdiagnosed as relatively more common entities, such as malignant mesothelioma. We describe a case of pleural synovial sarcoma in a patient with recurrent pleural effusion, treated initially presumptively as tuberculosis (TB).

CASE PRESENTATION: A 37 year female was referred for evaluation of a nonresolving left sided exudative hemorrhagic effusion. Her history was significant 6 kg. weight loss over 3 months, dry cough and breathlessness. She had thoracentesis previously at another center, with 1.5 L of a lymphocytic exudate drained and treated empirically as TB, as is common in the Indian subcontinent. A chest tube was placed later for recurrent large effusion. Clinical exam showed absent breath sounds on the left side, with a chest tube draining about 400 ml/day. Pericardial effusion was ruled out by echocardiogram. CT chest showed two 1-2 cm nodular lesions in right lung, large lobulated mass in left posterior hemi thorax, left hydropneumothorax with chest tube in situ, and adenopathy in left cardiophrenic space. We proceeded with medical thoracoscopy and drained about 1400 ml of hemorrhagic fluid. Thoracoscopy showed a hemorrhagic pleural surface, with a visceral pleural mass. Cautious biopsies were done, and the mass opened in layers, giving an onion peel-like appearance. Histopathology showed tumor cells with scanty ill-defined cytoplasm, spindle cells with oval nuclei and mild pleomorphism. Immunohistochemistry identified the tumor as pleural synovial sarcoma.The chest tubes were removed on day 3. She was started on chemotherapy consisting of Adriamycin and ifosfomide. She received 6 cycles of chemotherapy. She improved significantly, with remarkable decrease in the tumor size, and no recurrence of the effusion.

DISCUSSIONS: Primary pleural sarcomas comprise <1% of all primary lung malignancies.Synovial sarcomas are rare tumours, occasionally arising in the pleural cavity. Their histological characteristics may be mistaken for those of malignant mesothelioma or other spindle cell tumors. Diagnosis is made by histology and immunohistochemistry. Identification of the synovial sarcoma-specific chimeric transcript (SYT-SSX1 or SYT-SSX2), with immunoperoxidase studies, are critical in identifying cases of pleural monophasic synovial sarcoma. Synovial sarcoma is extremely aggressive with a high incidence of recurrence. Prognosis is poor; with an overall 5-year survival rate of 50%.Our patient has done well on the chemotherapy regimen outlined earlier.

CONCLUSION: This case is one of the few case reports of primary pleural synovial sarcoma described in literature. Interesting points include misidentification for commoner conditions like TB in the Indian subcontinent, which delays the diagnosis. Here, the role of medical thoracoscopy was vital in making an accurate diagnosis and in conjunction with accurate immunohistochemistry led to a diagnosis, which helped to streamline management. Another interesting issue was the sampling of a mass in the visceral pleura, which is not commonly done. This was possible due to a clear avascular surface tumor on the visceral pleura, which was biopsied cautiously, with no complications. In our case, arriving at the correct diagnosis, with contemporary interventional pulmonology methods, and chemotherapy has improved the outcome of this otherwise aggressive tumor.

DISCLOSURE: Lakshmi Priya Srinivasan, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):28A. doi:10.1378/chest.10909
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INTRODUCTION: Involvement of the respiratory system is unusual in Multiple Myeloma (MM). Patients with advanced MM can have lytic lesions in the bone leading to Myelomatous Pleural Effusion (MPE) and local pleural disease. There can also be mediastinal involvement with Extramedullary Plasmacytomas (EP) leading to pleural effusions. Simultaneous occurrence of both MPE and EP as the initial presentation for MM is extremely rare. We report a case with a large pleural effusion and mediastinal plasmacytoma as the initial presentation of MM.

CASE PRESENTATION: 74 year old female, past history of renal cell cancer (RCC) who had undergone a nephrectomy 3 years back and had a history of lumbar spine laminectomy and fusion secondary to degenerative disc disease 6 years back, presented to the hospital with a complaint of 3 months of worsening dyspnea on exertion and back pain. CT of the chest revealed a large right-sided effusion, pleural nodularity and large paraaortic and AP window lymph nodes along with a large paraspinal density surrounding the T4 vertebra. The effusion was tapped, found to be consistent with an exudate, and cytology showed numerous plasma cells with large multinucleated cells with open chromatin and prominent nucleoli. Immunohistochemical stains were also compatible with myeloma cells. Due to the history of RCC, the mediastinal nodes were also biopsied and the pathology found to be consistent with an extramedullary plasmacytoma. The patient underwent a bone marrow biopsy that showed variably cellular marrow with diffuse and nodular infiltrates of atypical plasma cells (30% of overall) consistent with plasma cell neoplasm. A serum protein electropheresis (SPEP) showed a monoclonal protein spike (IgG kappa, 26.1) and she was diagnosed with MM.

DISCUSSIONS: MM comprises 1% of all malignancies and 10% of all hematologic malignancy. Pleural effusions occur in about 6% of patients with MM myelomatous involvement occurs in less than 1% of cases. The majority of these patients have IgA type of monoclonal protein and the etiology of MPE is thought to be due to invasion from skeletal lesions, chest wall invasion of mediastinal masses or direct tumor deposits in the pleura. MPE is usually a late manifestation of the disease process and is associated with a more aggressive form of MM. The prognosis of these patients is very poor, with less than 4 months survival from the time of development of MPE according to published reports. EP is also a rare occurrence and only about 5% of patients with EP have concurrent MM. Most EP are found in the aerodigestive tract but mediastinal involvement is extremely rare. Our patient was unusual in that she not only presented with MPE and extramedullary plasmacytoma at the time of diagnosis of MM, but, also had IgG Kappa gammopathy that is not usually associated with pleural effusions. Pleurodesis and chemotherapy have been used for patients with MPE, however their survival is very short despite treatment. Our patient decided to choose hospice care and was discharged home where she passed away 8 weeks after diagnosis.

CONCLUSION: When faced with a pleural effusion, MPE must be considered in the differential and every effort made to accurately diagnose underlying MM. Patients with MM who do develop MPE have a worse prognosis and do not seem to respond well to currently available chemotherapeutic regimens.

DISCLOSURE: Amik Sodhi, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):29A. doi:10.1378/chest.10940
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INTRODUCTION: Pleural effusions in ovarian hyperstimulation syndrome (OHSS), whether transudative or exudative, can occur in up to 30% of cases. However, it is exceedingly uncommon for them to present as a tension hydrothorax requiring emergent intervention. We report on just such a case.

CASE PRESENTATION: A 29-year-old female presented to our emergency department (ED) for chest pain and dyspnea. She had no previous medical history. She was being followed by reproductive medicine for in vitro fertilization which was preceded by ovarian hyperstimulation with human chorionic gonadotropin (hCG) 17 days previously. She had two embryos successfully implanted 7 days before presentation. She had complained of some increased dyspnea several days prior to her ED visit as well as some intermittent nausea. In the ED she was in moderate respiratory distress with complaints of abdominal pain and nausea. She was found to be afebrile with respirations of 20-25 breaths per minute, heart rate 130 beats per minute, and blood pressure of 116/80 mm Hg. She had decreased breath sounds over the right hemi-thorax. Her oxygenation was 98% on 2L oxygen per minute. There were no other remarkable physical findings. Laboratory investigations revealed a white blood count 23.2 thousand per cubic millimeter, hematocrit 48.8%, albumin 3.0 g/dL, and hCG 220 mIU/mL. Computed tomography of the chest revealed a right tension hydrothorax and no pulmonary embolus. Trans-vaginal ultrasound revealed bilaterally enlarged ovaries. During her hospital stay she required six therapeutic thoracenteses for a total volume of 9.6L removed. Her pleural fluid analysis revealed an exudative effusion by Light’s criteria.

DISCUSSIONS: Defined as a loss of control over hyperstimulation, OHSS complicates up to 33% of hyperstimulation cases which ranges both in severity and presentation. Vascular endothelial derived growth factor is thought to mediate much of the fluid shifts in these cases through increased capillary permeability. There are several thoughts as to why pleural effusions may develop in these cases and why they develop more on the right than left. They may result from both the positive intra-abdominal pressures and presence of diaphragmatic defects. The ascitic fluid is then allowed to move along a pressure gradient from the abdomen into the pleural space. Also, right sided pleural effusions may occur more often due to decreased lymphatic drainage when compared to the left side. Interestingly, our patient had a relatively small amount of ascites; however, this may have in fact been due to the continuous movement of fluid into the pleural space. Only 4.5% of reported severe cases have required therapeutic thoracenteses. In its severe form patients with OHSS may show objective evidence of hemoconcentration, acute kidney injury, electrolyte abnormalities, and liver dysfunction. They can also show clinical signs of hypotension, abdominal pain, vomiting, and diarrhea. OHSS is a pro-thrombotic state and thromboembolism is the most feared complication. Fortunately, our patient did not suffer a pulmonary embolus, but she did have a tension hydrothorax requiring immediate thoracentesis Based on the reported literature our patient required the second largest therapeutic volume removed.

CONCLUSION: Severe OHSS cases presenting with massive hydrothoraces may benefit from early placement of a small chest tube with daily volume removal until fluid re-accumulation subsides.

DISCLOSURE: Leon Bass, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):30A. doi:10.1378/chest.10218
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INTRODUCTION: We describe a patient who underwent esophagectomy and gastric interposition, which was complicated post-operatively by mediastinitis and vertebral osteomyelitis. He developed cough and was found to have an endobronchial inflammatory polyp due to migration of a surgical staple into the airway.

CASE PRESENTATION: A 62 year old man, who recently underwent esophagectomy and gastric interposition for esophageal cancer, presented with a 10-day history of fever, back pain and cough. The cough was productive of yellow sputum. There was no hemoptysis. Physical exam revealed fever and tenderness over the thoracic spine. Chest computed tomography (CT) revealed evidence of mediastinitis, thoracic spine osteomyelitis and a 5 mm polypoid mass with a central focus of high attenuation in the left mainstem bronchus that was not present on prior study (figure 1). Flexible bronchoscopy showed a friable, smooth, mobile, polypoid mass partially obstructing the left mainstem bronchus (figure2). Forceps biopsies of the mass were obtained and sent for histopathology. After biopsy, a metallic foreign body (FB) was seen in the center of the polyp. The FB was removed using biopsy forceps. The mass was not completely excised and was still partially obstructing the left mainstem bronchus at the end of bronchoscopy. Pathology demonstrated squamous epithelium and acute on chronic inflammation consistent with fibro-connective tissue, surrounding a surgical staple. Patient’s cough resolved within 2 weeks after removal of FB without additional treatment. Follow-up Chest CT 3 weeks later showed complete resolution of the left mainstem bronchus mass.

DISCUSSIONS: The patient’s esophagectomy and gastric pull-up surgery was complicated by mediastinal-esophageal fistula and mediastinitis that led to tissue breakdown and migration of the surgical staple into the airway. Staple line breakdown from chronic infection and local tissue invasion had been reported in the literature. Chest CT 1 week prior to bronchoscopy showed findings suggestive of mediastinitis with no evidence of endobronchial lesion. The patient’s cough began around the time of this CT, persisted for 1 week, and a follow-up CT demonstrated an endobronchial mass. A FB was suggested on CT. The FB, but only a small part of the polyp, was successfully removed via bronchoscopy. This case is unusual in that the surgical staple migrated into the airway within 1 week and it was associated with the formation of an inflammatory polyp. Prior case reports have suggested that the use of inhaled and systemic corticosteroids (CS) after removal of the FB may help in resolution of the polyp and its mass effect (1, 2). In our case, CS was not used due to concern that this may interfere with healing of the esophageal fistula. In our case, removal of the FB itself resulted in disappearance of the inflammatory polyp and the patient’s cough (2).

CONCLUSION: Development of an endobronchial inflammatory polyp due to surgical staple migration is a potential post-operative airway complication in esophagectomy and gastric interposition. Removal of the FB via flexible bronchoscopy, as in this case, may lead to resolution of the polyp and symptoms, without addition of CS. The role of CT and flexible bronchoscopy in evaluation of cough after mediastinal intervention is also key.

DISCLOSURE: Abdulilah Arafeh, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):31A. doi:10.1378/chest.10159
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INTRODUCTION: Conduit redundancy and dilatation are well-recognized late sequelae of colon conduit interposition for esophageal reconstruction. We present a rare case of symptomatic cardiac compression secondary to a massively dilated substernal colon conduit. In an era of mixed surgical and medical Intensive care units with medical intensivists taking care of surgical patients, a thorough understanding of surgical emergencies is essential.

CASE PRESENTATION: The patient is a 49-year-old male who underwent substernal colon interposition at 5 years of age for congenital esophageal atresia. He presented with a 24 hour history of inability to swallow, shortness of breath, and a left-sided supraclavicular mass which could not be reduced. He reported transient episodes of similar symptoms over several months prior to this presentation, which had previously resolved after “pushing the left neck mass into the chest”. Physical examination disclosed an apprehensive patient with a systolic blood pressure of 96/62, heart rate of 120, and respiratory rate of 30. Neck exam revealed jugular venous distention and a large tender supraclavicular mass just inferior to the estimated level of the esophagocolonic anastomosis, which was not reducible (figure 1). Laboratory data was remarkable for a white blood cell count of 16,300 and a mild lactate acidemia with an arterial pH of 7.28. Computed tomography showed massive distention of the substernal colon interposition filled with liquid and particulate matter, with secondary compression of the heart, ascending aorta, and right pulmonary artery (figure 2).The patient was emergently taken to operating room for conduit removal. An extended sternotomy incision from the left neck superiorly to the umbilicus inferiorly was utilized for exposure. After sternotomy, the conduit appeared ischemic and massively dilated with liquid and particulate matter. The gastrocolic anastomosis was found to be widely patent however substantially smaller in diameter than the conduit itself. The conduit was removed expeditiously. Immediately after conduit removal, the central venous pressure was noted to drop from 23 mm Hg to 12 mm Hg. Simultaneously, the systolic blood pressure increased by 20 to 30 mm Hg with a decrease in heart rate. A cervical end-esophageal fistula and tube gastrostomy for feeding were established. The patient made an uneventful postoperative recovery and ultimately underwent a substernal gastric reconstruction through a redo sternotomy approach.

DISCUSSIONS: Colon conduit redundancy and dilatation following interposition for esophageal reconstruction ultimately requires surgical intervention in an estimated 8-22% of cases. Commonly reported symptoms include dysphagia, regurgitation, and aspiration. Redundancy with dilatation of colonic interposition conduits in the substernal position may additionally result in retrosternal pain and neck swelling. Based on this patient’s chronic symptoms resulting in this acute event, we speculate that a progressive cycle of increased retention of liquid and particulate matter and ongoing low-grade ischemia led to massive conduit dilatation and secondary cardiac compression. A contributory factor to heart and great vessel compression in this case may have included a relative lack of redundancy into either pleural space as the conduit remained in the substernal position over time. Although a component of sepsis may have contributed to this patient’s overall presentation, the immediate improvement in cardiac hemodynamics following conduit removal is highly suggestive of cardiac compression as the primary pathophysiology. A MEDLINE search of the English speaking literature found only one previous report of cardiac compression attributed to a dilated substernal colon conduit.

CONCLUSION: This patient represents a rare but life threatening complication of cardiac compression resulting from a substernal colonic conduit surgery performed over 40 years ago. Earlier recognition of symptoms in such patients is important, especially for intensivists, as a delay in surgical intervention for cases of impending cardiac compression could be fatal.

DISCLOSURE: Babar Khan, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):32A. doi:10.1378/chest.10312
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INTRODUCTION: Our patient is a 66 year old man who went for an outpatient MRI for chronic low back pain. Two weeks later he presented to the ER in shock. We present a case of delayed cardiac tamponade with a very rare iatrogenic cause.

CASE PRESENTATION: CASE REPORTA 66-year-old man presented to the ER with chest pain, low blood pressure and severe shortness of breath.His medical history was significant for well controlled hypertension and diabetes. His initial physical and lab work up was unrevealing. His cheat X ray and chest CT was normal. The presumptive diagnosis was ACS. Patient was transferred to ICU for further management. In the ICU his systolic pressures dropped to 60’s despite pressors and fluids. Patient was hypoperfusing, venous engorgement was noted on his anterior chest and his heart sounds seemed distant. A TTE revealed pericardial effusion with right ventricular collapse. Emergent thoracotomy and pericardial window was performed at bedside. About 400 cc of hemorrhagic fluid was drained; subsequently, hemodynamic stability was restored. A follow up echocardiogram revealed two echogenic densities coursing through the right atrium into the right ventricle. These densities were confirmed by TEE. On review of the chest CT obtained in ER, a possible foreign body was seen coursing from the right ventricle to the hepatic vein. Interventional radiology consult retrieved a guidewire via internal jugular vein access. The extracted guidewire was a small caliber, long wire and magnetic. The patient remained stable after the removal of the wire and was transferred to a step-down unit. After detailed review of his history, the patient had a motor vehicle accident 12 years previously. This was complicated by bilateral deep vein thromboses and PE. He had an IVC filter placed and possible central line placement.Most likely that is when the guidewire was left in the patient. He had no information about the wire.

DISCUSSIONS: Hemorrhagic pericardial effusions with tamponade have few etiologies. In a recent series from the United States, the authors evaluated 96 cases of cardiac tamponade with hemorrhagic pericardial effusions. 31 % required pericardiocentesis. In this series bloody pericardial effusion etiologies were mostly iatrogenic. Malignancy, complications of acute myocardial infarction, and idiopathic disease were also prevalent. Tuberculosis should be strongly considered in endemic areas outside the US. Foreign objects left inside the body causing cardiac tamponade are a very rare cause and mostly iatrogenic.

CONCLUSION: More than 5 million central lines are inserted in the US every year. One of the complications noted is loss of guidewire. There are no good statistics on how often this complication happens. We also don't know how many people are living with guidewires inside them but unaware of it. It is most likely that our patient was living with a 20 cm guide-wire inside him for the last 12 years. The MRI caused this wire to puncture his myocardium causing slow leaking of blood which caused a cardiac tamponade 2 weeks later. Prompt recognition and treatment saved his life.

DISCLOSURE: Yashvir Sangwan, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):33A. doi:10.1378/chest.11030
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INTRODUCTION: Bochdalek hernias are a rare entity, occurring in 1 out of 2,200 to 12,500 live births. Some may be detected early in childhood due to respiratory distress, but others may not be discovered until later on in life, if at all. This case report demonstrates the importance of Bochdalek hernias in the setting of abnormal thoracic imaging in symptomatic patients.

CASE PRESENTATION: 79 year old male with a past history of hyperlipidemia, melanoma post removal many years ago, and recent boating accident 6 months ago in which he sustained several rib fractures on his left side presents with 2 days of nausea, vomiting, diarrhea, and left upper quadrant abdominal pain which radiates to the flank. He denies fever, chills, blood in the stool, recent travel. His exam was positive for left upper quadrant tenderness, left basilar crackles, and fever. He was admitted, CT scan of the abdomen performed, which showed segmental thickening and inflammatory changes at the splenic flexure. He was started on broad spectrum antibiotics for concern of diverticulitis. During his hospitalization, he had sudden onset shortness of breath, and a chest film was obtained showing a pneumothorax. A chest tube was placed, and drained brown material which grew E. coli and Enterococcus. He became septic necessitating pressor support. A larger bore chest tube was placed with improvement in his status. He subsequently underwent VATS due to concern of empyema or rind seen on a repeat CT chest. Multiple chest tubes continued to drain large amounts of brown material. He then underwent a thoracotomy, where a diaphragmatic hernia was noted and his colon was found in the thorax, perforated and leaking feculent material. He had diaphragmatic hernia repair along with colectomy with subsequent lung re-expansion.

DISCUSSIONS: Bochdalek hernias arise from the failure of the pleuroperitoneal space to close prior to the return of bowel to the abdomen in week 8-10 of development. Although some are diagnosed at birth due to dyspnea or other respiratory symptoms, others remain asymptomatic or are acquired with increased intra-abdominal pressures such as retching, coughing, pregnancy, or trauma. There are approximately 105 case reports of symptomatic Bochdalek hernias in the adult population. CT scans appear to be the most sensitive for detection of hernias but are often mistaken for other intrathoracic pathology, complicating or delaying the diagnosis. In our patient, we theorize that he had a diaphragmatic opening due to a Bochdalek defect with subsequent herniation of bowel during his boating accident. The incarcerated bowel became necrotic and perforated causing his symptoms, including his pneumothorax and empyema. The lateral and posterior nature of Bochdalek hernias make detection during VATS difficult, as was the case in our patient.

CONCLUSION: Bochdalek hernia is an extremely rare cause of empyema in adults. Presentation and imaging may be nonspecific, making it challenging to diagnose. Surgical correction is recommended whenever it is encountered to correct or prevent complications.

DISCLOSURE: Kristine Kuhl, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):34A. doi:10.1378/chest.10746
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INTRODUCTION: Idiopathic fibrothorax may result in severe debility and is a treatment challenge. Decortication is not frequently considered as a routine component of management of fibrothorax(1).

CASE PRESENTATION: The patient is a 45 year old male treated at an outside institution for nonspecific interstitial pneumonitis with azathioprine and prednisone for 8 months prior to presenting to our institution for a second opinion. His co-morbidities included morbid obesity (BMI 52), obstructive sleep apnea requiring nocturnal CPAP and chronic back pain. He never smoked. He worked retail in a car repair shop for many years. At the time of presentation PFTs revealed a severe restrictive lung pattern and reduced diffusing capacity with hypercapnic respiratory failure requiring 5 liters of oxygen with activity. Chest CT scanning showed diffuse patchy ground glass and bilateral pleural scattered calcifications. He underwent BAL initially and infection was ruled out and then VATS assisted biopsy of the lung with pathology showing only mild pleural fibrosis and normal lung structure. He became more hypercapnic and required tracheostomy with nocturnal ventilator support with oxygen continuously. His hypercapnia stabilized in the mid 70s. He was assessed for surgical intervention by transplant surgery and they deemed him not a candidate based on his weight. After 120 lbs weight loss assisted with gastric sleeve surgery, no significant improvement in his respiratory failure was evident. Repeat imaging of the chest at this point clearly revealed diffusion pleural thickening. Serologic work up was negative but his back disease was deemed seronegative spondyloarthopathy by Rheumatology. He then underwent sequential bilateral decortication procedures. Three months after the procedures he had considerable improvement in functional status, pulmonary functions, arterial blood gases and almost complete resolution of his sleep disordered breathing, and he was liberated from oxygen. After more than a year of follow-up he remains off oxygen, pulmonary function tests and exercise capacity steadily improve with each visit and his hypercapnia has resolved. He retains mild OSA which requires CPAP at night.

DISCUSSIONS: We believe that this represents a case of idiopathic fibrothorax in a morbidly obese patient where treatment with pleural decortication resulted in a significant improvement in lung function and the overall health status, which was not fully appreciated until his profound weight loss alone did not correct the hypercapnia. There are only rare reports of treatment with decortication for idiopathic fibrosis (2).

CONCLUSION: Our experience shows that pleural decortication is a viable option in treating idiopathic fibrothorax.

DISCLOSURE: Muhammad Akbar, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):35A. doi:10.1378/chest.10917
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INTRODUCTION: Metastectomy for colon cancer has been proven as a survival prolonging procedure for both liver and lung metastases. Data on metastectomy of the less common sites, retroperitoneum, mesentery and mediastinum, is minimal and thus has not become a widely adopted as a treatment strategy. We report long-term survival of a patient with a history of colon cancer who underwent a mediastinal metastectomy.

CASE PRESENTATION: A 61 year-old male presented to our clinic with a mediastinal mass. Seven years prior to presentation, he underwent resection, which demonstrated T2N1 moderately differentiated mucinous adenocarcinoma. Postoperatively, he received six cycles of chemotherapy with 5-FU and leucovorin. Six years later, rising CEA levels prompted a PET scan which demonstrated isolated uptake in his abdomen. For this recurrence, he underwent resection of the abdominal mass and associated bowel. Pathology confirmed poorly differentiated infiltrating mucinous adenocarcinoma. Postoperatively, he underwent additional chemotherapy treatment with 5FU-leucovorin, Oxaliplatin and Avastin. One year later, CEA levels were again elevated. A PET scan revealed an isolated right paratracheal mass measuring 5.8 × 3.6 × 4.8 cm mass. He was taken to the operating room for muscle-sparing axillary thoracotomy with resection of the mediastinal mass and mediastinal lymphadenectomy. Pathology of the mass demonstrated multiple matted lymph nodes containing mucinous adenocarcinoma. However, seven lymph nodes were negative for malignancy. Postoperatively, he underwent adjuvant therapy and has remained disease free for five years.

DISCUSSIONS: Over 50% of patients undergoing colon resection for colon adenocarcinoma will have metastases1. The most common site of metastasis is the liver, followed by the lungs. Metastectomy in isolated hepatic and pulmonary metastases has improved 5-year survival up to 40% or higher2. The morbidity and mortality of mediastinal lymphadenectomy has decreased over the past several years. Currently, patients can be discharged on the first postoperative day after minimally invasive techniques, such as a transcervical mediastinal lymphadenectomy. Given the minimal morbidity and mortality associated with this procedure, the potential role of mediastinal lymphadectomy in the management of patients with metastatic cancer should be investigated further.

CONCLUSION: Progress in the diagnosis and treatment options allows cancer to be managed as a chronic disease. In patients with limited mediastinal disease, mediastinal lymphadectomy may afford a survival benefit. The procedure is associated with minimal morbidity and mortality. The oncologic and survival benefit of such a procedure should be evaluated.

DISCLOSURE: Trevor Upham, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):36A. doi:10.1378/chest.10915
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INTRODUCTION: Kaposi’s sarcoma (KS), an angioproliferative tumor, mainly involves mucocutaneous tissues. Extracutaneous spread to visceral organs including the lung can occur in acquired immunodeficiency syndrome (AIDS)-associated KS; however, pulmonary KS in the absence of mucocutaneous involvement is rare. Few cases of KS-associated immune reconstitution inflammatory syndrome (IRIS) have been reported. We report a case of probable KS-associated IRIS in a patient with AIDS who developed progressive respiratory failure one month after initiation of highly active antiretroviral therapy (HAART). At autopsy he was found to have pulmonary KS in the absence of mucocutaneous lesions.

CASE PRESENTATION: A 25 year-old man with AIDS presented to the emergency department with three days of dyspnea, nonproductive cough, hemoptysis, fevers and pleurisy. His CD4 count two months prior was 40 with a viral load of 403,000. He had started HAART one month prior to presentation. Physical examination revealed a non-toxic young male in mild respiratory distress. Notable findings were pulse 106, respirations 22, temperature 101.4, pulse oximetry 98%, and bibasilar crackles. He had no oral mucosal or skin lesions. Initial laboratory values included a white blood cell count of 5.1, platelets 31,000, CD4 of 170, and a viral load of 1500. Chest x-ray showed diffuse fine reticular interstitial opacities. CT scan revealed diffuse septal thickening, lobular and somewhat focal ground glass opacities, some nodular in appearance. Several of the nodular densities were surrounded by ground glass opacities. The differential diagnosis included alveolar hemorrhage, Pneumocystis jirovecii, bacterial, viral and other fungal infections. Bronchoscopy did not reveal any endobronchial lesions but the lavage fluid was mildly bloody. The patient was empirically treated for typical and opportunistic infections. Over the course of 2-3 weeks, he clinically deteriorated with worsening hypoxemia. Cultures remained negative. Repeat bronchoscopy again revealed no lesions or infection. He developed massive hemoptysis with subsequent respiratory failure and rapidly progressive shock with multi-system organ failure. At autopsy, gross examination revealed poorly demarcated pulmonary nodules. Microscopic examination of these nodules revealed an angiocentric pattern of spindle shaped tumor cells, proliferating endothelial cells and fibroblasts consistent with pulmonary KS.

DISCUSSIONS: KS remains the most common AIDS-associated cancer and may present with mucocutaneous lesions and visceral involvement. The clinical presentation of intrathoracic KS is nonspecific and may be indistinguishable from pneumonia. Readily identifiable endobronchial lesions are noted in 50%. Pulmonary KS in the absence of mucocutaneous involvement is rare however, ranging from 0-15% cases. Radiographic findings include peri-bronchovascular reticulonodular opacities, septal line thickening, consolidation and pleural effusions. Pulmonary KS carries a poor prognosis with a median survival of 2-10 months. Treatment is often palliative. Combination chemotherapy affords response rates of 30-50%. Radiation therapy benefits are short lived. HAART reduces the risk of developing KS and decreases growth rate of pre-existing KS and may improve median survival. KS-associated IRIS has been rarely reported. It usually occurs within two months of initiation of HAART and is characterized by the development of an inflammatory reaction with both progression and development of KS lesions. CT findings are similar to typical AIDS-related KS.

CONCLUSION: KS-associated IRIS should be considered in AIDS patients presenting with progressive respiratory abnormalities within 1-2 months of HAART initiation, even in the absence of mucocutaneous lesions or prior known disease. The clinical presentation may mimic infection. Radiographic findings include reticulonodular opacities, septal line thickening, consolidation, and pleural effusions. The course may be rapidly progressive and fatal.

DISCLOSURE: Rabih Halabi, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):37A. doi:10.1378/chest.9979
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INTRODUCTION: Rapidly fatal septic shock is well known with rickettsiae that do not grow on routine cultures and require different antibiotics. We describe here a case of septic and obstructive shock due to rickettsial infection that manifested with an atypical presentation (without rash).

CASE PRESENTATION: A 68 year old male with nonsignificant past medical history was transferred from outside hospital with worsening hemodynamic status, intubated, on mechanical ventilation and pressor support. Patient had presented with a history of high grade fevers, and non-productive cough for a week. Physical examination was then remarkable for crepitations at the base of the left lung. Patient had received ceftriaxone, and azithromycin as empiric coverage for presumptive diagnosis of community acquired pneumonia. Laboratory data on presentation revealed leukocytosis, thrombocytopenia, hyponatremia, a high anion gap metabolic acidosis and acute renal failure. Patient had an APACHE II score of 37 on presentation, and was admitted to the intensive care unit for escalation of care, and drotrecogin infusion. Cultures were sent, and empiric antibiotic therapy initiated with cefepime, vancomycin, azithromycin, and metronidazole. On day 4 of hospitalization, patient developed new onset hemodynamically unstable rapid atrial fibrillation converted to normal sinus rhythm by direct current cardioversion. EKG showed electrical alternans. 2 D echocardiogram revealed pericardial effusion with tamponade physiology. Subxiphoid pericardiocentesis drained 600 cc hemorrhagic fluid. Analysis revealed 1740000 red blood cells per cu.mm, consistent with hemopericardium. Serologic titers for rickettsia were sent, and doxycycline added to the regimen. Fluid cultures were negative for bacteria and acid fast bacilli (AFB). Rickettsial serologies revealed positive RMSF IgM (1:128), IgG (1:512), Ehrlichia IgG (>1:2048), and R typhi IgG (1:256).

DISCUSSIONS: Rickettsial diseases have a case-fatality rate of 23% unless treated early and appropriately. Early diagnosis remains a challenge due secondary to difficulty staining with ordinary bacterial stains. It is also difficult to be cultivated in cell-free medium, and growth requires living host cells (1). Epidemiological trends show fall in incidence in 1949 secondary to introduction of tetracycline antibiotics, with increased incidence lately due to a decline in use of tetracycline antibiotics as first line agents in many other infections. Rocky mountain “spotless” fever occurs more often in older and black patients. Pulmonary involvement is suggested by cough, and radiologic evidence of alveolar infiltrates, interstitial pneumonia, and pleural effusion. Pulmonary edema with impairment of ventilation, and adult respiratory distress syndrome (ARDS) requires oxygen therapy, and mechanical ventilation. Cardiac complications are seen in 5-25% of patients including hypotension due to shock, myocarditis and arrhythmias (2). Pericardial effusion has been reported as a manifestation of scrub typhus, with 58% incidence of pericarditis by autopsy in this rickettsial infection. Endothelial cell invasion by rickettsia during infection causes vasculitis, which may lead to hemorrhagic pericardial effusion. Early clinical diagnosis is key. Serology is the usual method for confirmation of diagnosis. The diagnostic titer is 1:64 for indirect immunofluorescence assay, the most sensitive, and specific test.

CONCLUSION: We saw severe hemorrhagic pericardial effusion causing tamponade in severe rickettsial sepsis. Cross-reactivity within the spotted fever or typhus group precludes speciation of rickettsiae. It is important to recheck for a change in the IgM titer 7-14 days after the initial specimen.

DISCLOSURE: Ankur Kalra, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):38A. doi:10.1378/chest.10013
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INTRODUCTION: On April 2010, novel influenza A (H1N1) virus (S-OIV) was identified in Mexico, the USA and elsewhere. Pulmonary changes commonly associated with this infection are consolidation, diffuse edema and hemorrhage. We present the case of a young H1N1 infected adult that developed uncommon pulmonary changes and represented a clinical challenge due to persistent air leak.

CASE PRESENTATION: Our patient is a 32 year old man, who was diagnosed with Influenza H1N1 virus infection and Staphylococcus aureus secondary bacterial pneumonia. He developed acute type I respiratory failure and was admitted to the ICU for mechanical ventilation and amine support. His treatment included oseltamivir, meropenem, vancomycin, amikacin and moxifloxacin.8 days after admission, chest X-rays showed a right-sided 20% pneumothorax for which a 28 Fr. chest tube was placed. Control chest X-rays proven pulmonary re-expansion. On the 13th day, X-rays showed right-sided pneumothorax recurrence and subcutaneous emphysema. Chest CT scan was performed on day 14th, which demonstrated persistence of the pneumothorax and a large right- sided cystic lesion. Pleural drainage showed persistent air leak. Due to clinical improvement, the patient was discharged on day 18 with a portable pleural drainage system and a scheduled re-hospitalization for definite management of the air leak.2 months after initial admission, the patient developed sudden right-sided chest pain and resting dyspnea. Chest CT scan showed a right-sided pneumothorax, along with persistence of the cystic lesion, bilateral lung parenchymal injury and fibrosis. A second 28 Fr. chest tube was placed and the patient's conditions markedly improved. Definite treatment was performed by means of a thoracoscopic resection of the cystic lesion and lysis of pleural adherences. Chest tubes were removed 5 days after surgery. Two days after, the patient was asymptomatic, X-rays showed pulmonary expansion and he was discharged.He was then treated as outpatient with inhaled bronchodilators and anti-inflammatory therapy, plus supplementary oxygen. He is currently a NYHA functional class I and is no longer on supplemental oxygen.

DISCUSSIONS: The clinical spectrum of the Influenza A H1N1 (S-OIV) infection is still being defined. Current available information is mainly based on large observational studies. Pathological changes associated with S-OIV infection are more often localized to the lungs; being the most common: lung consolidation, diffuse edema and variable degrees of hemorrhage.Secondary complications, such as primary viral pneumonia and secondary bacterial pneumonia may be responsible for severe illness in many patients. Staphylococcus aureus has been identified in Mexico as the most common cause of secondary bacterial pneumonia in ICU patients with H1N1 infection.The most common chest CT abnormalities in H1N1 affected patients are local patchy shadowing, bilateral or unilateral infiltrates and ground-glass opacities. The authors did not find any references of cystic lesions after H1N1 infection. As in the case presented, a pneumatocele is a cystic airspace within the lung that characteristically increases in size over a period of days to weeks. It is associated with infection, particularly staphylococcal pneumonia. This patient presented this uncommon complication, probably due to the secondary S. aureus pneumonia. Immediately after surgery, air leak ceased and the patient conditions progressively improved.

CONCLUSION: H1N1 spectrum is still being defined. It is important to be aware of uncommon complications like the one presented in this case, since they may require special therapeutic decisions that could markedly improve the patient’s outcome.

DISCLOSURE: Luis Martin-Del-Campo, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):39A. doi:10.1378/chest.9958
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INTRODUCTION: Empyema necessitatis (EN) occurs when an empyema extends through the parietal pleura into the surrounding tissues. EN has become less common with the routine drainage of empyema and antibiotic use. Most cases reported in the modern literature have been in immunocompromised patients.

CASE PRESENTATION: A homeless 59-year-old African-American man with chronic hepatitis C and hypertension presents with a finger abscess. During the interview, he mentions a left sided chest mass that has been present and slowly enlarging for several months. He reports a cough productive of yellowish sputum and weight loss of about thirty pounds over the past three months. He has no history of tobacco, alcohol, drug abuse, HIV or immunodeficiency. Upon exam, the patient has a soft, nontender, nonulcerated mass located on the anterior left chest wall measuring 14 cm in diameter. Blood counts and metabolic panels are within normal limits. HIV testing is negative. A chest radiograph and CT scan are performed. The Chest CT demonstrates a large mass communicating with the pleural space. The lung parenchyema has some nodular areas and areas of cavitation. Sputum sample reveal acid fast bacilli (AFB) and PCR confirms mycobacterium tuberculosis.

DISCUSSIONS: EN is a rare complication of pulmonary infection. EN is usually located on the anterior chest wall between the second and sixth intercostal spaces. However, this complication has been reported to occur in the bronchi, esophagus, breasts, diaphragm, retroperitoneum, and groin. The most common causative agent is mycobacterium tuberculosis with actinomyces being the next most likely. Cases have been described with streptococcus pneumoniae, staphylococcus aureus, gram negative bacilli, polymicrobial infections, blastomycosis, and neoplasia. Chest imaging usually shows a calcified well defined encapsulated pleural mass associated with an extra pleural mass. Our case highlights the connection between the pleural and extra-pleural collections, which is rarely seen due to the small size of the connection. Treatment consists of antimicrobials and drainage of the abscess. Before antibiotics, mortality was as high as 87% among those with tuberculosis as the causative agent.Our patient was started on antibiotic therapy with ethambutol, isoniazid, pyrazinamide, and rifampin. The chest wall abscess was drained by cardiothoracic surgery. Material from the abscess was AFB positive. Antibiotics were continued at a local tuberculosis hotel under direct supervision with follow-up by the health department and the infectious disease team.

CONCLUSION: Empyema necessitatis is a rare complication most often associated with tuberculosis infection. EN should be included in the differential when evaluating a chest wall mass, especially in a patient with constitutional symptoms, risk factors for tuberculosis infection or immune compromise.

DISCLOSURE: Scott Kellie, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):40A. doi:10.1378/chest.10855
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INTRODUCTION: Tuberculosis remains one of the leading causes of infectious morbidity and mortality in the world today. The presentation of miliary tuberculosis can be varied, ranging from septic shock to chronic illness. We present the case of a patient with tuberculosis involvement of the skin, sternum and ascending aorta resulting in an arterial bleed requiring emergent surgical repair.

CASE PRESENTATION: A 48 year old male from guyana presented to the emergency department with a complaint of profuse bleeding from a chest wall abscess after falling. The abscess had been present for 7 months prior, which the patient had been self treating with epsom salts unsuccessfully. Prior to this admission, he had not received medical care in over 20 years, but has progressively been getting weaker, with weight loss and night sweats for the last year. He denied any exposure to tuberculosis since immigrating to the United States 17 years earlier. Physical exam demonstrated bilateral axilla lymphadenopathy and his anterior chest was significant for a 2 cm and a 1.5 cm round area of abrasion to the right of the sternum over the 3rd intercostal space with associated skin atrophy, hyperpigmentation and erythema. Chest x-ray performed at admission demonstrated a thick wall cavitary lesion in the right upper lobe and bilateral apical cavities. While on the floor he developed a pulsatile bleed from his abscess site. CT of the chest demonstrated a pseudoaneurysm of the brachiocephalic artery extending into the retromanubrial region. A stent to the right subclavian artery was then placed by vascular surgery. The patient was then taken to the operating room for biopsies of lymph nodes and debridement of the anterior chest. Biopsies revealed acid fast bacilli stain positive granulomatous osteomyelitis of the sternum. Sputum cultures demonstrated mycobacterium tuberculosis.

DISCUSSIONS: Uncontrolled hematogenous dissemination of Mycobacterium tuberculosis is referred to as miliary tuberculosis. There is a high degree of variability to the clinical presentation of miliary tuberculosis, from septic shock and acute respiratory distress syndrome, to failure to thrive or fever of unknown origin. Cutaneous disease is rare in miliary tuberculosis. Tuberculosis cutis milaris disseminata, which consists of 5-10 mm macules and papules is the most common presentation of cutaneous tuberculosis. Mycotic aneurysims of the ascending or descending aorta are also associated with disseminated tuberculosis. Embolization to the aortic wall vasa vasorum during hematogenous spread, or spread from a lymph node or vertebral osteomyelitis have been proposed as potential mechanisms for involvement of the aorta. Aneurysm rupture has been reported after the initiation of antituberculos chemotherapy.

CONCLUSION: Tuberculosis remains one of the leading causes of infectious morbidity and mortality in the world today. Though our patient had been living in the United States for the last 17 years, and could not recall any recent contacts with tuberculosis, his birth in an endemic region of the world for tuberculosis placed him at risk for tuberculosis infection. Reactivation tuberculosis can present in it's miliary form, with a wide range of presentations. In this patient, his lack of medical care, and attempts at self-therapy delayed his diagnosis, resulting in the advanced stage of his disease. He was stared on rifampin, isoniazid, pyrazinamide and ethambutol. His repeat sputum cultures are negative,he has been gaining weight, and his wound on his chest has been healing by secondary closure.

DISCLOSURE: Faisal Uddin, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):41A. doi:10.1378/chest.11047
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INTRODUCTION: Legionella micdadei or “Pittsburgh pneumonia agent (PPA)” (1) is a rarely isolated organism that accounts for over 60% of infection caused by non-pneumophilia Legionella species. Initially identified as the etiologic agent in an outbreak of pneumonia at the Pittsburgh VA in the early 1980s, it has since been described to cause severe pulmonary infection both in the immune deficient and normal host. We report a case of asymptomatic pulmonary nodules caused by Legionella micdadei in a patient with newly diagnosed CNS lymphoma on corticosteroid therapy.

CASE PRESENTATION: A 64-year-old woman presented to an outside hospital with confusion. Brain imaging revealed a basal ganglia lesion and stereotactic brain biopsy confirmed primary CNS lymphoma. She was started on intravenous dexamethasone (4 mg every 6 hours) and transferred to our institution ten days later. The pulmonary service was consulted for an abnormal CT chest. She denied any respiratory complaints. Past medical history: rheumatic fever in childhood. Social history: former smoker (5 pack years); no history of intravenous drug use. She denied exposure to tuberculosis; PPD status was unknown. She had no recent travel, animal or toxin exposure. Physical examination was unremarkable. CT scan of the chest done on admission to our institution to evaluate extent of disease revealed multiple bilateral pulmonary nodules that were new compared to a chest CT done two weeks earlier. Lab data: WBC count 25.5; LDH 407. Blood cultures and fungal serologies including aspergillus galactomannan and beta-D-glucan were negative. Urine legionella antigen was negative. A 2D-ECHO showed moderate aortic stenosis; no vegetations visualized. CT-guided needle biopsy of one lung nodule was non-diagnostic. Despite a one week course of empiric levofloxacin, a repeat chest CT showed increase in the size of nodules. Bronchoscopy with bronchoalveolar lavage (BAL) and VATS wedge lung biopsy of left upper and lower lobes nodules was done. BAL cultures were negative. Pathology of the lung biopsies revealed an inflammatory infiltrate with micro-abscesses and areas of organizing pneumonia, and no evidence of malignancy. Stains for microorganisms (Gram, AFB, GMS and Fite) were negative. Culture of biopsy material grew gram-negative rods that were further identified as Legionella micdadei. The patient was treated with a 3-week course of levofloxacin with subsequent radiographic improvement of the lung nodules, and is currently receiving treatment for CNS lymphoma.

DISCUSSIONS: Legionella micdadei is a rarely isolated gram-negative rod that is often weakly acid-fast in fresh specimens. In an initial case series of 26 patients (1), 88% were hospital-acquired infections, 50% of patients were immunocompromised for various reasons and 46% had an underlying malignancy. A majority (>85%) were symptomatic with fever and cough. While Legionella micdadei typically causes lobar or segmental infiltrates, varied radiologic presentations have been described, including pleural effusions and cavitary pneumonia. Pathology shows a neutrophilic alveolitis, microabscesses, and macrophages with intracytoplasmic bacilli. Organizing pneumonia within areas of fibrotic nodules has also been described (2).

CONCLUSION: This is a unique case of asymptomatic pulmonary nodules caused by Legionella micdadei in a patient immunocompromised by steroid use and underlying malignancy. Since Legionella micdadei is a rarely isolated organism, a high index of suspicion is needed to make the diagnosis. Legionella micdadei pneumonia should be considered in the differential diagnosis of causes of multiple pulmonary nodules.

DISCLOSURE: Hala Moukhachen, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):42A. doi:10.1378/chest.9858
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INTRODUCTION: Pulmonary alveolar proteinosis (PAP) is rare in the pediatric population, yet similar to adult cases results in abnormal alveolar surfactant accumulation. Hemophagocytic lymphohistiocytosis (HLH) is characterized by macrophage hemophagocytosis of various cells and their precursors in the bone marrow and other tissues. Clinical symptoms include cytopenias, hepatosplenomegaly, and fever. HLH may be associated with malignancies, genetic, infectious or autoimmune diseases. We report a case of a patient with Juvenile Idiopathic Arthritis (JIA) and later onset of HLH who developed PAP.

CASE PRESENTATION: A 7-year-old boy diagnosed with JIA at age four and HLH at age six, developed hypoxia and infiltrates on computed tomography (CT) at diagnosis of HLH. He was treated with multiple chemotherapeutic agents and systemic steroids resulting in remission of HLH and resolution of hypoxia. On later admission, he presented with a 1 day history of fever, chills, rash and increased ferritin level consistent with worsening HLH. Despite lack of supplemental oxygen requirement, he was tachypneic and easily fatigued with mild exercise. Pulmonary function tests showed severe progressive restrictive lung disease with a total lung capacity of 0.87L (60%) compared to 1.26 L (101%) from 18 months prior. CT of the chest showed bilateral nonspecific reticular nodular prominence. A lung biopsy demonstrated pulmonary alveolar proteinosis with evidence of chronic interstitial inflammation. Within days, he developed increasing dyspnea on exertion, but was still not hypoxic. Whole lung lavage was performed to optimize lung function in preparation for bone marrow transplantation, which yielded clinical and radiographic improvement. Subsequently, his course was complicated with pulmonary hemorrhage and acute respiratory failure requiring high frequency oscillatory ventilation and eventual extracorporeal membrane oxygenation (ECMO). Despite repeated whole lung lavages on ECMO, he was unable to transition to conventional ventilation and support was withdrawn. Autopsy confirmed diagnosis of PAP.

DISCUSSIONS: Pulmonary alveolar proteinosis is a rare disorder in children with three recognized categories: congenital, idiopathic, and secondary. Congenital PAP is a disorder caused by genetic alterations in surfactant proteins and is treated with lung transplantation. Idiopathic PAP results from presence of an autoantibody and treatments include whole lung lavage and granulocyte macrophage colony-stimulating factor (GM-CSF). Secondary PAP can be associated with a multitude of diseases including infectious, hematologic, and autoimmune. Symptoms of secondary PAP include progressive exertional dyspnea, malaise and low-grade fever. Radiologic findings are generally worse than patient’s symptoms. Bronchoalveolar lavage and biopsy yield definitive diagnosis. Pathologic findings include an abnormal accumulation of alveolar surfactant with a molecular structure similar, but not identical, to that of natural surfactant. Defective alveolar macrophage function impairs surfactant clearance. Treatment of the underlying disorder is necessary for curative treatment of secondary PAP. Pulmonary fibrosis is a typical finding late in the course of the disease.

CONCLUSION: This patient has two potential causes for PAP: JIA and HLH. Pulmonary decline was not encountered prior to diagnosis of HLH leading us to believe HLH was re-activated causing secondary PAP. A literature review has shown rare adult cases, but no pediatric cases of PAP secondary to HLH. HLH is a disorder of macrophage activating system, while PAP can be a result of dysfunctional alveolar macrophages. Our report suggests PAP should be considered in patients with HLH and deteriorating lung function.

DISCLOSURE: Shimoni Dharia, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):43A. doi:10.1378/chest.9818
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INTRODUCTION: IgG4-positive multiorgan lymphoproliferative syndrome (IgG4+ MOLPS) is a new clinical entity characterized by elevated serum IgG4, plasma cell infiltrates in tissues and good response to corticosteroid therapy. IgG4+ MOLPS includes autoimmune pancreatitis, sclerosing cholangitis, inflammatory pseudotumors of the lung, liver and breast and many other inflammatory conditions in multiple organs. We report the case of a patient with sclerosing cholangitis, presenting with inflammatory pseudotumors of the lung in which the diagnosis was made after twelve years from the initial symptoms.

CASE PRESENTATION: This 70 year-old man was referred to our institution in November 2009 for the elucidation of recurrent pulmonary infiltrates. In 1998 he was diagnosed with sclerosing cholangitis after a cholecystectomy for gallstones. Few sessions of dilatation of bile ducts by endoscopic retrograde cholangiopancreatography were done and a good symptomatic control was referred with ursodeoxicolic acid. There was no inflammatory bowel disease associated. In 2004 a lower lobe pulmonary nodule detected in a routine abdominal CT (with no signs of liver disease, but changes in size and echogenicity of the pancreas, suggesting chronic pancreatitis) was ressected and diagnosed as an inflammatory pseudotumor. Four years later he developed recurrent episodes of dyspnea and fatigue, with chest CT showing, along the years, different image patterns, predominantly bilateral peripheral nodules or subpleural consolidations with ground-glass halo. The symptoms and images responded to prednisone with recrudescence of the clinical picture following tapering of the corticosteroid. A search for autoimmune diseases was done several times, always with negative results. As commorbidities, the patient suffered from diabetes since 2000 and became insulin-dependent just after diagnosis. The association of an indolent cholangitis, a presumable autoimmune pancreatitis with endocrine insufficiency and inflammatory pseudotumors of the lung led us to the hypothesis of IgG4+MOLPS. The serum IgG4 concentration was 936 UI (reference values: <140 UI). A revision of the pulmonary nodule biopsy revealed a dense fibrosis with plasmacytic infiltration and focal lymphoid aggregation. Small number of eosinophils were seen (<5/high power field in most areas). Some vessels were partially or completely obstructed by inflammatory cells. Most of the infiltrating plasma cells were positive for IgG4, and IgG4/IgG ratio was over 90%. There was no light chain restriction (normal range of kappa: lambda) and no signs of malignancy.

DISCUSSIONS: A high serum IgG4 linked disease was first described in 2001, associated with sclerosing pancreatitis. This report was followed by others showing a similar involvement of multiple organs, such as pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate, denominated IgG4-related sclerosing disease. Recently, a new clinical entity, IgG4+MOLPS, has been proposed as an etiology for IgG4 plasma cell infiltrates in multiple organs and good response to glucocorticoids, as shown in this case.

CONCLUSION: When faced with inflammatory pseudotumors (histologically corresponding to plasma cell granuloma) or migratory pulmonary infiltrates with good response to corticosteroid therapy, the diagnosis of IgG4+MOLPS should be considered in the differential diagnosis. This may lead to a correct treatment and avoid unnecessary lung resections.

DISCLOSURE: Olivia Dias, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):44A. doi:10.1378/chest.10155
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INTRODUCTION: Common causes of dyspnea in the first year following lung transplantation include infection, acute rejection, bronchiolitis obliterans and bronchial strictures. Recurrence of primary lung disease is uncommon and rarely clinically significant. We report a case of recurrence of fibrosing nonspecific interstitial pneumonitis in a transplanted lung.

CASE PRESENTATION: A 42-year old female with fibrotic Nonspecific Interstitial Pneumonitis (NSIP) underwent bilateral lung transplantation. Post-operative course was complicated by grade 3 primary graft dysfunction, prolonged ventilator dependence with tracheostomy, heparin induced thrombocytopenia and deep vein thrombosis. She was discharged home after 5 weeks on prednisone, tacrolimus and azathioprine. Following discharge her exercise tolerance and allograft function improved. She was able to exercise on the treadmill without oxygen. Eight weeks after transplantation she underwent diagnostic bronchoscopy for new onset shortness of breath and was found to have acute rejection (A2B0). Histiocytic infiltrates were noted on biopsy at that time and were thought to be consistent with early post transplant findings. She was treated with methylprednisolone and azathioprine was switched to mycophenolate. Follow up biopsies 3 weeks later showed A1B0 rejection with increasing intraalveolar macrophages and evidence of early interstitial fibrosis. All cultures were negative. Fluorescence in situ hybridization confirmed recipient origin of the infiltrating macrophages. Over the next several months the patient developed exercise induced hypoxemia and six month surveillance biopsies showed features of mixed desquamative and non-specific interstitial pneumonitis, pathologically identical to her native lung disease.

DISCUSSIONS: Recurrence of primary lung disease in a transplanted lung is rare complicating less than 1% of all lung transplants. While recurrence of systemic disease like sarcoidosis and lymphangiomyomatosis has been described, the course of disease after recurrence is usually indolent. Of the interstitial lung diseases, only two cases of recurrence of desquamative interstitial pneumonitis (DIP) and one of giant cell interstitial pneumonitis have been reported. The cause of recurrence is unknown. Possible etiologies include hypersensitivity, autoimmunity, viruses, induction therapy, recurrent aspiration, and smoking status of donor. In this case, pathologic accumulation of recipient macrophages was evident on transbronchial biopsies 2 months post transplant. This macrophage population continued to expand and subsequent biopsies revealed intraalveolar macrophage with concomitant interstitial fibrosis.

CONCLUSION: Recurrence of clinically significant interstitial lung disease after lung transplant is rare. Recurrence of interstitial pneumonitis occurred after a double lung transplant. Host factors likely play a major role, evidenced by pathologic accumulation of recipient macrophages.

DISCLOSURE: Surya Bhatt, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):45A. doi:10.1378/chest.11028
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INTRODUCTION: IgG4 related sclerosing disease is an inflammatory and fibrosing disorder, characterized by lymphoplasmacytic inflammation with infiltration of various organs, including the pancreas, bile ducts, lung, kidney, and retroperitoneum. We report a case of a young woman with IgG4 related systemic sclerosing disease, who presents with dysphagia mimicking esophageal cancer and pulmonary infiltrates.

CASE PRESENTATION: A 31 year old previously healthy Dominican female was referred for further evaluation of a 3-month history of progressive dysphagia and weight loss. She was a nonsmoker and had no known tuberculosis exposure; her PPD status was unknown. Physical examination revealed a cachectic appearing young female, but was otherwise unremarkable. Barium swallow revealed mid-esophageal constriction. CT chest revealed a mid-esophageal mass below the level of carina, scattered nodular infiltrates in the right upper and right middle lobes, as well as calcified mediastinal lymph nodes. PET scan revealed FDG uptake in the esophageal mass (SUV 5.2), a dilated upper esophagus and mild FDG uptake in the pulmonary nodules (SUV ranging 2.0-4.0). Upper GI endoscopy revealed mid-esophageal constriction secondary to extrinsic compression; no endoluminal mass lesions were detected. Bronchoscopy with endobronchial ultrasound guided mediastinal lymph node biopsy was nondiagnostic. VATS lung biopsy of the right upper and right middle lobes was performed. Histopathology revealed multinodular lymphoplasmacytic infiltrates with bronchiolocentric distribution and distortion of alveolar structures by diffuse sclerosing inflammation. Prominent perivascular sclerosing inflammation was present, obliterating small to medium-sized vessels. Immunostaining revealed strong staining for IgG4 with mixed infiltrate of lymphocytes, plasma cells, histiocytes and neutrophils. PPD was negative. Urine Histoplasma antigen was negative. A complete metabolic profile and complete blood count were within normal limits. ACE level was within normal limits and a rheumatologic panel was negative. Total serum IgG was elevated (2280 mg/dl, normal 600-1500), as was the serum IgG4 level (414 mg/dl, normal 6-121), thereby confirming a diagnosis of IgG4 related sclerosing disease. Treatment with prednisone was initiated (1 mg/kg daily). After nearly two months of therapy, the patient’s dysphagia resolved. Radiographic imaging revealed a decrease in the size of the mediastinal mass, improvement in the pulmonary infiltrates and a decrease in serum IgG4 titers.

DISCUSSIONS: Originally implicated in causing autoimmune pancreatitis, IgG4 related sclerosing disease has since been implicated in inflammation involving multiple anatomic sites, including the lung, kidney, retroperitoneum, mediastinum, biliary tract, salivary and lacrimal glands (1). Solid nodules, ground glass opacities, alveolar interstitial and bronchovascular infiltration are four characteristic patterns of lung involvement, although pleural disease and mediastinal adenopathy have been observed; most patients have more than one pattern of involvement (2). Diagnosis is based on histopathologic findings of polyclonal lymphoplasmacytic infiltration along intrapulmonary connective tissue in the interstitium, interlobular septa and bronchovascular bundles, with strongly positive immunostains for IgG4. Total serum IgG and IgG4 levels are elevated. Alternative diagnoses, such as connective tissue disorders, must be excluded. In our patient, the esophageal mass was attributed to mediastinal adenopathy resulting in extrinsic esophageal compression, and the pulmonary infiltrates to IgG4 related interstitial lung disease. The disease is generally steroid-responsive, although cyclosporine has been used anecdotally in at least one case of steroid resistance.

CONCLUSION: IgG4 related sclerosing disease should be entertained in the differential diagnosis of individuals presenting with non-malignant causes of mediastinal adenopathy and/or pulmonary infiltrates, characterized by polyclonal lymphoplasmacytic inflammation.

DISCLOSURE: Pramod Krishnamurthy, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):46A. doi:10.1378/chest.10305
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INTRODUCTION: Diffuse alveolar hemorrhage (DAH) can be an acute, life threatening presentation of several diseases - of which, the most common cause is pulmonary vasculitis. Microscopic polyangitis (MPA) is the least common cause of pauci-immune pulmonary capillaritis presenting with DAH and is associated with perinuclear antineutrophil cytoplasmic autoantibodies (pANCA). We present an unusual case of MPA which was pANCA negative by indirect immunofluorescence assay (IFA) and was positive for myeloperoxidase (MPO) by enzyme immunoassay (EIA) and required a renal biopsy for establishing the diagnosis.

CASE PRESENTATION: A 31 year-old non-smoker caucasian female with a history of Type I diabetes mellitus and hypothyroidism presented with fever, dyspnea and hemoptysis. Initial O2 saturation was 82% on room air; patient required oxygen supplementation with 40% venturi mask at 10 L/min to increase O2 saturation to 98%. Hemoglobin upon admission was 10.7 g/dl with MCV of 87 fl (normocytic anemia) and urinalysis was positive for hematuria. Chest X-Ray revealed diffuse alveolar infiltrates with bronchograms in all lobes of the right lung. Chest CT scan demonstrated diffuse alveolar infiltrates in all lobes of the right lung and infiltrates in the left upper and lower lobes. Patient was intubated and bronchoalveolar lavage (BAL) was performed. Serial aliquots during BAL demonstrated progressively hemorrhagic fluid in three separate bronchopulmonary segments. BAL was positive for hemosiderin laden macrophages. Creatinine steadily increased from a baseline of 1.2 mg/dl to 1.9 mg/dl with decreasing urine output. Hemodialysis was started on day 5 of admission. Laboratory evaluation revealed positive anti-nuclear antibody (1:160), with negative serological work-up for anti-cyclic citrullinated peptide (Anti-CCP), anti-glomerular basement membrane antibodies (anti-GBM), Anti-Smith antibodies, double stranded DNA, anti-Ro and pANCA and cytoplasmic staining ANCA (cANCA). Patient underwent renal biopsy that revealed glomerulonephritis with cellular crescents and necrosis consistent with pauci-immune glomerulonephritis. MPO detection by enzyme linked immunosorbent assay (ELISA) was positive and confirmed the diagnosis of MPA. Patient was started on methylprednisone 1000 mg IV daily for 5 days with lack in improvement of respiratory failure. Cyclophosphamide was started and patient was extubated after 2 days of initiating cyclophosphamide. She was discharged twenty-two days after admission. Two months after discharge, patient has regained her renal function and there have been no further episodes of hemoptysis.

DISCUSSIONS: Our patient presented with respiratory insufficiency and acute renal failure. Differential diagnosis of pulmonary-renal syndrome includes Wegner's granulomatosis (WG), Goodpasture's disease, systemic lupus erythematosus (SLE), Churg Strauss syndrome (CSS), Henoch Schonlein Purpura, and MPA. WG is ANCA positive in more than 90% of the cases, with cANCA positivity in more than 75% of the cases. Our patient did not have the involvement of the upper airways or the presence of necrotizing granulomas on biopsy making WG less likely. Goodpasture's disease is typically seen in men with history of smoking and almost all patient have anti-GBM antibodies detectable by ELISA . Renal biopsy findings of pauci-immune vasculitis were not consistent with SLE and patient lacked a history of asthma and eosinophilia suggestive of CSS. HSP occurs more commonly in children and is associated with rash in the lower extremities - features not seen in our patient. MPA was diagnosed in the presence of a negative pANCA by IFA - feature seen in less than 10% of all cases. In patients with features suggestive of pauci-immune capillaritis and absence of granulomas, a negative ANCA should be confirmed with EIA using purified MPO as substrate.

CONCLUSION: This is a unique case of pauci-immune glomerulonephritis that was confirmed as MPA on the basis of a positive EIA for MPO. Despite the absence of pANCA by IFA, MPA must be considered as part of the differential diagnosis for pauci-immune pulmonary capillaritis.

DISCLOSURE: Aditya Gupta, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):47A. doi:10.1378/chest.10748
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INTRODUCTION: Acute fibrinous and organizing pneumonia (AFOP) is a histologically distinct form of lung injury first described in 2002. Less than 20 cases have been reported since Beasley’s original description and case series. We report a case of idiopathic AFOP and review the literature on this poorly understood entity.

CASE PRESENTATION: A 55 year-old woman active smoker with well-controlled asthma presented with a complaint of progressive dyspnea for one week. On physical exam, she was afebrile with a respiratory rate of 20, oxygen saturation of 85% on ambient air and bilateral, diffuse crackles on chest auscultation. White blood cell count (WBC) was 6.2 K/mm3, and her chemistry and liver function panels were unremarkable. Chest radiograph and computed tomography of the chest were notable for bilateral patchy air-space opacification in the middle and lower lung fields. Despite antibiotic therapy, her respiratory status worsened over the next 24 hours and she required increased oxygen supplementation and ICU care. Bronchoscopy with bronchoalveolar lavage yielded negative cultures and a cell count of: WBC 565, 3% lymphocytes, 6% histiocytes, 26% macrophages, 63% granulocytes with 2% eosinophils. The patient was started on prednisone and slowly improved. An open lung biopsy was performed as radiographic findings persisted.Histology revealed: Presence of multiple intra-alveolar fibrin plugs. Organizing fibrinous pneumonia and acute fibrinous pleuritis. Background lung shows respiratory bronchiolitis.

DISCUSSIONS: In 2002, Beasley described AFOP, a form of acute lung injury with a histologic pattern that differed from diffuse alveolar damage (DAD), organizing pneumonia (OP) and eosinophilic pneumonia (EP). The dominant histological finding of AFOP is the presence of intra-alveolar fibrin (fibrin "balls") and patchy organizing pneumonia. The hyaline membranes that are identified in DAD are not observed. Fibroblastic Masson bodies within the alveolar spaces, ducts and bronchioles are present but not abundant, and eosinophils are rare. Among the 17 cases that comprised Beasley’s initial report of AFOP, the most common symptoms reported were dyspnea, fever, and cough. The most common radiographic finding was bilateral basilar infiltrates. Therapy included antibiotics and steroids. Nine cases were fulminant and died of the disease; eight were subacute. Since Beasley’s initial description, 12 individual case reports of AFOP have been published. All patients had symptoms for less than 2 months, with cough and dyspnea as the most common. Radiographic abnormalities were basal/lower lobe predominant in 6 patients and bilateral in 8 patients. Identified exposures or associated conditions included medications (abacavir and decitabine), hematopoietic stem cell transplant (HSCT), acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML) and juvenile dermatomyositis (JDM). In 6 cases, an attributable cause or association could not be identified. Patients were treated with medications including corticosteroids, mycophenolate mofetil, cyclophosphamide, and cyclosporine. Among the 12 case reports, two patients required mechanical ventilation, and two patients died. In addition to these individual case reports, a published description of autopsy findings in 20 patients with SARS reported that 6 of the 20 patients had AFOP on histology. An additional 6 patients had a combination of AFOP and DAD, while the remainder had DAD. Clinical and radiographic details were not reported.

CONCLUSION: AFOP represents a histologically distinct subset of lung injury. Our case represents the 7th case of idiopathic AFOP reported since Beasley’s initial description. Common findings in patients with AFOP are: dyspnea and cough acutely/subacutely, bilateral and basilar predominant radiographic abnormalities. AFOP can result from a variety of insults or be idiopathic. Its varied response to anti-inflammatory therapy suggests that it has a better prognosis than DAD. AFOP may be a part of a spectrum of lung injury that both clinically and histological lies between COP/BOOP and DAD.

DISCLOSURE: Rokhsara Rafii, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: pneumonia

Case Reports: Tuesday, November 2, 2010

Chest. 2010;138(4_MeetingAbstracts):48A. doi:10.1378/chest.10707
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INTRODUCTION: Tracheoesophageal fistula (TEF) is an uncommon problem that may present in adulthood as a latent manifestation of a congenital abnormality or as an acquired complication of a disease state, most commonly esophageal and lung malignancy. We report a case of massive tracheoesophageal fistula in a patient with rectal adenocarcinoma metastatic to the lungs, treated with radiation and a chemotherapy regimen including Bevacizumab.

CASE PRESENTATION: A 41 year-old white female presented to the hospital with two weeks of progressive, severe cough, sputum production as well as nausea and vomiting with any oral intake. She had a history of rectal adenocarcinoma metastatic to lung. Her initial cancer therapy two years prior included radiation followed by resection with adjuvant 5-fluorouracil, leucovorin, and oxiplatin. Since discovery of bilateral pulmonary metastases approximately sixteen months prior to presentation, she received 5-fluorouracil, leucovorin, irinotecan, bevacizumab, and capecitabine as well as stereotactic radiation to lung nodules, the last treatment occurring nine months prior. On admission, her chest x-ray revealed infiltrates in the right middle and right lower lobes. After no improvement despite several days of broad spectrum antibiotics a chest CT revealed a large tracheoesophageal fistula that appeared to communicate with a cavitary lung lesion. She was then transferred to our institution and underwent bronchoscopy. Bronchoscopy revealed complete obliteration of the mucosa circumferentially from mid trachea to proximal mainstem bronchi bilaterally. Further, obliteration of the carina and posterior membrane of the right upper lobe bronchus was noted with direct communication into the mediastinum and esophagus. There was no visible tumor and forceps biopsies were negative for malignancy. She subsequently underwent PEG tube insertion and an esophageal stent was placed. She was started empirically on amphotericin and voriconazole in addition to broad spectrum antibiotics. Her respiratory cultures grew only candida albicans but were otherwise negative. Four days following bronchoscopy her respiratory status declined and she required intubation. Ultimately, her family opted to withdraw care.

DISCUSSIONS: Tracheoesophageal fistula (TEF) remains a rare complication of malignancy with the majority of cases resulting from direct invasion by esophageal carcinoma or primary lung neoplasm. Less commonly, TEF results from instrumentation and local treatment including embolization or radiation. TEF carries a poor prognosis and prompt diagnosis and intervention is critical to minimize soiling of the airway and further complications. However, diagnosis can be difficult as patients typically present with pulmonary signs and symptoms mistakenly attributed to underlying malignancy or anti-neoplastic therapy. Bevacizumab is a frequently used component of the chemotherapy regimen for colorectal cancer and non-small cell lung cancer. Gastroenteral tract perforation and fistulization is a more commonly reported side effect of bevacizumab than respiratory complications. Rare reports of TEF following bevacizumab exist. A recent publication suggests that the anti-angiogenesis of Bevacizumab combined with mucosal injury of the esophagus contributes to TEF formation2;. Our patient is unique in the severity of tracheal injury when compared to other reported cases. Additionally, her radiation exposure was more remote than most, but not all, previously reported cases of TEF post bevacizumab.

CONCLUSION: In patients undergoing treatment for intra-thoracic malignancy who present with pulmonary complaints, the differential diagnosis is broad. Acquired TEF is a rare complication of malignancy but carries a poor prognosis and relies on early diagnosis for successful management. Our case suggests that a high suspicion for TEF should exist in patients with pulmonary complaints who received bevacizumab, even when radiation therapy is distant to presentation.

DISCLOSURE: Thomas Iden, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):49A. doi:10.1378/chest.10299
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INTRODUCTION: Stridor is a medical emergency that can be related to tracheal stenosis, vocal cord edema, foreign body presence or laryngeal dyskinesia. We present an unusual case of post-intubation stridor caused by obstructive fibrinous tracheal pseudomembrane (OFTP).

CASE PRESENTATION: A 38-year-old woman was admitted with peripartum cardiomyopathy and respiratory failure 18 days after Cesarean section. Her pregnancy had been complicated by pre-eclampsia, HELLP syndrome (hemolysis, elevated liver enzymes and low platelets) and placental abruption. She returned to hospital a week after discharge with worsening dyspnea and orthopnea requiring endotracheal intubation and mechanical ventilation for 3 days while her cardiac function was optimized. A week after extubation she developed acute onset of stridor. Laryngoscopy revealed no evidence of angioedema or vocal cord dysfunction. She had intermittent stridor for the next 12 hours despite racemic epinephrine and steroids. Pulmonary function testing (PFT) revealed a very severe obstructive ventilatory defect with a FEV1 of 0.65 L (19% of predicted). Flow volume loop revealed consistent plateaus in both the inspiratory and expiratory limbs indicative of a fixed airway obstruction . A bedside bronchoscopy revealed 90% subglottic narrowing. She was taken to the operating room for direct laryngoscopy and anticipation of emergent tracheostomy. Rigid bronchoscopy revealed an annular membrane of necrotic debris (~3 cm long x 0.3 cm thick) separated from the trachea by a longitudinal plane. This membrane obstructed the trachea in a ball-valve manner and was easily separated from the posterior wall of the trachea with cryotherapy and flexible bronchoscopy leaving a widely patent tracheal orifice. Histopathology revealed “membranous fragments of fibrino-inflammatory debris with focal areas of reactive squamous epithelium”. Her stridor resolved immediately. PFTs one week later revealed only moderate obstruction (FEV1 2.2 L ; 66% of predicted) and a normal flow volume loop.

DISCUSSIONS: OFTP is a rare potential complication of endotracheal intubation. Only a few case reports and one case series of 10 patients have described the condition, which presented as intermittent positional dyspnea or stridor starting between 3 hours to 9 days after short duration endotracheal intubation (<24 hours to 6 days). A thick whitish tubular pseudomembrane molded the tracheal wall and caused an intermittent ball-valve tracheal obstruction. In reported cases, mechanical removal was curative with avoidance of the development of secondary tracheal stenosis. It is postulated that OFTP may be the initial stage in a process triggered by ischemic damage from high pressure endotracheal cuffs leading to inflammation, infarction and ultimately necrosis of the tracheal mucosa that may eventually result in tracheal stenosis. Diagnosis of OFTP can be a challenge because the clinical picture is often misleading when symptoms of dyspnea and stridor are positional and intermittent secondary to the ball-valve obstruction from the pseudomembrane. Symptoms can be misdiagnosed as bronchial asthma, vocal cord dysfunction or glottic edema. Flow volume loops are a valuable non-invasive test used to diagnosis upper airway obstruction during symptoms. Bronchoscopy is key to the diagnosis and management but should be carried out in an operating room with surgeons trained in emergency airway access on stand by. Removal of the pseudomembrane completely resolves the problem.

CONCLUSION: This patient developed upper airway obstruction secondary to an OFTP after short-term endotracheal intubation. The development of intermittent and positional post-extubation stridor and a fixed obstruction on flow-volume loop should alert clinicians to include OFTP in the differential diagnosis of upper airway obstruction. Removal of the pseudomembrane by interventional bronchoscopic procedures is key to the management and resolution of symptoms.

DISCLOSURE: Brenda Rice, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: trachea , stridor
Chest. 2010;138(4_MeetingAbstracts):50A. doi:10.1378/chest.9914
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INTRODUCTION: Endotracheal schwannomas remain an exceedingly rare cause of airway obstruction. The first reported case of neurogenic intrapulmonary tumor was in 1914. Patients are generally asymptomatic until the tumor reaches a critical size, at which point, obstructive symptoms are seen. Since 1950, only a few dozen of these cases have been reported. We present a case of a man who presented with shortness of breath and cough and was found to have an obstructing tracheal schwannoma.

CASE PRESENTATION: A 70-year-old man presented to the emergency department with complaints of productive cough and dyspnea. His past medical history was significant for Chronic Obstructive Pulmonary Disease (COPD), 20-pack-year smoking history, hypertension, and hyperlipidemia. He was admitted for presumed acute COPD exacerbation and physical exam was notable for mild respiratory distress. Despite treatment with bronchodilators, antibiotics, and intravenous glucocorticoids, he continued to experience shortness of breath. While supine, stridor was audible and radiated to both lungs. A Computed Tomography (CT) scan of the neck and thorax revealed a 2.2 x 1.4 cm endotracheal mass. No lung nodules or lymphadenopathy was seen. Pulmonary Function Test (PFT) demonstrated flattening of the flow volume loop. Flexible bronchocopic evaluation revealed a pedunculated endobronchial mass obstructing 90% of the tracheal lumen. Rigid bronchoscopy was subsequently performed with excision of the tracheal mass. Characteristic histologic elements, and diffuse staining of the protein S-100, established the diagnosis of schwannoma. The remainder of his hospitalization was uneventful. Post resection PFT revealed resolution of the central airway obstruction. Surveillance bronchoscopy at one year was negative for recurrence of the lesion.

DISCUSSIONS: Primary tracheal tumors are less prevalent than bronchial tumors. Two thirds of all primary tracheal tumors are evenly divided between squamous cell carcinoma and adenoid cystic carcinoma. The remaining types of primary tracheal tumors encompass a wide spectrum of lesions including malignant, intermediate, and benign tumors (1). Primary neurogenic tumors of the trachea (schwannoma) are extremely uncommon and symptoms of airway obstruction predominate (2). Generally, patients are symptomatic only when more than half of the cross sectional area of the trachea is obstructed (1). In light of this, incidental masses may be found in entirely asymptomatic patients. The most common presenting symptom associated with these tumors is dyspnea. Chest X-Ray is interpreted as normal in 30-75% of patients, thus, leading to the inappropriate treatment of presumed asthma and/or COPD (1). When tracheal tumor is suspected, the most important and preferred test for diagnosis is rigid bronchoscopy (1). Compared to flexible bronchoscopy, rigid bronchoscopy provides more secure control of the obstructed airway and control of potential bleeding that may occur after biopsy (1). When present, schwannomas are typically circumscribed and encapsulated tumors (2). Histologically, schwannomas have regions of high and low cellularity called Antoni A and Antoni B areas, respectively. The protein S-100 can also be stained to confirm the diagnosis. Strong expression of S-100 is associated with benign tumors, whereas minimal or only focal expression of this protein is suggestive of a malignant lesion (2).

CONCLUSION: Despite their low prevalence, tracheal tumors should be suspected when considering patients with persistent symptoms of dyspnea and obstruction. Follow up surveillance bronchosopies are recommended as the long-term natural progression and recurrence rates of tracheal schwannomas are unknown.

DISCLOSURE: Gabriel Gomez, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: neurilemmoma , trachea
Chest. 2010;138(4_MeetingAbstracts):51A. doi:10.1378/chest.9460
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INTRODUCTION: Cervico-thoracic mass in the pediatric population is uncommon and has a broad differential diagnosis. We present a case of an extremely large cervico-thoracic mass causing airway obstruction in a 3-day-old otherwise healthy male infant.

CASE PRESENTATION: Our patient was a 3-day-old male infant born to a 17-year-old G1P1 female. Pregnancy was uncomplicated with routine prenatal care. Prenatal labs were notable for negative Group B streptococcus, hepatitis B, rubella and a nonreactive VDRL (Venereal Disease Research Laboratory.) He was born by vaginal delivery at 39 and 5/7 weeks gestation with APGARS of 9 and 9 at 1 and 5 minutes. Initial course in the newborn nursery was unremarkable with no signs of respiratory distress or feeding difficulty. He was discharged home after 2 days. Over the following 24 hours the patient developed difficulty feeding with increasing lethargy and intermittent apnea at home and was brought to the local emergency room for evaluation. On exam, he was found to have a large, soft neck mass with biphasic stridor. A CT scan noted a cystic lesion extending from the anterior mediastinum to the pre-vertebral space and superiorly into the hypopharyngeal region with a homogenous fluid layer. The mass caused prominent rightward and posterior laryngeal and tracheal deviation. Given this finding, he was transported to our institution for evaluation by pediatric otolaryngology. On arrival, he was on nasal cannula oxygen but continued to have intermittent desaturations, more prominent with agitation. Following evaluation by pediatric otolaryngology, he was taken to the operating room for mass resection. Airway control was obtained through awake intubation using rigid bronchoscopy after failed direct laryngoscopy. During resection, the lesion was noted to extend from the pyriform sinus and contained foul smelling, purulent fluid. Tissue pathology revealed a thymic cyst with components of thyroid and parathyroid tissue. The fluid grew multiple organisms including Viridans streptococci, Haemophilus parainfluenzae, and Streptococcus anginosus, consistent with oral flora. Post-operatively, he developed hypocalcaemia responsive to vitamin D and calcium supplementation, which was determined to be secondary to parathyroid tissue removal. Evaluation for 22q.11 deletion by FISH (fluorescent in situ hybridization) was negative. The patient recovered from his surgery without complications and was discharged from the hospital on day of life 13.

DISCUSSIONS: Differential diagnosis of cervico-thoracic masses includes congenital lesions, inflammatory lesions, traumatic lesions, and tumors. Congenital lesions are most frequent in the pediatric population with lymphangioma being most common. Congenital thymic cysts compose less than 1 percent of pediatric cervico-thoracic masses. Congenital thymic cysts arise from the anomalous development of the thymopharyngeal duct. The cyst occasionally connects to the pyriform sinus. In the only reported case in the literature, a 6-year-old presented with neck swelling and pain. Similar to our patient, airway control was a significant issue given the prominence of airway compression. Although final diagnosis in our patient was by pathology, he had features suggestive of a thymic cyst in the homogenous appearance of the fluid with an air fluid level. Nevertheless, it was important to work up immunologic and genetic etiologies given the early appearance of an infected, midline mass.

CONCLUSION: The differential diagnosis for cervico-thoracic lesions is broad. In addition to diagnosis, clinicians must carefully regard the airway impact of such masses. Clinical features and radiographic findings assist with diagnosis, but pathology may be required.

DISCLOSURE: Benson Hsu, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):52A. doi:10.1378/chest.11041
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INTRODUCTION: Esophageal cancer is one of the least studied and sixth most common cause of cancer death. Most patients present with advanced disease which has a poor prognosis and a 5-year survival rate ranging from < 10% - 14%. In addition to a limited life expectancy, patients may develop many potentially incapacitating complications such as malignant dysphagia, airway stenosis, esophagotracheal fistulation or other impediments related to therapeutic or palliative interventions.

CASE PRESENTATION: A 61 year old female presented to the Emergency Department with one week of worsening dyspnea. She reported a cough of similar duration but denied fever, chills or night sweats. She had a past history of advanced esophageal cancer with placement of a coated, expandable metallic esophageal stent six months prior for dysphagia related to esophageal obstruction. Initial laboratory data showed mild leucocytosis, while the admission chest x-ray showed a small left pleural effusion. The patient became unresponsive 6 hours after admission and was subsequently intubated. Her clinical status did initially improve however she had a prolonged hospital course that was complicated by the development of pulmonary embolism and atrial fibrillation with rapid ventricular response. On day 29 of her hospital course, she developed worsening respiratory distress and the ensuing chest x-ray showed complete opacification of the left hemithorax. The patient was transferred to the medical intensive care unit and a bronchoscopy was performed which revealed fistulation of the esophageal stent at the level of the left main bronchus superorly into the lumen of the trachea. No intervention could be performed at that time so the procedure was terminated. A subsequent CT chest performed revealed complete collapse of the left lung with worsening airspace disease of the right lung. Due to the patient’s grave prognosis, the family opted for comfort care. The patient subsequently expired the following day.

DISCUSSIONS: The majority of patients with esophageal cancer present with dysphagia (74%), odynophagia (17%) and weight loss (57%). Less common presentations which may indicate extensive and unresectable disease include dyspnea, cough, hoarseness and retrosternal, back or right upper abdominal pain. Less than 50% of patients are candidates for surgical resection. For the greater majority of patients, palliation is the treatment of choice. The placement of an esophageal stent is a common palliative modality used in the treatment of dysphagia resulting from esophageal tumor obstruction, particularly in patients with advanced disease and poor functional status, unable to tolerate chemotherapy or radiation therapy, or in whom prior therapeutic interventions failed. Over 90% of patients with dysphagia experience relief after receiving expandable metal stents. Nevertheless, placement of esophageal stents have inherent risks: a) intra-procedural complications comprise those related to conscious sedation, aspiration, stent malposition and esophageal perforation; b) immediate complications include chest pain, bleeding, foreign body sensation, perforation and tracheal compression leading to airway compromise; c) late complications such as in our patient can occur up to 2 years post-insertion and consist of stent migration, esophagotracheal fistulation, pneumomediatinum, bleeding, overgrowth of granulation tissue or tumor, gastroesophageal reflux, aspiration pneumonitis and stent occlusion. It is important to note that placement of these stents decrease the rate of early but not late complications; the latter being higher in patients previously treated with chemotherapy, radiation or both.

CONCLUSION: Expandable metallic stents are effective for the palliation of malignant esophageal obstruction. However, they are associated with a number of late and frequently life-threatening complications. These complications may not be completely unavoidable, nevertheless, early recognition is vital to effectively managing these patients with such a limited life expectancy.

DISCLOSURE: Ahmad Abdel-wahed, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):53A. doi:10.1378/chest.10290
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INTRODUCTION: Pulmonary artery sling is a congenital vascular anomaly in which the pulmonary arterial circulation to the lung arises from the pulmonary artery (PA) of the contralateral lung rather than a mainstem PA. Tracheal compression occurs as the anomalous pulmonary artery traverses the mediastinum posterior to the airway. This anomaly, while often seen in children, rarely remains undiagnosed into adulthood. We describe a case of PA sling presenting with respiratory symptoms in an adult.

CASE PRESENTATION: A 29 year old female developed severe dyspnea, cough and wheezing following caesarean section for the delivery of her third child. She had a history of quiescent asthma and seasonal allergies. The patient resides in a rented house with some mold, but endorses no recent travel or exposure history. She was treated with empiric corticosteroids and underwent bronchoscopy for broncho-alveolar lavage. Cultures grew Klebsiella and she was treated with a course of antibiotic therapy. Despite an appropriate anti-infective regimen, her symptoms worsened, with a persistent ‘barking’ cough and exertional dyspnea with exercise limitation. She underwent thorough allergy testing which revealed allergy to grasses, trees and mold. However, IgE levels, aspergillus specific antigens, ANCA and CRP were all negative, arguing against chronic allergic reaction, allergic broncopulmonary aspergillosis or Churg-Strauss vasulitis. A repeat dynamic bronchoscopy was performed to assess for a possible component of tracheobronchial malacia. This study revealed a focal collapse in the membranous wall of the distal trachea and proximal left mainstem bronchus. An airway computed tomography scan identified a vascular sling. The anomalous left PA originates from the proximal right PA then passes between the trachea and esophagus, resulting in extrinsic compression of the distal trachea. The patient underwent surgical resection of the vascular sling with re-anastomosis to the main PA. She has recovered well without persistent respiratory symptoms.

DISCUSSIONS: Vascular rings are rare and usually diagnosed early in life. Symptoms range from absent to significant respiratory distress, especially in the newborn. Airway symptoms predominate early in life while dysphagia may be the primary symptom in older patients. PA slings are among the least common of the vascular rings, comprising only 10% of diagnosed cases in large series (insert ref from CMH series here). This anomaly is created by formation of the left PA from the right sixth vascular arch (rather than the left), resulting in a left PA arising from the posterior aspect of the right PA. There is also a strong embryologic association between pulmonary artery sling and development of complete tracheal rings, accentuating the tracheal stenosis. Surgical repair is focused on the restoration of the normal anatomic relationship between the airway and pulmonary vasculature. Exposure is obtained by median sternotomy and cardiopulmonary bypass is usually required. The left PA is disconnected from the right and anastomosed to the proximal main PA. Careful examination of the trachea is required as segmental resection and reconstruction by slide tracheoplasty is often required due to the significant airway stenosis which often accompanies these anomalies (1). Routine transthoracic echocardiography is recommended to exclude associated congenital heart defects (2).

CONCLUSION: Diagnosis of vascular rings requires a high index of suspicion with typical symptoms of noisy breathing and respiratory distress. CT imaging with contrast is the preferred diagnostic modality to indentify the anomaly and define the anatomy. Surgical correction is the treatment of choice, often requiring tracheal reconstruction. The rare presentation of our patient in adulthood illustrates the spectrum of age and delay in symptoms that are possible.

DISCLOSURE: David Odell, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):54A. doi:10.1378/chest.10297
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INTRODUCTION: Bronchopleural fistula is an uncommon severe complication encountered post pneumonectomy and is associated with high morbidity and mortality. We describe the 4th report of successful closure of Bronchopleural fistula through Amplatzer ASD occlusion device in the entire literature.

CASE PRESENTATION: 66-year-old male underwent left-sided pneumonectomy for non-small-cell lung cancer in August 2009. Postoperative course was complicated by development of a Bronchopleural fistula leading to operative repair with latissimus dorsi and intercostal muscle flap. Recurrence of fistula was again documented in 3 weeks of the procedure and another open attempt was performed with evacuation of the chest cavity and an intercostal muscle flap was done to repair the defect inthe bronchus. Procedure was unsuccessful with recurrence of the Bronchopleural fistula in following 3 weeks. Patient was then hospitalized and IV antibiotics were instituted. A decision was then made to proceed with Endobronchial placement of Amplatzer ASD Occluder device.PROCEDURE: Patient was intubated with single lumen ET tube and general anesthesia was induced on January 17 2010. Bronchoscope was advanced and examination of left bronchial stump revealed the persistent Bronchopleural fistula. Serial measurements to measure the diameter of the aperture in the bronchus and fluoroscopic markers were placed on the skin of location of Bronchopleural fistula. Based on the careful measurements a 6-French guiding sheath was passed through the therapeutic bronchoscope and placed across the Bronchopleural fistula opening. Under fluoroscopic guidance the ASD occlusion device was then passed and deployed. To provide airtight occlusion 2 ml of Tissel and 2ml of BioGlue were infiltrated into the sub sinus of the device. Patient was then extubated successfully and was sent home.Follow up: Our patient has had 3 bronchoscopies since his procedure in January 2010 and has shown no evidence of recurrence of the Bronchopleural fistula. Excellent healing is documented on repeated bronchoscopies.

DISCUSSIONS: Bronchopleural fistula (BPF) after pneumonectomy represents the most dreaded complication in thoracic surgery. Although the incidence has decreased in the recent years due to understanding of the bronchial healing process , diagnosis and therapeutic management of these fistulas remains a formidable therapeutic challenge, which has been approached with a variety of surgical techniques. BPF may be treated by a range of surgical and medical techniques, including chest drain, Eloesser muscle flap, omental flap, transsternal bronchial closure, thoracoplasty, and prolonged antibiotic regimens.The use of bronchoscopy has been reported for the delivery of biological glue, coils, covered stents, and sealants. The degree of success is variable, and depends on the size and proximity of the fistula and the underlying disease. In this case, we describe a novel method of BPF closure using the Amplatzer device, which is commonly used for transcatheter closure of atrial septal defects (ASDs) with remarkable results when it was deployed endoscopically using flexible bronchoscopy. Kramer et al and Gulkarov et al have described successful use of this device in case of BPF post pneumonectomy .

CONCLUSION: The continued success at closure of BPF with Amplatzer warrants future studies to assess their use as the one of the first line treatment modalities for Bronchopleural fistula post pneumonectomy.

DISCLOSURE: Sanober Butt, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):55A. doi:10.1378/chest.10272
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INTRODUCTION: Fibroepithelial polyps of the lower respiratory tract are extremely rare with only a few reported cases(1). Surgical resection was typically used to make the diagnosis(2). We report a case of a patient with a fibroepithelial polyp diagnosed and treated by endobronchial resection after the benign nature of the lesion was suspected during bronchoscopic airway evaluation.

CASE PRESENTATION: A 68-year-old man presented to the emergency department with a one month history of retrosternal chest pain after eating. Initial imaging revealed a nodule in the lower esophagus which on biopsy proved to be squamous cell carcinoma. A chest CT done for staging purposes incidentally showed atelectasis of the right upper lobe and an endobronchial lesion in the right upper lobe bronchus. The patient denied cough, dyspnea, wheezing, hemoptysis or fever. He also denied receiving antibiotics for pneumonia in the past. Of note, he smoked half a pack of cigarettes per day for thirty years, and quit several weeks prior to presentation. On physical examination his vital signs were within normal limits. Chest examination showed normal percussion note and vesicular breath sounds bilaterally. Bronchoscopy showed a lobulated polyp with a glossy surface causing incomplete obstruction of the right upper lobe bronchus. With manipulation, the stalk of the polyp was noticed to be originating from the posterior wall of the anterior segment of right upper lobe bronchus. Multiple biopsies taken from the polyp showed benign respiratory mucosa. He subsequently underwent repeat bronchoscopy and the entire polyp was removed by using an electrocautery snare. After removal, the remaining stalk was cauterized with an electrocautery probe. Histopathological examination showed a polypoid lesion lined by benign metaplastic squamous epithelium. The stroma was composed of fibromyxoid connective tissue with vascular congestion, few inflammatory cells and a prominent adipocytic component. This was consistent with the diagnosis of fibroepithelial polyp.

DISCUSSIONS: Tumors in the tracheobronchial tree are typically malignant. While common in locations outside the lungs, fibroepithelial polyps are extremely rare benign tracheobronchial tumors. Although their etiology remains unclear, it is thought to be related to a chronic inflammatory process. Histopathologically these polyps consist of normal bronchial respiratory epithelium covering a fibrovascular stroma with scattered lymphocytes. Depending on their size and location, fibroepithelial polyps may be asymptomatic or cause wheezing, cough, dyspnea, or rarely hemoptysis. Partial or complete obstruction of the tracheobronchial tree can lead to recurrent pneumonia, bronchiectasis, and atelectasis. Radiologic imaging may identify these lesions with a soft tissue density in the tracheobronchial tree, or there may be indirect evidence of obstruction like distal atelectasis, bronchiectasis and ipsilateral volume loss. Endobronchially, they have a lobulated appearance with a glossy surface. With manipulation, a stalk arising out of the tracheobronchial wall can usually be identified. Endobronchial biopsy via flexible bronchoscope in most cases is non-diagnostic. However, complete endobronchial resection is usually possible and can yield definitive diagnosis and prompt relief of symptoms.

CONCLUSION: Even though fibroepithelial polyps are exceedingly rare endobronchial lesions, a high clinical suspicion in the right context may help avoid unnecessary surgery. Diagnosis and relief of symptoms can be obtained by flexible bronchoscopy and the appropriate instruments.

DISCLOSURE: Puncho Gurung, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: skin tag
Chest. 2010;138(4_MeetingAbstracts):56A. doi:10.1378/chest.10412
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INTRODUCTION: Benign bronchial stenosis is a well known complication of infection, sarcoidosis, chemical or thermal burns, prolonged unilateral lung intubation, post lung transplant or other bronchial injuries. It can produce significant morbidity and be potentially life-threatening when it produces critical airway narrowing. Frequency of restenosis depends on etiology of stricture and modality of prior treatment. We report a case of pregnant women with accelerated restenosis of right(R) proximal bronchus.

CASE PRESENTATION: 22 year-old woman was referred to clinic in January, 2010 with worsening dyspnea for two months. She has a history of a motor vehicle accident in March, 2008 when she sustained multiple injuries including pelvic and R side rib fractures with prolonged R sided pneumothorax, recurrent pneumonias and R main stem bronchus obstruction requiring balloon dilation and argon plasma coagulation (APC). After being discharged from a prolong rehab stay, she presented with stridor. She was only able to walk twenty steps without dyspnea. She denied hemoptysis, cough, sputum, fever, chills, sick contacts or tuberculosis exposure. She also denied smoking, substance or toxic chemicals exposures. There is no family history of any lung disease. On exam, she had tachycardia and inspiratory wheezing on R side. Pulmonary function tests showed moderately-severe obstruction without significant bronchodilator response. CT Chest showed severely narrowed proximal right mainstem bronchus. The stenosis was approximately one centimeter long. Laryngoscopy found no vocal cord dysfunction. Patient was very symptomatic and at risk for fetal hypoxia, thus outpatient bronchoscopy was performed which revealed extremely narrowed (3-4 mm) proximal R mainstem bronchus. Balloon dilatation with APC was performed on February 12, 2010. The patient was then hospitalized on March 7, 2010 for a R lung collapse due to restenosis of proximal R mainstem bronchus. Patient again underwent a balloon dilation of the R mainstem stenosis. Two week after this procedure, she returned with hypoxia and worsening dyspnea. Bronchoscopy at that time showed no visibly patent R mainstem airway, although dilation was performed with guide wire perforation and mechanical debridement with forceps. Due to increased risk of fetal hypoxia with recurrent restenosis, emergent Cesarean section was performed to deliver the infant. Patient was referred to cardiothoracic surgeory to remove proximal right bronchus segment. Three weeks after Cesarean section she underwent another balloon dilatation. Endobronchial biopsy revealed benign bronchial mucosa with no inflammation or granulation tissue.

DISCUSSIONS: Upon review of literature, we found no similar pathophysiology in pregnant women. We suspect the initial etiology of stenosis was prolonged R lung collapse. She accelerated proximal R bronchial stenosis during her pregnancy and peripartum state. We are unclear as to the cause for the recurrence. We propose that patient’s pregnant status contributed to promote fibrous tissue formation and cause rapid restenosis in this benign bronchial lesion. We must consider the hormonal influences on the rate of restenosis. She had stenosis at the same site in 2008, although at that time she did not require repetitive dilatation. More definitive and appropriate therapies such as surgical removal of stenotic segment and reconstruction is recommended for young patients who can tolerate this procedure. In a pregnant patient with limited management options, balloon dilation or APC can be a bridge until definitive treatment.

CONCLUSION: Clinicians should be vigilant about the restenosis rate of benign bronchial stenosis in pregnant patients. Since hypoxia can be detrimental to fetal survival, early fetal delivery may be required. It is imperative to have close follow-up with high-risk maternal-fetal medicine and pulmonary physicians.

DISCLOSURE: Paresh Patel, No Financial Disclosure Information; No Product/Research Disclosure Information

Topics: pregnancy , restenosis
Chest. 2010;138(4_MeetingAbstracts):57A. doi:10.1378/chest.10987
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INTRODUCTION: Endobronchial Schwannomas have been previously described. Although this tumor is typically benign, it has been associated with symptomatic airway obstruction and massive hemoptysis. Surgical resection remains the most common treatment for endobronchial Schwannoma (1). We describe the first reported case of an endobronchial Schwannoma treated with Argon Plasma Coagulation (APC) during flexible bronchoscopy.

CASE PRESENTATION: A 38 year-old female with history of asthma presented to the emergency department with progressive dyspnea, left sided pleuritic chest pain, and a non-productive cough. She denied recent hemoptysis, fever, weight loss, or night sweats. She reported occasional alcohol and marijuana use, but denied any use of tobacco or other illicit drugs. Her vital signs were normal and physical examination was unremarkable except for findings of abdominal obesity and a healed low transverse scar. Laboratory data were within normal limits. Room air arterial blood gas revealed mild respiratory alkalosis and chest Roentogram was consistent with hyper-expanded lung fields but no evidence of acute cardiopulmonary disease. A CT angiography of the chest ruled out acute pulmonary embolism but revealed a left lower lobe endobronchial mass. The patient was initially treated with bronchodilators and referred to pulmonary medicine for further evaluation. A Flexible bronchoscopy was performed and revealed a large, white, smooth, well-vascularized tumor causing 80% stenosis of the proximal left lower lobe bronchus. Endobronchial biopsies were obtained and showed spindle cell tumor with histological features of neurogenic tumor. Uniform and intense immunostaining for S-100 protein confirmed the diagnosis of endobronchial Schwannoma. Due to the location of the tumor, surgical resection would have required left pneumonectomy. In order to spare the patient a pneumonectomy, bronchoscopic endobronchial resection was successfully performed using APC. Complete tumor ablation lead to minimal residual stenosis with an estimated procedure time of 70 minutes. The patient reported an immediate improvement in her dyspnea. Follow up bronchoscopy done at 2 months revealed evidence of granulation tissue with fibrin deposition. This was ablated with APC applications. Surveillance bronchoscopy done at 6 months showed that her airways remained patent, and the patient reported continued relief from her dyspnea.

DISCUSSIONS: Endobronchial Schwannomas are rare benign neurogenic tumors. They can appear in any region of the tracheo-bronchial tree and association with neurofibromatosis has been described. Diagnosis is established by histology where degenerative changes and marked nuclear atypia can be seen. Endoscopic treatment has been proposed for small endoluminal Schwannoma with a low probability of recurrence. Nd-YAG laser resection and snare electrocautery are the current described endoscopic treatment of choice (2); however, APC has proven successful in the treatment of endobronchial neoplasms and its safety profile may be more favorable than the Nd-YAG laser. We report the first case of successful ablation of an endobronchial Schwannoma with APC and which effectively maintained patency of the airway at six months. This treatment has allowed our young patient to avoid a pneumonectomy.

CONCLUSION: Argon Plasma Coagulation can be safely used as an effective treatment for endobronchial Schwannomas.

DISCLOSURE: Kevin O’Neal, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):58A. doi:10.1378/chest.10690
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INTRODUCTION: Vocal cord dysfunction or paradoxical vocal cord movement (PVCM) is a laryngeal disorder characterized by paradoxical adduction of the vocal cords during inspiration and/or expiration. PVCM presents with shortness of breath, stridor, wheezing, and in severe cases can lead to acute respiratory failure. The etiology of PVCM includes central nervous system injury, inhaled irritants, laryngeal dystonias, conversion disorders, and psychological stresses. PVCM can also occur following cardiac surgery and is described in association with asthma. Diagnosis is made by laryngoscopic visualization of the vocal cords during inspiration showing discordant movement of the anterior and posterior segments. Botulinum toxin type A (BT-A) is used to treat a variety of conditions caused by muscular spasticity or rigidity. It provokes a chemical denervation of the neuro-muscular junction via inhibition of exocytosis of acetylcholine. Its use has been reported for the treatment of mild PVCM. We present a case of severe PVCM following cardiac surgery that was successfully treated with bronchoscopic vocal cords injection of BT-A.

CASE PRESENTATION: A 65 year-old male underwent coronary artery bypass surgery for the treatment of coronary artery disease. Post-operatively he suffered an acute ischemic stroke which led to a prolonged intubation and failed extubation. Bronchoscopy revealed PVCM and the patient underwent tracheostomy placement. Over a period of six months, the patient repeatedly removed his tracheostomy tube and required multiple surgical revisions and replacements. As a solution to the patient’s non-compliance, permanent tracheostomy was being considered. We proposed utilization of BT-A as a less invasive alternative to permanent tracheostomy. Using therapeutic bronchoscope, 0.2 cc of 12.5 U/ml of botulinum toxin A was injected into each vocal cord using a 25 gauge needle. Following a second session of BT-A injection one week later, the patient was successfully decannulated. He remained asymptomatic for a six-week follow up period.

DISCUSSIONS: To our knowledge, this is the first case to report the efficacy of BT-A injection in the treatment of post cardiac surgery PVCM s. We describe the successful use of BT-A in chronic PVCM after prolonged tracheostomy (6 months). Most reported cases of the use of BT-A in the treatment of PVCM have been in the acute setting, whereas most long-term therapeutic modalities consist of a combination of speech therapy, patient education, and psychotherapy. The mechanisms responsible for post cardiac surgery PVCM are not well understood. Proposed mechanisms include endotracheal intubation (traumatic or non-traumatic), traumatic injury from central line insertion, direct surgical trauma, and cold injury. Besides tracheostomy, treatment options for refractory and persistent post cardiac surgery PVCM include arytenoidectomy, or vocal cords injection with Teflon or autogenous fat. In normal subjects, the posterior cricoarytenoid (PCA) muscles are responsible for abduction of the vocal cords and the lateral cricoarytenoid (LCA) muscles produce adduction of the vocal cords. In our patients, the PCA muscles were likely permanently injured during the cardiac surgery or in the post operative period secondary to multiple and prolonged intubations, or his perioperative cerebro-vascular accident. This explains the inability of the vocal cords to normally abduct during inspiration; leading to unopposed adduction of the vocal cords by LCA muscles during the respiratory cycle. The injection of BT-A into the LCA muscles prohibited the tonic adduction of vocal cords during respiration and improved the subject’s symptoms.

CONCLUSION: In summary in post cardiac surgery PVCM, when symptoms are severe, persistent, and refractory to non-invasive therapeutic measures such as psychotherapy, speech therapy and axiolytics, a trial of BT-A injection into the LCA muscles might save the patient multiple intubations and permanent tracheostomy.

Chest. 2010;138(4_MeetingAbstracts):59A. doi:10.1378/chest.10978
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INTRODUCTION: Tracheal laceration is a rare and potentially deadly complication of endotracheal intubation. Primary surgical repair is considered the preferred treatment, however patient co-morbidities may preclude surgical intervention.

CASE PRESENTATION: A 77-year-old female was intubated by Emergency Medical technicians for respiratory arrest at a skilled nursing facility. By the time she was brought to the Emergency Department subcutaneous emphysema was noted and a CT scan of her chest showed a 4cm full-thickness tear in the mid trachea involving the membranous layer. Emergency thoracotomy with posterior tracheal repair was performed and a chest tube left in place. Air-leak persisted and then increased and the tracheal repair was found to have dehisced along the suture lines. A second surgery was performed for revision along with a tracheostomy. A long tracheostomy tube was used with the intent of bridging the tracheal repair site. She remained stable for a few days but when her air-leak increased again a repeat CT scan was obtained. It showed tracheal repair dehiscence again with the tracheostomy tube extending into the right pleural space through the defect in the right posterolateral tracheal wall. The patient developed sepsis and additional surgical interventions were deemed to be prohibitively risky. A decision was made to attempt tracheal stenting in an effort to reduce her air leak, but the tracheal tear was noted to extend down to the main carina and it was felt that a tracheal self expandable metal stent could not be used because of the extent of the tracheal defect. A customized silicone Y stent was then placed via rigid bronchoscopy, completely covering the tracheal laceration. Almost immediately, the air-leak decreased and her oxygenation improved. She continued to improve clinically over the following days, with resolution of her sepsis and pneumonia. She was eventually discharged to a long-term acute care facility.

DISCUSSIONS: Early surgical repair has traditionally been considered as the cornerstone of therapy; however non-operative management of tracheal laceration has been described. Surgical repair is particularly advocated for full thickness lacerations greater than two cm and those involving the carina. With the significant morbidity and mortality associated with surgical repair, non operative techniques may be necessary in some patients. Massimo et al reported successfully bridging tracheal tears with endotracheal tubes in 13 non operable patients. There are few case reports of self-expandable metal stents used to manage tracheal tears, but these cannot be used when the tear extends to the carina. Metal stents are also more difficult to remove. Silicone Y stents cover the carinal region and are easily removed even following long emplacement times. They are also less prone to migration compared with straight silicone stents. We believe this is the first report of the successful use of a silicone Y stent to manage a tracheal tear after failure of surgical attempts.

CONCLUSION: Surgical repair remains the primary management for tracheal tears. Silicone Y stent placement may be a helpful option in patients with large tracheal tears, who are not surgical candidates, or have failed surgery.

DISCLOSURE: Somnath Ghosh, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):60A. doi:10.1378/chest.10386
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INTRODUCTION: Oncocytoma is an uncommon tumor of epithelial origin. It is characteristically composed of large eosinophilic cells with small round nuclei and large nucleoli. They commonly occur in the kidney, salivary glands, and thyroid. Their occurrence in the lungs is extremely rare with less than 10 cases reported in the literature. We are reporting a case of 26 years-old man with persistent pulmonary infiltrates found to have pulmonary oncocytoma.

CASE PRESENTATION: A 26 year old nonsmoker male with a past medical history of depression, sinusitis, and hepatitis C infection with undetectable viral load, was initially referred to the chest clinic for a + PPD and an right mid lung filed infiltrate on chest x-ray. He complained of brownish sputum with blood tinged at the time for about two days. He denied any constitutional symptoms. A chest CT scan showed an infiltrate in the superior segment of his right lower lobe that was treated with a 14 day course of a macrolide. Sputum AFB smear and cultures were negative. A repeat CT scan showed persistent infiltrates for which he underwent fiberoptic bronchoscopy and transbronchial biopsy which was nondiagnostic. After two years of lost follow up, he had a CT scan which showed a mass which was more confluent, and a PET scan which showed borderline neoplastic activity (SUV of 2.7). Repeat bronchoscopy and biopsy was diagnostic of bronchial oncocytic hyperplasia. Case was discussed in the chest multidisciplinary conference and patient was scheduled to undergo right lower lobe lobectomy.

DISCUSSIONS: Oncocytomas are uncommon tumors composed of mitochondria-rich oxyphilic cells called oncocytes. They are usually considered to be benign lesions. High grade tumors with metastases are occasionally seen in a small percentage of cases. They were first described in the salivary glands of man in 1897. Later, they were also found in glands of the tongue, pharynx, esophagus, and trachea, with the parotid and the submandibular gland being the most common sites. Oncocytomas also account for about 5% of kidney tumors. The characteristic feature of an oncocyte is its granular eosinophilic cytoplasm, which is due to mitochondrial hyperplasia. As hyperplasia of other cytoplasmic organelles can produce cytoplasmic eosinophilia. Some case are problematic in terms of making an accurate diagnosis, due mainly to the large number of differential diagnoses involving tumors, ranging from overtly malignant to clearly benign. Sometimes electromicroscopic study may be essential to confirm such diagnosis.Fewer than ten case reports of pulmonary oncocytomas have been reported to our knowledge in the literature. Most reported patients were relatively asymptomatic. They were found to have a mass on chest X-Ray. The diagnosis was reached only after excision in the majority of cases. To our knowledge there was one case report of a diagnosis being obtained by transbronchial biopsy. Oncocytomas are usually benign tumors. They should be considered in the differential diagnosis of mass lesions in the lung, making the appropriate diagnosis is imperative as adequate surgical excision is usually curative.

CONCLUSION: The importance of identifying pulmonary oncocytomas lies not only in ruling out a metastatic tumor but also because of its ability to mimic pulmonary tumors with diverse clinical outcomes. Adequate surgical excision is usually curative.

DISCLOSURE: Gerald Tarirah, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):61A. doi:10.1378/chest.10310
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INTRODUCTION: Ganglioneuroma is a rare posterior mediastinal tumor which usually presents during childhood. It is a well differentiated benign tumor composed of mature ganglion cells. Ganglioneuroma is usually asymptomatic and when symptoms present they are secondary to mass effect from tumor extension. We describe an adult patient with thoracic ganglioneuroma who presented with chest tightness and shortness of breath. We also illustrate the clinical and histopathological features of a giant thoracic ganglioneuroma.

CASE PRESENTATION: A 27 year-old African American female with no past medical history presented with chest tightness and dyspnea on exertion for 2 months. She was hemodynamically stable and oxygen saturation was 99% on room air without signs of acute respiratory distress. Auscultation and percussion of the chest revealed decreased breath sounds and dullness on the right. Complete Blood Count, Basic metabolic panel and arterial blood gases were within normal limits. Chest x-ray revealed soft tissue mass density on the right mid and lower lung with a 25% right-sided pneumothorax. A CT scan of the chest revealed a soft tissue mass occupying two thirds of the right hemithorax adjacent to the pleura and the ribs. MRI of the thoracic spine confirmed a large right paraspinal tumor extending from T3 to T12 with extension into the neural foramina at multiple levels. Barium esophagogram showed displacement of the distal esophagus to the left with no esophageal involvement. Patient was evaluated by Pulmonary, Thoracic surgery andNeurosurgery services and underwent bronchoscopy, hemilaminectomy of T4-T7, right thoracotomy and resection of the giant pleural tumor. Histopathological examination revealed a ganglioneuroma invading the right pleura. Patient's symptoms resolved and she is doing very well.

DISCUSSIONS: The differential diagnosis of posterior medistinal mass includes neurogenic tumors. Ganglioneuroma is a neurogenic tumor that arises from sympathetic ganglion cells. The incidence of ganglioneuroma is unknown. The tumor is generally encapsulated and attached to a sympathic or intercostal nerve trunk. It may present with chest pain secondary to thoracic bone compression or erosion of the nerves, cough, dyspnea or hemoptysis. These symptoms are related to intrathoracic extension of the tumor. Intraspinal growth with intrathoracic extension is a rare complication of ganglioneuroma and should be approach by neurosurgical and thoracic procedures. Ct scan and MRI are useful to localize the tumor and define the extension. Complete resection is generally curative and local recurrence is uncommon.

CONCLUSION: Although it is rare, ganglioneuroma should be included in the differential diagnosis of posterior mediastinal and intrathoracic masses in young adults. Multidisciplinary team approach is necessary for the proper diagnosis and treatment of intrathoracic ganglioneuroma.

DISCLOSURE: Juan Rojas Balcazar, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):62A. doi:10.1378/chest.10509
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INTRODUCTION: Synovial sarcoma is a rare soft tissue tumor involving the extremities and limb girdles and is seen in young adults. It metastasizes in 30-40 % of cases and lung is a frequent site of metastases. Reported cases include presentations as a single large lung mass, multiple metastatic lung nodules- with or without involvement of the mediastinum, pericardium or diaphragm, and lung metastases simulating a granuloma. We report a case which presented as a spontaneous pneumothorax. Further investigations revealed a metastatic synovial sarcoma after 11 years of remission.

CASE PRESENTATION: A 38-year-old African American male presented to the hospital with acute onset of sharp right-sided chest pain and dyspnea. The initial chest film revealed a tiny right apical pneumothorax, less than 10%, which was managed conservatively in the hospital by observation and nasal-flow oxygen for the next 24 hours. The patient had no prior history of chest surgery, chest trauma, heart failure, recent pulmonary infection, or congenital lung abnormality.Past medical history included surgical resection and radiation of a synovial sarcoma presenting as a 6.7 x 7.3 cm oval-shaped mass in the right lower quadrant of the abdominal wall 13 years prior. Two years later a recurrence on the right iliac crest required a curative surgical resection followed by brachytherapy. He was reported to be in remission on regular follow up appointments for the next 5 years.Family history was unremarkable. Social history revealed that he was a non-smoker, a full time engineering student with no history of exposure to asbestos or environmental toxins.Physical exam: A healthy-appearing man, with normal breath sounds and exam unremarkable except for prior surgical scars.Lab data: Unremarkable except for a WBC count of 3.4 x 103 cells/μL with normal differential count. Hospital course: Further work up included a CT scan of the chest with pulmonary embolism protocol, negative for PE, but revealing a triangular 2.9 x 2 cm opacity in the right major fissure, raising the possibility of a pseudotumor due to fluid in the fissure. Symptoms resolved without further intervention, and the patient was discharged from the hospital.Subsequent course: He returned to the outpatient pulmonary clinic 10 days later. A follow-up CT scan revealed persistence of the opacity in the right major fissure, and another 1.3 cm pulmonary nodule in the left lower lobe. A CT guided biopsy of the right sided lesion revealed a metastatic synovial sarcoma of monophasic type. Cytogenetic evaluation using DNA FISH technology demonstrated a rearrangement of the SS18 (18q11.2) gene in 64% of nuclei. This probe hybridizes to band 18q11.2 (SS18) confirming translocation (X;18) for synovial sarcoma. A thoracic surgery consultation led to uneventful right axillary thoracotomy with wedge resection of the mass involving right middle and right lower lobe for metastasectomy with curative intent. A wedge resection of the left sided nodule is pending in the coming month.

DISCUSSIONS: Synovial sarcoma is a spindle cell tumor of soft tissues with a high propensity for pulmonary metastasis though primary pleural malignancy has also been described. Though our case represented metastatic disease, it is unusual in its presentation as a pneumothorax with only a few cases previously reported. The usual differential diagnosis of an opacity in the major fissure includes loculated fluid, blood, pus, and inflammatory cells, with malignancy less likely. In our case, concern for malignancy required careful history-taking and a high index of suspicion of a pneumothorax secondary to sub-pleural pulmonary metastasis.

CONCLUSION: Metastatic synovial sarcoma with pulmonary involvement can present after several years of remission and should be suspected in a patient with a known history of synovial cell sarcoma .

DISCLOSURE: Rupesh Dave, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):63A. doi:10.1378/chest.11074
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INTRODUCTION: Pleomorphic carcinoma of the lung is a rare malignant epithelial tumor that makes up only 0.3% to 1% of all malignant lung tumors and contains carcinomatous and sarcomatoid components. Due to its rarity few studies have been reported and its clinical and pathological features remain unclear. It is believed to spread at an early stage and to have an aggressive clinical course. We describe a case of this unique aggressive malignant tumor. The case will include chest imaging and the pathology slides (from pneumonectomy).

CASE PRESENTATION: A 60 year old cachectic male presented with cough, right side chest pain and weight loss. Physical examination revealed decreased breath sounds on the right side on auscultation. Initial laboratory analysis was essentially unremarkable. Chest x-ray revealed a 13x10 cm mass like density with smooth borders in the right side of the chest. Computed tomography (CT) of the chest showed a large heterogeneous mass with smooth margins in the right mid lung zone with areas of decreased attenuation consistent with necrosis. Internal intra lesional vessels were also identified. PET scan showed an area of malignant range FDG uptake in the right lower lung zone measuring up to 5.3SUV. No distant neoplastic range focal abnormalities were identified. Core biopsy obtained under CT guidance was inconclusive. Subsequently patient underwent right pneumonectomy with lymph node resection. Histopathology revealed a pleomorphic carcinoma with adenocarcinomatous, spindle cell, and clear cell features with extensive necrosis. Immunostaining showed the spindle cells were positive for both keratin and vimentin. No metastasis was found in the hilar or mediastinal lymph node. Pathologic stage was T2N0M0.

DISCUSSIONS: The recent WHO classification defines pleomorphic carcinoma as a poorly differentiated nonsmall cell lung cancer, namely squamous cell carcinoma, adenocarcinoma, or large cell carcinoma, containing spindle cells and/or giant cells, or carcinoma consisting only of spindle and giant cells. At least 10% of spindle cells, giant or both, should be present to classify a carcinoma as pleomorphic carcinoma. The actual nature of pleomorphic carcinoma has been debated extensively because of its rarity. It often presents in male smokers as large peripheral neoplasms with central low attenuation area and frequently invades the pleura and chest wall. Coughing and hemoptysis are the most common complaints although chest pain, dyspnea, fever have also been reported. Preoperative pathologic diagnosis in the small tissue samples is limited and diagnosis generally requires surgically resected samples. Biphasic appearance of the tumor also makes the diagnosis difficult and requires immunohistochemical staining. PC follows an aggressive clinical course with high local disease relapse rate after surgical resection and a high frequency of disseminated disease on initial diagnosis. There have been few reports regarding the use of chemotherapy and evidence of benefit of chemotherapy is also lacking. Advanced stage lymph node metastasis and histologically diagnosed massive coagulation necrosis (>25%) of the tumor predict poor disease free survival.

CONCLUSION: In conclusion, this rare disease should be considered to have a tremendously aggressive malignant behavior and a strict follow up observation must be continued in these patients. Further investigation of biologic features of pulmonary pleomorphic carcinoma and therapeutic operation is a high priority issue so that suitable treatment strategies can be planned. Unfortunately our patient developed brain metastasis and opted for hospice care.

DISCLOSURE: Sandeep Gupta, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):64A. doi:10.1378/chest.10865
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INTRODUCTION: Extracranial metastases of high-grade gliomas are traditionally thought to be a rare event, with a reported frequency of 0.44%. Recently published case reports have demonstrated distant metastases primarily from Glioblastoma Multiforme (WHO Grade-IV glioma) to regional lymph nodes, lungs, pleura, bone, liver; and rarely to skin, parotid and pancreas.(1,2) We describe a case of Anaplastic Oligoastrocytoma (WHO Grade-III Glioma) with rapidly progressive pulmonary metastasis after debulking of glial tumor.

CASE PRESENTATION: 51 years old previously healthy male admitted with heterogeneous enhancing right frontal lesion and midline shift on MRI brain. Patient underwent resection of tumor and placement of gliadel wafers followed by chemoradiation for cerebral mass. Histopathology confirmed a mixed anaplastic oligoastrocytoma, WHO grade-III Glioma. Four months later patient was admitted with increased symptoms, radiological progression of disease on MRI brain and a new onset cough. CXR showed a 2.5 X 2.2 cms nodule in the left lung. Resection and debulking of glioma was re-attempted. Multiple large blood vessels within the tumor bed were coagulated. Histopathology was similar to previous cerebral tumor (anaplastic oligoastrocytoma).Lung nodule was followed by serial CT scans of chest that demonstrated rapid progression and extensive spread. Thoracotomy and wedge resection of the lung mass was performed. Histopathology confirmed malignant neoplasm with abundant eosinophilic to pale cytoplasm, nuclear variability, prominent nucleoli, abundant misitoses and central necrosis typical of anaplastic tumors. Immunohistochemical stains ruled out Primary lung cancer and were positive for S-100, Vimentin, epithelial membrane antigen (EMA), thyroid transcription factor-1(TTF-1, cytoplasmic positivity only) and Glial fibrillary acidic protein (GFAP). Tumor cells did not stain for Synaptophysin, cytokeratin (CK), HMB45 and melan-A (A103) ruling out other lung secondaries. Patient received 4 cycles of Avastin with Irinotecan followed by PCV (vincristine, lomustine, procarbazine) regimen and radiation to chest. Despite aggressive chemo-radiation patient continued clinical and radiological progression of metastatic disease and died in 16 months.

DISCUSSIONS: Lower incidence of metastizing high-grade gliomas is attributed to absence of cerebral lymphatics, dense dural covering of cranial sinuses, immunological response of host organ to neuroglial tumor cells and short survival of patients. Previous literature suggests debulking surgery as a common characteristic feature in all cases except few. (1) We speculate that surgery and institution of chemotherapy in this patient may have provoked the metastatic cascade leading to vascular invasion, immunosupression and egress of anaplastic cells into the systemic circulation. An interesting feature of our patient is immunohistochemical expression of TFT 1 in pulmonary deposits, which is also expressed in structures of diencephalic origin. We are not sure if common embryonic origin of TTF-1 expression in lung anlage and neuroblast cells have a role in cell determination and metastatic spread complimenting “Seed and Soil Hypothesis” of metastatic spread. Our patient had mixed anaplastic oligoastrocytoma, WHO grade-III Glioma along with possible surgical contagion and TFT 1 signature on anaplastic cells suggesting a possibility of prevention and or early detection .

CONCLUSION: High-grade gliomas are rapidly progressive and can metastise extensively to lung parenchyma with out major respiratory symptoms. A high degree of clinical suspicion and radiological surveillance may provide early diagnosis. Further research on genomic signatures of pulmonary metastases may have an impact on early diagnosis and survival.

Chest. 2010;138(4_MeetingAbstracts):65A. doi:10.1378/chest.9509
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INTRODUCTION: Abdominal compartment syndrome (ACS) is most often recognized in surgical and trauma intensive care units and in association with large volume of fluid resuscitation. This is a rare case of ACS due to pneumoperitoneum occurring in a medical intensive care unit (MICU) during high frequency oscillatory ventilation (HFOV).

CASE PRESENTATION: A 67-year-old male developed acute respiratory distress syndrome requiring mechanical ventilation after elective arthroscopic knee surgery at a local community hospital. Aspiration was suspected perioperatively. The patient was transferred to our MICU from the community hospital. He had a history of hypertension, diabetes, coronary artery disease and smoking. Pertinent findings on examination were an obese male and left lower lobe crackles. Otherwise physical examination was unremarkable with no signs of congestive heart failure. PaO2/FiO2 ratio was 80. Chest radiography and computed tomography showed bilateral diffuse opacities. There was no pulmonary embolism. Due to persistent hypoxia despite diuretics, antibiotics and conventional modes of mechanical ventilation, HFOV was initiated. On day 5 of HFOV, the patient developed acute hypoxia and hypotension requiring vasopressors. Oliguria was also noted. Central venous pressure (CVP) was 18. Hypotension and oliguria were resistant to fluid resuscitation. Examination revealed BP 69/34 mm Hg. HFOV settings: amplitude 86 cm H2O, frequency 3 Hertz, mean airway pressure 45 cm H2O, FiO2 93%. Crepitus was noted on upper chest. Abdomen was distended, tympanic to percussion. Immediate chest radiograph revealed extensive subcutaneous emphysema and pneumomediastinum but no pneumothorax. Over the next 6 hours, due to increasing abdominal girth, a cross table lateral film was done that revealed free air in the abdomen suggestive of pneumoperiotoneum. Because of abdominal distension and concern for abdominal compartment syndrome, urinary bladder (UB) pressure was measured and was elevated at 43 mm Hg. Surgery team performed a bedside diagnostic peritoneal lavage. As soon as the peritoneum was entered, gush of air was released with no blood, bile or stool. After abdominal decompression, UB pressure decreased to 14 mm Hg and BP and urine output normalized.

DISCUSSIONS: The patient had ACS causing hypotension and oliguria due to large amount of free air in the peritoneum (pneumoperitoneum) as a result of barotrauma from HFOV. Barotrauma caused pneumomediastinum and subcutaneous emphysema with subsequent leakage of air into the peritoneal cavity. Intraabdominal pressure as detected by UB pressure above 20 mm Hg along with at least one new organ failure (both hypotension and oliguria in this patient) is highly suggestive of ACS. Hypotension was a result of direct compression of inferior vena cava from elevated abdominal pressure and decreased cardiac output from high intra-thoracic pressures. Oliguria was due to abdominal pressure causing reduction in the renal filtration gradient. Both hypotension and oliguria completely resolved with abdominal decompression. CVP is often falsely elevated both in ACS and with HFOV and must be interpreted with caution. High index of suspicion and early detection is key to diagnosing ACS which if unrecognized has a very high mortality.

CONCLUSION: ACS is increasingly recognized in the medical intensive care unit. This is an unusual case of pneumoperitoneum from HFOV barotrauma causing ACS. After a long hospital course, this patient developed multiorgan failure and died.

DISCLOSURE: Sucharita Kher, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):66A. doi:10.1378/chest.10792
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INTRODUCTION: Pulmonary arterial hypertension (PAH) can occur in patients with antiphospholipid antibody syndrome (APS). It is frequently secondary to chronic thromboemboli (CTEPH). This case details a patient with APS and IPAH presenting with acute PAH exacerbation and decompensated right ventricular (RV) failure associated with an APS flare.

CASE PRESENTATION: A 45 year old female presented with a history of APS and mild renal insufficiency managed with anticoagulation, azathioprine, and prednisone. She was initially seen in our clinic one year ago for worsening dyspnea upon exertion initially noticed two years prior. Evaluation confirmed PAH with a mPA of 45 mmHg and a Pwedge of 13 mmHg. A ventilation-perfusion scan and chest CT PE were negative. Ambrisentan was started however she remained symptomatic. Six months later a repeat RHC showed no significant changes in her hemodynamics. Echocardiogram continued to show normal left and right ventricular size and function with an increased RVSP indicating severe PH. Tadalfil was initiated. Two months later she was admitted to a local hospital with dyspnea, cough, occasional sputum, and hypoxia. She was treated with antibiotics, corticosteroids and transfused for anemia and thrombocytopenia. Her dyspnea, fatigue, and hypoxia continued and she was admitted to OSU. Upon admission she was afebrile, hemodynamically stable, and tachypnic with an O2 sat of 96% on 2L. Her lungs were clear and heart sounds were regular rate and rhythm. The patient’s abdomen was distended with concern for ascites and pitting edema was present in the lower extremities. Labs revealed a platelet count of 134 K/μL, BUN 73mg/dL, and creatinine of 1.59 mg/dL. Initiation of IV diuretics elicited minimal response and she subsequently developed acute renal failure (ARF), worsening thrombocytopenia, and increasing oxygen requirement. Chest X-Ray did not show pulmonary edema or infiltrates. Elevated D-dimer 3.7 μg/mL(<0.5), anti-cardiolipin antibody IgG 148.7 GPL (0-15), and depressed platelets (16 K/μL) in combination with ARF confirmed the diagnosis of catastrophic APS. Repeat echo showed RV dilatation with severely reduced function. Anticoagulation was maintained and high dose corticosteroids were initiated. Further intervention included nitric oxide (NO), dopamine, ultrafiltration and IV epoprostenol which resulted in temporary improvement of thrombocytopenia. Plasmapharesis elicited a dramatic improvement of renal function, resolution of RV failure, hemodynamic stabilization, and improved oxygenation. Thrombocytopenia recurred and rituximab was added.

DISCUSSIONS: The prevalence of pulmonary hypertension in patients with APS is estimated to be 1.8-3.5% 1. PH can be secondary to CTEPH or IPAH or on rare occasion the sole manifestation of APS. PAH is characterized by intimal thickening and proliferation, fibrosis and hypertrophy of the muscular layer with development of plexiform lesions, and thrombosis in situ. The etiology of the initial insult is unknown. Immunological processes have been implicated as PAH frequently complicates autoimmune mediated diseases. Antinuclear and anti-KU antibodies are often seen in patients in IPAH. Studies have shown improvement of PAH in lupus patients treated with aggressive immunosuppresion.2 Our patient was chronically immunosuppressed with azathioprine and prednisone and selective vasodilator therapy was added because of development of PAH despite immunosuppresion. Aggressive right heart failure management included volume removal, vasopressors, inhaled NO, initiation of intravenous epoprostenol, intensive immunosuppresion via high dose corticosteroids, plasmapharesis and anticoagulation. Rituximab was later added to address her persistent elevation of antibody titers and thrombocytopenia.

CONCLUSION: PAH may complicate APS. Prompt recognition and management of RV failure is imperative. Optimal therapy includes a multidisciplinary approach of aggressive immunosuppresion and vasodilator therapy.

DISCLOSURE: Jessica Kynyk, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):67A. doi:10.1378/chest.9835
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INTRODUCTION: Diabetic ketoacidosis(DKA) is a prothombotic state that is frequently observed as the presentation of new onset diabetes mellitus. Aortic arch thrombus has been rarely reported in patients with normal aortic wall architecture. We report the first case of aortic arch thrombus complicating DKA in the presence of normal aortic wall.

CASE PRESENTATION: A 53 year Afro-Caribbean man with no significant medical history presents to the emergency department with a chief complaint of nausea, vomiting, polyurea, polydypsia and weakness for the three days prior to admission. The patient was admitted to the MICU with new onset diabetes and DKA. On hospital day two the patient was symptomatically improved, and his DKA resolved. Just prior to transfer the nurse reports BP of 40/17 mmHg and informs the house-staff. The patient was found awake, alert, in no distress and without new complaints. On exam he was found to have cool upper extremities with absent radial pulses bilaterally but normal carotid and lower extremity pulses. Blood pressures taken from the lower extremities were found to be 130's/80's. The remainder of the exam was unchanged from admission. A stat bedside echocardiogram showed a mobile mass most likely representing a thrombus present at the transition from the transverse to descending aorta. His ejection fraction was normal. A CT aortogram showed a large filling defect in the distal ascending aorta extending into the aortic arch along its inferior wall without definite evidence of an intimal flap or pulmonary emboli. Additionally there were smaller filling defects found in the left axillary artery. He was anticoagulated with heparin and was transferred to cardiothoracic surgery service. On the following day thrombectomy was performed. Ten days later the patient was discharged home. Preliminary hypercoaguability work up was sent prior to administration of heparin. The patient’s lupus anticoagulant was positive. The pathological reports was consistent arotic thrombus.

DISCUSSIONS: Diabetic ketoacidosis (DKA) has been shown to be a prothombotic state. The risks of thrombus formation are increased by treatment of DKA are thought to be due to a relative deficiency of free protein C and protein S and a relative increase in von Willebrand Factor (vWF) which in turn activates vascular endothelium(1). Thoracic aortic mobile mural thrombus (TAMT) is a rare condition that is usually seen in elderly patients with extensive atherosclerotic disease, trauma or a hypercoaguable state. Most reported cases have been associated with atherosclerotic plaque with superimposed thrombus formation and are usually detected following cerebral, visceral or peripheral embolization(2). Our patient was a middle aged male that had been previously healthy. He had no personal or family history of DVT or abnormal clotting and his coagulation profile was normal on admission. His preliminary hypercoaguability work up revealed a positive lupus anticoagulant, without any consequences to date. The effect of hyperviscosity due to osmotic diuresis combined with alterations in the clotting cascaded caused by DKA and its treatment resulted in the formation of a large aortic arch clot in an individual who was already at risk. This case represents the perfect storm that was created by a predisposed individual whose risk was increased by an acquired prothombotic state created by DKA.

CONCLUSION: DKA is a hypercoaguable state. Clot formation can precipitate or complicate DKA. A high degree of suspicion is necessary to identify and treat such life threatening complications.

DISCLOSURE: Joseph Gorga, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):68A. doi:10.1378/chest.10898
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INTRODUCTION: Propofol infusion syndrome is an uncommon complication of Propofol infusion typically associated with high dose or prolonged administration. Clinical manifestations include metabolic acidosis, elevated creatinine kinase, hypotension, and organ dysfunction. Propofol has been advocated in the treatment of elevated intracranial pressure in hepatic encephalopathy to ease in neurologic exam monitoring.1 We report a case of fulminant hepatic failure complicated by propofol infusion syndrome.

CASE PRESENTATION: A 42-year-old previously healthy female presented with a diagnosis of acetaminophen-induced fulminant hepatic failure from a therapeutic misadventure. Exam was significant for grade III hepatic encephalopathy with tachycardia, hypoxia and hypotension. Labs at presentation included: AST 28,000, INR 2.6, Cr 2.7, pH 7.1, and lacate of 11.5. Liver transplant evaluation was initiated. Patient was aggressively resuscitated including mechanical ventilation, N-Acetylcysteine treatment and vasopressor support. An intracranial pressure monitor was placed and elevated pressures were treated with usual interventions including sedation with propofol. The total dose of propofol infused was 1.89 grams over 17 hours (range of 15-40 mcg/kg/min). Creatinine kinase elevation escalated, and metabolic acidosis and hypotension worsened. Propofol was discontinued. Patient experienced a PEA arrest followed by asystolic arrest 1.5 hours later. At autopsy findings of microvesicular infiltration of the liver, heart, and kidneys were consistent with the putative clinical diagnosis of Propofol infusion syndrome in the setting of multi-system organ failure.

DISCUSSIONS: Propofol infusion syndrome occurs rarely but often has very severe consequences. Typically the syndrome is associated with high doses (>67 mcg/kg/min) and prolonged infusions (>48 hours). Experimental and clinical evidence supports the hypothesis that Propofol uncouples the respiratory chain in the heart leading at least in part to the pathological consequences of the infusion syndrome2. Furthermore Propofol infusion syndrome is exacerbated by low carbohydrate supply2, a common finding in hepatic failure. Propofol has been previously used to treat elevated intracranial pressures including in fulminant hepatic failure. Caution is warranted in the setting of vasopressor use with worsening acidosis. Creatinine kinase elevation can assist in the diagnosis.

CONCLUSION: In spite of being a relatively uncommon complication, Propofol infusion syndrome should be considered in any patient receiving an infusion especially given the potential for catastrophic outcomes. A high index of suspicion should be maintained in the critically ill patient with acute hepatic failure and if confirmed an alternative sedative agent should be utilized.

DISCLOSURE: Jody Olson, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):69A. doi:10.1378/chest.10052
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INTRODUCTION: Hypotension can be one of the manifestations of several types of transfusion reactions, including acute hemolysis, bacterial contamination, transfusion related acute lung injury (TRALI), and anaphylaxis. These reactions present with other characteristic symptoms, or signs. In some cases though, hypotension is the only manifestation of a transfusion reaction. This reaction predominantly occurs in patients on angiotensin converting enzyme (ACE) inhibitors. This reaction is referred to as Acute Hypotensive Transfusion Reaction (AHTR). We are reporting a case of AHTR observed in a patient on ACE inhibitor therapy.

CASE PRESENTATION: A 81 year old male presented to ER with altered mental status for 2-3 days. Past medical history was significant for diabetes mellitus, chronic kidney disease, and coronary artery disease. The patient was lethargic and slow to respond but oriented to time, place and person with no focal neurologic deficit. Pertinent laboratory data included hemoglobin of 14.6 g/dL, white blood cell count of 16100/cu. mm, and serum creatinine of 2.6 mg/dL. Blood cultures grew methicillin resistant Staphylococcus aureus (MRSA) and vancomycin was initiated. During the in-hospital course, the patient’s hemoglobin steadily started to decline. The patient was transfused with two units of packed red blood cells when hemoglobin dropped below 8 g/dL. Esophagogastroduodenoscopy, and colonoscopy were unremarkable. It was decided to initiate ACE inhibitor therapy. Patient’s hemoglobin declined despite previous transfusion, and two more units of packed red blood cells were ordered. Fifteen minutes following initiation of current blood transfusion, the patient started having dizziness. The blood pressure had dropped to 67/37 mm Hg. The blood transfusion was immediately stopped, and intravenous fluid bolus was given. Ten to fifteen minutes following the cessation of blood transfusion, the patient started feeling well and the blood pressure normalized to 101/70 mm Hg. Analysis of the donor and recipient blood products revealed no cross-reacting antibodies. It was, however, observed though that the patient was initiated on ACE inhibitor therapy, and that the second set of blood transfusion was ordered following the administration of ACE inhibitor. The onset of hypotension was abrupt with commencement of blood transfusion, with rapid resolution of hypotension once the transfusion was stopped. This phenomenon is referred to as Acute Hypotensive Transfusion Reaction (AHTR), and ACE inhibitors are known to play a key role in the pathogenesis of this phenomenon.

DISCUSSIONS: AHTR depends on knowing bradykinin (BK) function and metabolism. The starting point of the activation process requires interaction of activated factor XII with negatively charged surfaces such as blood filters. Factor XIIa transforms prekallikrein into kallikrein. Kallikrein is responsible for generating BK from HMWK. BK stimulates normally present B2 receptors on the endothelium and mediates the pharmacological effects of bradykinin, and prostaglandin PGI2. Bradykinins are hydrolyzed by several metallopeptidases. Angiotensin converting enzyme (ACE) is responsible for 75% of BK inactivation. In the presence of ACE inhibition through anti-hypertensive medications, larger amounts of des-Arg9-BK can form, manifesting in hypotension. Hypotension related to the use of ACE inhibitors in patients undergoing apheresis procedures has been well documented, and the medication is recommended to be discontinued at least 24 hours prior to the patient undergoing apheresis. Owen and colleagues reported on 301 patients undergoing plasma exchange, 15 of them taking ACE inhibitors. All 15 patients experienced hypotension or flushing during a procedure.

CONCLUSION: Once an episode of AHTR occurs, the most important measure is to immediately stop the transfusion. Symptoms will usually subside quickly as the transfusion is discontinued. The patient should not be re-challenged with that same product.

DISCLOSURE: Ankur Kalra, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):70A. doi:10.1378/chest.10176
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INTRODUCTION: Acute eosinophilic pneumonia (AEP) is an uncommon cause of acute hypoxemic respiratory failure in otherwise healthy young adults and is exceedingly rare in children. While it can be life-threatening, the need for extracorporeal membrane oxygenation (ECMO) support in this setting has not been previously described. We, therefore, are presenting a case of severe AEP in an adolescent who was successfully managed with ECMO support.

CASE PRESENTATION: A 17 year old, 116 kg, otherwise healthy white male presented with 2 days of high fevers, myalgias, headache, and dry cough. Risk factors for lung disease included recent initiation of smoking and occupational exposure to pigeon droppings. On admission to a referring hospital, he was hypoxic with bilateral pulmonary infiltrates on chest radiograph. He was started empirically on vancomycin, ceftriaxone, doxycycline, azithromycin, and caspofungin as well as BiPap support. On hospital day 3 he was transferred to our PICU due to progressive respiratory distress. On PICU arrival, his respiratory rate was 40 on BiPap 20/14, with a PaO2/FiO2 (P/F) ratio of 100, and x-ray findings consistent with ARDS. He was intubated and underwent rapid escalation in support, including inhaled nitric oxide and high frequency oscillatory ventilation. His P/F ratios remained around 100 with oxygenation index values around 40. Within 24 hours of PICU admission he was placed on veno-venous ECMO. The next day, bronchoscopy was performed with analysis of BAL fluid revealing 4543 WBC/mm3 of which 51% were eosinophils. A diagnosis of idiopathic acute eosinophilic pneumonia was made, with immediate institution of methylprednisolone 125mg IV q6 hr x 14 days followed by a taper. Extensive testing for infectious agents was negative. The BAL WBC count dropped to 314/mm3 (with no eosinophils) by day 5 of steroid treatment and his lung compliance improved. He required serial bronchoscopy to remove tenacious mucous plugs during his 14 day ECMO course. The patient’s ICU course was complicated by ventilator-associated pneumonia and severe myoneuropathy of critical illness necessitating tracheostomy, chronic mechanical ventilation, and inpatient rehabilitation. He was, however, ultimately discharged home 114 days after his initial presentation, ambulatory, decannulated, and without an oxygen requirement.

DISCUSSIONS: While uncommon in children, AEP is rapidly reversible with prompt treatment. Lack of recognition of the disease can result in a more severe, life-threatening course. The diagnostic criteria of AEP include BAL fluid with >25% eosinophils (or compatible lung biopsy) plus clinical criteria including acute febrile respiratory illness of <1 week, bilateral diffuse infiltrates on chest radiograph, and hypoxemia. Other causes of pulmonary eosinophilia must be excluded. Our patient had severe hypoxemic respiratory failure with ARDS and clinical instability that delayed bronchoscopy. Stabilization with ECMO support allowed for successful bronchoscopy, and BAL results led to a diagnosis of AEP. As expected, high dose corticosteroid therapy led to improvement, although our patient experienced many of the complications associated with high-dose glucocorticoid therapy. To our knowledge, this is the first described case of AEP requiring ECMO rescue.

CONCLUSION: Acute eosinophilic pneumonia remains an uncommon cause of acute respiratory failure, particularly in children, but should remain a consideration in children and adults with rapid onset, refractory respiratory failure of unknown etiology. Successful management requires early bronchoscopy for diagnosis, and high-dose corticosteroid therapy. ECMO represents a therapeutic option to provide respiratory support while diagnosis is sought in AEP or other causes of respiratory failure. However, early consideration of AEP in previously healthy children presenting with fever and rapidly progressive, diffuse hypoxemic lung disease may allow for more timely diagnosis and treatment of this disorder - before ECMO therapy is needed.

DISCLOSURE: Todd Karsies, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):71A. doi:10.1378/chest.9804
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INTRODUCTION: In patients with massive variceal bleeding, placement of a Minnesota tube or similar device is sometimes necessary to tamponade culprit varices at the gastroesophageal junction. Traditionally the gastric balloon is inflated in two stages: first 50mL of air is insufflated after the tube has been advanced to the 50cm mark; after radiographic confirmation of gastric balloon position, 300mL of air is added and traction is applied. Simple auscultation for air infused through the gastric port has been associated with serious complications arising from unrecognized balloon malposition, and is therefore not recommended as a substitute for radiographic confirmation. We describe two cases of variceal bleeding where Minnesota tube placement was confirmed using bedside ultrasonography by the treating intensivists, allowing balloon inflation and traction to be applied safely before portable radiography was available.

CASE PRESENTATION: Case 1. A 52-year-old woman with a history of orthotopic liver transplantation was transferred to our facility for management of suspected graft rejection. On her ninth hospital day, she had an episode of hypotension and a gastric lavage demonstrated bright red blood. Her heart rate was 130 beats per minute and blood pressure was 100/55 mmHg. In the next hour, after receiving two units of packed red blood cells, 2 units of fresh frozen plasma and 1 six-pack of pooled platelets, the patient’s hemoglobin dropped from 9g/dL to 5.2g/dL. A Minnesota tube was inserted. A 5-2 MHz 65-mm broadband curvilinear transducer was placed transversely over the patient’s left upper quadrant after insertion of the Minnesota tube. Echogenic contrast was easily visualized after infusion of 10mL of agitated saline through the gastric port of the device. Examination of the patient’s left upper quadrant with a 12-4 MHz 42-mm broadband linear transducer revealed an acoustic shadow within the stomach, corresponding to the device tubing. Case 2. A 63-year-old morbidly obese man with a history of alcoholic cirrhosis was transferred from an outside hospital for management of presumed variceal bleeding. In the 4 hours prior to arrival he had received six units of packed red blood cells, six units of fresh frozen plasma and two six-packs of pooled platelets. His heart rate was 135 beats per minute and blood pressure was 95/50 mmHg. The initial hemoglobin was 11.9 g/dL, and lactate was 11.3 mMol/L. A Minnesota tube was inserted. A 5-2 MHz 65-mm broadband curvilinear transducer was placed transversely over the patient’s left upper quadrant. Echogenic contrast was visualized after infusion of 10mL of agitated saline through the gastric port of the device. In addition, the gastric balloon was visualized prior to agitated saline infusion, though with some difficulty. In contrast to the first case, the Minnesota tubing was not visualized with the 12-4 MHz 42-mm broadband linear transducer.

DISCUSSIONS: To the best of our knowledge, these two cases are the first published descriptions of Minnesota tube placement confirmation with ultrasound by the treating clinician. In both cases, the exam was performed contemporaneously with resuscitations of hemodynamically unstable patients. For the purpose of verifying a gastric position of the tamponade balloon, ultrasound served as a substitute for portable radiology - and therefore facilitated earlier control of hemorrhage. It may not always be possible to confirm placement using ultrasound on account of body habitus or overlying bowel loops, and in those cases radiographs should still be obtained.

CONCLUSION: Minnesota tube placement can be confirmed using bedside ultrasound by the treating clinician, obviating the need to wait for portable radiology prior to tamponade balloon inflation. In hemodynamically unstable patients this may be clinically significant by facilitating more rapid control of variceal hemorrhage.

DISCLOSURE: David Wallace, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):72A. doi:10.1378/chest.9421
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INTRODUCTION: One of the benefits of thoracic ultrasound (US) is the ability to quickly rule out a significant pneumothorax (PTX) in the critically ill patient. Thoracic US has shown to be much more sensitive than chest radiography for detecting PTX. Despite this fact, thoracic US is still not routinely used for this purpose. We are presenting a case that demonstrates the potential utility of thoracic US in a common clinical scenario where the physician needs to decide whether to place a chest tube, obtain further imaging, or observe the patient.

CASE PRESENTATION: A 53 year-old woman was brought to the emergency department (ED) with history of blunt chest trauma from a fall. She complained of left-sided chest pain and was hypotensive on arrival. Physical examination showed tenderness in the left axillary region. An ultrasound exam was performed to evaluate the abdomen and chest. The abdominal US was unremarkable. The anterior thoracic US was significant for no lung sliding bilaterally, suggesting bilateral PTXs. An upright chest radiograph confirmed a left-sided PTX, but there was no sign of a right-sided PTX. She was volume resuscitated and a left-sided chest tube was placed urgently. However, she continued to remain hypotensive. A second and more extensive right thoracic US exam was then performed with the patient in supine position. Imaging at several locations showed no lung sliding superior to the nipple line and anterior to the mid-axillary line. CT scan of the chest was obtained which confirmed the bilateral PTXs. Right-sided chest tube was placed and the hypotension was immediately resolved.

DISCUSSIONS: Thoracic US is an evolving diagnostic modality for a variety of pulmonary pathologies, including PTX. “Lung sliding” is the sonographic term for the normal to-and-fro movement between the visceral pleura on the lung surface and the parietal pleura on the chest wall. Presence of lung sliding always rules out PTX, whereas absence of lung sliding is highly suggestive of PTX. When the lung sliding is absent in an unstable patient chest tube placement should be considered without further imaging. If the patient is stable, “lung point sign” should be sought, which is 100% diagnostic for PTX. Lung point sign is the sonographic term for detecting the leading edge, where the lung is intermittently touching the chest wall. Due to its high sensitivity, it is common to identify small PTXs with thoracic US, which cannot be identified by plain radiography and may simply be observed in non-ventilated patients. Examining one location bilaterally decreases the sensitivity of the exam but a clinically significant PTX should initially be identified by this method. For a more detailed exam, the probe is moved to several thoracic locations. This makes it possible to estimate the size of the PTX as well as to detect the lung point sign by mapping the leading edge of the lung. Finding the lung point sign in the absence of sliding sign is pathognomonic for PTX, whereas the absence of sliding sign by itself can be caused by several other conditions including pleural adhesions, bronchial intubation, pulmonary infiltrate or contusion, ARDS, and atelectasis.

CONCLUSION: This case highlights the fact that thoracic US can be much more sensitive than plain radiography for detecting PTXs. We suggest that in an unstable patient with no lung sliding, it is reasonable to place a chest tube immediately based on the thoracic US findings alone in an appropriate clinical setting. In stable patients with no lung sliding, lung point sign should be sought or a thoracic CT scan obtained if the plain radiography does not confirm a PTX.

DISCLOSURE: Michael Markos, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):73A. doi:10.1378/chest.10527
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INTRODUCTION: Endovascular therapy plays an important role in the treatment of brain arteriovenous malformations (BAVMs). Ethylene vinyl alcohol copolymer (Onyx) Liquid embolization material has been used to treat high grade BAVMs pre-operatively to decrease size and flow. We report a case in which onyx use was complicated by the development of Superior Vena cava syndrome.

CASE PRESENTATION: A 74 year old man presented to our hospital for treatment of a symptomatic dural arteriovenous malformation at the right posterior fossa. He had episodes of altered mental status, headaches, dizziness and seizures which lead to the diagnosis of the AVM. His past medical history was significant for Multiple Myeloma for which he was treated with radiation therapy and chemotherapy. He had a right brachiocephalic and vena cava stent placed for venous stenosis caused by a previous chemoport. The chemoport was no longer in situ. The patient was treated with selective embolization of the AVM through the right middle meningeal artery with onyx liquid embolization. Post procedure the venous flow through the AVM was significantly decreased.Three weeks later the patient was admitted to our hospital for respiratory failure secondary to upper airway obstruction. Stridor and vocal cord edema was noted during intubation. Physical examination was significant for bilateral upper extremity non-pitting edema, plethora of the face and varices on the chest wall. Examination of the heart and lungs were normal.Chest radiography revealed a linear structure extending from the SVC stent into the right atrium. A CT of the chest confirmed the linear structure starting from the stent extending into the right atrium. The patient successfully underwent interventional radiology (IR) guided removal of the material by a femoral vein approach. The material was determined to be onyx. The patient had developed a significant stent stenosis and venous thrombosis. The venous pressure in the right internal jugular vein was 33 mmHg, compared to 15 mmHg in the femoral vein. After anticoagulation and angioplasty of the stent with removal of the onyx material the right internal jugular venous pressure decreased to 22 mmHg. The upper extremity edema and facial swelling improved and the patient was extubated. The patient was discharged with no further complications.

DISCUSSIONS: Onyx is available in three different formulations each with a different viscosity. Onyx 18 and 20 are generally used for embolization of a plexiform nidus and onyx 34 is used for embolization of large arteriovenous shunts. It is less adhesive and polymerizes slowly thus improving ease of use. The complication rate ranges from 6.9 -16.5%.Superior vena cava syndrome (SVCS) is the clinical manifestation of superior vena cava (SVC) obstruction either by obstruction or compression from outside the vessel. Malignancies account for the majority of cases. More recently, the incidence of SVC syndrome due to obstruction has risen due to increased use of intravascular devices.To the best of our knowledge this is the first case of SVC syndrome reported as a complication of embolization with onyx. The onyx material attached to the preexisting venous stent and caused an occlusion of the SVC. The symptoms dramatically improved with removal of the material and restoration of the venous flow.

CONCLUSION: We present a case of a SVC mass caused by embolization liquid from treatment of a brain AVM. This was associated with development of severe acute SVC syndrome. The symptoms promptly resolved with the removal of the onyx material from the superior vena cava.

DISCLOSURE: Kavan Ramachandran, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):74A. doi:10.1378/chest.9355
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INTRODUCTION: Indwelling catheter fecal diversion systems such as the Zassi Bowel Management System are commonly used in intensive care units in patients with diarrhea or fecal incontinence. The ability to contain and divert stool can be challenging and is important in the prevention of infection and skin breakdown as well as reducing nursing burden. The system is composed of a tension cuff, which resides in the rectal vault, and a drainage system. The system is largely reported as safe and the use of the device is increasing. The manufacturer states that the device can be used up to twenty nine days and lists infection, obstruction, perforation, pressure necrosis, fecal leakage, and loss of sphincter tone as possible complications of the device. Mucosal pressure necrosis and ulceration are known risks of the device, however, reported cases are rare. Since using the device at our institution, we have seen three cases of rectal bleeding that appears to be related to the devise. The complication of pressure necrosis may be under reported and warrant further study. This report is of severe life-threatening bleeding due to the Zassi Bowel Management System.

CASE PRESENTATION: A sixty year old man with multiple medical problems presented to the intensive care unit with sepsis and respiratory failure. The patient had a report of a normal colonoscopy approximately one month prior to his hospital admission. He was resuscited, treated with antibiotics, and weaning from the ventilator. After several days in the intensive care unit he developed significant diarrhea. Multiple stool studies were negative for infection and a Zassi Bowel Management System was placed. After seven days with the system in place he developed acute hematochezia to the point that he was transfused packed red blood cells. A colonoscopy was performed and the patient was found to have significant amount of active bleeding. He had two large ulcerations covering one third of the circumference jut proximal to the anorectal verge and one visible bleeding vessel. Multiple epinephrine injections, haemostatic clipping and use of argon plasma coagulator (ACP) were required to control bleeding. A full colonoscopy and upper endoscopy revealed no other source of bleeding. The patient required a total of nine units of packed red blood cells. Once homeostasis was achieved the rest of his ICU stay was unremarkable.

DISCUSSIONS: We report a case in which a patient with an indwelling catheter fecal diversion system developed severe clinically significant rectal ulceration and bleeding seven days after placement of the device. This is a rare complication of the system that may be under-reported.

CONCLUSION: •We report a case of life-threatening rectal bleeding due to pressure necrosis from an indwelling catheter fecal diversion system. Two less severe cases have been reported at our institution.•Although few reports of this complication are in the literature, the risk is real and may be underreported.•Further studies to determine which patients are at increased risk of developing this complication are warranted.

DISCLOSURE: Veena Nandwani, No Financial Disclosure Information; No Product/Research Disclosure Information

Chest. 2010;138(4_MeetingAbstracts):75A. doi:10.1378/chest.10531
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INTRODUCTION: Infective endocarditis in patients with prosthetic valves is a serious life threatening infection, that in many cases need surgical removal of the valve and prolonged antibiotics. Gemella species are normal commensals of the oral mucosa, but can cause soft tissue infections, endocarditis and meningitis. We report the first case of prosthetic valve endocarditis associated with perivalvular abscess and aorto atrial fistula caused by Gemella sanguinis.

CASE PRESENTATION: A 28 year old female with a history of rheumatic heart disease manifested by mitral stenosis and aortic regurgitation underwent bivalvular replacement with a St.Jude's valve. Eight months later she developed fever and chills for 2 weeks that did not subside with 1 week of outpatient antibiotics. She also had intermittent irregular palpitations for 1 week. There were no sick contacts, recent travel, pets at home or recent dental procedures. On initial examination, body temperature was 101.4 F, pulse 110/min, and blood pressure 110/60 mm Hg. Oral exam showed severe dental caries. Electrocardiogram on admission showed first degree A-V block. Cardiovascular exam was significant for an early diastolic murmur best heard in the aortic region. Blood cultures were drawn and empiric antibiotics were started. Trans esophageal echocardiogram showed 1cm mobile echo density on the left ventricular side of the aortic valve ring, suggestive of a vegetation and increased thickness of the aortic wall on the posterior aspect with a soft tissue echo density suggestive of a perivalvular abscess. Blood cultures grew Gemella Sanguinis. The antibiotics were adjusted to Ceftriaxone and Gentamicin based on sensitivities. On day 12 of hospitalization she underwent sternotomy with removal of mechanical aortic valve with debridement of aortic root with pericardial patch repair. Introoperatively an aorto atrial fistula was diagnosed and was closed with an aortic homograft and re implantation of the coronary artery. Patient had an uneventful post operative hospital course and was discharged home for 6 weeks of antibiotics.

DISCUSSIONS: Gemella is a catalase negative, facultative anaerobic gram positive coccus which is a commensal of oral mucosa. Gemella species are rare causes of infective endocarditis with approximately 45 cases being reported in the literature. Among these, only three cases were caused by Gemella sanguinis. Gemella sanguinis was first identified in 1998 in a descriptive study of six specimen isolates from bacteremic patients, one among who had sub acute bacterial endocarditis. Since then two more cases of infective endocarditis with Gemella sanguinis have been reported in the literature. All the three cases were infection of native valves. This case is unique in that, it is the first reported case of Gemella sanguinis mechanical valve endocarditis associated with severe anatomical destruction manifested by both peri valvular abscess and aorto atrial fistula. Risk factors for infection with Gemella spp include dental disease and underlying heart disease. Few cases of infection associated with intravenous drug use and gastrointestinal procedures were also reported. Our patient had multiple dental caries and had underlying heart disease.

CONCLUSION: Gemella sanguinis infective endocarditis is a rare cause of endocarditis. Risk factors include poor dental hygiene and structural heart disease. Treatment is crucial to prevent serious complications.

DISCLOSURE: Prashant Gundre, No Financial Disclosure Information; No Product/Research Disclosure Information

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