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INTRODUCTION: Acute coronary syndrome due to coronary artery aneurysm is well known. The coronary artery aneurysm is usually due to atherosclerotic disease, but may be caused by either dissection or recent intervention or vasculitis. We describe a case of acute coronary syndrome caused by coronary artery mycotic aneurysm caused by methicillin resistant Staphylococcus aureus (MRSA).
CASE PRESENTATION: A 61 year old man was admitted with angina, fever, chills and rigors, and poor appetite. He was status post coronary artery bypass graft (CABG in 1985) and status post 14 coronary artery stents with the most recent two stents placed 8 months ago. He also had diabetes and end-stage kidney disease requiring hemodialysis. Ten days prior to admission he was hospitalized at another facility for MRSA bacteremia that persisted despite adequate intravenous antimicrobial therapy. During that prior hospitalization, he reported pus drained from his fistula. He became febrile shortly after admission. His admitting physical examination only revealed a non-functional AV fistula in the left arm. The laboratory work showed positive cardiac enzymes, however, the EKG was negative for ischemia (Non-ST Elevation Myocardial Infarction- NSTEMI). Because the blood cultures yielded MRSA, the NSTEMI was managed medically. The AV fistula was removed and yielded MRSA, but the bacteremia persisted. Trans-esophageal echocardiogram was negative for vegetation. The patient was injected with 1 mCi of In-111 labeled autologous leukocytes intravenously. Whole body scan and spot images of the chest were taken at 24 hours. These images revealed increased uptake in the anterior mediastinum. SPECT images of chest were also taken for better localization and possible correlation with recommended CT. A CT scan of chest was obtained which revealed several findings corresponding to location of abnormality on the WBC scan. There was a 3.9 × 2.8 cm area of increased density with two fluid collections measuring 2.3 × 1.6 cm and 3.7 × 3.7 cm (Image 1). The enhancing nodule appears to communicate through a thin channel with a dilated vessel seen anterior to the aorta just superior to and possibly communicating with the right ventricle. Multiple bypass clips were seen in the area of these fluid collections. Cardiac catheterization revealed aneurysms of the left main, left circumflex, saphenous vein graft (SVG) to diagonal and right coronary artery (RCA) (Image 2). It appeared that the aneurysm had ruptured with fistulous communication from SVG to RCA to possibly the left atrium and ventricle. A diagnosis of mycotic aneurysm of the coronaries was made; the patient refused surgery.
DISCUSSIONS: This is the first case report describing the diagnosis of Coronary Mycotic Aneurysm with aid of WBC scan, complemented with CAT scan of the chest and cardiac catheterization. Previously, the mycotic aneurysm has only been described either on autopsy or pathology after the resection of the mycotic aneurysm. Mycotic aneurysm is a rare entity associated with a high mortality and morbidity. Risk factors include recent invasive procedure, or seeding of coronary stents or endocarditis. Early identification of mycotic aneurysm is associated with better prognosis.
CONCLUSION: A high index of suspicion is required for diagnosing mycotic aneurysm in the patients with history of CABG or percutaneous coronary intervention, who present with acute coronary syndrome and persistent bacteremia with no identifiable source of infection.
DISCLOSURE: Nadish Garg, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Necrotizing eosinophilic myocarditis (also known as hypersensitivity myocarditis) has been associated with numerous medications, but there are no case reports in the literature that associates tumor necrosis factor alpha antagonists with this disease. We present a case of a woman with fatal necrotizing eosinophilic myocarditis temporally related to adalimumab.
CASE PRESENTATION: The patient is a 51 year old female with history of relapsing polychondritis who presented seven years prior to admission with wheezing and was diagnosed with asthma. The patient developed tracheal narrowing and chest CT scan 10 months prior to presentation revealed severe tracheal narrowing (5×7mm) at the level of the aortic arch and narrowing of the bronchus intermedius to 2mm. Bronchoscopy revealed fixed airway obstructions not amenable to stent placement. The patient had been on numerous immunosuppressive medications including etanercept, cyclophosphamide, methrotrexate and most recently prednisone 6mg and adalimumab for 3–4 months, along with beta agonist inhalers. The patient presented with complaints of 2–3 days of nausea and weakness with poor appetite. On admission, she was afebrile, BP 70/50, P 160, O2 99% on 2L NC, RR 18bpm. Relevant labs included WBC 10.5 with normal differential, troponin 59 ng/mL, AST 1998 U/L, ALT 616 U/L, albumin 1.4gm/dL, ABG 7.49/26/62/19.8. EKG revealed a supraventricular arrhythmia and adenosine, cardizem and lopressor were given without success. Electrical cardioversion was performed multiple times and she continued to be hypotensive and tachycardic. After phenylephrine was given, amiodarone was administered to medically convert the arrhythmia. Transthoracic echocardiography showed severe left ventricular hypertrophy and hypokinesis, severely decreased ejection fraction, without valvular abnormalities. The patient became bradycardic and developed pulseless electrical activity and resuscitation was unsuccessful. An autopsy showed severe diffuse myocardial chronic inflammatory infiltrates consisting of lymphocytes, macrophages and numerous eosinophils consistent with necrotizing eosinophilic pancarditis (Graphic 1). The autopsy revealed fibrosis of cartilage and severe tracheomalacia consistent with relapsing polychondritis.
DISCUSSIONS: Myocarditis can be classified as fulminant with severe ventricular systolic dysfunction and acute (nonfulminant) with less ventricular dysfunction but is paradoxically associated with a worse prognosis. Our patient presented with a fulminant form of hypersensitivity myocarditis, called necrotizing eosinophilic myocarditis, where patients develop severe heart failure that develops within days to a week. Cases usually occur in the setting of viral or parasitic infection, or upon initiation of a new medication (including beta lactams and sulfonamides, thiazides, anticonvulsants, and nonsteroidal agents). Fever and rash are common upon presentation along with signs and symptoms of acute myocardial infarction, with chest pain, ST-segment elevation, and elevated troponin. Peripheral eosinophilia may be mild or absent in many cases. Echocardiography typically reveals normal chamber size (reflecting the sudden onset of the process and lack of time for dilatation), increased wall thickness, and severe, biventricular diffuse systolic dysfunction. A pericardial effusion is seen in 75% of cases, occasionally causing tamponade. The mortality exceeds 50%, and the median survival is only a few days. The treatment for fulminant myocarditis includes high-dose corticosteroids, inotropes and mechanical ventricular support. Our patient did not have the typical presentation of fever, rash, chest pain or eosinophilia, and the autopsy results did not suggest a viral or parasitic infection. Despite the atypical presentation, the autopsy confirmed necrotizing eosinophilic myocarditis.
CONCLUSION: Necrotizing eosinophilic myocarditis is a fulminant form of hypersensitivity myocarditis that has been linked with numerous medications. Adalimumab has been shown to cause new onset heart failure or worsening of chronic heart failure, but has not been associated with a drug induced hypersensitivity myocarditis. In addition, hypersensitivity myocarditis has not been previously described in patients with relapsing polychondritis. We hypothesize that our patient's fulminant course can be attributed to her use of adalimumab for her underlying disease.
DISCLOSURE: Kristy Bauer, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Cardiac tumors are rare. Metastatic deposits are more common than primary cardiac tumors. Moreover, primary mediastinal neoplasias are infrequent, corresponding to less than 10% of all thoracic neoplasias. We report, to our knowledge, the first case of an invasive thymoma invading the epicardial vessels and myocardial tissue causing acute vessel occlusion and ST-segment elevation myocardial infarction (STEMI.).
CASE PRESENTATION: A 39 year old African American male with a history of an unresected thymoma presented to our institution with a four week history of progressive dyspnea and chest pain. Physical examination revealed a blood pressure of 128/80 mmHg, bilateral rhonchi and wheezing, with occasional rales at the bases. There were no other abnormal findings on cardiac or other systemic examinations. The electrocardiogram showed ST-segment elevation in the antero-lateral leads. Emergent coronary angiography revealed a vascular structure completely occluding the left anterior descending artery. Coronary intervention was not attempted. The patient was placed on Intra-aortic balloon counter-pulsation and referred for surgical evaluation. Further imaging using computed tomography showed the large mass with scattered calcifications completely occluding the left middle mediastinum with anterior mediastinal lymphadenopathy. A tissue biopsy was obtained from the left atrial mass via a salvage left Chamberlain procedure and the histopathological sample confirmed an invasive thymoma (oval/spindle shaped cells with bland nuclei positive for cytokeratin, CD3, CD5, CD99 and Ki67.). The patient's hospital course was complicated by bradyarrythmia, hypotension and eventually death. Autopsy findings included a 13 cm thymoma extending to the level of the superior and anterior mediastinum, compressing the left main bronchus, encasing the great vessels and coronary arteries, and invading the left and right atria. Extrathoracic metastasis was present in the liver. Final staging according to the Masaoka Classification was malignant thymoma Stage IV B.
DISCUSSIONS: In general, metastatic tumors to the heart are more common than primary cardiac neoplasms. They are seen in approximately 10–15% of autopsy patients with invasive cancers. The most common malignancies that metastasize to the heart are lung and breast carcinoma, malignant melanoma, lymphoma, and leukemia. They reach the heart by lymphatic and hematogenous spread or by direct invasion. Metastatic disease of the heart can present with congestive heart failure, pericarditis, or with an arrhythmia. Metastatic involvement of the coronary vessels is extremely rare. We report a case of invasive thymoma to the myocardium and coronary vessels resulting in acute STEMI. To our knowledge, this is the first reported case of coronary vessel involvement by an invasive thymoma. Thymoma is the most common primary tumors of the anterior mediastinum. One third of thymomas are invasive. Invasive thymomas most commonly involve the pleura, pericardium, lungs, and recurrent laryngeal nerve. Cardiac metastasis is rare. Invasive thymomas are often slow growing. Optimal therapy depends on the stage of the tumor. It is thought that surgical resection and radiation alone are not effective for management of Stage IV B tumors. A multidisciplinary approach has been proposed with the use of surgical resection and a combination of chemo, radio, and steroid therapy. STEMI is predominately caused by coronary artery atherosclerotic plaque rupture and subsequent occlusive thrombus formation. This illustrates yet another mechanism of acute vessel occlusion by external compression of the coronary arteries. Tumor debulking and subsequent bypass grafting would be the preferred strategy in this situation.
CONCLUSION: In conclusion, this case represents two rare phenomena namely invasive thymoma and external compression of a native coronary vessel resulting in myocardial infarction.
DISCLOSURE: Pooja Raju, None.
INTRODUCTION: The Fontan procedure is an anatomically complex cardiac revision performed in children with univentricular congenital heart diseases, including tricuspid atresia. The surgery has undergone multiple revisions since its inception by Dr. Francis Fontan in 1971, with a resulting evolution in its complication profile. We present the case of a 23-year-old female who presents with a well-known and life-threatening complication of the Fontan procedure.
CASE PRESENTATION: K.L. is a 23 yo female who presented to clinic with 1 week of SOB and palpitations. Shortly after birth, she was found to have tricuspid atresia. She underwent a Blalock-Taussig shunt at 6 weeks of age. At age 4, she underwent a Fontan procedure with right atrial to pulmonary artery anastomosis and ligation of the BT shunt. Her post-Fontan history was complicated by atrial tachyarrhythmias that were treated medically. On presentation, the patient appears uncomfortable and tachypneic. Her lungs are clear and her cardiac exam reveals an irregular tachycardia with no JVD, gallop, or RV heave. She required high-flow oxygen to maintain normal oxygenation. EKG revealed atrial tachycardia with a 2:1 AV block. An urgent TEE for planned cardioversion showed a 3 cm X 2 cm right atrial thrombus. A followup CTA revealed bilateral lobar and segmental acute pulmonary emboli. She was started on a high-dose heparin drip and given two courses of systemic TPA, the second initiated after the patient developed worsening hypoxia and a bedside TTE showed disruption of the right atrial clot with downstream embolization. The patient also underwent direct infusion of TPA into the right atrium for 60 hours. Given the dependence of the Fontan circuit on low pulmonary artery pressures, the patient was started on sildenafil and inhaled nitric oxide. By discharge, CTA showed a significant decrease in clot burden, TEE showed resolution of the right atrial thrombus, and the patient was not hypoxemic. The patient was discharged on coumadin, enoxaparin, and sildenafil 30 mg tid. She was referred for a Fontan revision to an extracardiac conduit with Maze procedure.
DISCUSSIONS: Our patient's complex cardiac history began when she was born with tricuspid atresia. a univentricular system with parallel systemic and pulmonary circulations. The Blalock-Taussig shunt is a palliative procedure that manufactures a shunt between the subclavian and pulmonary arteries to divert more blood to the lungs for oxygenation. This intervention is commonly performed as a bridge to the Fontan procedure that is usually delayed until later in life to allow reduction of pulmonary vascular resistance and enlarging of the vena cava. The classic Fontan procedure creates a communication between the pulmonary artery and the right atrium, effectively bypassing the right ventricle altogether and allowing the systemic venous circulation to flow directly into the pulmonary arterial system. A more updated version of the Fontan is the total cavopulmonary connection, which is a 2-stage operation that connects the caval circulation directly to the pulmonary artery, bypassing both the right atrium and the right ventricle. The cavopulomonary connection evolved out of an understanding of some of the common complications of the classic Fontan procedure, including atrial arrhythmias and cardiac thrombus with pulmonary embolism. The atrial arrhythmias are attributed to postsurgical scarring and sinus node injury, and have been shown to have low recurrence rates in Fontan patients who undergo conversion to the cavopulmonary Fontan with atrial arrhythmia surgery (cryoablation vs Maze). Cardiac thromboemboli have been attributed to a low-flow state and atrial arrhythmias. Decreasing the incidence of atrial arrhythmias, and bypassing both the right atrium and right ventricle should theoretically decrease the incidence of cardiac thrombotic events, although further studies are still necessary.
CONCLUSION: This case demonstrates the creativity required to manage complex and hemodynamically sensitive post-Fontan patients with life-threatening complications.
DISCLOSURE: Airie Kim, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Selenium deficiency is known to cause cardiomyopathy. There have been several cases of selenium deficiency reported in total parenteral nutrition (TPN) users. The following case presents a young female with selenium deficiency and cardiomyopathy that corrected after selenium supplementation.
CASE PRESENTATION: The patient is a 34 year old African American female with past medical history of gastric bypass for weight loss 9 years ago, deep vein thrombosis and pulmonary embolism 2 years ago. Her clinical course was complicated by small bowel obstruction which required exploratory laporatomy, small bowel resection and ligation of intraabdominal adhesion. A percutaneous gastrostomy tube was placed for malnutrition secondary to major depression and anorexia. She was bed bound for several months prior to this admission and developed a sacral decubitus ulcer. She presented with back pain and hypotension secondary to an infected stage IV decubitus ulcer. The patient was admitted to ICU for treatment of septic shock with antibiotics and pressors, then subsequently developed respiratory failure and required mechanical ventilation. Chest radiograph revealed bilateral opacities with small bilateral pleural effusions. A transthoracic echocardiogram showed normal left ventricular size with reduced ejection fraction (EF) of 15%, which decreased from 25% on the previous echocardiogram 2 months ago. The differential diagnosis of her cardiomyopathy included nutritional cardiomyopathy. Laboratory data revealed a selenium level 54 mcg/L (Normal range: 110–160 mcg/L), total carnitine level 47 αmol/L (Normal range: 25–55 μmol/L), and vitamin B1 97 nmol/L (Normal range: 87–280 nmol/L). Selenium 200 αcg per day was started intravenously. The patient was successfully extubated. Remeron was started for her depression. After 20 days of selenium supplementation her selenium level improved to 90 mcg/L. Repeat echocardiogram showed normal left ventricular size and function. The patient improved and no longer required blood pressure support medications, and eventually she was discharged to a rehabilitation facility.
DISCUSSIONS: Selenium deficiency induced cardiomyopathy is well known as Keshan disease in areas of low selenium intakes, such as China. Cardiomyopathy due to acquired selenium deficiency in nonendemic area has been reported among patients on TPN. Although our patient has never been on TPN, nutritional cardiomyopathy was considered as a possible diagnosis because of her poor nutritional status. The diagnosis of selenium deficiency was made based on low selenium level. Her echocardiogram showed severely reduced LV function but no dilation, which is usually seen in selenium deficiency induced cardiomyopathy. Since LV function recovered to normal after selenium supplementation, selenium deficiency was the likely cause of her cardiomyopathy.
CONCLUSION: Even without a history of TPN use selenium deficiency should be considered in the differential diagnosis when patients with malnutrition have cardiomyopathy. Patients status post rouex-en Y gastric bypass who exhibit depression, anorexia, and otherwise unexplained cardiomyopathy should be evaluated for selenium deficiency.
DISCLOSURE: Mayuko Fukunaga, None.
INTRODUCTION: Introduced in 1988, allogeneic cord blood stem cell transplant (CBSCT) is still used infrequently. Its benefits over other sources of stem cell transplant include lower incidences of graft-versus-host disease and viral transmissions, and potentially greater availability. Few well-documented adverse events related to CBSCT infusion have been reported. This report highlights a potentially life-threatening adverse effect.
CASE PRESENTATION: A thirty-four year old, blood type A, Hispanic woman, healthy except for acute myelogenous leukemia in second remission, was admitted for two dimethylsulfoxide (DMSO)-containing CBSCT infusions. She has no cardiac disease risk factors, and her normal pre-transplant echocardiogram showed an ejection fraction (EF) of 66%. Conditioning included total body irradiation, cyclophosphamide, and fludarabine. On the transplant day, each product was reconstituted to 500 ml with dextran and human albumin. The first unit contained ABO-type B cells. After 50ml was infused, she complained of chest pain. The transfusion was held. An electrocardiogram showed premature atrial contractions but no ischemic changes. Preliminary readings of a bedside echocardiogram indicated an EF of 50%. The transplant was resumed uneventfully. During the infusion of the second unit containing type O cells, the patient developed dyspnea, tachycardia, and an oxygen saturation of 85% despite supplemental oxygen. Her blood pressure remained normal. In the intensive care unit, the chest x-ray (CXR) showed bilateral infiltrates and cardiomegaly, both new compared to baseline. The patient received intravenous furosemide and her oxygenation improved. Troponin peaked at 7.7 ng/ml 18 hours later, at which time the final review of the prior echocardiogram revealed an EF of 35% and global hypokinesis. By then the patient was asymptomatic with resolving pulmonary infiltrates and decreased cardiac silhouette on CXR. Repeat echocardiogram several days later showed an EF of 50%, but persistent hypokinesis of the basal-mid septum. The patient engrafted successfully and remains in remission without further cardiac events eight months after the transplant.
DISCUSSIONS: Entities including acute coronary syndrome, pulmonary embolism, chemotherapy-related cardiotoxicity, and acute transfusion reaction were considered. However, the patient's lack of risk factors, normal baseline cardiac function, quick onset and resolution of symptoms, and predominantly left-sided heart failure make these unlikely. Given the transient cardiomyopathy, Takotsubo cardiomyopathy was also considered. However, the echocardiogram showed global hypokinesis, and there were no obvious catecholamine surge triggers. Since the symptoms began almost immediately upon infusion, the etiology seems related to the infusion contents. Dextran and albumin are not reported to cause these reactions. DMSO has previously been implicated in stem cell transplant complications. However, the amount of DMSO infused during CBSCT was similar to that infused in autologous peripheral stem cell transplants (PSCT), and there are no reported cases of DMSO-related cardiomyopathy associated with PSCT. Other possibilities seem more likely. The presence of maternal anti-HLA antibodies in the cord blood may have caused transfusion related acute lung injury. Also, cytokines, such as TNF-α, IL-1β, and IL-6, are implicated in heart failure, peripartum cardiomyopathy, and sepsis-induced myocardial depression, and may have been present from either the maternal or fetal circulation due to peripartum stress. Granulocytes and other cells in the cord blood may also have lysed during processing, releasing cytokines leading to myocardial suppression. Since cardiomyopathy has not been reported with PSCT or bone marrow transplant, perhaps something unique to cord blood stem cells triggered the cytokine release.
CONCLUSION: We infer a strong association between our patient's transient cardiomyopathy and the infusion process. The mechanisms are unclear, but are possibly related to antibody or cytokine presence. CBSCT is still a novel treatment for patients lacking HLA-matched donors, and expanded FDA regulation is expected. Analysis of this and similar cases may provide insights into modification of current protocols to improve safety and decrease complications.
DISCLOSURE: C Lee, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: ICU admission for acute abdomen secondary to PEG tube related complications are not common. We present a clinical condition where the entire PEG tube was internally displaced and cause intestinal obstruction.
CASE PRESENTATION: This is an 85 year old, obese, demented female patient who presented to us with abdominal distension and pain with constipation. She had a PEG tube placed four years ago for the dysphagia during the recovery phase of neck surgery. Six months later she had signifcant improvment and started to take the food orally with no difficulty. While continuing to eat by mouth, she did not visit her physician regularly for proper care of the PEG tube. An year later, the external flange which hold the PEG tube fell off and subsequently the PEG tube too disappeared. The patient and her family perceived that the tube had fallen off. The PEG site wound was left for natural healing with occasional dressing. She continued her routine without much difficulty, eating by mouth until 3 days prior to this admission, when she developed constipation, abdominal pain and distension. She was found to be in moderate pain with distended and tender abdomen with hyperactive bowels and foul smelling discharging wound at the original PEG site. CT abdomen revealed the entire PEG tube was found in the ileum causing complete small bowell obstruction. During the explorative laporotomy the PEG tube was seen obstructing the distal ileum with proximal dilatation of bowel without any ruptured viscus or evidence of peritonitis. The PEG tube was removed and the surgery was uneventful. Patient went home with no further complication.
DISCUSSIONS: Placement of PEG tube has been a common practise in geriatric population who have deranged swallowing mechanism for variety of reasons. The procedure has been considered safe with minor complications. Tube blockage, infected tube insertion site, abscess, dislodgement, bleeding, breaking of tube have all been described as complications. However internal displacement of entire PEG tube is a rare but a serious complication could cause high morbidity and in some cases even mortality specially in elderly, demented patients.
CONCLUSION: Regular follow up is essential for the care of PEG tube along with assisted or supervised feeding in elderly, demented patients to prevent serious and potentially life threatening complications. These patients generally does not have adequate supportive care available at home. Institutionalization is another possible way to provide adequate supervision to prevent these complications.
DISCLOSURE: Subakeesan Pathmanathan, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: There are multiple case reports of Sickle Cell Trait (SCT) with rhabdomyolysis in literature but only two case reports appear during Pubmed search having SCT with compartment syndrome (CS) and there is only one case report of SCT with compartment syndrome and rhabdomyolysis. We are presenting a case of a young army recruit with SCT who developed rhabdomyolysis and CS after strenuous exercise.
CASE PRESENTATION: A 25 year old African American active duty recruit presented to emergency department with sudden onset of bilateral leg pain following a 1.5 mile run. A similar episode of lesser severity was reported few months prior requiring no intervention. He had history of sickle cell trait (SCT). He had a family history of SCT and no drug or alcohol use. He complained of acute leg pains and was restless but cooperative. Physical examination revealed bilateral tense thighs and lower extremities with decreased lower extremity pulses on palpation. His initial laboratory workup revealed hyperkalemia, acute renal failure, very low bicarbonate and an elevated lactic acid. He was also coagulopathic and showed evidence of DIC with decreasing platelets. His hematocrit dropped to 17 within a few hours and initial CPK was found to be 70K. This quickly rose to over 200K. He was aggressively resuscitated in the medical ICU with IV fluids, packed red blood cells and fresh frozen plasma. Due to the DIC and coagulopathy, fasciotomy in legs to relieve the compartment syndrome was delayed for a few days. His ICU course was complicated by sepsis and ARDS while intubated, both of which he recovered from initially. Subsequently, he developed renal failure requiring hemodialysis. His ICU course was marked by an initial recovery followed by septic shock and subsequent death.
DISCUSSIONS: It has been well documented, over many years, the issue of acute rhabdomyolysis in African American military recruits who show evidence of sickle cell trait(SCT). In asymptomatic patients sickling is known to occur in low oxygen tension environment-most notably in the spleen and in the renal medulla. Severe exertion can cause rhabdomyolysis, limited compartment syndrome and renal failure in healthy individuals without sickle cell trait. Sickling diathesis does lower the threshold for the occurrence of compartment syndromes. In SCT patients during severe exertion, the plasma water loss causes hemoconcentration which causes increased blood viscosity and slowing of flow. This in turn causes oxygen deprivation to overworked muscles which promotes erythrocyte sickling and lactic acidosis. They then start to develop rhabdomyolysis which leads to compartment syndrome and sets up vicious cycle which fuels each other. They also develop myoglobinuria, acute renal failure, DIC, further acidosis and electrolyte abnormalities. These complications do not occur in all people with SCT. There are a number of possible risk factors why some are more susceptible to acute rhabdomyolysis and sudden death. The risk factors are deconditioning, age, exposure to extreme of temperatures, high altitude, use of weight loss supplements and last, a rare but significant factor is the co-existence of G-6-PD deficiency.
CONCLUSION: Patients with SCT are prone to develop acute rhabdomyolysis and CS after strenuous exercise. CS may be more frequent than reported and should be suspected in patients with SCT who present with rhabdomyolysis. At the same time, patients with SCT should be counseled about risk factors including strenuous exercise associated with rhabdomyolysis and compartment syndrome.
DISCLOSURE: Siddharth Shah, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Originally developed as an anti-depressant, linezolid is an oxazolidone antibiotic. Indicated for the treatment of vancomycin-resistant enterococcus and methicillin-resistant staphylococcus aureus, it has the unique property of Monoamine Oxidase (MAO) inhibition. The triad defining the hyperserotonergic state of serotonin syndrome (SS) includes mental status changes, autonomic instability, and neuromuscular irregularities. SS is well-documented in the setting of combination therapy with linezolid and other Selective Serotonin Reuptake Inhibitors (SSRI); however, to date there has been only one published case report on the interaction between diphenhydramine and linezolid. Diphenhydramine inhibits post-synaptic reuptake of serotonin.
CASE PRESENTATION: A 71-year-old woman, with a past medical history of congestive heart failure, and osteoarthritis, admitted to the hospital for an infected total knee arthroplasty for intravenous vancomycin, debridement, and myofasiocutaneous flap. Due to failure to heal, the hardware was removed, and a vancomycin-impregnated cement spacer was placed in the joint space. Initial wound cultures demonstrated ampicillin-sensitive Enterococcus faecalis. After 3 weeks of therapy with Vancomycin, the subsequent cultures were positive for vancomycin-resistant Enterococcus faecalis, and therapy was changed to linezolid and daptomycin. On day 32 she developed a maculopapular rash on her trunk and proximal extremities proven to be a drug eruption on biopsy. The vancomycin spacer was removed and she was started on intravenous diphenhydramine. The vesicles and bulla resolved over few days. On day 45, she was extubated, and showed signs of delirium with symptoms of paranoia, vivid auditory and visual hallucinations. She was tachycardic, febrile with autonomic instability and tremor. Concern for SS led to tapering of antimicrobial regimen to daptomycin. Measured serum serotonin prior to discontinuation was 2008 ng/ml (reference values 100–225), and subsequently fell to <25 ng/ml. Her neurological status improved over the course of three days, and at the time of ICU discharge she was alert and oriented to person, place and situation, hallucination-free, with hemodynamic stability.
DISCUSSIONS: SS is a potentially life-threatening adverse drug reaction that results from therapeutic drug use, self-poisoning or interaction between serotonergic agents and MAO inhibitors. SS can manifest as a wide spectrum of clinical symptoms, but is characterized generally by the triad of mental status changes, neurological abnormalities and autonomic instability. Often, this syndrome is under diagnosed as physicians are unaware of its clinical diagnosis The time course in our patient, as well as the coexisting symptoms, makes simple delirium unlikely. The possibilities are the enhanced toxic effect of diphenhydramine versus SS precipitated by enhancing the effect of diphenhydramine by linezolid. Linezolid is a reversible inhibitor of MAO A and B. MAO is responsible for the catabolism of catecholamines via oxidative deamination. Inhibition of this enzyme results in increased levels of norepinephrine, serotonin, and dopamine, with generalized stimulation of the sympathetic nervous system (SNS). Signs and symptoms of agitation, hypertension, hyperthermia, and tachycardia may result from severe cerebral excitation associated with increased central dopaminergic hyperactivity from high blood concentrations of MAO inhibitors.By impairing the body's ability to degrade catecholamines, linezolid has the potential to enhance the toxic effects of drugs that suppress the parasympathetic nervous system (PNS) leading to increased risk of CNS toxicity from over stimulation of the SNS (1). Diphenhydramine is such a PNS blocker, by blocking the muscarinic receptors, and inhibits post-synaptic reuptake of serotonin leading to generalized stimulation of the SNS. In fact, it was this discovery in the 1960s that led to the search for other SSRIs.
CONCLUSION: The SS witnessed in this patient was possibly the result of an interaction between linezolid, a nonselective MAO inhibitor, and diphenhydramine, an anticholinergic agent.
DISCLOSURE: Melissa Whitmill, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Spontaneous splenic rupture (SSR) is a rare but potentially life threatening condition demanding rapid diagnosis and treatment.
CASE PRESENTATION: 72-year-old Caucasian female with history of chronic obstructive pulmonary disease (COPD), hypertension and hypothyroidism presented to an outside hospital with complaints of shortness of breath and cough. She was treated for COPD exacerbation and community acquired pneumonia with steroids, bronchodilators and antibiotics. During the second day of her hospital stay she complained of chest pain and her electrocardiogram was suggestive of non-ST elevation myocardial infarction. Patient was then transferred to our facility for further management. She was given Lovenox 75 milligrams twice a day as a part of her treatment for ACS. On fourth hospital day she was found to be hypotensive with blood pressure 60/40 millimeters of mercury and was transferred to intensive care unit. Laboratory data revealed white cell count 25,000 per cubic millimeter, hemoglobin 9.8 grams per deciliter (gm/dl), hematocrit 29%, platelet count 170,000 per microliter, lactic acid 7.2 millimoles per liter, PT 18 seconds, INR 1.6, PTT 39 seconds. Differential diagnosis included septic and cardiogenic shock. She was fluid resuscitated, started on vasopressors for cardiovascular support and intubated for increase work of breathing. Six hours later hemoglobin and hematocrit dropped to 5.5 gm/dl and 15.9%. Suspected hemorrhagic shock was treated with packed red blood cells (PRBC) and fresh frozen plasma (FFP) transfusions. Computed Tomography (CT) Abdomen/Pelvis with oral contrast showed bilateral rectus sheath hematomas and large collection of intra-abdominal fluid/blood. Emergent exploratory laparotomy was performed and 4.5 liters of blood was evacuated from the peritoneal cavity. Spleen was found to be ruptured and splenectomy was performed with good control of the hemorrhage. Rectus sheath hematomas were evacuated. Pathologic examination of the spleen revealed a normal size spleen (126gm) with capsular disruption, areas of focal congestion, and no specific pathologic change. Patient's condition improved and she was eventually discharged to a rehabilitation facility.
DISCUSSIONS: Spontaneous splenic rupture unlike traumatic splenic rupture is a rare and thus a less recognized clinical entity. Orloff and Peskin (1) identified four criteria that define spontaneous splenic rupture: no history of trauma, no perisplenic adhesions suggestive of previous trauma, the absence of disease that affects the spleen itself and normal microscopic and macroscopic appearance of spleen. Negative serology for viral antibody titers suggestive of recent infection with types that affect spleen was added as a fifth criterion. Our patient fulfilled first four criteria, however viral serologies were not obtained. Many theories have been postulated to explain SSR that occurs in the absence of disease or trauma. A sudden increase in intra-abdominal pressure associated with exercise, coughing, or vomiting have been implicated as a potential cause of SSR. In our patient the cough was mild and intermittent. Splenic rupture typically manifests as abdominal pain and/or a falling hematocrit or shock. Laparotomy remains the diagnostic gold standard. CT scan is 89 to 96% sensitive and can more precisely localize the bleeding, however, in this case report the CT scan did not show splenic rupture but it did reveal bilateral rectus sheath hematomas and large amount of intra-abdominal fluid/blood. There are separate case reports of either abdominal wall hematoma or spontaneous splenic rupture where low molecular weight heparin (LMWH) was the only identifiable risk factor. In our case report both of these conditions were coexistent and no risk factor other than lovenox therapy could be identified.
CONCLUSION: This case reminds us that high index of suspicion is prudent to recognize spontaneous splenic rupture as a cause of shock in a patient on LMWH therapy.
DISCLOSURE: Soophia Khan, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Adrenal insufficiency related to adrenal hemorrhage is uncommon. Association of heparin induced thrombocytopenia (HIT) and antiphospholipid antibody syndrome (APL) with adrenal hemorrhage is well-known. However, combination of all three concomitantly presents the diagnostic and therapeutic challenge.
CASE PRESENTATION: A 49 yr old woman was transferred to our facility with hypotension. She initially presented with deep-vein thrombosis and pulmonary embolism. She was treated with unfractionated heparin and coumadin and transferred to a skilled nursing facility for rehabilitation. Her workup revealed APL with positive anticardiolipin antibodies (IgG and IgA). One week later, she developed tachycardia, chest pain, and shortness of breath. She was found to have an elevated troponin and underwent a cardiac catheterization after reversal of INR. Coronary arteries are patent. A repeat lower extremity Doppler performed revealed an extension of her deep vein thrombosis. She was treated with enoxaparin and then coumadin. Two days after initiating anticoagulation, she was found to have a decrease in her platelet count (95,000 from 222,000) and a platelet factor (PF4) ELISA test was strongly positive. Enoxaparin was discontinued and lepirudin was started with a target activated partial thromboplastin time (aPTT) of 45–60 seconds. The following day, she developed a sudden onset of hypotension. A baseline PTT was 46.6 sec (normal: 22–36 seconds) prior to any form of anticoagulation. A random cortisol level was 1.5 mcg/dl. A cosyntropin stimulation test was consistent with adrenal insufficiency. She was treated with hydrocortisone. An abdominal CT scan was performed (Figure 1). Figure 1: Contrast CT scan of the abdomen showing enlargement of both adrenal glands with a density of roughly 50 Hounsfield units. The right adrenal gland (arrow) measures 4 cm; the left adrenal gland measures 4.7 cm.
DISCUSSIONS: Adrenal insufficiency is commonly associated with hypotension. Clinical features of adrenal hemorrhage include abdominal pain (55%), hypotension (54%), fever (40%), nausea and vomiting (31%), weakness or fatigue (31%), and lethargy or altered mental status (19%). APL is characterized by clinical evidence of arterial or venous thrombosis associated with thrombocytopenia. HIT is characterized by a fall in platelet count by fifty percent of the baseline count associated with exposure to heparin caused by platelet activating antibodies. Bilateral adrenal hemorrhage has been described as a finding characteristic of the thrombotic complications associated with HIT in up to 5%. The association of adrenal insufficiency and thrombotic disease due to either APL or HIT is related to the unique nature of the vascular anatomy of the adrenal glands. The treatment of thrombotic complications associated with HIT includes the use of non-heparin products such as direct thrombin inhibitors (e.g. argatroban, bivalirudin, and lepirudin) and the anti-Factor Xa (FXa) inhibitor (fondaparinux). Argatroban and lepirudin are administered intravenously and require laboratory monitoring with the aPTT to maintain the appropriate anticoagulation level. A laboratory method of monitoring patients with APL in the presence of an elevated aPTT is the dilute thrombin time monitoring. This monitoring system is not widely available and limited data exists in terms of its efficacy. Fixed dose weight based argatroban regimen without laboratory monitoring is one potential management strategy with limited experience. Use of fondaparinux has been reported to successfully bridge to warfarin anticoagulation in a small group of critically ill patients in whom HIT was suspected without any laboratory monitoring. Our patient was successfully treated with fondaparinux.
CONCLUSION: Hypotension secondary to adrenal hemorrhage is an unusual complication associated with heparin induced thrombocytopenia. Adrenal vein thrombosis is the mechanism causing such hemorrhage. Anticoagulation with either direct thrombin inhibitors or anti-FXa inhibitors should be considered. Due to inability to monitor direct thrombin inhibitors in setting of elevated aPTT with APL and HIT, fondaprinux may be used.
DISCLOSURE: Maulik Patel, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Adult Onset Still's Disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It classically presents as fever or unknown origin (FUO) with a constellation of symptoms and signs. The diagnosis requires high index of suspicion after excluding other diseases. We are presenting a case of fulminant AOSD with pleuropericarditis and pulmonary capillaritis which is not common.
CASE PRESENTATION: A 60-year-old Japanese man was admitted with a 2 week history of fevers, sore throat, fatigue, myalgia, right knee arthralgia and a 10-pound weight loss. He had a fever of 104.5°F, heart rate of 130/min, respiratory rate of 32/min and blood pressure of 150/70 mmHg. Physical examination revealed pericardial friction rubs, bibasilar lung crackles and right knee tenderness without effusion. There was no hepatosplenomegaly. The white blood cell (WBC) count was 37 × 103 cells/mm3 (88% neutrophils and 7% bands). The hemoglobin, hematocrit and platelet counts were normal. Blood urea nitrogen, serum creatinine, urinalysis and liver function tests were normal. C-reactive protein (CRP) was 26.20 mg/dl (< 0.80 mg/dl) and erythrocyte sedimentation rate (ESR) was 65 mm/hr (0–10 mm). All infectious work up was negative. Antinuclear antibody (ANA) profiles, antineutrophilic cytoplasmic antibodies (ANCAs), rheumatoid factor (RF) and angiotensin converting enzyme (ACE) level were all normal. The serum ferritin level was above 8000 ng/ml (22–322 ng/ml). The first chest CT (fig 1A) showed an infiltration of the posterior segment of the right upper lobe. However, the repeated chest CT for clinical worsening showed an interval development of pericardial and bilateral pleural effusions (fig 1B). A thoracoscopic biopsy of right lung demonstrated focal capillaritis and hemosiderin-laden macrophages which suggested an alveolar hemorrhage.
DISCUSSIONS: After exclusion of infectious etiologies, common connective tissue diseases or vasculitides and malignancies, the diagnosis of an Adult Onset Still's Disease (AOSD) was the most likely especially with markedly elevated serum ferritin. AOSD is a rare systemic inflammatory disorder of unknown etiology and classically characterized by "quotidian or double-quotidian" fever, arthritis/arthralgia, and a salmon-pink evanescent rash. Estimated incidence of AOSD is 0.16 cases per 100,000 persons per year. The disease tends to affect younger people; the onset is between 16- 35 years of age. Manifestations include fever (94–100%), arthralgia or arthritis (64–100%), myalgia (56–84%), rash (51–94%), sore throat (35–92%), splenomegaly (14–65%), lymphadenopathy (32–74%), pleuritis (12–53%), and pericarditis (10–37%). Laboratory study usually reveals marked leukocytosis. A serum ferritin level above 1000 ng/ml has been used to suggest AOSD, although a reduction of its glycosylated fraction (<20%) is more specific. Treatments include NSAIDs, steroids, disease modifying antirheumatic drugs (DMARDs) such as methotrexate, intravenous immunoglobulin, anti-tumor necrotic factor agents (eg infliximab or etanercept), anakinra (IL-1 receptor inhibitor) and rituximab (CD20- antibody).Our patient met three major (fever, arthralgia and leukocytosis) and two minor (sore throat, negative ANA and RF) diagnostic criteria. He had a fulminant course with pleuropericarditis which required a pericardiocentesis and a focal pulmonary capillaritis. After trials of multiple agents, ultimately, his disease was in remission with a combination of high dose prednisone and subcutaneous anakinra.
CONCLUSION: The AOSD is a very rare cause of FUO that should be considered as a diagnosis of exclusion in patients for whom extensive evaluation has ruled out infections, common autoimmune diseases or vasculitides and malignancy. Pulmonary capillaritis is a rare presentation. Treatments include multiple immunomodulating agents.
DISCLOSURE: Atikun Limsukon, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Bullous lung disease is commonly seen in patients with COPD. We report a case of a giant lower lobe bulla in a young patient with bronchial asthma.
CASE PRESENTATION: A 19-year-old female presented with a two-week history of sharp pleuritic right-sided chest pain associated with worsening dyspnea. She had a history of asthma diagnosed at age 8, but was well controlled with inhaled fluticasone and albuterol as needed. She denied recent wheezing and did not feel better after albuterol use. No history of tobacco, drug or alcohol use. Family history was negative. No recent travel vigorous exercise was reported. A chest x-ray obtained by her primary care practitioner one week prior to admission was reportedly "normal". Physical examination revealed minimal air entry to the right base, but minimal wheezing. ABG on room air revealed pH 7.41, pCO2 37, pO2 99. Chest x-ray revealed a radiolucent zone in the right base. CT of the chest revealed a 13 cm bulla at the right base associated with right middle lobe atelectasis and shift of the mediastinum to the left. Alpha 1 antitrypsin levels were normal, genetic testing for cystic fibrosis was negative. Due to the worsening of the symptoms despite maximal therapy for her asthma, an open bullectomy was performed with improvement of her symptoms. The space occupied by the bulla was occupied by normal lung tissue. At six months follow up she is asymptomatic with well-controlled asthma.
DISCUSSIONS: A lung bulla is defined as an emphysematous space of more than 1 cm in diameter in the inflated lung. Its wall is formed by pleura, connective tissue and compressed lung parenchyma. They can be morphologically categorized into three types. Type 1 has a narrow neck and is well demarcated by pleura. There is a small amount of lung that is over distended. They commonly originate from subpleural locations in the apex of the upper lobe or along the costophrenic angle of the middle lobe or lingual. Type 2 bullae have a broad neck and are frequently located on the anterior and diaphragmatic surface. They have a high predilection for developing a pneumothorax. Type 3 represent an exaggerated form of emphysema and lie deep within the lung parenchyma. Bullae can also be subdivided as either primary associated with normal pulmonary parenchyma, or secondary associated with obstructive lung disease and emphysema. Most bullae do not require surgical resection. Patients with symptoms such as chest pain, dyspnea, and hemoptysis associated with the bullae that have not corrected with medical management may be treated surgically. Recurrent infections of the bullae require surgical bullectomy. Bullectomy is also indicated if the bulla is large enough to cause mediastinal shift and atelectasis of the contralateral lung.
CONCLUSION: Rapidly enlarging bulla may rarely complicate asthma. Bullectomy relieves symptoms and restores normal lung function.
DISCLOSURE: Yatin Mehta, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Recurrent respiratory symptoms in a child may result from infectious or noninfectious etiologies, including congenital airway abnormalities. This is the case of a previously healthy child who was referred for pulmonary evaluation because of recurrent pneumonia.
CASE PRESENTATION: A 13-year-old boy was healthy until one year prior to presentation when he was diagnosed with four separate episodes of radiographic proven right upper lobe pneumonias. Theses illnesses presented with fever, productive cough, and right-sided chest pain. Symptoms resolved intermittently with oral antibiotics. He denied hemoptysis, dyspnea, respiratory symptoms when eating, and weight loss. The child was athletic with no physical activity limitations. He had no tracheal surgeries, but was intubated once at three years of age for an adenotonsillectomy due to recurrent pharyngitis. Perinatal and family histories were non contributory. On exam, the patient had normal vital signs. He was well developed and his physical examination was unremarkable. The lungs were clear to auscultation over the tidal range. There was no digital clubbing. Spirometry revealed a mild obstructive pattern with no bronchodilator response. Chest radiographs revealed a persistent abnormality in the right upper lobe and lucency to the right of the trachea (Figure 1). Due to the abnormal radiographic findings, a chest computed tomography (CT) scan was obtained and demonstrated numerous diverticula or out pouches of the trachea in the right posterolateral region of the superior mediastinum (Figures 2 and 3). At least one fistulous communication was seen between the diverticula and the tracheal lumen. A barium esophagram showed no abnormalities; in particular, there was no evidence of a tracheoesophageal fistula. A flexible bronchoscopy revealed numerous, well-circumscribed diverticula along the posterior membranous tracheal wall and mainstem bronchi (Figure 4). The majority of diverticula were several millimeters in diameter. Injection of methylene blue into the trachea and diverticula did not result in its appearance in the esophagus or stomach. A concurrent esophagoscopy was unremarkable.
DISCUSSIONS: Recurrent respiratory tract diseases in children raise the suspicion of congenital airway anomalies, such as tracheoesophageal fistula, tracheal bronchus, or bronchopulmonary sequestration. Additionally, a rarely encountered finding is tracheal diverticulosis, characterized by multiple evaginations of the tracheal wall arising from congenital or acquired tracheal wall abnormalities. Although only a few adult cases have been reported, to our knowledge there are no reports describing children with such a profound number of tracheal diverticula, as seen in this patient, with no associated tracheomegaly. Tracheal diverticula are rarely seen in clinical practice and typically present in adulthood as an acquired single tracheal outpouching associated with chronic bronchopulmonary disease, diagnosed via radiographs or bronchoscopy. The diverticula typically occur between the cartilaginous and muscular portions of the tracheal wall along the right posterolateral region. On radiographs, the diverticula can appear as isolated paratracheal air cysts or communicating with the tracheal lumen, as in this patient. The cause of tracheal diverticulosis remains speculative, and may occur in isolation or in association with other congenital anomalies, such as tracheobronchomegaly (Mounier-Kuhn syndrome), in which there is herniation of the tracheal mucous membrane through an inherent muscular defect. Clinically, tracheal diverticula can lead to recurrent respiratory tract infections with cough, dyspnea, and chest pain. Supportive therapy, such as chest physiotherapy and aggressive treatment of respiratory infections, is aimed at minimizing the damage caused by the stasis of secretions in the diverticula.
CONCLUSION: Tracheal diverticulosis should be considered a cause of chronic respiratory symptoms and recurrent respiratory infections in children and adults. Thus, careful analysis of the central airways on radiographic studies and direct visualization should occur. Although most cases present in adulthood with a long history of respiratory symptoms, this case highlights the findings in a symptomatic child.
DISCLOSURE: Jason Caboot, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Right sided aortic arch (RSAA), aberrant left subclavian artery (aLSA), dilated origin of the LSA (Kommerell's diverticulum), and persistent left superior vena cava (LSVC) usually manifests in individuals younger than 35 years. Symptoms of tracheal or esophageal compression are common manifestations. These vascular malformations were discovered in an elderly patient who developed respiratory distress after tracheostomy tube (TT) change.
CASE PRESENTATION: A 70-year male with COPD, on home BiPAP ventilation for sixteen months was admitted to MICU following TT change to an uncuffed long distal tube. He was cyanotic, tachypneic, tachycardic, and anxious. Venous gasometry revealed: pH 7.33, pCO2 74.3 mmHg, and pO2 26 mmHg on FiO2 0.4. TT was changed to a cuffed one, and his respiratory distress improved. Bronchoscopy showed 80% occlusion at the distal end of the TT by a protuberant membranous trachea. CT chest demonstrated RSAA with, aLSA, and Kommerell's diverticulum causing anterior esophageal and tracheal displacement. On sagittal views, the TT tube was seen to be partially occluded by the displaced membranous trachea. In addition, a persistent LSVC was found. Later, a short distal TT was placed 2 cm proximal to the membranous protuberance. Thereafter respiratory symptoms improved.
DISCUSSIONS: There are limited reports of the constellation of vascular anomalies found in our patient. Autopsy series indicate a 3% frequency of anomalies of the aortic arch and its branches. These anomalies are usually asymptomatic, being an incidental radiological finding, except when they cause tracheal or esophageal compression. Respiratory symptoms are more common in children, whereas dysphagia is the most common symptom in older patients. Tracheal compression by a Kommerell's diverticulum mimicking bronchial asthma has also been described. Pain in the thorax, shoulders, and neck, as well as ischemia of the left upper extremity has been reported. Initial suspicion of these vascular malformations can be confirmed by CT or MRI. Arteriography is reserved for patients requiring corrective surgery. Resection of the diverticulum and the aberrant vessel followed by reanastomosis with one of the nearby large vessels usually has satisfactory results. To our knowledge, respiratory distress secondary to TT occlusion in these patients, and the presence of LSVC in association with RSAA and aLSA have not been described.
CONCLUSION: The presence of a right sided aortic arch with an aberrant left subclavian artery and Kommerell's diverticulum has to be considered in patients endotracheally intubated presenting with occlusion of the distal opening of the endotracheal tube.
DISCLOSURE: Shahida Bashir, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Double aortic arch (DAA) is the most common clinically recognized form of vascular ring, in which the trachea and esophagus are encircled by the aortic arch and its branches. The overall incidence is unknown. Half to two-thirds of patients undergoing vascular ring repair have a DAA; of these 42% have respiratory symptoms, one third have dysphagia and one third are asymptomatic. Surgical division of the arch (usually on the left) is associated with prompt relief of symptoms.
CASE PRESENTATION: A 14 year old girl presented with a history of intermittent cough only in the winter daytime since 2 months of age, and peanut allergy. In January 2008 she received intravenous steroids and epinephrine for presumed anaphylaxis associated with airway obstruction. She was prescribed inhaled bronchodilators, without benefit. She has had no problems with dysphagia and had a negative review of systems. On physical examination, she appeared well nourished, in no acute distress with normal vital signs and the rest of her physical examination was also normal. Spirometric findings were typical of a fixed intrathoracic airway obstruction. The chest radiograph showed characteristic narrowing in the intrathoracic trachea and air trapping. Echocardiogram revealed normal intracardiac anatomy. A contrast-enhanced computed tomography (CT) scan complemented by three dimensional CT reconstruction revealed DAA with a dominant right sided arch and tracheal narrowing in the mid thoracic trachea.
DISCUSSIONS: In DAA, the ascending aorta divides into left (anterior) and right (posterior) arches passing to either side of the trachea, the right arch being dominant in 70 % of cases. Associated cardiovascular anomalies are uncommon, but when present are usually cyanotic, including tetralogy of Fallot and transposition of great arteries. Usually diagnosed in infancy or early childhood, DAA commonly manifests with symptoms of stridor, dyspnea, cough and recurrent respiratory infections. Patients may experience dysphagia related to esophageal compression, which may manifest as vomiting and feeding intolerance. The diagnosis is suggested on the plain chest radiograph and barium swallow, and confirmed by CT or MRI scanning, which clearly demonstrate the arterial branching pattern and the location and extent of airway and esophageal obstruction. A 3-dimensional reconstruction of the aorta and airways can be a useful tool for preoperative planning. Echocardiography may be helpful in the evaluation of associated cardiovascular anomalies. The long-term prognosis for patients with repaired DAA is quite good. Persistent respiratory symptoms are the most common adverse outcomes. Pulmonary function testing reveals persistent upper airway obstruction in some patients. In one case series report, surgical relief of asymptomatic vascular rings was advocated in children to reduce the risk of tracheomalacia in adult life.
CONCLUSION: In summary, DAA is an important cause of persistent respiratory symptoms in infants, children or in adults. Early diagnosis and timely intervention can minimize airway complications. Further investigations including chest radiography, pulmonary function test and other imaging techniques are warranted.
DISCLOSURE: Sintra Phumethum, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Congenital cystic adenomatoid malformation (CCAM) is a congenital lung lesion rarely diagnosed in adulthood. We describe a patient with CCAM found to have mixed features of two congenital lung lesions.
CASE PRESENTATION: A 23 year-old female was hospitalized with fever, right upper quadrant pain, and cough. CT scan revealed multiple cystic lesions with air fluid levels in the right lower lobe (image 1). She denied any prior history of pulmonary illness, and her medical history was unremarkable. Blood and sputum cultures were negative. CT scan 3 years prior showed pre-existing cystic lung disease in right lower lobe (image 2).She underwent right-sided thoracotomy. Operative findings included dense consolidation of right lower lobe with multiple abscess cavities. A large arterial feeding vessel was discovered originating from the aorta. Right lower lobectomy was performed and the patient was given a preliminary diagnosis of pulmonary sequestration (BPS). Pathologic examination revealed alveoli with absent cartilage, and cystic structures lined with pseudostratified, ciliated columnar respiratory epithelium (image 3). These histologic features are consistent with congenital cystic adenomatoid malformation (CCAM), a rare congenital lung lesion. This lesion is most frequently diagnosed antenatally, and is very rarely found in adults. The finding of a systemic arterial blood supply represents an exceedingly rare mixed lesion consisting of CCAM and BPS.
DISCUSSIONS: CCAM is rare, occurring in 1 in 25,000–30,000 pregnancies. It results from an insult to the developing bronchopulmonary foregut during the first 6–7 weeks of gestation, resulting in abnormal bronchioalveolar airspaces and absence of alveolar cartilage. Stocker et al. classified this lesion into three histologic types: Type I consists of multiple large cysts (up to 10cm) or a single large cyst. This is the most common (50–70%). Type II contains multiple small cystic structures less than 2 cm. Type III is a bulky, solid mass with cysts less than 0.5 cm. (1) Up to 90% of CCAM lesions are diagnosed antenatally by ultrasound or x-ray, and most are successfully treated by surgical resection in infancy. Very rarely, CCAM can remain undiagnosed into adulthood. Morelli et al. reviewed 45 cases of postnatal and adult CCAM. The most common clinical findings included asymptomatic presentation, recurrent pneumonia, cough, or pneumothorax. Surgical resection is the treatment of choice due to the wide range of etiologies for cystic lung disease in the adult. Histologic confirmation is imperative to exclude malignancy or other causes. (1) Cass et al. report 6 cases of fetal CCAM-BPS lesions diagnosed prenatally, and have described this as a "hybrid" lesion. There are 19 cases of CCAM-BPS lesions in the pediatric literature, all were diagnosed prenatally. All were surgically resected by two years of age, with a survival rate of 94%. (2) At present, there are no adult case reports of a lung lesion with histologic features CCAM and systemic arterial blood supply.
CONCLUSION: Congenital lesions must be considered in an adult with focal cystic lung disease. Surgical resection is essential for definitive diagnosis, and prognosis is excellent. This is a unique case of CCAM with a systemic arterial vessel. This very rare lesion has been described in fetal and pediatric cases but to our knowledge none have been reported in an adult.
DISCLOSURE: Carrie Samiec, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: We present a case of a rare congenital pulmonary vascular anomaly presenting as pulmonary hypertension in an adult.
CASE PRESENTATION: A 52-year-old woman presented to the emergency room with a two week history of progressively worsening shortness of breath and bilateral lower extremity edema. She was afebrile, with a blood pressure of 130/82 mm Hg, heart rate of 91 beats per minute, respiratory rate of 23 breaths per minute, and pulse oxygen saturation of 91% on 4 liters via nasal cannula. Physical examination was significant for mild jugular venous distension, no cardiac murmurs, clear lung sounds, and 1+ pitting edema to mid-calf bilaterally. A chest radiograph revealed left pulmonary artery enlargement. Transthoracic echocardiography showed a pulmonary artery pressure (PAP) of 120/50 mm Hg and an ejection fraction of 55%. A bubble study was negative for any intracardiac shunt. There was no evidence of collagen vascular disease, underlying pulmonary or hepatic disease, or HIV infection. As part of an evaluation for thromboembolic disease, lower extremity duplex ultrasound was negative, but a ventilation-perfusion lung scan showed absence of perfusion to the right lung, with normal ventilation [Fig 1]. A CT pulmonary angiogram [Fig 2] showed no intra-arterial filling defects, but massive enlargement of the main pulmonary arterial trunk and left pulmonary artery with proximal right pulmonary artery interruption. Intercostal and chest wall arteries on the right were dilated consistent with systemic collateral flow to the right lung. Right heart catheterization and pulmonary angiography confirmed isolated proximal right main artery interruption with a PAP of 92/29 and mean PAP of 58 mm Hg. The patient was diagnosed with pulmonary arterial hypertension (PAH) complicating congenital proximal pulmonary artery interruption. Because of the lack of visualization of a distal pulmonary artery and the chronic severe nature of the pulmonary hypertension, she was not considered a surgical candidate. She was medically optimized on sildenafil, lasix, digoxin, and anticoagulation.
DISCUSSIONS: Proximal interruption of the pulmonary artery is a rare congenital anomaly. Although previously termed unilateral absence of the pulmonary artery because of the apparent termination before or at the hilum, proximal interruption is a more accurate description since the vascular network within the ipsilateral lung is intact and patent. The affected lung is perfused via systemic collateral vessels.1,2 Cardiac malformations are less frequent with right-sided proximal interruption. A subgroup of patients with right proximal pulmonary artery interruption without cardiac anomalies or pulmonary hypertension in infancy usually survive to adulthood. These individuals experience recurrent hemoptysis or pulmonary infections. Many patients remain asymptomatic through adulthood, the condition first being recognized during evaluation for abnormal chest radiograph. Our patient presented with severe PAH and cor pulmonale due to right proximal pulmonary artery interruption. Since she had been asymptomatic most of her life, PAH most likely developed over time as a complication of high flow through the pulmonary arterial system in the left unaffected lung. Our case stresses the importance of seeking underlying conditions in patients who present with PAH. Our patient's congenital anomaly was discovered during a work-up for thromboembolic disease as a possible cause for severe pulmonary hypertension. Since the pathophysiology of pulmonary hypertension was similar in our patient, we initiated treatment with sildenafil.
CONCLUSION: This is an unusual case of pulmonary hypertension, which in the course of work-up for an underlying cause was found to be due to a rare congenital anomaly. It illustrates the importance of the recommended investigation for associated conditions in patients with newly recognized pulmonary arterial hypertension.
DISCLOSURE: John Cho, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Kombucha "mushroom" tea, sold in popular health food stores, is touted to have healing properties, including boosting immunity in HIV. Here, we present a case of severe hyperthermia, lactic acidosis, and acute renal failure in a 22 year old male within 15 hours of Kombucha tea ingestion.
CASE PRESENTATION: A 22 year old Filipino gay male with a recent diagnosis of HIV, was seen in the ER after developing acute onset of shaking fevers and chills. One week prior to admit, the patient purchased a bottle of unpasteurized Kombucha tea, as he believed it could boost his immune system. The patient consumed this tea the night before admission, sharing a 1 liter bottle with an HIV negative friend. His life partner reports he had a tactile temperature that night, though appeared well in the morning. Eight hours later, the patient began shaking uncontrollably, became short of breath and was febrile to 103.0F. Review of systems was otherwise negative. The patient was diagnosed with HIV two weeks prior to admit, at which time his CD4 count was 414. HAART had not been initiated. He also has known anaphylaxis to peanuts. Both he and his life partner adamantly denied alcohol, tobacco, or illicit drug use. In the ER, the patient was febrile to 104.1F, HR 180s, BP 192/105. The patient became combative and confused, requiring sedation and intubation for airway control. Pupils were dilated but reactive. Neurological assessment was nonfocal. The remainder of his physical exam was negative.Laboratories revealed a lactate of 12.9, ammonia level 214, AST 57. LFTs were otherwise normal. ABG showed 7.32/31/446/16/100%. Chemistries showed a bicarb 16, AG 25, and Cr 2.1. Urine toxin and ethanol levels were negative. Head CT was negative. Lumbar puncture testing was normal. Empiric antibiotic therapy for meningitis and encephalitis was initiated and quickly discontinued, per infectious disease recommendations, as the patient remained afebrile after admit without evidence of infection. Within 36 hours of Kombucha tea ingestion, the patient dramatically improved with supportive care. He was extubated and discharged home on hospital day #2.
DISCUSSIONS: Kombucha "mushroom" tea, synonymous with Manchurian or Kargasok tea, is fermented black tea in a yeast-bacteria medium. In the HIV community, it is popular for its reported ability to improve T cell counts. These claims have not been confirmed via objective scientific investigation. Review of the literature reveals little data on the medicinal effects, side effects, or potential toxicity of this tea. There are several case reports of serious, and in some instances, fatal, hepatic dysfunction and lactic acidosis, within close proximity to Kombucha tea ingestion. Microbiological studies have revealed evidence of numerous types of yeast and bacteria present during the fermentation process, including Aspergillus. Leeching of toxic material into this tea during the fermentation process, particularly with home-brewed tea, is of particular concern. Lead toxicity has been linked to fermentation of Kombucha tea in a lead-based ceramic pot.
CONCLUSION: While Kombucha tea is considered a healthy elixir, there currently is no scientific data to support this claim. The limited evidence currently available raises considerable concern that Kombucha tea may pose serious health risks, particularly in the immunodeficient population. Consumption of this tea should be discouraged, as it may be associated with life-threatening lactic acidosis.
DISCLOSURE: Alison Kole, None.
INTRODUCTION: About 2000 cases of fulminant hepatic failure occur annually in the United States and the mortality rate ranges from 30–80% despite maximal supportive care. The most feared complication is the development of cerebral edema leading to raised intracranial tension and subsequent brain herniation and death. The prognosis of patients with raised ICP in the setting of acute liver failure remains grim without emergent liver transplantation. Therapeutic mild hypothermia has been used as a bridge to transplantation, but has not been studied as an independent therapeutic measure in this setting. We report a case of fulminant liver failure and cerebral edema from acetaminophen which was treated with moderate hypothermia.
CASE PRESENTATION: Our index patient was admitted to the medical ICU of our tertiary care institution after being found unresponsive with acetaminophen toxicity. She had ingested 56 grams of acetaminophen over a 2 day period. On initial presentation, patient had stage 3 hepatic encephalopathy, and her depressed mental status was noted 24 hours preceding admission. Initial evaluation confirmed fulminant hepatic failure from acetaminophen, and cerebral edema. Our patient was treated with hyperosmolar therapy, hyperventilation, sedation and chemical paralysis. Her intracranial pressure (ICP) remained elevated despite maximal medical therapy. Therapeutic hypothermia was initiated at that point, with patient listed for emergency orthotropic liver transplantation. Over the next few days, her hepatic synthetic functions began to improve, but continued to have high ICP whenever reversal of hypothermia was attempted on days three and four. On day five she tolerated re-warming well without increase in her ICP. She was transferred out of the ICU on day 9, and discharged on day 16 from the hospital. On the day of discharge she had near complete recovery of neurological and hepatic functions.
DISCUSSIONS: There is an emerging body of literature that supports the use of mild to moderate therapeutic hypothermia for the therapy of cerebral edema and intracranial hypertension associated with FHF. Although data on the impact of raised ICP on survival in liver failure are scant, a mortality of more than 90% is expected in patients whose ICP cannot be controlled by conventional means (1). In a study of 315 patients with acute liver injury secondary to Tylenol overdose, 50% of deaths in the group that did not meet liver transplantation criteria and were deemed to have a good prognosis otherwise expired from cerebral herniation (2). These data suggest that brain herniation can occur even in those whose liver is recovering. The only definitive treatment for patients whose hepatic function is unlikely to recover is liver transplantation. It is thus imperative to aggressively control ICP in patients who are suitable transplantation candidates as a bridge to liver transplant. This intervention also permits those who have a good prognosis adequate time for hepatic recovery.
CONCLUSION: In patients with fulminant hepatic failure and cerebral edema from the same, prolonged therapeutic hypothermia could potentially be used as a life saving therapy without liver transplantation. Therapeutic hypothermia decreases cerebral edema by multiple mechanisms. A clinical trial of hypothermia in patients with acute liver failure is warranted to further evaluate the use of this potentially lifesaving therapy.
DISCLOSURE: Shibin Jacob, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Evans syndrome is a rare condition in the adult population and is defined by the presence of autoimmune hemolytic anemia with thrombocytopenia and occasionally neutropenia We report the case of an individual with catastrophic hemolyses in a 24-year-old man with Evan's syndrome which was successfully treated with the anti-CD 20 monoclonal antibody rituximab, after failure of conventional therapies.
CASE PRESENTATION: A 24 year-old man with Evans Syndrome presented with a 3-day history of fever, diaphoresis, and cough. The past medical history was significant for long term anemia, thrombocytopenia and splenectomy. On physical examination he was a thin man in mild respiratory distress. Temperature was 38.9°C, blood pressure 117/58 mmHg, pulse rate of 132 /min, and respiratory rate of 27 /min. Oxygen saturation was 95% while receiving 4 liters/ min per nasal cannula. There was Eugophony and wheezing over the right lower lung field. The remainder of the physical examination was unremarkable. Laboratory workup revealed hemoglobin of 8.2 g/dl, white blood cell count of 21,100 /ml (59% neutrophils, 30% band forms, 6% lymphocytes, and 5% monocytes), and platelets of 32,000/mm 3. Peripheral smear showed ovalocytes, spherocytes, anysocitosis and poikilocytosis. Reticulocytes were elevated (9.9%), liver panel was normal except for an increase in total bilirubin and indirect bilirubin (2.6 mg/dl); coombs test, direct and indirect, were both positive. Hemoglobin electrophoresis, HIV, HEP C, and B serologies, complement and ANA were all normal. A chest radiograph showed patchy opacifications in the right middle lung zone with air bronchograms. The patient was admitted to the ICU with a community-acquired pneumonia and acute autoimmune hemolytic anemia. Antibiotic treatment was initiated with ceftriaxone and azitromycin. He received bronchodilators, supplemental oxygen, methylprenilosone 125 mg IV every 6 hours, IVIG (600 MG /KG) QD. Four hours later his hemoglobin decreased to 4 g/dl. He required transfusions over the following 24 hours. On Hospital day 2 dyspnea worsened and he required mechanical ventilation. Hemoglobin failed to raise over 5 g/dl despite transfusion of 18 units of PRBC's, indirect bilirubin increased to 9.0 mg/dl and the patient became jaundiced. Due to lack of response to IVIG and methylprednisolone, a trial of 375 mg of rituximab were given. There was significant hematological improvement after a single dose, (Hg of 10 mg/dl and platelets to 165,000 /mm3). Patient did not required furthered transfusions. We were able to extubate patient by day 3. The patient was discharged home in stable condition after 10 days from admission.
DISCUSSIONS: Evan syndrome is characterized by a chronic course with frequent exacerbations and remissions. Conventional treatment includes corticosteroid, splenectomy, IVIG or immunosuppressive agents such as cyclosporine, mycophenolate mofetil, vincristine, and danazole. More recently some cases have been treated with rituximab. Rituximab has gained widespread acceptance in the management of B-Cell malignancies. Rituximab is a monoclonal antibody against CD-20. Binding of CD-20 receptor by rituximab depletes B Cells in the circulation and in the lymphoid tissue. In this case rituximab was able to stop the production of auto antibodies allowing to stop the cycle of autoimmune hemolyses restoring immune tolerance.
CONCLUSION: In very severe cases of autoimmune hemolytic anemia refractory to conventional therapies rituximab may be an effective option.
DISCLOSURE: Shigeki Saito, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: We present a rare case of severe sepsis with purpura fulminans and multi-organ dysfunction from Capnocytophaga canimorsus following a dog bite in a patient without known risk factors.
CASE PRESENTATION: 48 year old white man with no significant past medical history presented to the Emergency Department with nausea, vomiting, abdominal pain, fever and numbness in his legs 24 hours after a dog bite. He rapidly developed respiratory distress requiring intubation and mechanical ventilation. He was febrile with marginal blood pressure requiring fluid resuscitation. Physical exam was significant for cyanosis of the lips, dry mucous membranes, dog bite on the right hand and positive Babinski with negative meningeal signs. Labs showed WBC-17, H/H-15.5/44.3, plt-9,000, BUN-35, creat-3.7, serum lactate of 7.4, the rest of metabolic panel was within normal limits. He developed DIC within a day of his presentation associated with purpuric skin rash. Peripheral smear showed abundant Gram negative bacilli within the neutrophils and extracellularly. Broad spectrum antibiotics were started early upon presentation. His ICU course was complicated by non ST elevation MI with peak Troponin I level of 8.79 and disseminated intravascular coagulation (DIC). His renal function deteriorated further requiring hemodialysis. Both blood cultures drawn on the day of presentation grew Capnocytophaga canimorsus which was identified by DNA sequencing. Antibiotics were de-escalated to ceftriaxone and levofloxacin. The patient received platelet and blood transfusions for DIC and was treated with plasmapheresis for a total of 4 sessions. He improved dramatically within 2 weeks of his presentation with resolution of renal failure and hematologic abnormalities. On follow up visit in the clinic the patient had complete resolution of all symptoms and regained his baseline functional status.
DISCUSSIONS: Capnocytophaga canimorsus is a fastidious gram-negative rod that is present in the normal oral flora of dogs and cats. It was first isolated by Butler and colleagues in 1977 from a patient with a dog bite. It can cause fulminant sepsis, meningitis and endocarditis. Risk factors include chronic lung disease, splenectomy, immunosuppression and alcoholism. The mortality rate for C. canimorsus septicemia is 30–36%. The increasing frequency of C. canimorsus may be related to more pet owners, greater animal bites and enhanced lab techniques to isolate the organism. C. canimorsus should be suspected in any case following a dog bite as prompt therapy especially in high risk population may prevent a potentially fatal course. Due to the slow growth of this bacterium in culture, C. canimorsus is often difficult to isolate and identify, therefore Gram-staining of a peripheral blood smear may provide an early diagnosis thus avoiding delay before appropriate antibiotic therapy is started.
CONCLUSION: A dog bite leading to septicemia is rare in the normal population. Clinicians should consider C. canimorsus in patients presenting with sepsis and a history of dog bite or animal exposure. Since the organism grows slowly, the clinical laboratory should be alerted to its potential presence. Finally, physicians should inform patients with immune suppression and splenectomy that dogs or animal contact can be important risk factors for Capnocytophaga.
DISCLOSURE: S Martin, None.
INTRODUCTION: Diffuse alveolar hemorrhage (DAH) occurs in about 5% of hematopoietic stem cell transplant (HSCT) recipients with a mortality rate of 30 - 70% (1). We describe a patient with recurrent episodes of DAH occurring late in the post-HSCT course.
CASE PRESENTATION: A 14 year old boy with aplastic anemia was treated with a matched-sibling non-myeloablative allogenic HSCT. His post-transplant course was complicated by acute graft-versus-host disease (GVHD) of the gut, skin and eyes as well cyclosporine-associated posterior reversible leukoencephalopathy syndrome. On day 110, he developed abdominal pain and on hospital day 3 underwent a laparoscopic cholecystectomy for acalculous cholecystitis. On post-operative day 1, his oxygen requirement and work of breathing increased and a chest radiograph showed bilateral alveolar infiltrates without effusions (Figure 1). Diuretics and antibiotics were administered. His immunosuppressive regimen consisted of cyclosporine and prednisone. On post-op day 3, he required intubation, a fraction of inspired oxygen (FiO2) of 0.70 with 12 cm of H2O positive-end expiratory pressure (PEEP). He had bloody tracheal secretions with a platelet count of 58,000/αL and a prothrombin time and activated partial thromboplastin time of 24.4 sec and 59.5 sec, respectively. An echocardiogram showed normal left ventricular function and chest computerized tomography showed dense bilateral, centrally-located lung consolidations (Figure 2). Tracheal aspirates were negative for bacterial or fungal pathogens but did reveal hemosiderin-laden macrophages. A presumptive diagnosis of DAH was made and he was treated with platelets, fresh frozen plasma, recombinant factor VIIa (rVIIa) and methylprednisolone (3mg/kg/day). Within 24 hours, his FiO2 was 0.40 with 5 cm of H2O of PEEP and he was extubated (post-op day 7). Repeat chest radiographs showed improvement in his air space disease. The patient subsequently experienced four episodes of hypoxic respiratory failure on post operative days 13, 21, 33 and 39, all consistent with DAH. Each episode followed tapering of methylprednisolone from 3mg/kg to 0.5mg/kg over 7 days. These episodes responded promptly to rVIIa infusion and increases in steroid doses. Bronchoscopy with bronchoalveolar lavage (BAL) was performed during his third episode. Active bleeding was observed in multiple segments bilaterally and the lavage was bloody. BAL cultures were negative for bacterial, fungal or viral pathogens and cytology showed hemosiderin-laden macrophages that stained positive for intracellular iron. Due to the refractory DAH with steroid and rVIIa therapy, two doses of rituximab were given to treat his GVHD and the bleeding resolved without recurrence.
DISCUSSIONS: In the absence of infection, DAH after HSCT may be a manifestation of GVHD resulting from idiopathic lung syndrome, diffuse alveolar damage or capillaritis. Increasing immunosuppression with corticosteroids has been the mainstay of DAH therapy. The unique features of this case are the multiple episodes of DAH that were responsive to rVIIa and the use of rituximab resulting in a successful clinical outcome. Recently, rVIIa has been used successfully in the HSCT population for a variety of hemorrhagic conditions, including DAH (2). Rituximab is an anti-CD20 antibody that has been used to treat refractory GVHD. Animal models suggest a link between severe GVHD and DAH, therefore rituximab may influence the inflammatory process in DAH as well.
CONCLUSION: Diffuse alveolar hemorrhage after HSCT may be treated with rVIIa. In refractory cases, the addition of rituximab to treat a pulmonary manifestation of GVHD, may provide an additional therapeutic approach.
DISCLOSURE: Jason Elinoff, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: A 5-month-old male infant presented with fever, vomiting, deterioration of consciousness and respiratory failure. He had a difficult intubation with multiple attempts and subsequently multiple failed extubations. Bronchoscopy revealed bilateral vocal cord paralysis, finally diagnosed as infant botulism.
CASE PRESENTATION: The patient was brought to a local community hospital with a history of increasing drowsiness. He had had 4 days of fever and 1 day of vomiting prior to admission. Physical examination revealed an afebrile infant with cold extremities and lethargy. His Glasgow Coma Scale was 6. He had tachycardia, tachypnea and respiratory distress. Lungs were clear to auscultation. He went on to develop respiratory failure and experienced cardiac arrest (of 23 minutes duration) due to hypoxia during a difficult intubation. Cardiovascular stability was maintained with a dopamine infusion prior to referral. Upon arrival, his Glasgow Coma Scale was 3 and significant hypotonia was present. His pupils were 5 mm, non-reactive. Broad spectrum antibiotics and acyclovir were administered. Initial investigations including complete blood count, urinalysis, electrolytes, renal function and liver function tests were unremarkable. Chest radiography, neuroimaging, and echocardiogram were all within normal limits. His blood, nasopharyngeal swab, non-bronchoscopic bronchoalveolar lavage and urine cultures were all negative. He was fully awake and alert on the third day after intubation. As he had a good cough and a gag reflex was excellent, the decision was made to extubate him on the 4th day of admission. Biphasic stridor, increased work of breathing and paradoxical movement of chest and abdomen were observed. He was re-intubated within 2 hours. Subsequent extubations were attempted on the 5th and the 7th days after admission. Neither attempt was successful. His neurological examination was within normal limits except he had a persistent mild truncal hypotonia. The repeat head and neck CT scan were unremarkable. Chest CT scan revealed only bibasilar and right apical subsegmental atelectasis. The ultrasonography of diaphragm revealed normal diaphragmatic movement. Flexible bronchoscopy was performed and bilateral vocal cord paralysis was discovered. Two weeks after admission, extubation was again attempted. The patient suddenly developed biphasic stridor and acute respiratory distress. The flexible bronchoscopy was performed and revealed immobilized left vocal cord and partial movement of the right vocal cord. The patient was re-intubated with a plan to re-evaluate in 1 week before considering tracheostomy. A successful extubation was performed on day 20 of admission. Stool Clostridium Botulinum toxin B came back positive. There was no history of honey or canned-food ingestion. The hypotonia was gradually improved without major neurological impairment. He was discharged home after 1 month of hospitalization.
DISCUSSIONS: Infant botulism is the most frequent form of human botulism caused by Clostridium botulinum which is colonized in small intestines before hematogenous spread. Most of affected infants presents between 1–6 months of age. The definite diagnosis can be made from positive botulism toxin or spores in stool. Enteric toxin causes intestinal immotility while neurotoxin blocks pre-synaptic Acetylcholine release, causing bulbar palsy, skeletal muscle weakness, flaccid paralysis and respiratory arrest. Most of the patients recovered without sequalae. Botulism immunoglobulin is now available and may be useful if it is given within 2 weeks after onset.
CONCLUSION: Botulism should be considered in an infant who presents with poor feeding, lethargy, weakness and respiratory failure.
DISCLOSURE: Harutai Kamalaporn, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Lung volume reduction surgery has been shown to be an effective treatment for severe heterogenous emphysema, where there is hyperinflation and parenchymal destruction in one area of the lung and relative preservation of tissue and function in the remaining compressed lung. This case illustrates an unusual situation where the relatively preserved lung was compressed and compromised by the emphysematous hyperinflation and also by a large intrapleural mass. It also demonstrates that very low forced expiratory volume within 1 second (FEV1) is not a contraindication for thoracic surgery if recruitment of viable lung tissue is expected as a result of the surgery.
CASE PRESENTATION: A 66 year old female with severe emphysema presented with gradually worsening dyspnea on exertion over the course of 2 years. A chest x-ray in March 2006 was interpreted as showing elevation of the right hemidiaphragm (image 1). She became increasingly cachectic (body mass index = 14.7) and ultimately oxygen dependent by March 2008. Chest computed tomography scan was obtained showing a large inhomogeneous mass in the base of the right chest cavity with compression of surrounding lung tissue. Her FEV1 was 0.68 Liters (28% predicted), oxygen saturation on room air was 72%, and arterial partial pressure of oxygen (paO2) on room air was 50 torr. She underwent a right thoracotomy under general anesthesia supplemented with a thoracic epidural. A pedunculated 1.5 kilogram benign solitary fibrous tumor of the pleura (SFTP) arising from the visceral pleura of the right lower lobe was removed (image 2). An apical volume reduction-like wedge resection of the most diseased portions of the right upper lobe was performed to assist with lung mobilization difficulties due to chronic pleural adhesions. Post-operative recovery was uncomplicated and an increase in paO2 to 70 torr on room air was noted.
DISCUSSIONS: Solitary fibrous tumors are rare tumors of mesenchymal origin which can arise from a number of anatomical locations. Most commonly they are found originating from the visceral pleura. Nearly 800 cases of SFTP have been reported in the literature to date. Approximately 12% of SFTP are malignant, based mainly on the tumor's histopathologic appearance. Clinically, malignant SFTP are prone to reoccur locally, metastatic disease is rare. Complete surgical resection, when possible, is the treatment of choice in all variants of SFTP, and complete resectability with tumor-free margins is the single most important predictor of favorable outcome.Generally, an expected post-operative FEV1 of less than 0.8 Liters or 40% of predicted is considered a contraindication to thoracic surgery because of the high risk of post-operative morbidity and mortality. However, each case must be evaluated for individual factors that may predict otherwise. In the described case, not only was the underlying mass considered resectable, careful evaluation of the available images suggested that a significant amount of viable lung tissue was being compromised by the mass effect. Removal of the mass would permit reexpansion of the underlying compressed lung with likely relatively preserved function. It was also felt that diaphragmatic function would be likely improved with the removal of the 1.5 kilogram mass impeding its normal movement.
CONCLUSION: Evaluation of patients' suitability for thoracic surgery is of vital importance, especially in cases of severe lung disease. Consideration should be given to cases otherwise deemed inoperable if, by the nature of the surgery (improved mechanics of breathing, volume reduction of nonfunctioning lung, etc.) the lung function is predicted to improve.
DISCLOSURE: Lorenzo Klein, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Nocardiosis is an invasive disease caused by members of the aerobic Actinomycetes genus, found in soil and organic matter. There are approximately 500 to 1,000 cases a year in the United States. This is an underestimation because Nocardia is not a reportable disease. An increasing number of Nocardia infections have been noted in the past 20 years as a result of the increasing numbers of immunocompromised hosts and improvement in laboratory techniques (1).
CASE PRESENTATION: A 45-year-old gentleman presented with fever of 104F 8/06, severe pleuritic pain, cough, dyspnea and sweating for several days. Past medical history: asthma since childhood, long standing extensive pulmonary fibrosis secondary to stage 4 sarcoidosis diagnosed by TBB 1997, active pulmonary tuberculosis while in prison treated 08/04-08/05 with 4 medications, with repeated sputum AFB smears and cultures becoming negative on treatment. A left pleural effusion was noted on 1/06 for which thoracentesis demonstrated a transudate negative for all cultures. MEDICATIONS: prednisone 30 mg daily, hydroxychloroquine 200 mg and home oxygen. FAMILY HISTORY: positive for sarcoidosis in the older sister. SOCIAL HISTORY: denies tobacco, alcohol or drug abuse. HOSPITAL COURSE: 110/85, HR 132, temperature 103.4, RR 18 Diaphoretic, mild respiratory distress. Left lower lobe crackles, wheezing. LABS: WBC 16.9, Chest x-ray: Bilateral densities and left lower lobe large opacity. CT of chest: bilateral interstitial changes, loculated left pleural effusion. The patient was admitted for pneumonia. IV Moxifloxacin and ceftriaxone were started and prednisone continued. The patient subsequently developed chills, and greater tachycardia, and was transferred to ICU. He then underwent thoracotomy, decortication and drainage. Biopsy showed organizing fibrinous pleuritis with negative AFB and GMS. Antibiotic therapy now included streptomycin, Bactrim, acyclovir, Rocephin and Biaxin. Culture of pleural peel grew Norcardia species, which was identified by CDC as N cyriacigeorgica. Susceptibility testing showed the following: amikacin, (MIC 1 susceptible); imipenem, (MIC 8 intermediate) ceftriaxone, (MIC 2 susceptible); sulfonamides, (MIC 8 susceptible); amoxicillin/clavulanate (MIC 16/8 intermediate), Vancomycin (MIC > 32 resistant). Accordingly Bactrim was added. The patient improved clinically. Chest x-ray showed resolution of pleural effusion.
DISCUSSIONS: Nocardia cyriacigeorgica is a recently described species. Of Nocardia species, the most frequently involved in human infections are members of the Nocardia asteroides complex. The members of this complex were subclassified into six different drug susceptibility types. Numerous new species of the Nocardia asteroides complex have been recently described including N. cyriacigeorgica . This species corresponds to strains of drug pattern type VI. It has been rarely reported in human infections so far. Pulmonary nocardiosis can be acute, subacute, or chronic. Acute norcardiosis infection can present as an isolated lung abscess or necrotizing pneumonia. Cavitating lesions can cause empyema or complicated parapneumonic effusions. The indolent progression of nocardiosis mimics other chronic granulomatous infections or pulmonary malignancies. Medical therapy with Sulfonamide remains the initial treatment and the mainstay. Clinical improvement is noted in one week and antibiotic levels reveal excellent tissue penetration. Several other antibiotics have been reported to be successful, but tetracycline derivatives (eg, minocycline), aminoglycosides, and carbapenems (imipenem-cilastatin, meropenem) have been the safest and most effective alternatives. Surgical therapy is recommended in patients who have localized abscesses, including CNS or empyema.
CONCLUSION: Pulmonary nocardiosis can present as an acute, subacute, or chronic condition with many different clinical presentations. Disseminated nocardiosis can occur in any patient, but is usually present in immunosuppressed patients with predominant involvement of the meninges and brain tissue. Nocardia infections should be suspected when pulmonary, skin, or disseminated disease occurs in immunosuppressed patients.
DISCLOSURE: Sherif Latef, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Primary spontaneous pneumothorax is a common condition often occurring in tall individuals and smokers. Familial primary spontaneous pneumothorax is a rare occurrence often associated with autosomal dominant inheritance and other organ system involvement.
CASE PRESENTATION: 63 year old female previously in good health presented to the emergency department with complaint of chest pain and shortness of breath. Vital signs were with in normal range with temperature 98.9 degrees Fahrenheit, heart rate 84 beats per minute, respiration 18 breaths per minute, blood pressure 120 mmHg systolic over 70 mmHg diastolic. Pulse oxymetry was 96 percent on room air. Patient was a non smoker, employed as a teacher. Physical examination demonstrated normal S1 and S2 heart sounds with regular rate and rhythm, lung sounds were diminished on the right as compared to the left side. Neurological exam was grossly normal; extremities did not show clubbing, cyanosis or edema. Laboratory evaluation revealed no abnormalities with complete blood count, serial cardiac enzymes or complete metabolic panel. Electrocardiography showed normal sinus rhythm with no underlying pathology. Chest roentgenography postero-anterior view unveiled a large right pneumothorax. Further questioning into past medical and family history established that patient has a daughter and a first cousin both of whom experienced primary spontaneous pneumothorax. In addition genetic testing prompted by occurrence of spontaneous pneumothorax in daughter and first cousin confirmed diagnosis of Birt Hogg Dube syndrome. An autosomal dominant gene responsible for transmission of BHD syndrome was mapped to chromosome 17.
DISCUSSIONS: In 1977 three Canadian physicians, Birt, Hogg and Dube described benign skin lesions, fibrofolliculomas, in members of one family. Multiple or bilateral renal carcinomas, pulmonary cysts and spontaneous pneumothoraces have been reported as manifestations of BHD syndrome. The autosomal dominant inheritance has been identified and mapped to chromosome 17 P11.2. BHD syndrome is an uncommon condition in the world and USA. It is an important consideration in any patient with primary spontaneous pneumothorax and familial history of such. Screening with renal ultrasound, Computed Tomography of chest, abdomen and pelvis is considered to prevent complications. Patient described above was treated with chest tube placement, experienced noted improvement of her pneumothorax as seen on the follow up chest radiograph. Talc pleurodesis procedure was performed to prevent recurrence of spontaneous pneumothorax on right side. Computed Tomography of chest and mediastinum revealed multiple pulmonary cysts and bullae diffuse through lung fields. Patient tolerated pleurodesis procedure well without unexpected complications. Upon discharge she was given instructions to avoid altitude climbing, and unnecessary valsalva like maneuvers. Follow up appointment to pulmonary care specialist was made.
CONCLUSION: This clinical synopsis illustrates a rare genetic disorder that may pose a medical quandary for primary care provider as well as for a consulting specialist. The rare entity of familial spontaneous pneumothorax most commonly carries an autosomal dominant inheritance which is associated with multiple organ involvement. In the case of Birt Hogg Dube screening for renal cancer, testing of relatives for affected genotype and counseling of offspring should be provided.
DISCLOSURE: Alexander Shalshin, None.
INTRODUCTION: Transbronchial biopsies are generally safe to perform with the most frequent complications being pneumothorax and pulmonary hemorrhage (1). Other rare complications include empyema, respiratory failure and death. We report a case of a visceral pleural hematoma presenting as a pleural mass following transbronchial biopsy in a patient with systemic amyloidosis.
CASE PRESENTATION: A 66 year-old with a history of amyloidosis presented to the hospital with pneumonia for which bronchoscopy and transbronchial biopsy were eventually performed. During follow-up, he was noted to have an enlarging pleural effusion associated with a pleural-based mass. CT demonstrated an enlarging mass projecting off of the visceral pleura and bedside ultrasound confirmed the presence of a homogenous, septated lesion. Two thoracenteses revealed an exudative pleural effusion without malignancy or infection. A VATS biopsy of the pleura accompanied by removal of the pleural-based mass was performed and confirmed the presence of a visceral pleural hematoma. Given that this was absent immediately prior to transbronchial biopsy and appeared thereafter, the visceral pleural hematoma was felt secondary to the transbronchial biopsy.
DISCUSSIONS: Transbronchial biopsies may have complications in the parenchyma (hemorrhage) or pleura (pneumothorax). Thoracic hematomas, on the other hand, typically develop from trauma, although spontaneous occurrences and hematomas associated with surgery, pneumothorax, and tuberculosis have been described (2). Amyloidosis is associated with fragile blood vessels that result from amyloid deposition in the walls of the vessels and is also associated with bleeding problems, including bleeding after procedures. In this case, transbronchial biopsy in a patient with pulmonary amyloidosis led to the development of a visceral pleural hematoma. It was expanding slowly, presumptively similar to reported cases of chronic expanding hematoma of the thorax.
CONCLUSION: Transbronchial biopsies may be associated with bleeding and pleural complications, typically pneumothorax. In this case, transbronchial biopsy in a patient with pulmonary amyloidosis led to the development of a visceral pleural hematoma presenting as a pleural-based mass. A history of prior transbronchial biopsy should be ascertained in the evaluation of patients with solitary pleural tumors.
DISCLOSURE: Jonathan Puchalski, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Pulmonary fungal infections and empyema thoracis are emerging clinical entities. According to the CDC national database, Candida species has become the 6th most common cause of nosocomial infections (7.2 %). Of all Candida infections, C.albicans and C.tropicalis account for the bulk of these infections (85%). Candida lusitaniae is an extremely rare but emerging nosocomial infection. We describe a 55-year-old female who was found to have bronchioloalveolar carcinoma and coexistent C. lusitaniae pleuropulmonary infection.
CASE PRESENTATION: A 55-year-old African-American actively smoking female presented with a three week history of cough, whitish sputum production and shortness of breath. Her past medical history was significant for Type 2 diabetes, recently treated COPD exacerbation with corticosteroids, untreated Hepatitis C and remote intravenous drug abuse (30 years ago). Her physical exam revealed a thin female (BMI 18) with crackles and decreased breath sounds over the right lower lobe. Radiographic studies identified a right middle and right lower lobe infiltrate with associated pleural effusion, which had worsened when compared to a chest X-ray done in 2007. High resolution computed tomography (HRCT) revealed right-sided pleural effusion with associated bilateral reticular opacities primarily in the right lung field associated with marked irregular septal thickening and small cystic air spaces. An ultrasound-guided thoracentesis revealed a serosanguinous exudative effusion with 85% lymphocytes. Despite adequate antibiotic coverage for health care associated pneumonia, her condition deteriorated requiring transfer to the Intensive Care Unit. She was intubated for worsening shortness of breath. A fiberoptic bronchoscopy with bronchoalveolar lavage (BAL) and brushing was performed. Pleural fluid and BAL specimens sent four days apart grew C. lusitaniae. Blood cultures were sterile. Cytopathology revealed adenocarcinoma of bronchioloalveolar cell type in BAL, brushing and pleural fluid.
DISCUSSIONS: Review of the literature identified certain risk factors for pleural fungal infections, all of which were present in our patient. These include malignancy, diabetes, recent corticosteroid use, and active smoking. The diagnosis of saprophytic fungal bronchopulmonary infections is difficult to confirm, as sputum and to some extent BAL specimens are unreliable. Our patient grew Candida lusitaniae in both BAL and pleural fluid confirming the diagnosis. C. lusitaniae infection primarily presents as fungal sepsis or candidemia. It is extremely rare to isolate fungi in pleural fluid (11 % in one study of 140 patients with pulmonary fungal infections). An extensive literature review in multiple databases showed that this is probably the first reported case of C.lusitaniae pleuropulmonary infection, and possibly the first fungal pleuropulmonary infection coexisting with bronchioloalveolar carcinoma.
CONCLUSION: With increasing numbers of immunocompromised patients due to prolonged survival in HIV/AIDS patients, overall improvement in management strategies for cancer patients, and use of immunosuppressive medications, the recognition of this pathogen as a source for emerging nosocomial infections is very important. This organism is frequently resistant to amphotericin B (one of the only 3 candida species resistant to this drug); therefore appropriate initial antifungal therapy is potentially lifesaving. After six days of intravenous fluconazole our patient was successfully extubated and clinically stable.
DISCLOSURE: Ashish Tikotekar, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Chylothorax is characterized by the collection of high lipid content fluid within the pleural space, usually due to thoracic duct disruption. Trauma is the cause in 30–50% of cases. Non-traumatic chylothorax is most often the result of lymphadenopathy. Similarly, disruption of chyle flow within the peritoneal cavity can result in chylous ascites. Chylothorax can occur as a result of chylous ascites as the negative intrapleural pressure draws chyle through diaphragmatic defects into the pleural space. In this case, we present a rare cause of chylous ascites, the nephrotic syndrome, resulting in chylothorax.
CASE PRESENTATION: A 53 y/o white female presented to the emergency department with a complaint of dyspnea. She had a history of CLL and matched unrelated donor stem cell transplant. Three weeks before presentating, she had a bronchoscopy with brochioalveolar lavage (BAL) to evaluate progressive dyspnea and bibasilar infiltrates. Viral culture from the BAL was positive for adenovirus. In response to this culture, cidofovir was initiated 8 days prior to presentation. At presentation, respirations were 20 per minute with room air SpO2 88%. She was without lymphadenopathy, hepatomegaly, or splenomegaly. Cardiovascular exam was unremarkable. There were diminished breath sounds with dullness to percussion to the mid lung fields on the right. A spiral CT scan showed a large right pleural effusion and moderate abdominal ascites. Thoracentesis revealed milky white fluid which with a triglyceride level of 2093 mg/dL. A paracentesis revealed a triglyceride level of 468 mg/dL. Lymphoscintigraphy verified an abdominal origination of the chylous fluid. A bone marrow biopsy revealed normal marrow, without evidence of CLL. Urine collection revealed 3.23 grams of protein in 24 hours. Other etiologies of chylous ascites were excluded, and she was diagnosed with chylothorax due to chylous ascites resulting from nephrotic syndrome. Cidofovir, a known cause of nephrotic syndrome, was discontinued, and the chylothorax resolved. A repeat 24 hour urine collection 27 days later revealed 0.97 grams of protein.
DISCUSSIONS: Up to 50% of patients receiving cidofovir in clinical trials developed proteinuria or a significant reduction in creatinine clearance. In a recent trial evaluating cidofovir for adenovirus infection after stem cell transplantation, 9% of patients developed proteinuria. In the largest case series of nephrotic syndrome with ascites, 30 patients underwent paracentesis 2. 52% had chylous or milky peritoneal fluid. The mechanism of chylous ascites in nephrotic patients remains unclear. One theory holds that the hypercoagulable state of nephrotic syndrome results in vena cava thrombosis and secondary lymphatic obstruction. Another suggests that bowel edema from hypoalbuminemia changes the permeability of mucosal lymphatics, resulting in leakage of chylomicrons into the peritoneal space. Once chyle is present in the peritoneal space, it is free to transfer through diaphragmatic defects aided by negative intrathoracic pressure during inspiration. Extensive evaluation in this case failed to reveal a more common cause of chylothorax. Onset chronologically followed initiation of cidofovir and was associated with nephrotic syndrome. Resolution followed reduction in proteinuria after discontinuation of the drug.
CONCLUSION: Chylous ascites is a well described complication of the nephrotic syndrome. We report a rare case of nephrotic syndrome as a cause of chylothorax. In patients with nephrotic syndrome and chylothorax, investigation for an abdominal origin of chylothorax has important clinical implications, as exploration of the chest for diagnosis may be avoided.
DISCLOSURE: Joel Mermis, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Tumor emboli to the pulmonary vasculature has been described in hepatocellular, renal cell, gastric, breast and prostate carcinoma. We report a case of a distantly treated urothelial carcinoma with a metastatic presentation of tumor emboli causing progressive pulmonary hypertension.
CASE PRESENTATION: A 69 year old man presented with a complaint of dyspnea, pleuritic chest pain and blood-streaked sputum for 3 days. His past medical history was significant for urothelial carcinoma of the left renal pelvis (high-grade, pT3NxM0) successfully treated 1 year ago with nephrectomy and partial ureterectomy followed by adjuvant chemotherapy with carboplatin and gemcitabine. He had been in good health up until this admission. Oxyhemoglobin saturation (Sa02) was 91% in room air. Physical examination was significant for right-sided crackles. Chest X-ray showed bibasilar interstitial markings. Chest CT revealed pulmonary emboli (PE) in the bilateral lower lobe segments, nodular and confluent ground glass opacities in the bibasilar and right upper lobes. Echocardiogram demonstrated normal left ventricular function with a dilated and poorly functioning right ventricle (RV) and a calculated pulmonary artery systolic pressure (PASP) of 67 mmHg. The patient was anticoagulated for PE and given a 14 day course of moxifloxacin for presumed pneumonia and discharged. The patient returned 10 days later complaining of worsening hemoptysis. Repeat chest CT showed no interval change. His hemoptysis was presumed to be due to pulmonary infarction. His hemoptysis improved without intervention and he was discharged on anticoagulation. Three weeks later, the patient returned to the emergency department, complaining of increasing dyspnea and persistent hemoptysis. He was found to be tachypneic with a respiratory rate of 24 breaths per minute and SaO2 of 91%. Physical exam again showed crackles over the right lung. Laboratory values demonstrated a white blood cell count 10,600/mm3 and an INR of 2.1. Repeat chest CT again revealed the previously noted multiple PEs and further progression of ground glass opacities to the left upper lobe. Abdominal CT did not show any evidence of local or metastatic recurrence of urothelial carcinoma. Repeat echocardiogram showed worsening PASP of 87 mmHg with persistent RV dysfunction. Treatment for presumed hospital acquired pneumonia was begun with a combination of ticarcillin/clavulanate and vancomycin. Anticoagulation therapy for PE was continued. His dyspnea continued to worsen requiring increasing FiO2. Open lung biopsy was performed on the 14th day of admission. During the biopsies, the patient developed a refractory shock state and subsequently died the following day. Pathologic examination of the lung biopsies revealed metastatic urothelial carcinoma predominantly present in the form of widespread intra-arterial emboli.
DISCUSSIONS: Tumor emboli from urothelial carcinoma is an uncommon occurrence. Only two prior cases have been reported in the literature. This underreporting may be due to the fact that RV failure in such cases is often thought to be due to venous thromboemboli. Pathologic confirmation is generally not performed. One possible clue of tumor pulmonary emboli is that patients will continue to have refractory dyspnea and worsening pulmonary hypertension despite adequate anticoagulation. This case was unique for that fact that the patient had no evidence of tumor recurrence. Tumor emboli was considered as a possible diagnosis due to his lack of improvement. Unfortunately, the definitive diagnosis was made late in the clinical course.
CONCLUSION: When patients with a history of cancer present with dyspnea and pulmonary hypertension, tumor emboli to pulmonary vasculature should be considered in the differential diagnosis. If clinical improvement is not observed after an appropriate period of anticoagulation, lung biopsy should be promptly considered. Only early diagnosis of tumor emboli may prevent fatal disease as there is a reluctance to retreat with chemo therapy without proof of progression.
DISCLOSURE: Taro Minami, None.
INTRODUCTION: Vascular endothelial growth factor (VEGF) plays a central role in the maintenance, differentiation and function of endothelial cells. It has been demonstrated in animal models that VEGF receptor blockade causes severe pulmonary hypertension associated with pre-capillary arterial occlusion by proliferating endothelial cells 1. Bevacizumab, a recombinant humanized anti-VEGF monoclonal antibody is approved by the United States Food and Drug Administration (FDA) for cancer treatment. We present the case of a patient who developed severe pulmonary hypertension while on treatment with bevacizumab for ovarian cancer.
CASE PRESENTATION: A 65 year-old Caucasian woman with a medical history of diabetes mellitus type 2, hypertension, hyperlipidemia, hypothyroidism and metastatic ovarian cancer, initially underwent neo-adjuvant chemotherapy with paclitaxel and carboplatin before undergoing extensive surgery. After surgery, numerous chemotherapeutic agents were used to induce remission, including intraperitoneal carboplatin, doxorubicin, tamoxifen, gemcitabine. Finally, due to a lack of response, bevacizumab and cyclophosphamide regimen was initiated. Eighteen months after intiation of bevacizumab, the patient presented with gradually worsening dyspnea. Echocardiogram, which was previously normal, revealed an ejection fraction of 55% and new finding of severely dilated right ventricle with an estimated right ventricular systolic pressure (RVSP) of 81 mm Hg. CT scan of the chest was negative for any parenchymal or vascular disease. Ventilation-perfusion lung scan showed a low probability for a pulmonary embolism. ×-natriuretic peptide level was 268 pg/ml. She had no history of liver disease, anorexigen use, and HIV testing was negative. Right heart catheterization revealed a pulmonary artery mean pressure (mPAP) of 52 mm Hg, occlusion pressure (PAOP) of 12 mm Hg, vascular resistance (PVR)-8 Wood units and cardiac index of 2.16 l/min/m2. Suspecting bevacizumab induced pulmonary hypertension, bevacizumab was discontinued and sildenafil 20 mg three times daily started. Unfortunately, her dyspnea and hypoxemia continued to worsen, despite increasing the dose of sildenafil. Right heart catheterization, six weeks later, revealed increased mPAP to 80 mm Hg and PVR to 21 Wood units with decreased cardiac index to 1.13 l/min/m2. With invasive hemodynamic monitoring sildenafil was increased to 80 mg TID, and inhaled iloprost initiated. Lack of hemodynamic response prompted initiation of intravenous epoprostenol. However, the patient's condition continued to deteriorate, ultimately leading to her demise.
DISCUSSIONS: VEGF inhibitors are being increasingly used today for treatment of metastatic colorectal cancer; non-squamous, non-small cell lung cancer; metastatic breast cancer, ovarian cancer, renal cell cancer and age-related macular degeneration. In a trial of bevacizumab for ovarian cancer, two of the seventy patients developed pulmonary hypertension; however this was not reported to be severe or fatal 2. Severe pulmonary hypertension in our patient was attributable to treatment with bevacizumab. This pulmonary hypertension showed lack of response to conventional therapy and was rapidly progressive.
CONCLUSION: To the best of our knowledge, this is the first report of severe fatal pulmonary hypertension attributable to vascular endothelial growth factor inhibitor use. With increasing use of these agents, we advocate increased awareness of this potential life threatening complication. Serial echocardiograms and other appropriate investigative modalities should be undertaken at the earliest sign or symptom suggestive of development of pulmonary hypertension.
DISCLOSURE: Manica Sodhi, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: The mechanism of right to left shunting of blood though a patent foramen ovale (PFO) in the absence of elevated right-sided pressures is controversial.
CASE PRESENTATION: A 79 year-old female was admitted to our institution due to acute ischemic stroke. Her hospital course was complicated by Clostridium difficile colitis with megacolon, requiring left hemicolectomy. 2 months into her hospitalization, she experienced recurrent attacks of shortness of breath and cyanosis whenever she assume a sitting position. Physical examination was only remarkable for a moderate thoracic kyphoscoliosis. When supine and while breathing room air pulse oximetry showed oxyhemoglobin saturation of 95%. After few minutes of sitting in a chair, she became cyanotic and her oxygen saturation decreased to 82% and did not improve with 100% oxygen, however, it was completely normalized when she resumed a supine position. Her chest roentogram and electrocardiogram were normal. A chest computed tomographic angioscan showed no evidence of pulmonary embolism or intrapulmonary arteriovenous malformations. A transthoracic echocardiography study combined with agitated saline injection (Figure 1) confirmed the presence of a PFO with preferential opening during the sitting position. Arterial blood gases was measured at various inclines (0, 30, 60 and 90°) while breathing 100% oxygen with a tightfitting non-rebreathing mask to calculate the shunt fraction (Figure 2). Right heart catheterization revealed an isolated positional changes in right atrial pressure from 2 mm H2O (supine) to 8 mm H2O (sitting) with normal right ventricle and pulmonary artery pressures. Patient was seen six weeks after transcutaneous closure of the PFO where she was asymptomatic and had normal oxygen saturations during both supine and erect positions.
DISCUSSIONS: Orthodeoxia-platypnea syndrome has been reported in association with different cardiac, pulmonary, and extrathoracic conditions, however, it is most commonly associated with an interatrial right-to-left shunt through a PFO, atrial septal defect (ASD), or fenestrated atrial septal aneurysm usually in the presence of pulmonary hypertension (1). The mechanism of intracardiac right to left shunt in the absence of pulmonary hypertension is still controversial and several mechanisms have been proposed. First, an isolated increase in right atrial pressure may reverse the interatrial gradient and can be due to an external compression of the right atrium or decreased right ventricular or right atrium compliance(2–8). Second, several conditions (atrial septal distortion and horizontalization, persistent Eustachian valve, or a high atrial septal defect) may lead to preferential blood flow streaming directly to the left atrium (3,7,9,10). We demonstrated an isolated preferential elevation in the right atrial pressure during upright position. This phenomenon has been proposed as a possible mechanism for a right to left shunt despite normal right-sided pressure. To our knowledge, this is the first case in the English literature to confirm this association and to document a proportional relationship between the degree of body inclination and the amount of right to left shunt. The combined effect of an elongated aorta and kyphoscoliosis may have contributed to the selective stretching and opening of the patent foramen ovale during the erect position in our patient as well as to the increase in right atrial pressure. Although both conditions may have been present for a long time in this patient, her recent major abdominal surgery may have worsened her intrathoracic geometry probably by manipulation of the aorta.
CONCLUSION: In patients with PFO and normal pulmonary pressures, right to left shunt may occur due to an isolated positional increase in the RA pressures.
DISCLOSURE: Saleh Alazemi, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Interferon induced pulmonary arterial hypertension (PAH) as a side effect is rare.
CASE PRESENTATION: A 40-year-old woman had undergone excision of a superficial spreading melanoma of the rima ani. Excised sentinel lymph nodes were unaffected. None of the staging examinations revealed metastatic spread. The medical history of the patient was unremarkable and she was not on any medication. Her family history was negative for cardiovascular disease. Because of the high-risk nature of the melanoma, the patient started long-term adjuvant therapy with Interferon alpha2b (IFNα2b). After 30 months of treatment the patient reported increasing dyspnea on exertion and a nonproductive cough accompanied by malaise and edema of the lower legs. Electrocardiography showed sinus tachycardia and right axis deviation. A chest x-ray showed signs of right ventricular dilatation and pleural effusion on the right side. No pneumonic infiltrates were seen. Abdominal sonography revealed ascites. Transthoracic echocardiography showed right ventricular hypertrophy and dilatation, PAH with a calculated systolic pulmonary artery pressure (PAP syst) of 80 mmHg and tricuspid insufficiency grade II-III with morphologically normal valves, a reduced right ventricular ejection fraction of 40%, a hypokinetic right ventricle and pericardial effusion without signs of tamponade. Laboratory work-up showed slightly increased levels of d-dimers and liver enzymes, while inflammatory markers were within the normal range. There were no signs of vasculitis, hypercoagulability or rheumatologic disorders. A CT angiogram of the chest was negative for pulmonary embolism, alveolar or interstitial lung diseases. Diagnostic right heart catheter revealed a PAPmean of 56 mmHg (PAP syst 87 mmHg), a pulmonary vascular resistance (PVR) of 1.128 dyn × sec × cm-5, an impaired cardiac index and a 3 fold increased total peripheral resistance. Testing of pulmonary vasoreactivity showed a reduction of PAP mean from 56 mmHg to 26 mmHg with the PDE-5-inhibitor sildenafil. Therefore, treatment with sildenafil was initiated. After one month, tricuspid insufficiency improved to grade I-II. After six months right ventricular hypertrophy and dilatation were reduced. We attempted to terminate sildenafil. However, PAP mean increased promptly to 57 mmHg. Reduction of PVR was higher in vardenafil vs. sildenafil; therefore the therapy was switched from sildenafil to vardenafil 10 mg twice daily. 24 months after the onset of PAH the patient resumed working. Vardenafil is still necessary to lower her PAP as demonstrated with transthoracic echocardiography: after pausing vardenafil for two days the PAP syst increased about 40 mmHg, but decreased again 120 minutes after administration of vardenafil. Currently she is only complaining of dyspnea with heavy exercise (WHO Class II symptoms). Her last PAP was 50 mm Hg and PVR was 1,5 WU. Regarding the melanoma she remains relapse-free.
DISCUSSIONS: IFNα2 is an accepted adjuvant treatment for patients with high risk melanoma. Studies on the importance of inflammatory mediators, such as chemokines, in the lungs of PAH patients have led to a possible inflammatory component in the development of PAH. This might be of relevance in IFN induced PAH since IFNα2 is known to induce expression of various chemokines. So far only 4 cases of IFN-induced PAH have been reported. To the best of our knowledge this is the first documented case where PAH was not reversible after termination of IFN alpha therapy requiring continuous vasodilator therapy. In our case treatment with PDE-5-inhibitors had a long-lasting beneficial effect.
CONCLUSION: If IFN alpha treated patients develop respiratory symptoms, PAH should be considered in the differential diagnosis.
DISCLOSURE: Alexander Panda, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Newer therapies for idiopathic pulmonary arterial hypertension (IPAH) have reduced the need for lung transplantation (LT), which is now reserved for patients with IPAH unresponsive to medical therapy. We report a patient with IPAH who underwent bilateral LT (BLT) and who, less than a year post-LT, developed severe recurrent IPAH and right heart failure.
CASE PRESENTATION: Our patient was a 47 year old female with a history of IPAH who underwent BLT in 12/05. Prior to LT, echocardiography showed a severely dilated right ventricle (RV) with moderately depressed function, severe right atrial enlargement, and normal left ventricular size and function. Hemodynamic data (HD) was consistent with severe IPAH. No risk factors for pulmonary hypertension (PH) were identified. Prior to LT, our patient was treated with bosentan, aldactone, digoxin, warfarin, furosemide and ultimately 10 months of intravenous treprostinil. Despite these medications, clinical deterioration continued, and BLT was completed. Immediate post-operative HD normalized. A post-LT echocardiography demonstrated that RV size and function had also normalized. All PH therapies were stopped, and standard immunosuppressant therapies including tacrolimus were maintained. Histopathology of her native lungs revealed diffuse vasculopathy of the small and medium-sized pulmonary arteries with significant medial hypertrophy and mild intimal hyperplasia. There were no obvious thrombi or plexiform lesions. Large vessel atherosclerotic change consistent with long-standing severe PH was observed. The patient did well for 9 months before developing progressive dyspnea and hypoxemia. Extensive evaluation, including bronchoscopy with transbronchial biopsies failed to demonstrate any anastomotic narrowing or acute cellular rejection. MRI imaging of her thorax and a V/Q scan did not demonstrate significant pulmonary shunting or acute or chronic thromboembolic disease. Echocardiography was negative for intracardiac shunting, but the RV was again markedly dilated. Data from right heart catheterization confirmed severe recurrent PAH. Intravenous epoprostenol, aggressive diuresis, and digoxin were initiated. Unfortunately, despite aggressive medical management, the patient developed worsening right heart failure and died. Autopsy was completed, and histopathology of the allograft revealed massive intimal hyperplasia predominantly in the medium-sized vessels with relative sparing of the small vessels. There was no significant medial hypertrophy, intra-luminal microthrombi or evidence of plexiform lesions.
DISCUSSIONS: Newer medications for the treatment of IPAH have decreased the need for LT, yet it remains the only available modality that offers a patient with IPAH the chance for cure. Diseases leading to PH have been reported to recur after LT, such as sarcoidosis and lymphangioleiomyomatosis, and recurrent PH after LT for IPAH has been reported resulting from post-operative anatomical factors. To our knowledge, however, there has never been a report of IPAH recurring after LT. Other etiologies for PAH could not be identified. Recurrent IPAH may have been secondary to pre-existing but unsuspected donor IPAH, which may have been accelerated post-LT, or to unidentified systemic circulating factors that initiated and accelerated IPAH in the allograft.
CONCLUSION: This 47 year old patient with known IPAH who underwent BLT developed severe recurrent PAH within one year of LT. Right heart catheterization, extensive clinical evaluation, and histopathology of the allograft on autopsy were highly suggestive of recurrent IPAH. This appears to be the first reported case of IPAH recurring in the lung allograft after LT. This observation highlights the need for further investigation into factors involved in the initiation and progression of IPAH.
DISCLOSURE: Saeher Muzaffar, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Percutaneous vertebroplasty involves an injection of acrylic cement, polymethyl metaacrylate (PMMA), into a diseased vertebra for partial vertebral body remodeling and lumbar pain relief. Osteoporosis, fractures, metastatic tumors, multiple myeloma and vertebral hemangiomas are the main indications of the procedure. We report the case of a patient who developed pulmonary hypertension following migration of PMMA into the pulmonary arteries eight weeks following percutaneous vertebroplasty.
CASE PRESENTATION: This 54 year old woman developed right upper quadrant abdominal pain associated with nausea and vomiting. She had neither chest pain nor shortness of breath. She has a history of asthma, vocal cord dysfunction, and aortic valve disease (requiring an aortic valve replacement). She is maintained on coumadin for the valve. She underwent a kyphoplasty for osteoporotic compression fracture of multiple vertebral bodies eight weeks prior. Her examination revealed normal vital signs, a clear chest exam, and a crisp valvular heart sound. A computed tomography scan of the abdomen revealed hyperdense material in the inferior vena cava, lumbar veins, and right interlobar pulmonary artery (Figure 1). PFTs showed an FEV1 of 1.9 L (77%) and FVC 2.33(74%), FEV1/FVC of 82, TLC of 3.63(84%), RV of 1.29(84%) and a DLCO of 17.1(74%). A transthoracic echocardiogram revealed normal right ventricle size and systolic function with a pulmonary artery pressure of 50 mm Hg. Pulmonary artery pressure five months prior as determined by echocardiography was 36 mm Hg. As she remained clinically stable she was discharged continuing anticoagulation with coumadin. Followup six months later revealed no increase in symptoms and no clinical changes.
DISCUSSIONS: Pulmonary hypertension is a rarely reported complication associated with cement embolization. Percutaneous vertebroplasty was first described by Galibert et al. in 1984 for the treatment of vertebral hemangioma and is now being used for the treatment of severe osteoporosis. Under CT or image guidance, polymethylmetaacrylate (PMMA) is injected into the vertebral body transcutaneously. Although clinical trials have proven percutaneous vertebroplasty to be efficient and safe procedure, associated complications occur in up to ten percent of cases. The major hazard of this technique is caused by cement extravasations. Cement embolization can occur as a result of insufficient polymerization of the PMMA at the time of injection allowing migration to the inferior vena cava, incorrect needle position with the respect to the basivertebral vein or overfilling of the vertebral body allowing cement migration into the venous system (1). Reported complications include transient worsening of pain, infections, bleeding, and injuries to the nerve roots or adjacent organs. If PMMA leaks into the spinal canal or neural foramen, partial or complete paraplegia can occur. Most patients with minor venous leaks, and even those with pulmonary emboli detected by chest radiographs remain asymptomatic. However, some fatal complications, including ARDS, fatal pulmonary embolism, paradoxical cerebral embolism, penetration of the right ventricle and renal artery embolism have been reported (2). There is little reported evidence of the clinical course following cement embolization.
CONCLUSION: Embolization of cement following a vertebroplasty can occur. While often asymptomatic in nature in some instances fatal complications have been noted. Pulmonary hypertension following such embolization has been reported only rarely. In cases of suspected embolization, despite the lack of clinical symptoms, echocardiographic evaluation of the right ventricle with determination of pulmonary artery pressure measurements should be performed. Subsequent followup of clinical symptoms and pulmonary artery pressure will be important to help determine the natural history of this disease.
DISCLOSURE: Ghazaleh Bigdeli, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: The solitary fibrous tumours of the pleura (SFPT) are uncommon and discovered fortuitously or in patients with non-specific respiratory symptoms. When associated with hypoglycaemia it is commonly referred as Doege-Potter syndrome. This syndrome is described in 2–4% of SFPTs. We present two cases of Doege-Potter syndrome with increased production of "big" IGF-II.
CASE PRESENTATION: Case 1. A 68-years-old woman was presented with a large pleural mass and low blood glucosae level (1,4 mmol/L). She had a long history of headache and decreased consciousness with one-year worsening dyspnoea and rihgt-sided chest pain. The chest x-ray (CXR) and CT revealed an enormous opacity occuping almost the entire right hemithorax. After transthoracic fine-needle aspiration, hystological and immunohistochemical analysis diagnosed a benign SFPT. Although surgical resection of such hudge tumour with hypoglycaemia is usually currative, our patient declined surgery and opted for conservative treatment of intravenous glucosa.Case 2. This is a case report of a 55-year-old woman with a large SFTP who was admitted with a loss of consciousness due to hypoglycemia (0.2 mmol/L), transient dysarthria and right-sided hemiparesis. She had 10-year history of dizziness, headaches and impaired concentration, and 12-months history of progressive dyspnea, right-sided chest pain, urinary incontinence, confusion and impaired consciousness. A possibility of a disease of the central nervous system was ruled out by CT and MRI scans. CXR and CT indicated a huge heterogenous mass lesion at the right thoracic cavity. Transthoracic fine-needle aspiration was performed. Based on macroscopic appearance, histological pattern and immunoprofile, the tumor was diagnosed as benign SFTP. Pulmonary function tests demonstrated a moderate restrictive pattern of impaired ventilation. Doppler echocardiography demonstrated moderate pulmonary hypertension. She underwent a standard posterolateral thoracotomy with complete excision of the tumor. There was a large lobulated and pedunculated tumor at the base of pulmonary ligament not adherent to surrounding structures, with subsequent atelectasis of the right middle and lower lobes. Postoperative recovery was complete and uneventful with normal blood glucose levels. Three years after the surgery, there were no symptoms or signs of tumor relapse.
DISCUSSIONS: In 1930, Doege first described the tumor of non-beta cells associated with hypoglycemia, presenting the patient with fibrous tumor of mediastinum. Brusseli et al. in 1981 published the study on eight SFTP patients among whom 4% had hypoglycemia. Giant fibrous tumors cause hypoglycemia by different mechanisms, such as: IGF-II secretion, increased consumption of glucose in so huge tumor, proliferation of insulin receptors in tumor cells, reduced gluconeogenesis and lower effective glucagon secretion. IGF-II secretion is thought to be the most likely mechanism of hypoglycemia origin in patients with SFTP because some solitary fibrous tumors produce excessive quantity of "big" IGF-II because of sudden expression of pro-IGF-II gene in tumor cells. In presented cases, benign SFTPs were diagnosed after fine-needle biopsy, using the histological and immunohistochemical analysis - tumor cells exhibited diffuse cytoplasmic positivity of vimentin, were positive for bcl-2 with no cytonuclear abnormalities. Strong and uniform reactivity of CD34 were manifested in spindle and round cells. The tumors' cells showed immunoreactivity for IGF-II and E-domain of pro-IGF-II in its Golgi areas. Therefore, we assumed that hypoglycemia has been caused by increased production of "big" IGF-II. In order to examine the other possible causes of hypoglycemia in both patients, we performed the encrinological tests and excluded the most common causes of hypoglycemia (insulinoma, exogenous insulin, adrenal insufficiency, hypopituitarism).
CONCLUSION: The presence of SFPTs with IGF-II secretion possibly producing insulin like neuropeptides, were considered the most likely mechanism of hypoglycemia origin. Clinicians should be aware of this rare, but important cause of hypoglycemia.
DISCLOSURE: Branislava Milenkovic, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Bronchopleural fistula (BPF) is a condition that results from numerous etiologies and typically occurs in the setting of multiple comorbid conditions that complicate the evaluation and treatment of a patient. Central BPF's are typically a result of pneumonectomy or trauma and can usually be visualized with bronchoscopy, whereas, peripheral BPF's usually occur in the setting of suppurative lung infection, neoplasm, or trauma. Large peripheral BPF's can be localized with computed tomography (CT) scanning except in the setting of bullous disease1. Standard ventilation scintography has been used to diagnose and localize moderate sized peripheral BPF's, but its utility in the setting of a large BPF is questionable.
CASE PRESENTATION: A 58 y/o male was referred from an outlying facility for treatment of an empyema. He initially presented complaining of progressive dyspnea, cough with foul smelling sputum, weight loss, and fever. A CT scan revealed a large loculated pneumothorax with an air fluid level, a right middle lobe opacity, and mediastinal adenopathy. He had a history of chronic pancreatitis from ETOH abuse and a 30 pack year smoking history and had quit smoking about four months prior to admission. Physical examination revealed an ill appearing, alert, afebrile male in no distress. He had a pulse of 64 bpm; BP 132/61; R 18; T 98.4; SaO2 96% on room air; Ht 73"; Wt 69 Kg. Cardiopulmonary exam revealed diminished breath sounds throughout with tympanic percussion over the right chest. Abdominal exam revealed ascites without tenderness to palpation. Laboratory data which revealed an albumin of 2.3 and hemoglobin of 10.8. Sputum cultures were positive for alcaligenes sp. and he was continued on piperacillin/tazobactam. Bronchoscopy and video assisted thoracotomy with decortication was performed. Pathology revealed adenocarcinoma in the resected lung tissue. He was staged IIIB and his oncologist deferred chemotherapy due to his poor performance status. The patient had a persistent air leak post thoracotomy and returned to surgery for bronchoscopy and talc pleurodesis. A BPF was suspected and could not be localized. Pleurodesis was unsuccessful. He was not a candidate for pneumonectomy due to his performance status and residual pulmonary function. The fistula was not identified with CT scan. A ventilation lung scan was done with planar imaging which revealed a broad focus in the mid to apical portions of the right lung with homogenous distribution in the left. Single photon emission computed tomography (SPECT) imaging was then performed and by correlating the transverse and coronal images with CT demonstrated that the leak was in the superior and posterior portion of the right upper lobe, which was the area of the surgical clips from his previous thoracotomy. He underwent fiberoptic bronchoscopy with the placement of IBV® valves in the segments of the right upper lobe. The leak gradually decreased over the course of seven days and the patient discharged to home with a chest tube and a heimleich valve.
DISCUSSIONS: The case illustrates the difficulty that can occur in the management of a BPF. The patient had a BPF that was unable to be localized on CT or ventilation scintography. Using SPECT imaging the presence of BPF was confirmed and its precise location defined. This allowed for the directed placement of IBV for noninvasive management.
CONCLUSION: SPECT imaging can be a valuable tool in the diagnosis and localization of BPF in the non-operable patient who maybe a candidate for IBV.
DISCLOSURE: Michael Hull, None.
INTRODUCTION: Solitary fibrous tumors of the pleura (SFTP) are mesenchymal neoplasms of the pleural mesothelium usually arising from the visceral pleura. They are rare with only 850 cases having been reported in the literature so far. Both a benign and a malignant form of SFTP exist, in a ratio of 7:1. They are normally asymptomatic and are often incidentally detected radiologically except when their size produces compression-related clinical symptoms(such as dyspnea and cough.We report a case of SFTP masquerading as an extrapulmonary tumor of the posterior mediastinum.
CASE PRESENTATION: A 65 year old woman with a history of arterial hypertension and uterine fibromyomatosis was admitted to hospital for a scheduled operation of total hysterectomy. On routine preoperative evaluation, a slight bulging to the left of the superior mediastinal margin was noted on chest x-ray. The patient was otherwise asymptomatic. MRI revealed a paravertebral tumor of heterogeneous density, with a diameter of 4 cm in the upper left side of posterior mediastinum resembling a possible neurogenic tumor. After I.V. injection of gadolinium an enhancement of signal intensity was notified. An exploratory video-assisted thoracoscopy (VAT) confirmed the presence of a solid tumor of high mobility arising from the visceral pleura via a stalk at the left lung apex. The wedge resection was expanded to apical posterior segmentectomy. The tumor was finally removed thoracoscopically. It was, a well circumscribed partially encapsulated tumor, 4 cm in diameter with multinodular, whitish and firm appearance in cut section. Histology showed an architectural pattern characterized by a combination of alternative hypocellular and hypercellular areas separated from each other by thick bands of hyalinized somewhat keloidal collagen and branching haemangioericytoma –like vessels. Histologic differential diagnoses included SFTP, synovial sarcoma, cellular angiofibroma, neurofibroma and spindle cell lipoma. Immunohistochemical tests were positive for vimentin, CD34, CD 99, Bcl-2, and SMA, and negative for actin, desmin, S-100 and CD68. Final diagnosis, based on these tests was SFTP.
DISCUSSIONS: SFTPs most often originate in the visceral pleura (80–90%). The tumors which are almost always benign, most commonly arise as a pedanculated discrete mass usually attached to the visceral pleura as in the case we report. Their resection requires only a margin of normal lung tissue. Less commonly, SFTP arises as a sessile tumor developing from the parietal pleura of the chest wall, diaphragm, or mediastinum. These tumors are more prone to recur and require a wide local extrapleural excision. Occasionally this kind of tumors could masquerade mediastinal masses. To the best of our knowledge there have been no reported cases of visceral pleura origin of SFTPs masquerading mediastinal tumors as was the case of our patient. SFTPs are normally difficult to diagnose before surgery with fine needle aspiration. We believe that all undiagnosed thoracic tumors should first be evaluated by video-assisted thoracoscopy. The cavity can be fully explored, the neoplasm biopsied and diagnosed, and a decision made as to the best approach for excising (if possible) the tumorùall without the invasiveness of a thoracotomy. Tumors (whether lung tumors or not, malignant or not) can be resected using endoscopic methods by following the protocols of oncological surgery: extracting the excised tissue in a plastic bag to avoid seeding the chest wall.
CONCLUSION: SFTP are normally difficult to diagnose before surgery with fine needle aspiration. We believe that all undiagnosed thoracic tumors should first be evaluated by VAT with the neoplasm being biopsied and a decision made as to the best approach for excising.
DISCLOSURE: Stylianos Michaelides, None.
INTRODUCTION: A pleural mass in a young female has a limited differential diagnosis. We present a case of an enlarging pleural mass associated with chest pain.
CASE PRESENTATION: A 19-year-old girl presented with persistent right-sided chest pain. Past medical history was significant for admission to another hospital six months earlier for acute dyspnea and chest pain. She was diagnosed with a spontaneous right pneumothorax and a chest tube was inserted with reported difficulty. The chest tube was removed two days later and she was discharged. Since her discharge, she reported pain at the site of insertion of the chest tube, which she described as persistent, occasionally sharp, and partly relieved by ibuprofen. There was no relation of the pain to her menstrual cycle. She denied fever, weight loss, dyspnea, or cough. Review of systems was negative. Physical exam was unremarkable. Routine blood tests including a complete metabolic profile and blood count were within normal limits. A chest radiograph was unremarkable. Computed tomography (CT) of the chest revealed a 2.7 × 1.3 cm area of pleural thickening. Tuberculin skin test was negative. Due to persistence of the pain, the chest CT was repeated 7 months later and showed that the pleural based process had enlarged to 4.3 × 1.9 cm with central necrosis . She underwent video-assisted thoracic surgery (VATS) for resection of the pleural mass. On pathologic examination, there was extensive chronic inflammation and fibrous connective tissue surrounding retained foreign bodies, which were identified as two fragments of latex from a medical glove measuring 2.2 × 1.7 cm and 0.9 × 0.5 cm. She made a complete recovery.
DISCUSSIONS: The differential diagnosis of a pleural mass in a young female includes primary tumor of the pleura, metastatic disease, lymphoma, infection, endometriosis, and as in this case, retained foreign body. Though clinical clues may occasionally be helpful, a definite diagnosis is often made via pathology. The retained foreign body, in this case a 'glove-boma' (to allude to the related condition of gossypiboma –retained cotton surgical sponge) induces an aseptic foreign body reaction with subsequent fibrosis and granuloma formation. Because of its rare occurrence and non-specific clinical and radiographic presentations, the diagnosis is unlikely to be considered.
CONCLUSION: The diagnosis of retained foreign body may be easily overlooked and should be considered in patients who have had previous invasive procedures performed. Early recognition would allow for prompt treatment.
DISCLOSURE: Subani Chandra, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Benign mesenchymomas are soft tissue tumors that are composed of, in addition to fibrous tissue, multiple cellular elements not commonly associated together. Benign mesenchymomas are rare in adulthood and few cases have been reported. We report a case of benign, pleural-based mesenchymoma in an adult.
CASE PRESENTATION: A 41 year old, non-smoker, Asian woman was admitted to our hospital with a five month history of progressive dyspnea, cough, hemoptysis, right sided chest pain, and a twenty pounds weight loss. Physical examination was normal except for significantly diminished right sided air entry by chest auscultation, with associated dullness to percussion and decrease in tactile and vocal fremitus. Chest roentgenogram and computed tomography (CT) were performed and revealed a large hetereogeneous low density mass in the right hemithorax possibly originating from the pleura, causing a right-to-left mediastinal shift and partial right lung collapse. Subsequent fiber-optic bronchoscopy (FOB) demonstrated evidence of external compression of the basal segment of the right lower lobe of the lung; bronchial washing and brushing results yielded chronic bronchial inflammation without malignant cells. CT guided biopsy of the mass was performed but results were inconclusive. A right thoracotomy was performed and revealed a 2.2 kilogram, well encapsulated mass that measured 21 centimeters in its largest dimension and attached to the parietal pleura; no adjacent extension or invasion was visualized. After resection, repeat FOB showed resolution of the external compression of the basal segment of the right lower lobe without evidence of endobronchial lesions. Microscopic examination and immuno-histochemical staining revealed tumor positivity for anti-vimentin, anti-desmin, anti-smooth muscle, CD34, and negativity for CD99, BCL-2 and S-100. These results were consistent with benign mesenchymoma of the pleura, alternately termed myochondrolipoma.
DISCUSSIONS: Mesenchymomas are rare neoplasms that are malignant in approximately 75% of cases and most commonly present in childhood. First described by Stout, the tumor consists of, in addition to the fibrous tissue which is present in all mesenchymomas, two or more different cellular elements that are mesenchymal derivatives. While malignant tumors may cause distant metastases, benign tumors usually recur locally. Ionescu and Eskenasy reported a case of a benign thoracic mesenchymoma that recurred four times over the 30 years post initial resection. Prior case reports have noted tumor occurrence in various locations, including the urogenital tract, retroperitoneum, breast, liver, rectus femoris and gluteal muscles, lung, and mediastinum. A thorough literature review revealed only two prior reported cases of benign mesenchymoma of the pleura.
CONCLUSION: Smooth muscle, chondrocytes and adipose tissue were the three mesenchymal derivatives of our patient's tumor. The tumor was fully encapsulated and radically resected without microscopic evidence of malignancy. Thus, the patient is undergoing chest imaging every three months to ensure non-recurrence of the tumor. To date, she remains asymptomatic and imaging studies show no evidence of recurrence.
DISCLOSURE: Adel Blamoun, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Calcifying fibrous tumor (CFT) is an uncommon lesion that was first described in 1988 as a fibrous tumor of childhood and has been described in somatic soft tissue with pathologic features of densely collagenized fibrous tumor with psammomatous and dystrophic calcification accompanied by a lymphoplasmacytic infiltrate. CFT of the pleura is a very rare entity that was first described in 1996 and only 10 cases have been reported in the literature. We describe a case of multiple CFTs of the pleura in an asymptomatic 29-year-old female.
CASE PRESENTATION: A 29-year-old active duty USAF female was referred to our clinic for evaluation of multiple pulmonary masses of the right lower lung. Her past medical history was significant for PPD conversion when she was deployed to Korea five years prior to presentation. The patient was without pulmonary complaints or constitutional symptoms. Travel history was significant for military installations in the southeast U.S. and a one-year tour in Korea. A screening chest x-ray for the history of PPD conversion revealed multiple right lower lung masses; CT chest revealed multiple large right basilar calcified masses with the largest measuring 2.3 × 4.5 cm. A bone scan was unremarkable and alpha-fetal protein and beta-hCG were negative. A CT-guided biopsy showed hyalinized and calcified fibrous tissue with entrapped vascular structures without evidence of malignancy. The tissue was CD34 positive but Congo red, keratin, BCL-2 and S-100 stains were negative as well as tissue cultures. The patient subsequently had a surgical biopsy that revealed dense hyalinized collagen with scattered inflammatory cells and psammomatous calcifications consistent with calcifying fibrous tumor of the pleura. There was no evidence of extension into the surrounding lung.
DISCUSSIONS: Calcifying fibrous tumors (CFTs) are unusual tumors typically found in children and young adults. They have been reported to occur in subcutaneous and deep soft tissues of the extremities and trunk, groin, scrotum, mediastinum, myocardium, paratracheal region, peritoneum, neck, mesentery, omentum, serosa, lung, bone, and gallbladder. CFTs of the pleural are very rare and were first described in 1996 with 10 total cases reported in the literature to date. There are no specific clinical laboratory or imaging studies that distinguish CFT of the pleura from other intrathoracic lesions. The differential diagnosis includes metastasis from osteosarcoma, chondral harmatoma, fibrous tumor of pleura, calcified pleural plaques, calicified pleural metastsis and calcified granuloma. Previously described cases have required surgical diagnosis in 9 and CT-guided biopsy in one. The first report of CFT consisted of a series of 3 patients each with unencapsulated circumscribed masses of hyalinized collagenous fibrotic tissue interspersed with lymphoplasmacytic infiltrates and calcifications with psammomatous features. Lesions were limited to the pleura without extension into the lung parenchyma; multiple lesions are even more uncommon. Calcifying fibrous tumor of the pleura is distinct from other pleural lesions and was incorporated into the WHO classification of lung tumors as a distinct entity. Immunohistochemical staining has been reported to show spindle cells diffusely positive for vimentin, CD34, and negative for epithelial membrane antigen, keratin, smooth-muscle actin, desmin, S-100 protein and anaplastic lymphoma kinase-1. As with extrapleural lesions, treatment has consisted of local excision without reports of recurrence.
CONCLUSION: Calcifying fibrous tumor of the pleura is a rare tumor that can mimic other pleural lesions. Patients are asymptomatic with incidental pulmonary nodules or masses found on radiographic studies. Diagnosis requires surgical excision with histologic confirmation demonstrating densely collagenized fibrous tumor with psammomatous and dystrophic calcification accompanied by a lymphoplasmacytic infiltrate without extension into the lung parenchyma. Similar to extrapleural lesions, treatment with complete surgical excision appears to be curative.
DISCLOSURE: Andrew Hsing, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Pneumocephalus from a subarachnoid-pleural fistula following resection of a pulmonary neoplasm is a rare postoperative occurrence. It is usually described in literature following open or closed spinal cord injury and is less frequent following a thoracic surgery.
CASE PRESENTATION: This is a case of a 72-year-old gentleman who underwent pneumonectomy in April 2005 for a recurrent bronchogenic carcinoma treated two years prior by left lobectomy and rib resection. Findings on resection included an extremely thick and partly calcified fibrotic pleura at the site of rib resection and a lesion in the left lower lobe bronchial stump adjacent to the aorta, which revealed a squamous cell carcinoma. No pleural-subarachnoid fistula was noted intra-operatively. After an uneventful post-surgical course the patient was discharged but readmitted for recurrent falls. The patient's blood pressure was 122/88, pulse 90, respirations 18, and a temperature of 98°F. There was no indication of infection on spinal fluid analysis. On neurological exam patient was lethargic and Glasgow coma score was 13 (15 prior to surgery). Cranial nerves were intact except for flattened right nasolabial fold. The right arm had 3/5 motor strength and cerebellar function could not be assessed. CT scan of the head revealed a pneumocephalus that, after conservative management, resolved spontaneously.
DISCUSSIONS: We present a new case of subarachnoid-pleural fistula leading to a pneumocephalus following pulmonary neoplasm resection that responded to conservative management. Bronchogenic carcinoma requiring rib resection exposed the patient to risk of a dural tear. Postoperative pneumocephalus usually presents in 1 to 8 weeks. Findings include headache and altered mental status. In this case, the patient returned with symptoms within 7 days. Myelography followed by CT is the definitive test for diagnosing fistulae and treatment depends on the neurological status of the patient. At times lumbar drainage and surgical repair of the fistula may be required, however cases frequently resolve spontaneously such as ours. Antibiotics may be used to prevent infection. If a tension pneumocephalus is present with neurological effects, the fistula should be drained. In addition to a thoracotomy or thoracoplasty, and depending on the location of the subdural effusion, the repair can also be through a posterior-laminectomy and placement of an intradural or extradural-patch.
CONCLUSION: Pneumocephalus following a thoracotomy is rare. However, as demonstrated in this case, care should be taken during tumor resection to prevent damage to the dural sleeve. Any patient presenting with neurological deficits following thoracic surgery should be assessed for the possibility of this entity.
DISCLOSURE: Joseph Ng, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Sinonasal teratocarcinosarcoma (SNTCS) is a very rare, highly aggressive, rapidly growing tumor that metastasizes to cervical lymph nodes, lungs and the airway. There have been only 63 cases reported to date in the literature. The tumor shows both epithelial and mesenchymal elements including nests of fetal-appearing, non-keratinizing clear cell squamous epithelium. SNTCS affects patients ages 18–79 with an approximate 8:1 male predominance. Three year survival is about 40%. The most common complaints at presentation include nasal obstruction, epistaxis, facial pain, proptosis and visual field deficits. Metastasis to the lungs and airways can cause dyspnea and obstruction of the main bronchi creating challenging situations. We report a case of SNTCS with a very large metastatic mass to the right main bronchus with near-total bronchial obstruction that was completely removed using endoscopic techniques.
CASE PRESENTATION: A 76 year old male with a history of atrial fibrillation, prior stroke and SNTCS first diagnosed in 2003 presented with significant shortness of breath. He had initially been treated with partial resection and radiation therapy. The tumor recurred in 2005 affecting the distal trachea and was treated with laser removal and radiation. He now presented with cough, dyspnea, and weight loss. Admission physical exam showed respiratory distress and decreased breath sounds in the right hemithorax. He had no lymphadenopathy and no visible nasal tumor. CT chest demonstrated a large endobronchial lesion with complete occlusion of right main bronchus. He had no other lesions. He underwent flexible and rigid bronchoscopy. A large glistening mass was noted obstructing the right main bronchus just distal to the carina. Initial biopsy was done and frozen section confirmed metastatic SNTCS. Endobronchial resection was then undertaken. We first circumferentially injected the mucosa around the tumor with dilute epinephrine to achieve hemostasis. The Injection Gold Probe Device, an injection therapy and bipolar electrohemostasis catheter with irrigation capabilities was then used to perform a complete mucosal resection of the tumor. The tumor was too large to be removed through a rigid bronchoscope and had to be removed en-masse with the scope. The patient was re-intubated and inspection of the resection bed showed good hemostasis. Final pathology confirmed this to be a 2.8 × 1.3 cm SNTCS. A second bronchoscopic examination five days later was performed. Additional biopsies from the base of the resected tumor as well as resection margins did not show residual tumor. The patient had a dramatic improvement in his symptoms and was subsequently discharged.
DISCUSSIONS: The cornerstones of treatment for SNTCS include surgical resection as well as radiation therapy with 45–70 Gy. Chemotherapy is rarely effective in this condition. Often, surgical resection is combined with adjuvant radiation therapy, after local resection and debulking have been performed. Options for endobronchial tumor resection include cold biopsy forceps removal, removal with electrocautery and laser destruction. Endobronchial therapy must be individualized depending on tumor size and location.
CONCLUSION: SNTCS is a rare, highly aggressive, rapidly growing tumor. Airway metastasis with bronchial obstruction is a noted complication and efforts should be directed towards symptom palliation. We advocate complete mucosal excision of these tumors, followed by adjuvant radiation therapy.
DISCLOSURE: Alfredo Astua, None.
INTRODUCTION: Mediastinal mature teratomas (MTT) in adults are uncommon typically asymptomatic tumors that arise in the anterior mediastinum. Usually, they are benign and resectable. Seldomly, they rupture into adjacent cardiothoracic structures. Hemoptysis as a presenting symptom is rare. Among adults, bacterial infection complicating MMT rupture has not been described. We report a man who presented with hemoptysis and infected MMT.
CASE PRESENTATION: A 58 year old man from Ghana was hospitalized following five days of hemoptysis, cough, dyspnea, and pleuritic left chest pain. Twelve years earlier he was treated for pneumonia. The left upper hemithorax revealed reduced tactile fremitus, dullness, and monophonic rhonchi. Routine laboratory tests were normal. A tuberculin skin test was non-reactive. Sputum smears and cultures revealed no tuberculosis. Blood cultures were without growth. A chest radiograph revealed a large opacity with multiloculated lucencies superior and adjacent to the left heart border. Non-contrast computerized tomography (CT) of the chest showed a 6.2 cm X 7.0 cm X 4.0 cm necrotic cavitating heterogeneous anterior mediastinal mass containing fat, soft tissue, and gas that suggested bronchial rupture. The mass appeared to occlude the anterior segmental bronchus of the left upper lobe, invade the left hilum posteriorly, and extend directly into pericardium. Figure 1. Non-contrast chest CT showing the mediastinal mass extending into pericardium. Fiberoptic bronchoscopy revealed left upper lobe bronchial edema and, at its origin, left lower lobe narrowing by external compression. All segmental and subsegmental bronchi were patent and without lesions. Transthoracic needle biopsies of the mass demonstrated thymic tissue, respiratory epithelium, and both apocrine and eccrine sweat glands, consistent with mature cystic teratoma. No malignant cells were identified. MMT tissue grew Hemophilus influenza. Figure 2. Photomicrographs showing respiratory epithelium (Left) and apocrine and eccrine sweat glands (Right) in the MMT biopsies (10X). During median sternotomy, tumor was seen infiltrating through ruptured pericardium and around both ventricles. The left anterior descending coronary artery coarsed through the mass. Complete dissection of the tumor from the heart was impossible. Hemoptysis, chest pain, and dyspnea sub-sided during treatment with pipercillin-tazobactam. Repeat chest CT six months later showed no significant interval change. Nine months post-operatively he felt well without symptoms.
DISCUSSIONS: Our patient with MMT is unique because (a) he presented with hemoptysis (rare) and (b) he had bacterial infection of the MMT (not described before in adults). Hemoptysis and chest CT evidence of gas in the MMT reflected tumor and tracheobronchial tree rupture. Mechanisms for rupture include MMT infection and release of proteolytic enzymes from pancreatic/intestinal mucosal tissues contained in the MMT. We found no evidence of infection beyond the MMT itself to explain its rupture; e.g., pneumonia, and therefore, we speculate that rupture resulted from MMT proteolytic enzyme release and led to hemoptysis and MMT infection. Pancreatic tissue, the most common one found in MMT, was not found in our small needle biopsy specimen, but the unsampled tumor likely contained pancreatic/intestinal mucosal tissue. Even though the MMT in our patient was benign and histologically mature, complete resection, the treatment of choice, was impossible because the MMT had infiltrated pericardium and encased both ventricles and the coronary arteries. Tumor resection under cardiopulmonary bypass, reconstruction of the loculated ventricles, and heart-lung transplant are future treatment considerations.
CONCLUSION: We report a mediastinal mature teratoma with a combination of rare features not previously described: the presentation of a MMT with hemoptysis, Hemophilus influenza infection in an adult, bronchial and intrapericardial rupture, and unresectable nature of a benign tumor.
DISCLOSURE: Karthik Jothianandan, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Pulmonary embolectomy is highly effective on hemodynamically unstable patients with massive pulmonary embolism.. We present a case of massive pulmonary embolism associated with cardiac arrest who was successfully treated with pulmonary embolectomy.
CASE PRESENTATION: A 21-year-old college student presented to the emergency department after a syncopal episode. He complained of chest pain and shortness of breath. A chest computed tomography scan showed massive bilateral pulmonary artery emboli(figure 1). Thrombolytics were administered and shortly thereafter he suffered cardiopulmonary arrest. The patient was taken emergently to the operating room while undergoing cardiopulmonary resuscitation. The patient was placed on cardiopulmonary bypass while receiving chest compressions. Pulmonary embolectomy was performed through incisions on the right and left pulmonary arteries. There were 2 large fully occlusive clots in the right and left main pulmonary arteries (figure 2). Valsalva maneuvers and direct compression of the lungs were performed to aid in extracting distal pulmonary clots. After the embolectomy, the heart resumed sinus rhythm. There was moderate right ventricle dysfunction that required milrinone and nitric oxide support. An inferior vena caval filter was placed prior to his return to the ICU. The patient woke up and had no neurological deficit. He was extubated the following day. He had bilateral lower extremity duplex with a small thrombus noted in his left saphenous vein. No hypercoagulable state was identified. The patient was fully anticoagulated and discharged home on post operative day #8. Figure 1: CT PULMONARY ANGIOGRAM shows large central pulmonary embolism with occlusion of both right and left pulmonary arteries. Figure 2: Emboli removed from this patient.
DISCUSSIONS: Massive pulmonary embolism is associated with cardiopulmonary collapse . The mortality for patients who present on cardiac arrest is at least 67%. Cardiac arrest is not only a marker of hemodynamic severity and poor prognosis of the embolism but it also is associated with high risk of neurological injury.
CONCLUSION: This case shows that an aggressive approach on selected patients with massive pulmonary embolism and cardiac arrest may be justified. Pulmonary embolectomy in patient with cardiac arrest should be consider in young patients, with limited comorbidities, and with short periods of cardiopulmonary resuscitation. Adherence to those criteria will improve outcomes and allow for complete chance of neurologic recovery. If the likelihood of neurological recovery is high, mechanical circulatory support for a failing right ventricle should be considered.
DISCLOSURE: Paul Vesco, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Severe scoliosis distorts thoracic anatomy and produce marked asymmetry within the chest cavities. These characteristics contribute to concerns for donor-to-recipient size mismatch in lung transplantation. For this reason, some patients with severe scoliosis end stage lung disease are denied lung transplantation (LTx). Two cases of patients with severe scoliosis who underwent successful double LTx are presented.
CASE PRESENTATION: Our first patient was a 53 year old female who developed end stage interstitial lung disease secondary to sarcoidosis. Work-up showed a normal forced expiratory volume in 1 second percent of predicted (FEV1%) 74%, forced vital capacity percent of predicted (FVC%) 79% and diffusion lung capacity for carbon monoxide (DLCO) 19% of predicted. The patient desaturated to 88% using 6 liters of oxygen by nasal cannula (NC). A quantitative ventilation-perfusion (VQ) scan demonstrated a right to left distribution of 70% and 30%, respectively. Pulmonary artery systolic pressure (PASP) was 70 mmHg. She had severe scoliosis with a Cobb angle of 80 degrees (Figure 1A). Due to her pulmonary hypertension, double LTx was recommended. Double LTx was performed with an unremarkable hospital course. She was discharged on postoperative day 10. Nine months after surgery, her chest radiograph was clear (Figure 1B). With an FEV1% of 68%, she required no supplemental oxygen, did not desaturate and reported an excellent quality of life. Our second patient was a 46 year old female who developed end-stage bronchiectasis due to congenital primary ciliary dyskinesia. Work-up showed FEV1% of 29% and FVC% of 32%. The patient was unable to tolerate DLCO measurement. With 6L of oxygen by NC, she desaturated on exertion to 90%. VQ scan demonstrated a right to left distribution of 73% and 27%, respectively. PASP was normal. She also had severe scoliosis with a Cobb angle of 72 degrees (Figure 2A). Due to her recurrent respiratory infections and bilateral bronchiectasis, double LTx was recommended. The postoperative course was complicated by myopathy, reoperation for bleeding and prolonged airleak. The patient was discharged on postoperative day 30. Eight months after surgery, her chest radiograph was clear (figure 2B), FEV1% 36% and no supplemental oxygen is required. Bronchoscopy demonstrated moderate, right mainstem stenosis which has not required any intervention.
DISCUSSIONS: Severe scoliosis is considered a technical contraindication to lung transplantation. Asymmetry of the hemithoraces is thought to prohibit appropriate graft size matching and chest wall distortion may cause poor functional outcome. This, however, was not our experience. No significant technical problems were encountered for either patient during lung extraction or implantation. Interestingly, the second patient developed a complication directly related to her scoliosis: right-sided airway narrowing secondary to dynamic airway compression against her scoliotic vertebral body. This seemed to negatively impact her FEV1% which is less than expected. Fortunately, this has not required intervention and the patient reports a good quality of life.
CONCLUSION: We conclude severe scoliosis alone should not preclude patients from double lung transplantation. Although surgically challenging, double LTx in patients with severe scoliosis is possible with outcomes similar to non-scoliosis patients.
DISCLOSURE: Jang Wen Su, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Vascular complications are rare but serious causes of morbidity and mortality following lung transplantation. Pulmonary artery stenosis at the anastomosis can cause dyspnea and pulmonary hypertension in transplant patients. Diagnosis of pulmonary artery stenosis can be achieved using ventilation perfusion scanning, echocardiography and pulmonary angiogram. In the literature, most anastomotic complications have been surgically repaired. We report a case of significant pulmonary artery stenosis in a single lung transplant recipient successfully treated with angioplasty and stent placement.
CASE PRESENTATION: A 70 year old male with a history of coronary artery disease who underwent left single lung transplantation for idiopathic pulmonary fibrosis developed exertional dyspnea 2 weeks after transplantation. The patient had an uneventful surgery and immediate post-operative course. When the patient was re-admitted for dyspnea, EKG, cardiac enzymes and CT angiogram were negative. Ventilation perfusion scan showed diffusely decreased perfusion and ventilation in the left lung with perfusion worse than ventilation. Split pulmonary function testing was done revealing 16.7% function of the left lung. Bronchoscopy was performed showing granulation tissue at the left mainstem bronchus anastomosis which was debrided in the operating room. After discharge he remained with symptomatic dyspnea and oxygen requirement. Four weeks post-transplant, repeat bronchoscopy was done demonstrating mucous plugging but no bronchus stenosis or rejection on biopsy. Echocardiography showed normal left and right ventricular function and a pulmonary artery systolic pressure of 30 mmHg. Arterial blood gas revealed a pH of 7.433, a PaCO2 of 32.6, PaO2 of 70.1 on room air with a normal methemoglobin level. A repeat bronchoscopy was done 6 weeks post-transplant showing normal bronchial anatomy and a biopsy negative for rejection. Seven weeks post-transplant, patient was seen in clinic with continued symptoms and a New York Heart Association III functional classification. At that time his oxygen saturation on 3L of oxygen per minute was 92%. He had pulmonary function testing revealing an FEV1 of 2.59L (88%), FVC of 3.63L (97%). Patient was then sent for pulmonary angiogram which demonstrated a severe stenosis at the anstomosis of the pulmonary artery. The pulmonary artery pressure in the pre-stenotic segment was 25 mmHg and 2–4 mmHg in the post-stenotic segment, making a transtenotic gradient of 21–23 mmHg. A 10mm diameter stent was placed and inflated using a balloon. There was improvement in room air saturation during the procedure from 88% to 96%. Patient's 6 min walk distance changed from 1150 feet requiring 4L O2 to 1125 feet without any O2 supplementation after stenting.
DISCUSSIONS: Vascular stenosis is uncommon after lung transplantation. The incidence of pulmonary vascular stenosis in one series was reported to be 1.75%. (1). Unilateral vascular stenosis in double lung transplants may go unnoticed secondary to the opposite lung compensating for the defect. Nearly all reported clinically significant cases were in single lung transplants. Diagnosis is usually suspected based on otherwise unexplained symptoms and preferential perfusion to the native lung on ventilation/perfusion scans. Diagnosis is confirmed with pulmonary angiogram. Until 1994 surgical repair was the only therapy and had associated morbidity and mortality risk. In 1994, Ferretti was the first to successfully percutaneously stent a stenosis using a balloon expandable stent. Since that time there have been several reports of this method as a surgery sparing treatment.
CONCLUSION: In post-lung transplant patients with otherwise unexplained dyspnea or pulmonary arterial hypertension, pulmonary artery stenosis should be considered in the differential. Percutaneous stenting of the artery is an effective treatment which avoids risky surgery.
DISCLOSURE: Lisa Kopas, None.
INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) describes a cytokine storm that is due to uncontrolled accumulation of activated T-lymphocytes and activated histiocytes. This results in organ infiltration with these cells, and subsequent hemophagocytosis of erythrocytes, leukocytes and platelets. In its most severe form, HLH leads to a sepsis-like picture and multiorgan failure (MOF). We report the case of an adult male that presented with severe adenovirus pneumonia (AVP) resulting in HLH with acute respiratory distress syndrome (ARDS) and MOF. This association may at least in part explain the recently observed increase in the incidence of fatal adenoviral infections.
CASE PRESENTATION: A 22-year old male with a remote history of juvenile rheumatoid arthritis (JRA) was admitted with a 3-day history of dyspnea, productive cough and fever that did not respond to outpatient treatment with a macrolide. The patient had not been on immunosuppressive medications for >2 years. Chest CT on admission revealed diffuse dense bilateral infiltrates (Fig. 1). Despite treatment with broad spectrum antibiotics, the patient remained febrile and developed rapidly pogressive hypoxic respiratory failure requiring mechanical ventilation. Bronchoalveolar lavage (BAL) initially did not reveal any pathogens. The patient developed shock, acute renal failure and metabolic acidosis, requiring multiple pressors and renal replacement therapy. Stress-dose hydrocortisone was added without improvement. Four days after bronchoscopy, the BAL viral culture grew adenovirus. The patient continued to exhibit severe hypoxia as well as hypercarbia and died of respiratory failure on hospital day 12. During the patient's clinical deterioration, ferritin increased from 757 to 9490 ng/ml, and platelets decreased from 180.000 to 36.000/mm3. Disseminated intravascular coagulation was excluded. The patient also developed hypertriglyceridemia (499 mg/dl) and anemia (8.6 mg/dl). The bone marrow biopsy did not show any evidence of hemophagocytosis. However, the postmortem examination revealed macrophages with hemophagocytosis and lymphophagocytosis in a hilar lymph node (Fig. 2). Decreased natural killer (NK) cell activity was detected in the patient's blood, although this result was not available until after the patient's demise. Based on these results, a postmortem diagnosis of adenovirus-induced HLH was made.
DISCUSSIONS: In immunocompetent patients, adenovirus infection usually causes self-limited upper respiratory tract infections. While cases of severe AVP in immunocompetent adults have been described, the overall incidence is extremely rare. Recently, several cases of fatal AVP in previously healthy patients were reported. Some of these were linked to adenovirus serotype 14. HLH, while being associated with viral pathogens such as herpes simplex or Ebstein-Barr virus, malignancies, autoimmune diseases, drugs, genetic and familial conditions, has also been associated with adenoviral infections. The diagnosis of HLH is based on a combination of clinical and laboratory criteria. These include fever, splenomegaly, cytopenia of ≥2 cell lines, hyperferritinemia, hypertriglyceridemia, increased soluble interleukin-2 receptor levels, decreased or absent NK-cell activity and hemophagocytosis in bone marrow, cerebrospinal fluid or lymph nodes. The syndrome is often misdiagnosed as sepsis, thereby precluding the potentially curative treatment with corticosteroids, immunosuppressants and/or chemotherapeutic agents. It is conceivable that the recently observed increase in virulence of adenoviral infections may at least in part be due to an association with HLH. The patient's history of JRA may have made him particularly susceptible.
CONCLUSION: HLH is a syndrome of life-threatening hyperinflammation that may be misdiagnosed as sepsis. The association of HLH with severe AVP observed in our patient suggests a potential mechanism for the recently reported increase in the incidence of extremely virulent adenoviral infections.
DISCLOSURE: Tim Lahm, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Bronchioloalveolar carcinoma (BAC) is a subtype of pulmonary adenocarcinoma, with growth along intact alveolar septa (lepidic growth pattern). BAC can be classified into mucinous, nonmucinous, and mixed or indeterminate cell types. It has been found that the prognosis of nonmucinous BAC is better than that of mucinous BAC, probably because the mucinous type tends to spread aerogenously and forms infiltrating, multifocal, or satellite tumors. Radiographically, it may present as a solitary peripheral lung nodule, lobar consolidation or multiple pulmonary nodules. We present a unique case of muconous bronchioloalveolar carcinoma presented radiologically as an innumerable sharply defined tiny nodules throughout both lung fields mimicking a miliary infectious etiology and its fulminant course.
CASE PRESENTATION: A 68 year old gentleman with hypertension, diabetes mellitus and a 35 pack year smoking history presented to the emergency room with left sided pleuritic chest pain for the past 1 week associated with mild shortness of breath and nonproductive cough. He denied hemoptysis, fever or chills. He admitted to a 40 pound weight loss over the past 5 months. Physical exam was significant for tachycardia and bilateral rhonchi. Chest radiograph showed miliary like pattern and CT of the chest revealed random distribution of innumerable sharply defined tiny nodules throughout both lungs (Fig 1). Urinary antigen for Histoplasmosis capsulatum, Aspergillosus, HIV test and 3 sets of sputum AFB smears were negative. Diagnostic bronchoscopy revealed no endobronchial lesions with non diagnostic transbronchial biopsies. During this time he progressively became more dyspneic and hypoxic, he was intubated and required ventilaory support. Despite these efforts he continued to have progressive respiratory failure and eventually went into cardiac arrest and expired. Autopsy confirmed extensive bronchioalveolar carcinoma (Fig 2) with metastasis to the mediastinal lymph nodes, liver and adrenal glands.
DISCUSSIONS: BAC was formerly considered a mystery tumor because of its various radiologic, clinical, and cytopathologic manifestations accounting for 3% of all lung malignancies. The solitary nodular form of BAC is the most common and it is seen in 38%, the lobar is seen in 24% of cases, and multilobar condolidation in 31% whereas the diffuse nodular form is the least common presentation and it is reported in 7% of cases. The CT scan appearance of diffuse nodular bronchioloalveolar carcinoma is diverse, and includes poorly or well-defined nodules and multiple poorly defined areas of ground-glass attenuation or consolidation which is not seen in our case. These nodules are usually distributed predominantly in a centrilobular fashion and it is rarely distributed randomly (Fig 1), whereas miliary tuberculosis and pulmonary metastasis usually have a diffuse random distribution. BAC should be considered in the differential diagnosis of solitary or multiple pulmonary nodules and acute or chronic alveolar diseases.
CONCLUSION: The multinodular form of bronchioloalveolar carcinoma should be differentiated from metastatic lung carcinoma, fungal infection, granulomatous disease, lymphoma, and disseminated pulmonary tuberculosis. The diffuse form (multinodules, diffuse, or infiltrative) tends to be relentlessly progressive with a worse prognosis regardless of intervention.
DISCLOSURE: Fadi Al Khankan, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Benign fistulae between the central airways and the neo-esophagus are a rare complication of esophagectomy. The subsequent recurrent bronchopneumonia can be life-threatening. There is no standardized therapy for post-esophagectomy fistulae. Reports of management range from observation to stenting to resection of the neo-esophagus.
CASE PRESENTATION: A 69-year-old man was referred to the interventional pulmonology service for evaluation of tracheo-esophageal fistula. The patient had been diagnosed with adenocarcinoma of the esophagus 14 months earlier and had undergone primary radiation and chemotherapy followed 6 months later by transhiatal esophagectomy. Four months after surgery he was admitted for aspiration pneumonia. Computed tomography (CT) scan of the chest at that time revealed a fistula between the right mainstem bronchus (RMB) and the neo-esophagus. Adequate esophagoscopy could not be performed because of a stricture at the cervical anastamosis. Flexible bronchoscopy revealed a 6mm fistula track in the medial wall of the RMB at the level of the right upper lobe bronchus. The surrounding mucosa appeared normal with no suggestion of recurrent malignancy or necrosis. The fistula was traversed, confirming a direct communication with the neo-esophagus. Initial treatment was attempted with an uncovered self-expandable metallic stent (Ultraflex®) with the anticipation that the stent would induce the formation of granulation tissue resulting in closure of the fistula. Follow-up bronchoscopy one month later revealed appropriate stent position with inflammatory changes at the fistula opening in the RMB but no change in diameter of the fistula. Several weeks later the patient developed right lower lobe (RLL) pneumonia with associated respiratory failure and septic shock. Flexible bronchoscopy revealed continuous leakage of secretions from the fistula into the airways of the RLL. Once hemodynamically stable, the patient underwent rigid bronchoscopy. The metallic stent was removed. An Amplatzer® Septal Occluder with an 18mm distal (neo-esophageal) disk, a 6mm waist and a 16mm proximal (bronchial) disk was deployed across the fistula. After deployment, all airways were patent and no further bronchial contamination from the fistula was visualized. The patient was subsequently liberated from the ventilator and discharged home. Several weeks later, the patient was readmitted with altered mentation after dilation of the upper esophageal stricture. He was diagnosed with Streptococcus milleri bacteremia and multiple brain abscesses. During treatment, he developed aspiration pneumonia and subsequent respiratory failure requiring mechanical ventilation. Repeat flexible bronchoscopy at that time (five weeks after initial placement) revealed good position of the Amplatzer® device with no evidence of leaking at the fistula site. The patient was subsequently transitioned to comfort-focused care and died two weeks later.
DISCUSSIONS: The Amplatzer® Septal Occluder is a biocompatible dual-disk device composed of nitinol metal and polyester fabric. It is designed for percutaneous deployment in the closure of atrial septal defects. The variety of sizes available and established efficacy make the Amplatzer® device suitable for use in the airways. Advantages of this device are the complete mechanical seal that it produces over the defect and the induction of tissue granulation to facilitate long-term closure. Our review of the literature yielded two case reports of using Amplatzer® devices in the airways: to close a post-surgical bronchopleural fistula and an esophagorespiratory fistula. We describe what we believe to be the first use of rigid bronchoscopy to deploy an Amplatzer® device for closure of an acquired fistula between the airways and digestive tract. Rigid bronchoscopy enabled direct visualization of deployment as well as confirmation of cessation of airway contamination once the fistula was closed.
CONCLUSION: The Amplatzer® Septal Occluder can be deployed via rigid bronchoscopy for closure of benign fistulae between the central airways and the digestive tract.
DISCLOSURE: David Green, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Granular cell tumor (GCT) of the bronchus is an uncommon benign tumor that can cause complications due to obstruction of the airways. We report the first case of bronchial GCT treated successfully with argon plasma coagulation (APC), without evidence of its recurrence 3 months later.
CASE PRESENTATION: A 54-year-old man, former smoker, and with history of hypertension, spontaneous pneumothorax in distant past, hepatitis C related cirrhosis of the liver, portal hypertension with esophageal varices and status post banding was evaluated for intermittent mild hemoptysis, of 1 year duration. Physical examination revealed normal nasal mucosa without any evidence of bleeding. There was no cervical lymphadenopathy. Examination of the chest revealed broncho-vesicular breath sounds in all lung fields, without presence of any crackles or wheezing. The cardiovascular examination revealed normal S1, S2, and regular rhythm. There was no peripheral edema. The prothrombin time (10.6 seconds) and platelet count (142 thousand/mm3) were normal. Pulmonary function testing revealed normal forced vital capacity of 6.01 L (113% predicted) and forced expiratory volume in 1 second of 4.13 (98% predicted). High resolution computed tomography of the chest revealed oval thickening (11.5mm × 7.8mm) of the inferior wall of the bronchus intermedius suggestive of a growth. Fiberoptic bronchoscopy revealed a small, variegated, non-bleeding lesion in the inferior aspect of the bronchus intermedius. This was located proximal to the right superior basal segment take-off and at the base of the accessory segment without causing its occlusion. Differential diagnoses included bronchial carcinoid, mucoepidermoid tumor, chondroid hamartoma, bronchogenic carcinoma etc. Histopathology of the tumor biopsies revealed polygonal cells with granular eosinophilic cytoplasm and uniform appearing hyperchromatic, centrally originating nuclei consistent with GCT. Immunohistochemistry was positive for S-100. Since, the patient was symptomatic and no other source of bleeding was identified, decision was made to achieve endobronchial resection. Endobronchial resection of the tumor was performed, in 2 sittings, using ERBE USA Inc. APC system with argon flow rate of 2 L/minute to achieve adequate tumor destruction and coagulation. A follow up bronchoscopy 3 months later did not reveal recurrence of tumor. The patient's hemoptysis resolved completely.
DISCUSSIONS: GCT of the tracheobronchial tree is uncommon and believed to be Schwann cell origin. Immunohistochemistry is crucial in the diagnosis. There is no consensus with regards to effective treatment modality for GCT's of the airways. A recent review of all the registered cases of GCT's in the Dutch Network and National Database for Pathology suggests very benign course of the disease1. The authors favor endobronchial therapy. Daniel et. al.2, in their review of the literature, found that all tumors removed bronchoscopically whose diameter was ≥ 1 cm recurred. However, none of the patients died because of the tumor recurrence. Full thickness involvement of the tracheal wall correlated with tumor size, and likely explained their recurrence. Surgical morbidity and mortality must be weighed before making a decision for treatment. APC provides a controlled, limited penetration into the tissue and good control of bleeding. APC is suitable for treating bronchial segments, taking off at acute angles, as argon flows quite flexibly around bends and corners. Also, it is cheaper, portable, and safely used with flexible bronchoscope.
CONCLUSION: Granular cell tumor of the bronchus is a benign tumor and endobronchial resection with argon plasma coagulation is an effective new treatment modality. Follow up bronchoscopy for tumor recurrence is recommended.
DISCLOSURE: Ashutosh Sachdeva, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Recurrent respiratory papillomatosis (RRP) is a benign, often multifocal neoplasm caused by the human papilloma virus (HPV), characterized by the formation of recurrent, epithelial neoplastic lesions in the larynx, but in 2 to 5% of the cases can affect the lower airways where they can produce airway obstruction. Treatment usually involves repeated surgical debulking with carbon dioxide (CO2) laser or the microdebrider with medical interventions reserved as adjuvant therapies. We describe a case with RRP involving all the distal airways using a combination of argon plasma coagulator (APC) and intralesional cidofovir.
CASE PRESENTATION: A 60 year old male presented with a five year history of progressive dyspnea and wheezing. An outside bronchoscopy reported partial obstruction of both main bronchus by multiple endobronchial lesions. Two different biopsies confirmed the diagnosis of RRP. His physical exam was remarkable for the presence of dyspnea at rest and bilateral wheezing. Our initial bronchoscopy showed a near complete obstruction of both main bronchus due to tumor growth (Figure 1 A and B), which extended to all the distal airways (Figure 1 C and D). APC was used to successfully desiccate all the intrabronchial lesions. Once the airways were patent (Figure 2) we used a Wang needle to inject cidofovir into the few visible lesions encrusted on the bronchial walls. The patient had a complete resolution of his symptoms and remains asymptomatic with repeat bronchoscopy performed at 18 and 36 months showed no recurrence of endobronchial lesions.
DISCUSSIONS: The treatment of the RRP lesions is challenging and usually involves repeated surgical debulking with CO2 laser or increasingly with the microdebrider. Adjuvant treatments include cidofovir, indole-3-carbinol, ribavirin, mumps vaccine, and photodynamic therapy. As illustrated in this case, APC can be used as an alternative treatment for RRP. The APC uses a high frequency electric current fed from a probe tip, that fits inside the working channel of the bronchoscope, through ionized argon plasma that causes superficial thermal coagulation of tissue. Argon flow was set between 0.5 and 1.0 L/min, and the current between 40 to 60 Watts. The probe was placed between 3 and 5 mm from and in a tangential position to the lesion of interest. Coagulation was activated by a foot pedal and repeatedly applied for 1- to 5-seconds intervals until the lesion appeared to be desiccated. Loose debris was removed with suction or forceps. Because argon coagulation is dependent on the water content of the targeted tissue, desiccation of the treated area prevents deeper thermal effect and damage to underlying structures. The APC offers significant advantages, including tissue damage is mild and predictable, argon coagulation does not result in tissue carbonization or vaporization, and can be used in the distal airways. It is cheaper than CO2 laser and requires much less training. There are only two reports of the use of APC in RRP but in both cases papillomas were limited to the trachea [1, 2]. Our report is unique in two aspects: first it describes an extreme case of RRP with involvement of all the segments of all the distal airways and second, this is the first report in which combination of APC and intralesional cidofovir is used.
CONCLUSION: APC may be an alternative for the treatment of RRP, especially with distal airway involvement. Whether the concomitant use of intralesional cidofovir will be effective requires further studies.
DISCLOSURE: Vichaya Arunthari, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Recurrent respiratory papillomatosis (RRP), benign tumors of the airway with potential to undergo malignant transformation to squamous cell carcinoma (in 3–4%), are caused by the human papilloma virus (HPV). These tumors usually involve the larynx, epiglottis, sub glottis, vocal cords and trachea. Pulmonary parenchymal and endobronchial involvement is seldom seen. We describe a case of endobronchial obstruction caused by RRP in a non-compliant patient.
CASE PRESENTATION: A 36-year male smoker with a past medical history significant of recurrent respiratory papillomatosis (RRP), s/p multiple laser excisions and microdebridement over the last ten years, presented with a two weeks history of hoarseness, productive cough and shortness of breath. Examination revealed an acutely ill man in moderate respiratory distress with respiratory rate of 30/minute, heart rate of 114/minute, blood pressure of 166/82 and oxygen saturation (SpO2) of 89%, increasing to 94% with supplemental oxygen. Chest examination revealed bilateral inspiratory and expiratory wheezing and stridorous breath sounds, prominent over the neck and the upper airway. The remaining physical examination was noncontributory. Complete blood count, metabolic panel and coagulation panel were normal, as was a portable single view chest radiograph. The patient became progressively hypoxic and developed respiratory failure, with arterial blood gas showing pH 7.214, pCO2 73mmHg and pO2 105mmHg. Non-invasive ventilation was not well tolerated. Direct laryngoscopic visualization of the upper airway during intubation showed multiple polyps in the supraglottic region and on the right vocal cord. He was treated with intravenous steroids and bronchodilators. Thoracic computed tomography demonstrated diffuse nodular opacities within the trachea and the bronchi, occluding the right main stem bronchus and bronchus intermedius, multiple scattered small nodules in the left lower lobe, left upper lobe and right upper lobe, including one with cavitation, and sub centimeter hilar lymphadenopathy. The next day, in the OR, fiberoptic bronchoscopy showed multiple papillomas in the supraglottic region, right vocal cord, trachea, right main bronchus, and right bronchus intermedius, the latter occluding the airway. The RUL and left-sided bronchi were patent. The patient underwent direct laryngoscopy and rigid bronchoscopy with excision and microdebridement of the upper airway, tracheal and endobronchial papillomas. The histopathology of the excised tissue showed laryngeal and tracheal papillomas consistent with the diagnosis of recurrent respiratory papillomatosis and revealed diffuse low as well as high grade dysplasia. Immuno-histochemistry was positive for HPV 6 and 11. After he was extubated and he reported considerable improvement of his symptoms.
DISCUSSIONS: Recurrent respiratory papillomatosis (RRP) are rare benign respiratory tumors with bimodal age distribution with peak age of diagnosis around 4 years in children and third to fourth decade in adults. RRP is caused by HPV subtypes 6 and 11. They are typically seen in the larynx, supraglottic area and trachea but rarely extend to the bronchial tree and lung parenchyma, presenting as nodules that may cavitate. Most common symptoms in adult patients include hoarseness of voice, sore throat, cough, and dyspnea. Bronchial RRP may be misdiagnosed as asthma, croup or bronchitis. The course of RRP varies from spontaneous remission to recurrence of papillomas, requiring multiple laser or surgical interventions. Our patient had respiratory failure due to endobronchial obstruction caused by RRP. He was not a candidate for laser resection at this time, due to the extensive endobronchial involvement. Excision of papillomas and microdebridement through rigid bronchoscopy in our patient was successful and he had significant improvement of his symptoms.
CONCLUSION: Recurrent respiratory papillomatosis (RRP), although uncommon, should be diagnosed early and treated aggressively with either laser resection or surgery. If left alone they have the potential to become malignant, and increased morbidity and mortality is seen in patients, with endobronchial obstruction.
DISCLOSURE: Jaswinderpal Sandhu, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: One of the most poorly understood areas for TBNA targets is the A-P window. A common misperception is that this area is too dangerous to attempt TBNA given large vascular structures bordering the region. The fear is unsupported as few reported complications from TBNA other than small bleeding at the puncture site exist.1 We report a case utilizing TBNA with an extended length needle in combination with fluoroscopic guidance and successful sampling of the lateral A-P window.
CASE PRESENTATION: A 47 year-old male with a 40 pack/year smoking history was referred for diagnosis of a PET positive A-P window lymph node (fig 1.a, 1.b). Although there were several areas within the A-P window that contained lymph nodes, the PET scan only showed activity in the lateral portion of the A-P window (fig 1.a, 1.b). Additionally, the patient had severe bilateral emphysema (fig 1.c). TBNA of the A-P window was the procedure of choice given the high risk for pneumothorax from transthoracic needle biopsy. Several conventional TBNA attempts were made in the A-P window station without diagnostic material. After failing to reach the lateral lymph node with a standard 1.5 cm TBNA needle, an extended length Vizishot transbronchial needle made by Olympus was used at the same site under fluoroscopic guidance to direct the needle tip to the most lateral area of the A-P window (fig 2). This specimen was positive for malignant cells. In this case, we were able to secure the diagnosis of non-small cell carcinoma with minimal risk to the patient.
DISCUSSIONS: The case above illustrates important issues regarding TBNA of the A-P window, including techniques for sampling even the most lateral areas of the A-P window. With conventional bronchoscopic needles, the lymph nodes immediately adjacent to the left tracheal wall are most likely sampled when performing TBNA in the A-P window. Using ATS nomenclature, these lymph nodes are considered left paratracheal lymph nodes while lymph nodes lateral to the ligamentum arteriosum are considered "true" A-P window lymph nodes. We propose a practical, conceptual framework to optimize TBNA strategies and increase the yield at this station. As such, the A-P window would be divided into thirds: the medial, middle, and lateral sections. While the medial and middle thirds may be sampled with conventional 1.5 cm needles, the most lateral section is completely inaccessible when approached through the tracheal wall. With an extended length needle and fluoroscopic guidance, this area is now available for aspiration. While endobronchial ultrasound (EBUS) is capable of sampling the A-P window, and the same extendable Vizishot needle is used with the EBUS bronchoscope, the angle of penetration is not 90 degrees as is often needed to access the lateral A-P window. The aorta often blocks a 45 degree angle approach to the lateral A-P window. As was shown in the earliest flexible needle TBNA reports, yet rarely used since, an extended length "telescopic" needle is an effective tool for TBNA.2.
CONCLUSION: Often, diagnosis of A-P window lymph nodes can be the determining factor for whether a patient is a surgical candidate. In some patients, the risk for diagnostic procedures other than TBNA can be quite high. Improving our understanding of this lymph node station and the techniques to safely and successfully perform TBNA in the A-P window may have a significant impact on quality of care for our patients.
DISCLOSURE: Daniel Kim, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Complications from transbronchial needle aspiration (TBNA) of lymph nodes are rare and can include minor bleeding, hypoxemia and bronchospasm. We report a case of endobronchial ultrasound (EBUS)-guided TBNA complicated by descending mediastinitis and purulent pericarditis.
CASE PRESENTATION: A 53-year-old female presented for outpatient consultation with symptoms of recurrent bronchitis and a long history of mediastinal lymphadenopathy. Thoracic CT revealed mediastinal and hilar lymph-adenopathy. The patient underwent EBUS-TBNA of the station 4R node to confirm a diagnosis of pulmonary sarcoidosis. Biopsy needle placement was confirmed using EBUS and four passes were made into the node. There were no immediate peri-procedural complications and pathology revealed only lymphoid elements. One week later, the patient presented to an outside pulmonologist with complaints of chest pain and high-grade fevers. A chest CT was obtained showing a new heterogeneous paratracheal mass at the level of the 4R lymph node and a small pericardial effusion. She was then transferred to our institution for treatment of mediastinitis. On mediastinoscopy, a large firm lymph node collection was discovered without suppuration or hematoma and biopsy revealed non-caseating granulomas with negative stains for fungal and acid fast organisms. She was hospitalized for three days and her fever resolved with vancomycin, gentamycin and piperacillin/tazobactam. Several days after discharge home without antibiotics, the patient again developed chest pain made worse when lying on her left side and deep inspiration, fevers, chills, joint pains and delirium. Her ECG showed low voltage with diffuse ST segment elevation and pulsus paradoxus was noted on exam. Echocardiography revealed a moderate circumferential pericardial effusion and mild hemodynamic compromise. She was promptly readmitted to our institution and treated with vancomycin, gentamycin and ceftriaxone. After catheter removal of 750 mL of purulent pericardial fluid with a white blood cell count of greater than 18,000 (98% polymorphonuclear cells) and no organisms on fluid culture, rapid clinical improvement ensued. Ceftriaxone was continued for 4 weeks. Follow-up transthoracic echocardiogram revealed improvement of the pericardial effusion.
DISCUSSIONS: Descending mediastinitis usually results from a complication of oropharyngeal infection or from extension of adjacent infection. Purulent pericarditis, equally rare and life threatening, usually results from contiguous pulmonary or chest wall infection such as pneumonia or empyema, hematogenous spread and direct infection from trauma or surgery. The most common organisms include S. aureus, other Gram positive organisms or fungal species. While others have reported mediastinitis through inoculation by infected transbronchial needles during TBNA and purulent pericarditis in the setting of gene therapy injection for treatment of lung cancer, none of these cases have involved standard EBUS-TBNA. The most likely mechanism for the spread of infection in this case would be translocation of airway bacteria into the pericardial space.
CONCLUSION: We report to our knowledge, the first case of mediastinitis and purulent pericarditis resulting from EBUS-TBNA lymph node biopsy illustrating the importance of considering this potential complication. Given the advantages and greater sampling accuracy of EBUS-TBNA, it will likely continue to be performed in much greater numbers which underscores the consideration of this rare complication.
DISCLOSURE: Henry Ostman, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Angiosarcoma is the most common primary malignant heart tumor with frequent metastasis to lungs at the time of diagnosis. However it is a rare cause of hemoptysis.
CASE PRESENTATION: A 50 year old previously healthy man with a 20-pack year smoking history was transferred for management of cough of one month duration and hemoptysis over the previous few days. The cough, which was initially productive of only thick white sputum, now demonstrated blood streaking. He also complained of low grade, subjective fevers and mild exertional dyspnea. He did not have chest pain, diaphoresis, lower extremity edema or swelling, tachycardia, or nasal symptoms. His physical exam was unremarkable. A CT scan with contrast demonstrated multiple bilateral pulmonary emboli and diffuse lung nodules with surrounding pulmonary infiltrates (Fig1.a.b.c). Routine laboratory data were unremarkable. Given concern for intraparenchymal bleeing due to the nodular process, the decision was made to place an inferior vena cava filter and forgo anticoagulation. A Bronchoscopy revealed normal appearing bronchial mucosa throughout both lungs. Bronchoalveolar lavage revealed sanguinous return from the right middle lobe bronchial segments consistent with alveolar hemorrhage. Bronchoalveolar lavage, fine needle aspiration of subcarinal lymph nodes and transbronchial biopsies were performed and were non-diagnostic. The work up for connective tissue diseases including Wegner's granulomatosis was negative as well. Over the subsequent few days, the patient appeared to be improving with cessation of his hemoptysis. An S3 gallop was auscultated and elevated jugular venous pressure was noted. An echocardiogram demonstrated a pericardial effusion and suggestive tamponade physiology. A pericardiocentesis revealed hemorrhagic fluid, and a subsequent pericardial window with simultaneous biopsy of lung nodules were performed. The lung biopsy revealed (Fig2) extensive involvement of the pulmonary vasculature with epithelioid and spindle-shaped cells, the majority of which had a high nuclear pleomorphism and mitotic rates, forming rudimentary vascular channels. The pulmonary vessels were filled with tumor emboli. Immunohistochemistry demonstrated the neoplastic cells to be positive for the endothelial cell markers CD31 and CD34 and negative for the epithelial cell marker cytokeratin supporting the diagnosis of angiosarcoma. A follow up echocardiogram was performed to assess the effectiveness of the pericardial window and a right atrial mass was suspected. Cardiac CT angiography revealed the mass to be located around right atrium and ventricle and encasing the right coronary artery consistent with a diagnosis of primary cardiac angiosarcoma with lung metastasis. The patient was enrolled in a clinical trial using sorafenib as the primary chemotherapeutic modality.
DISCUSSIONS: Angiosarcoma is a rare tumor of mesenchymal origin more commonly seen in males between the third and fifth decades of life. Cardiac angiosarcomas are most commonly found in the right atrium in contrast to benign tumors which are found in the left atrium. Clinical diagnosis of this rare entity is often difficult due to vague symptomatology at presentation. Although most of the cases are accompanied by hemoptysis, the non-specificity of this symptom leads to angiosarcoma rarely being considered in the differential diagnosis. In most of the cases, metastases are present at the time of diagnosis and are predominantly found in the lungs, liver, bone, lymph nodes, and central nervous system. Angiosarcoma carries a poor prognosis with a mean survival of less than a year following diagnosis. Effective treatment strategies have yet to be established.
CONCLUSION: Cardiac angiosarcoma, though a rare condition, should appear in the differential diagnosis of otherwise healthy patients presenting with hemoptysis and multiple pulmonary nodules. Optimal treatment strategies have not been developed.
DISCLOSURE: Sobia Farooq, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: We report an interesting presentation of renal leiomyosarcoma, a rare renal tumor.
CASE PRESENTATION: A 64 year-old woman presented with cough and dyspnea for 6 months. The patient donated her kidney to her daughter 10 years back, who had end-stage renal disease due to systemic lupus erythematosus. Chest radiograph of the patient was normal before donating kidney. Two years after the renal transplant, patient's daughter was found to have a lesion in the transplanted kidney during workup for proteinuria. Biopsy of the lesion showed leiomyosarcoma, which was surgically resected. Two months later, patient's daughter had recurrence of leiomyosarcoma, which responded to further surgical resection and chemotherapy. The patient denied smoking and alcohol abuse. Physical examination was normal. Routine blood tests were normal. Chest radiograph showed a right upper lobe lung mass. Computed tomography of the chest showed 3.6 × 2.5 cm mass in right upper lobe with spiculated borders. Positron emission tomography of the chest showed increased uptake in the right upper lobe lung mass. The patient underwent wedge resection of the lung mass with mediastinal lymph node dissection. Histopathology of right lung mass showed low-grade leiomyosarcoma without mediastinal lymph node involvement. A final diagnosis of primary leiomyosarcoma of the kidney with solitary lung metastasis was made.
DISCUSSIONS: Leiomyosarcoma is a rare renal tumor. Renal leiomyosarcoma, like other types of sarcomas, tend to displace rather than invade the parenchyma, and it is characterized by rapid growth rate, frequent metastasis, and high local and systemic recurrence rates. High-grade sarcomas often metastasize, the lungs being the primary site of spread, and prognosis is poor. The majority of the patients die due to the progression of the disease within a matter of months. Low-grade sarcomas tend to pursue a more indolent course, although local recurrences are common. For the renal sarcomas, the best treatment modality is radical nephrectomy. Leiomyosarcomas, even when confined to the kidney, have a poor prognosis in general with 5-year survival rates of 29–36% (1). In a case series on genitourinary leiomyosarcomas, the primary tumor site in 131 patients was the bladder in 20, the kidney in 26, paratesticular in 57, the prostate in 21 and other in 7. The most common histological subtypes were leiomyosarcoma in 29% of cases and liposarcoma in 26%. 78% of lesions were high grade (2). Sarcomas in solid organ transplant patients appear to have aggressive features with 62% being high grade and 40% metastatic at the time of primary diagnosis with a recurrence rate of 30%. The development of the leiomyosarcoma was more aggressive in the patient's daughter most likely due to her immunosuppressive therapy.
CONCLUSION: The clinical presentation of renal leiomyosarcoma in our case is interesting. It is possible that our patient had primary leiomyosarcoma of the donated kidney with micrometastases to lung 10 years back, which developed into a lesion in the donated kidney in her daughter. Slow growing low-grade leiomyosarcoma developed into a metastatic solitary lung mass 10 years later in the patient. The patient's daughter had more aggressive manifestation of leiomyosarcoma in the transplanted kidney due to immunosuppressive therapy. We are not aware of similar presentation of renal leiomyosarcoma in the medical literature.
DISCLOSURE: Bhavneesh Sharma, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Well differentiated Fetal Adenocarcinoma of the lung is an exceedingly rare form of adenocarcinoma with incidence ranging from 0.25 to 0.5% of all lung tumors. Very few cases have been reported in the literature.
CASE PRESENTATION: The patient is a 50 year old Mexican male with a past medical history of tobacco use referred to our outpatient pulmonary clinic for hemoptysis for the past month. He describes coughing one to two ounces of bright red blood approximately twice daily. There is no associated chest pain, dyspnea, fevers, chills or night sweats. The patient does however report a weight loss of approximately 20 pounds over the past 6 months. Initial PPD done at primary care physician's office was negative and chest x-ray at that time showed large left upper lobe mass with upper lobe collapse. Patient is not currently taking any medications. He has a 30 pk year smoking history and currently works odd jobs. He migrated to the US about 20 years ago and is currently married with 2 children. The patient denies any recent travel history. Upon examination in the office, vital signs were stable, patient was a febrile with an oxygen saturation of 97% on room air. Auscultation of the chest revealed decrease breath sounds on the left with good air entry on the right. A CT scan of the chest with IV contrast showed a 4.6 cm left upper lobe mass with complete collapse of the left upper lobe (fig 1). Patient subsequently underwent bronchoscopy which revealed a partially obstructing left mainstem endobronchial lesion. The lesion was biopsied and pathology revealed atypical cells suggestive of malignancy. Cardiothoracic surgery was consulted and patient underwent medastinoscopy with lymph node sampling. Pathology results revealed pulmonary adenocarcinoma of fetal type with positive inferior and superior bilateral lymph nodes. (fig 2) Subsequent testing revealed no other metastasis and the patient was staged as IIIb.
DISCUSSIONS: Well differentiated fetal adenocarcinoma is a rare lung neoplasm. Initially it was considered a "pulmonary blastoma" of monophasic type, but current WHO classification described it as an adenocarcinoma. The male to female ratio is equal and patients usually present in the 4th decade. The tumor most often less than 5 cm and thoracic adenopathy is associated with a poor prognosis. Standard treatment is surgery, but in advanced inoperable cases such as our patient, radiotherapy along with combined chemotherapy has been described. A review of the literature reveals a mean survival of 14.7 months.
CONCLUSION: Well differentiated Fetal Adenocarcinoma of the lung is a rare form of lung cancer. There are very few publications on this type of lung cancer. More research is needed identification and treatment of this malignancy. as well as diagnosing this difficult pathology.
DISCLOSURE: Maximo Lama, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Low-grade endometrial stromal sarcoma (LGESS) is an uncommon uterine neoplasm, which has a highly recurrent nature. We report a case of LGESS which presented with vaginal bleeding and incidental chest radiograph findings of multiple pulmonary nodules which on work up revealed as pulmonary metastasis of LGESS.
CASE PRESENTATION: 43 years old Caucasian female presented to her primary care physician for 6 months history of Menometrorrhagia,continuous vaginal bleeding for past 25 days, Dyspnea on exertion, excessive fatigue for 2 months and lower abdominal pain with vague lump for 3–4 months. Her past medical history was significant for DUB. She was receiving no medications. She has 30 pack year smoking history. Positive physical findings limited to significant pallor, Non tender palpable mass palpable in the hypogastric area up to the umbilicus. Significant Laboratory findings include Iron deficiency Anemia (Hemoglobin-5.4g/dl), Chest radiograph(Fig-1) showed multiple, non-calcified, variable sized, round-shaped nodules throughout both lungs. Patient was admitted to the hospital for blood transfusion due to anemia. CT Scan of Chest (Fig-2) showed bilateral numerous noncalcified nodules in lungs. The largest nodule in the left lung is about 1.5 cm. Uterus remarkably enlarged measuring 12.4 cm X 10.7 cm. Dilation and curettage was done which was negative for malignancy. PET scan was performed for the same reason and reported as, markedly enlarged uterus with a moderately hypermetabolic endometrial mass. Multiple pulmonary nodules had no significant FDG uptake to suggest an FDG-avid malignancy. Cardiothoracic surgery was consulted. Patient underwent Video Assisted Thoracoscopic Surgery with wedge biopsy of the lung nodules(Fig-3) showing metastatic LGESS. The tumor cells stained uniformly for CD10, estrogen receptor (ER), progesterone receptor (PR) and vimentin. Patient then underwent Total Abdominal Hysterectomy with Bilateral Salpingo Oophorectomy. Lower uterine segment was involved by the tumor. Histopathology showed endometrial stromal cells with no mitotic activity (characteristic feature of low grade). Neoplastic cells are focally disposed in a whorled pattern around numerous arterioles. Tumor growth was observed in the myometrial veins and in lymphatics. Diagnosis of LGESS was confirmed and tumor was staged as IV-B LGESS. Patient was started on hormonal therapy (progestin) after TAH.
DISCUSSIONS: The etiology of multiple pulmonary nodules is broad, with metastatic disease being the most common. Metastatic ESS should be included in the differential diagnosis of nonepithelial neoplasms in women. ESS represents 0.2% of all uterine malignancies and up to 25% of primary uterine sarcomas. Histologically, they can be divided into High & Low grades based on their mitotic count (more or less than 10 mitoses per 10 high power fields, respectively). The microscopic appearance of LGESS is characteristic with sheets of densely packed, uniform, small spindle or ovoid cells, often with prominent hyalinization within stromal vessels. The tumor also has a tendency to extend into blood vessels and lymphatic's and may be expressed as worm like masses(1). Lung metastases occur commonly and have been noted at the initial diagnosis of ESS as well as many years even after the removal of the primary. LGESS has a relatively indolent clinical course with a 5-year survival of approximately 60%. Favorable prognostic characteristics include early tumor stage, low myometrial invasion and low mitotic rate. Treatment options include hysterectomy for removal of primary and hormonal therapy for metastasis. Progestins have been the most widely used agents aiming at the inhibition of estrogen-induced growth promotion(2).
CONCLUSION: This case report is a perfect example of common clinical presentation of a rare and indolent tumour, LGESS which often metastasis to the lungs. Due to the rarity of the presentation, the condition is often missed or diagnosed late.
DISCLOSURE: Srikanth Davuluri, None.
INTRODUCTION: Pulmonary metastases of prostate adenocarcinoma have been well described, but endobronchial lesions are rare. Although neuroendocrine differentiation of prostate cancer is common, it is rarely seen in the context of pulmonary metastases. We present a case of metastatic prostate cancer initially presenting as bronchial carcinoid.
CASE PRESENTATION: A 73 year old East Indian man was referred for assessment of an abnormal chest x-ray. Past medical history was significant for type II diabetes and stage T3c prostate cancer 10 years earlier, treated with radiation and hormone therapy. Recurrences 4 years and 2 years after the initial diagnosis were treated with orchidectomy and flutamide therapy, respectively. There was no smoking history or known asbestos exposure. He had recently traveled to India at which time he developed an illness consisting of dyspnea, fevers, positional chest pain, fatigue, anorexia associated with a 35 lbs weight loss over 3–6 months and a right-sided pleural effusion. There was no history of cough or wheeze, but he did have 2 episodes of hemoptysis. While in India, thoracentesis demonstrated a sterile exudate and he received 6 months of therapy for presumed pleural tuberculosis. Other results from India were unavailable. After returning to Canada, chest x-ray showed a persistent pleural effusion and thickening with associated volume loss. Pulmonary function testing revealed a restrictive process. A repeat attempt at thoracentesis was unsuccessful. Sputum for acid fast bacilli was negative. Computed tomography of the chest demonstrated a right hilar mass (4.4 × 4.3 cm), pleural thickening contiguous with the mass, subcarinal lymph nodes and a small right pleural effusion. Bronchoscopy revealed a smooth, shiny right middle lobe endobronchial mass. Brushings and biopsies of the mass suggested typical carcinoid based on histologic morphology and positive chromogranin and synaptophysin staining. Because the clinical picture was inconsistent with primary pulmonary carcinoid, a pleural biopsy was subsequently performed. The biopsy was weakly positive for prostate specific antigen (PSA) and strongly positive for alpha-methylacyl-CoA racemase (AMACR), consistent with metastatic prostate adenocarcinoma. The carcinoid samples were negative for prostate specific markers. Serum PSA was elevated at 133.6 and bone scan revealed bony metastases.
DISCUSSIONS: Approximately 5% of patients with prostate cancer have radiologic evidence of pulmonary metastases at diagnosis, but clinical detection of lung metastases is infrequent, as the majority of patients remain asymptomatic from pulmonary lesions. Focal or extensive histologic neuroendocrine differentiation of prostate cancer can be seen in up to 50% of prostate tumors by immunohistochemistry, and generally portends a poor prognosis and response to therapy. There are at least three reported cases in the literature of metastatic prostate cancer mimicking pulmonary carcinoid tumors (1). It has been suggested that long-term exposure to hormonal therapy can select for neuroendocrine differentiation in both primary and metastatic tumor deposits. The carcinoid mass in this case did not stain for prostate specific markers, consistent with the possibility of a synchronous primary of lung origin. Indeed, epidemiologic studies have also suggested an almost three-fold higher than expected incidence of prostate cancer in patients diagnosed with primary pulmonary carcinoid tumors (2). The reason for this association is unclear, but may relate to an underlying genetic tendency toward the development of endocrinologic tumors or to unidentified environmental or hormonal factors.
CONCLUSION: This case highlights the importance of being aware of the potential for prostate cancer to undergo neuroendocrine differentiation and the association of pulmonary carcinoid tumors with prostate cancer.
DISCLOSURE: Mitesh Thakrar, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Pulmonary synovial sarcomas are extremely rare tumors with no predilection for tumor location, usually affecting young males. Their clinical prognosis is largely unknown. We describe the first case of primary synovial sarcoma of the lung with direct, intracardiac extension.
CASE PRESENTATION: A 53 y/o WM treated with mantle radiation for Hodgkin's Lymphoma 13 years ago presented with 6 weeks of nonproductive cough. He denied dyspnea and ran 3 miles on a treadmill daily without limitation. He denied chest pain, palpitations, orthopnea, fevers, chills or gastroesophageal reflux symptoms. Past medical history was significant for only Hodgkin's lymphoma. He never smoked, and denied alcohol or illicit drug use. Review of systems was unremarkable. Vital signs and physical examination were notable for diminished breath sounds in the right upper chest. Heart exam was normal. Chemistries and CBC were unremarkable. Echocardiogram revealed a normal ejection fraction and a large left atrial mass 4.4 × 2.6 cm originating from the right superior pulmonary vein and appeared to traverse the mitral valve with moderate mitral regurgitation. PFTs showed mild restriction with a normal diffusing capacity. Chest X-ray demonstrated a 10 X 9 cm large round opacity in the right upper lobe and anterior mediastinum. MRI demonstrated a large mass in the right upper lobe with invasion of the right superior pulmonary vein and left atrium. The patient subsequently underwent a right pneumonectomy and primary resection of the left atrial and pulmonary vein invasion. On gross examination of the right lung, there was a well circumscribed tan white soft mass (8 X 8 X 7.5 cm) with focal areas of necrosis. The intracardiac mass was oval shaped, nodular tissue with a smooth surface (7 X 3 X 2 cm) weighing 28 grams. Microscopically, it was monophasic pulmonary synovial sarcoma which stained positive for vimentin, BCL-2 and epithelial membrane antigen. Molecular studies performed on submitted tissue were positive for the SYT-SSX gene.
DISCUSSIONS: Synovial sarcomas are soft tissue tumors of uncertain histogenesis, usually arising in the vicinity of large joints. Rarely, they have been described in the head/neck, chest and abdomen. Pulmonary sarcomas are rare (<0.5% of lung tumors) and are usually metastases from other primaries (leiomyosarcomas, fibrosarcomas and heamangiopericytomas). Usually, they present as a large (2–15 cms) well defined mass in an asymptomatic patient found on routine X-ray. About 60 cases have been reported so far in literature and only 11 have been confirmed by the definitive molecular detection of SYT-SSX fusion gene transcription. T(X;18)(p11.2;q11.2) translocation results from fusion of SYT gene on Chr 18 to either SSX1 or SSX2 on Chr X. SYT-SSX1 has a worse prognosis with a 5 yr survival rate of 42 vs. 89% (for SSX2). Histologically, they are classified into four subtypes: biphasic, monophasic fibrous, monophasic epithelial, and poorly differentiated. These tumors stain frequently for epithelial markers such as cytokeratin and/or epithelial membrane antigen; vimentin and CD99. Diagnosis can be made on clinical, histological and immunohistochemical evaluation, and molecular testing is not required in all cases. Clinical course is largely unknown with a reported mortality rate up to 55% in a large case series of 25 patients. Intracardiac extension has not been previously reported. Our patient had successful extraction of the intracardiac mass with right pneumonectomy, and remains in clinical remission 6 months post-operatively.
CONCLUSION: This is the first described case of primary synovial sarcoma of the lung with an intracardiac extension. Since prior reported cases showed no particular predilection of tumor location, we suggest cardiac evaluation for synovial sarcomas with mediastinal involvement.
DISCLOSURE: Veena Devarakonda, None.
INTRODUCTION: Respiratory failure (RF) with recurrent pneumonia in the ICU can have devastating consequences for the patient. Search for an underlying cause should always prompt thinking of the common and then not so common causes, especially in the post operative thorasic patient. Known complications of esophagectomy can include chylothorax and bronchopleural fistula (BPF). Having both complications in the same patient leading to recurrent aspiration of the chyle into the bronchial tree, recurrent pneumonia and persistent RF is very rare.
CASE PRESENTATION: A 65 year old man with a history of hypertension, was suffering from dysphagia secondary to a fungating growth in the distal esophagus extending into the gastroesophageal junction. Pathology revealed poorly differentiated adenocarcinoma on biopsy, with no metastasis. Preoperatively, the patient received neoadjuvant treatment consisting of chemotherapy, and external beam radiation. Ivor-Lewis esophagectomy with split-stomach fundoplication was performed.His post-operative course was complicated by fever, persistent right-sided pleural effusion, and pulmonary infiltrates on chest radiography and computed tomography. RF persisted and mechanical ventilation (MV) support was required. Multiple tube thoracostomies were performed for the persistent pleural effusion. Bronchoscopy was performed, which revealed two small-sized BPF. The larger fistula, measuring 4–5 mm in diameter, located on the posterior wall of the proximal left mainstem bronchus. The smaller fistula measured 1–2 mm in size and was located proximal to the larger lesion. Significant secretions with active influx of fluid from the pleural space into the tracheobronchial tree through the fistulas were found. Comparative analysis of bronchial wash and pleural effusion showed similar analysis. Rigid bronchoscopy with contrast bronchography confirmed the communicating BPF. Fibrin glue was injected into the lesions and subsequently an ultraflex covered stent was placed in the left mainstem bronchus. Follow up bronchoscopy two weeks later revealed that the fistulas were enlarging. A larger stent was then placed. Repeat bronchoscopy two weeks later established stability of the stent with appropriate healing of fistulas.
DISCUSSIONS: BPF and chylothorax are known complications of esophagectomy. Having both in the same patient can lead to devastating consequences. Recurrent pneumonia with persistent RF and difficulty weaning from MV was the initial challenge in this patient, which prompted further investigations.BPF is a relatively rare complication, with significant morbidity and mortality. Different etiologies include infection, malignancy, trauma, and adult respiratory distress syndrome. Most commonly BPF results from operative complication following pulmonary resection. Treatment of BPF can be surgical or medical. Surgical options include direct stump closure with muscle or omental flap, thoracoplasty, or chronic open drainage. Bronchoscopy with use of sealants and/or a mechanical occlusion device is the hallmark of medical therapy especially for high risk or debilitated patients. Various different sealants can be used. Mechanical occlusion, with stents or coils, has been used either alone or in conjunction with sealants to achieve closure. BPFs > 8 mm are not appropriate for endoscopic management, those ≤;1 mm have the best success rate. High-output chylothorax may lead to life-threatening complications such as respiratory failure, immune suppression, malnutrition, and nosocomial infections. Non-operative management of chylothorax includes fat-free diet with medium-chain triglycerides to decrease the output, drainage of the effusion via tube thoracostomy, enteric rest with total parental nutrition, aggressive diuresis, and somatostatin or octreotide injections. Surgical management includes thoracic duct repair or ligation via an open or thorascopic approach, fibrin glue application, pleurodesis, pleuroperitoneal shunting, and percutaneous embolization of the thoracic duct.
CONCLUSION: BPF, a potentially treatable condition, should be suspected and investigated in the presence of chylothorax with persistent RF and pneumonia.
DISCLOSURE: Hamed Mataria, None.
INTRODUCTION: Self-Expanding Metallic Stents (SEMS) are an increasingly common pulmonary procedure due to their easy flexible bronchoscopic insertion. Airway stenting offers a valuable tool for treatment of Central Airway Obstruction (CAO) secondary to malignant or benign disease. However, in patients with benign airway disease, stent complications are upward to 50%, prompting a 2005 FDA advisory emphasizing patient selection criteria. Injudicious use in non-malignant disease increases the frequency of severe early and late complications due to strut fracture, mucosal erosion, migration, or granulation tissue stenosis –requiring urgent interventional trained pulmonary expertise in SEMS extraction. In this case, we report on the inappropriate deployment and life-threatening complication of a SEMS for benign trachealmalacia and the multi-disciplinary experience of its endoscopic removal.
CASE PRESENTATION: This 69 year old male from Louisiana has "newly diagnosed" asthma with frequent intermittent wheezing symptoms for many years. Since 2001, progressive unexplained dyspnea is now limiting his activities and after a hospitalization for pneumonia an outpatient bronchoscopy shows tracheamalacia with mild tracheal stenosis. In 2004, recurrent pneumonias prompt a repeat bronchoscopy with deployment of a SEMS into the involved tracheal area. The patient symptoms improve and no further pulmonary follow-up occurs until late 2007. At this time, the patient suffers a mild acute ischemic stroke and respiratory distress while traveling in Colorado. A chest CT scan rules out pulmonary embolism but notes "airway abnormalities." The patient is stabilized and returns to Louisiana. Referral to our interventional pulmonary program finds a patient with monophonic wheezes over the anterior chest with imaging evidence of a SEMS in the right main stem bronchus and soft tissue partially filling the stent, critically occluding the airway. Rigid bronchoscopy is performed the next day in coordination with a multi-disciplinary team including Otolaryngology and Thoracic surgeons for endoscopic SEMS extraction. The migrated, fractured, and severely granulated SEMS proves difficult to remove requiring forceful traction, bracing of the airway wall with the rigid scope, and intentional fracturing of the well-embedded barbs for piecemeal removal over several hours. Thoracotomy, loss of airway, or death does not occur, but the patient requires a temporary custom-sized silicone Y-stent post-operatively. With close outpatient follow-up, the patient has minimal airway symptoms and currently remains stent free.
DISCUSSIONS: Although the SEMS manufacturer recommends open surgical removal, an endoscopic approach is achievable only when performed under the auspices of a multi-disciplinary airway center of excellence. Life-threatening complications are real. Our SEMS extraction experience eerily matches that described by others as "like rolling spaghetti on a fork, but much more difficult and at least equally as messy." Indications for stent removal include: high-grade obstructing granulation tissue with strut fracture and/or mucus retention, stent migration, mucus plugging, stent infection, and strut fracture with associated pain. Mean hospital duration post stent removal is 8 days. Retained stent pieces, mucosal tears with bleeding, reobstruction requiring silicone stenting, prolonged mechanical ventilation, and pneumothoraxes are the most common complications. Metallic stents are not considered first-line therapy for benign airway obstruction. In one study, 60% of patients did not require further intervention after stent extraction, implying an initial false need for SEMS deployment. Finally, primary surgical airway reconstruction as an alternative is usually not available after SEMS placement and subsequent extraction.
CONCLUSION: The FDA advisory highlights the limited role, if any, for SEMS in benign airway pathology. Besides the inherent long-term risks associated with SEMS, this case emphasizes the labor intensive, multi-disciplinary approach required for a high-risk endoscopic procedure to be successful. A scenario likely never anticipated at time of initial SEMS deployment.
DISCLOSURE: Michael Czarnecki, None.
INTRODUCTION: Pneumonectomy has been called a "disease unto itself," and is associated with significant morbidity and mortality. Many complications occur early, but long-term sequelae are also possible. Proper surgical technique remains an important factor in outcomes after pneumonectomy.
CASE PRESENTATION: 68-year-old man underwent a left pneumonectomy 7 years previously, at another institution, for a T2 N0 M0 squamous cell carcinoma. Two years after resection, a chest x-ray demonstrated an air-fluid level in a previously opacified hemithorax. During the past year, he developed a productive cough with a scant amount of blood. Initial treatment with antibiotics prior to referral was unsuccessful in improving his symptoms. He was referred to our institution where a work-up for presumed bronchopleural fistula (BPF) was initiated. Computed tomography scan demonstrated an air-fluid level in the left pleural space and suggested an elongated bronchial stump. Bronchoscopy showed a 4 cm left mainstem bronchial stump without evidence of a frank BPF or tumor recurrence.We performed a left thoracotomy. Air bubbles emanating from the mediastinum on positive pressure ventilation confirmed the diagnosis of BPF and assisted in guiding dissection of the left bronchial stump in the scarred mediastinum. Reresection of the left bronchus was carried back to a level flush with the carina. The new left bronchial stump was covered by a pedicled ipsilateral serratus anterior muscle flap. The chest was initially packed open due to the chronic pleural space infection. It was ultimately closed 2 weeks later using the modified Clagett technique.
DISCUSSIONS: The clinical scenario of cough and air-fluid level in the post-pneumonectomy space heralds the presence of a BPF. Despite the lack of overt bronchoscopic findings, the clinical suspicion was sufficiently high to proceed to thoracotomy for definitive surgical repair. Long bronchial stump syndrome occurs when redundant main-stem bronchus remains after pneumonectomy. Often, this occurs when the pneumonectomy is performed as a series of lobectomies, instead of at the hilum. The stump acts as a reservoir for secretions and a nidus for infection leading to complications including BPF and empyema.
CONCLUSION: Complications following pneumonectomy may occur years later. Meticulous surgical technique along with appropriate postoperative care can reduce the rate of morbidity. Long-bronchial stump syndrome can be prevented by transecting the bronchus flush with the carina.
DISCLOSURE: Jess Thompson, None.
INTRODUCTION: An elderly man with decreased mental status aspirated a 28 French Nasopharyngeal Airway (NPA) over a suction catheter and developed inspiratory stridor. This is the only documented video of NPA aspiration and its immediate management. This case highlights an uncommon complication of a commonly used device. NPA aspiration was first documented in 1985 and remains an important consideration for patient safety.
CASE PRESENTATION: An 84 year-old man with GCS 5 had a 28 French Robertazzi NPA (Rusch) lubricated with Surgilube placed. His nurse passed a 14 French Medline Suction Catheter via the NPA. Once the catheter was inserted to a depth of 13–15 cm, the nurse applied suction and turned to view the monitor. Upon turning back to view the patient, the nasal airway was missing, with the suction catheter still in place at the same depth. Vitals signs were unchanged. Inspiratory stridor developed when suction catheter was removed. The attending arrived within 5 minutes. The oropharynx was evaluated with a flashlight, tongue depressor, and then a laryngoscope with a Macintosh #3 blade. Bloody secretions were present with no gag reflex. An intubating fiberoptic scope, Video Cart, and a tongue retractor revealed a grade one view with no gag, no cough, and image 1. Video shows the NPA flange above the glottis, with inspiration causing inward bowing of the flange, slight inward migration, with concurrent inspiratory stridor. The tip of the NPA was in the trachea. Direct laryngoscopy was performed with a Macintosh blade #4 video laryngoscope providing a grade one view. The NPA was removed with forceps. The stridor resolved immediately.
DISCUSSIONS: The first documented NPA aspiration was in 1985 by Hayes et al with flange located 4cm below glottis and tip more distal, requiring rigid bronchoscopy for removal. Milam et al documented a patient in whom the NPA "disappeared" with no change in respiratory status or chest film. Autopsy revealed distal tip of NPA at carina and flange at level of larynx. Ho et al documented an NPA aspiration while the nurse advanced the suction catheter via the NPA with a "snug fit". The NPA was retrieved with a hemostat under direct laryngoscopy after aerosolized lidocaine and intravenous midazolam. Yokoyama et al described a patient with an NPA slipping "through his nasal cavity into his trachea" during pharyngeal suctioning. Despite no radio-opaque stripe the NPA was seen in the trachea and projecting into the bronchus intermedius on chest film. A general anesthetic was given to remove the NPA with Jackson's laryngeal scope and forceps. Our case describes a similar clinical scenario of an elderly male with declining mental status, poor gag/cough reflexes, and standard placement of a common NPA. It is interesting that the pharyngeal suction catheter appears to have acted as a slide directing the NPA directly into the larynx. This is the first video documentation reported of a NPA aspiration, with collapsing flange causing stridor, and its immediate removal. Complications of aspiration of an NPA may include non-recognition of the aspiration, sub-mucosal tunneling, vomiting, airway obstruction, laryngospasm, atelectasis, infection, and airway trauma/bleeding; associated increased work of breathing, hypoxemia, and death.
CONCLUSION: In our institution, 446 of this type of NPA were utilized in 2007. Our department is investigating other reports of NPA aspiration. The aspiration of a commonly used NPA is a significant issue for patient safety with no intervention despite 23 years of literature describing this complication. These cases are an indication for re-evaluation of NPA design and use, as the original design did not intend for this device to be a conduit for suction catheters and has flawed construction for such use.
DISCLOSURE: Carlos Brun, None.
INTRODUCTION: We present a case of a patient who developed an acute Lobar Torsion within hours of undergoing a Double Lung transplant.
CASE PRESENTATION: A 65 year old female underwent Double Lung transplant for IPF - with Ischaemic times of 4 hours for the Left lung and 5 hours for the right lung. After the surgery, the patient was moved in a stable condition to the ICU. 4 hours later, the Transplant Fellow was called in to see the patient because of increasing Oxygen requirements. The patient had decreased Breath sounds in the Left Upper lobe on exam and had O2 Saturation of 93% on a FiO2 of 100%. Her Pulmonary Artery Pressures were 67/20. A stat CXR was ordered - that showed evidence of a dense opacity in the Left Upper Lobe. A hematoma was suspected and a Bronchoscopy done immediately. It revealed that the Left Main Bronchus was inaccessible with a fish mouth appearance. The patient was immediately rushed back into the OR and the torsion was corrected. Post surgery, the patient did well and the CXray cleared up in 2 days time - showing that a Pulmonary Infarction, which could have been disastrous in this setting, had been prevented by the speedy intervention.
DISCUSSIONS: Acute Lobar Torsion is a rare but serious complication after a Transplant - with very few cases reported in the literature. As the tracheobronchial tree is twisted in lung torsion, compromise of the pulmonary arterial, venous, and bronchial circulation develops. It is assumed that the risk of Lobar torsion is higher after a Transplant because of the division of the Pulmonary ligament of the donor lung and the size difference between the donor and recepient chest cavity that could potentiate torsion of the donor lung. High Clinical suspicion and prompt diagnosis by Bronchoscopy are essential to intervene early in order to prevent Pulmonary Infarction. The treatment is Surgical Detorsion or removal of the affected Lobe.
CONCLUSION: The possibility of acute lobar torsion should be considered in lung transplant recipients who demonstrate evidence of acute respiratory insufficiency in the early post-operative period.
DISCLOSURE: Vinu Abraham, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: The United States Consumer Product Safety Commission recalled Stand N' Seal "Spray-On" Grout Sealer after 88 reports of adverse reactions after using the aerosolized sealant. 28 individuals sought medical attention for respiratory symptoms with 13 individuals requiring medical treatment. This case documents the clinical course of a patient from the time of exposure to this aerosolized sealant up to the diagnosis of Reactive Airways Dysfunction Syndrome (RADS).
CASE PRESENTATION: A 43 year old male smoker with no subjective or objective evidence of pulmonary disease or atopy used Stand N' Seal "Spray-On" Grout Sealer in an enclosed room within his house. Within a few hours of exposure to this aerosolized sealant he developed severe cough and shortness of breath. By the time the patient reached the Emergency Department he was found to be tachypneic, tachycardic, and hypoxic. The patient's initial chest x-ray revealed bilateral perihilar and infrahilar airspace disease. He was treated with albuterol and ipratropium nebulization, intravenous methylprednisolone, and then transferred to the Intensive Care Unit as he was still requiring 100% FiO2 to keep his oxygen saturation barely above 90% on arterial blood gas. While hospitalized, he was treated with oxygen, albuterol nebulization, and oral dexamethasone 10mg every 8 hours. Upon discharge the patient did not require oxygen, he had improvement in his respiratory symptoms, and was instructed to take a 10 day course of oral prednisone. The patient followed in the Pulmonary Clinic one week after discharge. He noted definitive improvement but continued to have intermittent wheezing and chest tightness which improved with the use of his albuterol inhaler. He had no tobacco use since his hospitalization. A repeat chest x-ray was normal. Spirometry done showed a forced vital capacity (FVC) of 3.13 liters (64% of predicted), forced expiratory volume in one second (FEV1) of 2.42 liters (62% of predicted), and a normal ratio of the FEV1/FVC, consistent with restrictive physiology. He subsequently had full pulmonary function tests which, in addition to a restrictive physiology, revealed a significant bronchodilator response (320 ml and 13% improvement in FEV1) and a reduced single-breath diffusion capacity for carbon monoxide of 31.5 ml/mmHg/min (68% of predicted). The patient returned for follow-up one month later with complaints of intermittent cough and shortness of breath in response to strong odors, fumes, cold air, and exertion. He had remained tobacco free. He then underwent cardiopulmonary exercise testing which showed no ventilatory mechanical limitation, gas exchange abnormality, or diffusion impairment. Sixteen weeks after the initial exposure, the patient had a methacholine challenge test and his FEV1 decreased from 2.80 liters to 2.19 liters (22% reduction) following the administration of methacholine at a concentration of 1 mg/ml. Given these findings, the patient was started on inhaled fluticasone/salmeterol 500/50mcg and diagnosed with RADS.
DISCUSSIONS: The risk of developing RADS after an inhalational exposure to a toxic substance has been difficult to quantify. This patient fulfills the seven diagnostic criteria for RADS. To our knowledge, none of the reported patients who were exposed to Stand N' Seal "Spray-On" Grout Sealer have official documentation of the development of RADS.
CONCLUSION: The case emphasizes the importance that physicians must recognize that inhalation exposures to toxic substances within the home, not just the workplace, can lead to the development of RADS.
DISCLOSURE: Amanda Godfrey, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Cavitary degeneration of pulmonary malignancy is well described in the literature. Cystic presentation of malignancy is less common. Here, we present a case of a giant intrathoracic cyst determined to be a de novo presentation of an adenocarcinoma.
CASE PRESENTATION: A 78 year-old Vietnamese man with past history of tuberculosis, and parasitic liver abscess (E. histolytica) presented with complaints of right leg swelling and dry cough. He denied chest pain, fever, or significant dyspnea, but did admit to intermittent streaky hemoptysis. He was a 60 pack-year smoker. There was no significant social, family, occupational, or exposure history. Physical examination revealed an oxygen saturation of 93% on room air with stable vital signs. There was no digital clubbing, cyanosis, or tracheal deviation. The lung fields revealed scant bilateral basal crackles. The right leg was visibly swollen. A venous ultrasound confirmed an above knee deep venous thrombosis in the right leg. A chest film revealed a large multilobulated smooth-edged opacity with mass effect in the left hemithorax. Chest films from ten years previously were reported as normal. Contrast-enhanced computed tomography (CT) of the chest revealed a thin-walled 21 cm by 11cm by 10 cm cystic mass in the medial left hemithorax without associated adenopathy or apparent solid component. Initial diagnostic considerations included congenital cysts, hydatid (ecchinoccal) cyst, intrathoracic pancreatic pseudocyst, cystic tuberculoma, and malignancy. Echinococcal serology was negative. Chest MRI confirmed the cystic nature of the lesion but demonstrated fine septations not seen on CT, suggesting a complex nature to the cyst. Mycobacterial smears and cultures obtained during fiberoptic bronchoscopy were negative. Bronchoscopy was unremarkable aside from a small region of mildly edematous bronchial mucosa located at the orifice to the left upper lobe. Mucosal biopsies taken from this site were consistent with adenocarcinoma. However, given that the appearance of the large cystic mass was atypical for malignancy, cyst aspiration and positron emission tomography (PET) scanning were arranged. PET revealed heterogenous uptake of the entire cystic lesion, as well as focal uptake localized to mediastinal lymph nodes and lumbar spine, suggestive of metastatic disease. Transthoracic fine-needle aspiration of the cyst yielded bloody fluid containing abnormal cells consistent with adenocarcinoma. Post aspiration of the cyst, a previously unidentified solid component was seen and biopsied and showed non-mucinous adenocarcinoma. Several days post aspiration, prior to initiation of treatment, the patient's clinical condition declined and he died.
DISCUSSIONS: This case represents only the third described case in the English literature of non-mucinous pulmonary adenocarcinoma presenting as a cystic chest lesion.1 It is the first case to describe such a large and thin-walled lesion. The two previous cases of cystic adenocarcinoma describe complex cysts with visible septations and walls composed of malignant cells. In the present case, however, the septations were not apparent on contrast-enhanced CT, and the solid component was not appreciated until the cyst had been aspirated. Cystic change is less common than cavitary degeneration but has been described in bronchoalveolar carcinoma, pulmonary mucinous cystadenocarcinoma, chondrosarcoma, and adenocarcinoma. It has been hypothesized that cystic adenocarcinoma may develop if malignant cells extend along the walls of alveoli, permitting cyst formation and enlargement without the more common central necrosis that is often seen in the setting of large pulmonary malignancies.
CONCLUSION: Lung cancer can present de novo as a cystic chest lesion and should be included in the differential diagnosis of even simple appearing cystic chest lesions.
DISCLOSURE: Paul Heffernan, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Traumatic pulmonary pseudocyst (TPP) is an uncommon manifestation of blunt chest trauma in young adults. This should be considered in the differential diagnosis in patients sustaining blunt chest trauma.
CASE PRESENTATION: A healthy 27 year old man was seen after blunt trauma to his right chest while playing rugby. He was complaining of right lower chest pain and some streaky hemoptysis. There was no history of dyspnea, fever, chills, nightsweats. He had no prior pulmonary disease. He was not on any medication and denied alcohol abuse or use of recreational drugs. On examination he was comfortable at rest with normal vital signs. There was no lymphadenopathy or clubbing of fingers. There was mild chest wall tenderness on the lateral lower right chest. Chest was clear to auscultation. A chest radiograph was unremarkable and did not show rib fractures. A chest CT scan was obtained. Figure 1 shows areas of pulmonary contusion on the affected side of the right chest. Figure 2 shows a pulmonary pseudocyst on the right lower lung.
DISCUSSIONS: TPP manifests within the first 24 hours after blunt chest trauma in the majority of cases or up to fourteen days post-injury. Symptoms are non-specific and include cough, chest pain, hemoptysis, and shortness of breath. Physical examination findings are unremarkable and are usually restricted to crackles on the affected chest. Most reported cases of TPPs have concurrent lung injuries such as pulmonary contusion, hematoma, hemopneumothorax, and pneumothorax.1 Chest radiographs have a diagnostic yield ranging from 24–50% whereas chest CT scans can have a yield of 100%. TPPs, which may be uni- or bilateral, are usually subpleural lesions commonly affecting the lower lobes. Contused pulmonary parenchyma surrounds these thin, irregularly walled lesions, which lack epithelial lining. TPPs may contain air-fluid levels that result from bleeding from the surrounding vessels.1TPP can occur at almost any age group but majority of cases are 30 years old or younger. When a young adult gets involved in a blunt trauma, compression of an elastic thorax with resultant negative intrathoracic pressure on chest recoil causes parenchymal laceration, and because of the normal elastic recoil of the lung, air and fluid escape into the contusion-induced cavities. In younger patients, the greater compliance of the chest wall allows better transmission of the force of impact to the lung, and presumably is responsible for the higher frequency of this lesion in young adults.2 TPPs usually follow a benign course. Pseudocysts change in size and shape and may become larger during the first two weeks of observation. They eventually get smaller until complete resolution in 1–6 months.2 For the most part, pseudocyts are treated conservatively. For uncomplicated cases, serial chest X-rays are sufficient until resolution of the cysts, unless the patient has persistent fever or leukocytosis suggesting super-infection. Small infected cysts may respond to antibiotics alone or may require CT–guided percutaneous drainage if with clinical deterioration. Surgical resection is recommended if the infected cysts are more than 6 cm or if with massive pulmonary hemorrhage.1 TPPs usually have an excellent prognosis since majority of the cases are uncomplicated.
CONCLUSION: TPP is an uncommon manifestation seen in young adults after blunt chest trauma. It generally follows a benign clinical course but complications necessitating antibiotics and surgical drainage may arise.
DISCLOSURE: Jose Angelo De Dios, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Mounier-Kuhn syndrome (MKS) is a rare disorder of the central airways with less than 100 reported cases. We report a patient with MKS infected with multiple nontuberculous mycobacteria.
CASE PRESENTATION: A 48-year-old homeless man with a 15-pack year smoking history and diabetes mellitus presented with the complaint of dyspnea and cough for two weeks. The patient had a history of recurrent pulmonary infections and previous hospitalizations for pneumonia. Chest X-ray revealed right apical bullae with bilateral interstitial changes and bronchiectasis mostly in the upper lung zones. Given his clinical symptoms and his social circumstances, the patient was assessed for active tuberculosis. Induced sputum was positive for acid-fast bacilli and rifampin, isoniazid, pyrazinamide and ethambutol were started. The patient was HIV-negative. Chest CT showed enlarged central airways with a tracheal diameter of 3.6 cm. The right main bronchus measured 2.5 cm and left main bronchus was 2.3 cm. Central and peripheral bronchiectasis, bilateral nodules and bilateral scattered bullae were also noted. Sputum cultures repeatedly grew M. kansasii, M. fortuitum and M. avium intracellulare complex. M. tuberculosis was not cultured and sputum PCR was negative. Azithromycin was replaced with pyrazinamide with the plan to treat until he was culture negative for one year.
DISCUSSIONS: First described in 1932, Mounier-Kuhn syndrome is characterized by enlargement of the trachea and central bronchi with recurrent respiratory tract infections. Pathologically, there is loss or atrophy of the longitudinal elastic fibers and muscularis mucosa in the trachea and bronchi leading to dilatation and increased compliance of the airways. The etiology of MKS is unknown with the majority of cases reported in adulthood. The diagnosis of MKS is made when the diameter of the trachea is greater than 3.0 cm and right and left main stem bronchi are larger than 2.4 and 2.3 cm respectively. Three sub-types are described based on the configuration of the central airways. Our patient fits into the first category typified by symmetrical enlargement of the trachea and main bronchi. Patients with MKS often present with recurrent pulmonary infections. Collapsibility of the airways, especially during expiration, can lead to retained secretions, chronic infections, bronchiectasis and pulmonary fibrosis. We believe that this mechanism explains the etiology of our patient's repeated infections. Nontuberculous mycobacteria are reported with increased frequency in patients with underlying lung disease. It is not surprising that our patient was infected with mycobacteria as patients with COPD and bronchiectasis are often colonized. It is unusual, however, to be infected with multiple mycobacterial species as demonstrated by repeated positive cultures. Patients with these infections often present with symptoms of their underlying disease. To make the diagnosis, the patient needs to have clinical and radiographic findings consistent with mycobacterial lung disease along with two sputum samples, bronchoalveolar lavage or biopsy cultures showing the organism. Our patient had multiple sputum samples that grew M. kansasii, M. fortuitum and M. avium intracellulare with a chest CT showing nodules and bronchiectasis suggesting true infection rather than colonization.
CONCLUSION: MKS should be in the differential in patients with recurrent pulmonary infections. The diagnosis can be made by chest CT. Although nontuberculous mycobacterial infections are often seen in patients with bronchiectasis this is the first reported case in which a patient with MKS was infected with multiple mycobacteria. It is important that patients with MKS be evaluated for these organisms, as it may be an explanation for worsening symptoms.
DISCLOSURE: Santhi Iyer, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: We present a case of a 48 year old Egyptian-born woman with initial presentation of persistent malaise, fever and an intermittent non-productive cough of 2 years duration.
CASE PRESENTATION: The patient's past medical history is significant only for a cholecystectomy with no chronic use of medications or allergies. She is a lifelong non-smoker and alcohol drinker. She emigrated from Egypt to Ohio, where she worked as an epidemiologist but no known infectious or environmental exposures. Due to the persistence of her symptoms, a chest x-ray and subsequent CAT Scan (CT) was obtained which revealed diffuse cervical, hilar and abdominal adenopathy and no pulmonary parenchymal disease. A fine needle aspiration (FNA) of the cervical lymph node revealed granulomatous adenitis with evidence of necrosis. She was eventually started on corticosteroid therapy for presumptive diagnosis of sarcoidosis (prednisone 30mg daily for two months) but therapy was halted as she had interval increase in the size of her cervical nodes. She was referred to our center where she had a biopsy of the left axillary lymph node (which was positron emission tomography (PET) positive) revealing non-caseating granulomas. The pathology obtained from a subsequent mediastinoscopy revealed granulomas with focal caseating necrosis, with negative work up for infectious etiology, including acid fast bacteria (AFB). Nearly one year after her initial symptoms, she continued to have diffuse increase in her lymphadenopathy and symptomatic hepatosplenomegaly. After a negative bone marrow biopsy, she later underwent splenectomy for a rapidly enlarging spleen for suspicion of occult lymphoma. The spleen pathology revealed focally necrotizing granulomatous splenitis, with negative stains for AFB and fungus. These results were confirmed with negative polymerase chain reaction (PCR) for mycobacterial DNA as well as negative flow cytometry. She subsequently underwent an extensive infectious disease evaluation which was negative for brucellosis, histoplasmosis, schistosomiasis, bartonella and Q fever. Her rheumatologic evaluation was also non-diagnostic which included normal values for anti-nuclear antibody (ANA), double stranded DNA (dsDNA), and rheumatoid factor. Other laboratory values revealed a normal serum angiotensin converting enzyme (ACE), albumin, and gamma globulin levels but mildly elevated alkaline phosphatase.
DISCUSSIONS: After her extensive workup, the patient was given a presumptive diagnosis of sarcoidosis with features of necrotizing sarcoid granulomatosis (NSG) predominantly involving the lymphatic system. NSG was first described by Liebow in 1973, and currently defined as involvement of the bronchovascular bundle with vasculitis and parenchymal necrosis (usually of the ischemic type) 1. It is generally considered a variant of nodular sarcoid and thus a diagnosis of exclusion. Clinically patients present with fevers, malaise, dyspnea with multiple pulmonary nodules2. However, unlike the classic presentation of NSG, our patient did not have pulmonary infiltrates and was essentially asymptomatic from a pulmonary standpoint with the exception of an intermittent cough. Furthermore, her imaging was free of any pulmonary nodules, thus her pathology lacked the classic vasculitis-induced ischemic necrosis. Kikuchi-Fujimoto disease as well as Katayama fever were considered in our differential diagnosis, but were ruled out, respectively, due to evidence of granulomatous necrosis and negative serology for schistosomiasis.
CONCLUSION: This patient's case is unusual as the majority of her disease remains extra-pulmonary leading to diffuse lymphadenopathy, hepatosplenomegaly, with disabling fever and malaise. Our working and unifying diagnosis remains sarcoidosis with atypical features of NSG. As a result of her unrelenting clinical course, the patient was eventually started on prednisone, hydroxychloroquine and methotrexate, with repeat PET scan revealing decreased uptake in the mediastinum, with stabilization of her symptoms.
DISCLOSURE: Ali Massoumi, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Benign metastasizing leiomyoma (BML) is a rare clinical entity first described in 1939 by PE Steiner as "a tumor composed histologically, in both the primary growth and its metastasis, of benign appearing, fully differentiated smooth muscle cells and dense connective tissue". The term benign metastasizing leimyoma was later coined by JP Horstman to distinguish the myometrial origin of the disease. Since its initial description, approximately 75 cases have been reported in the medical literature. Here, we report a case of BML in a pre-menopausal, 42 year-old woman who presented with an abnormal chest radiograph.
CASE PRESENTATION: A 42 year-old African-American woman was evaluated for an abnormal chest radiograph. She denied cough, fever, chills, night sweats, weight loss, fatigue or limited exercise. Her past medical history was significant for large uterine fibroids, positive PPD and two prior D&Cs. She has never smoked or been exposed to chemicals or dusts and has lived in Queens for 30 years. She has no known allergies and takes ibuprofen prn. On exam she appeared well and vital signs were normal. Exam was normal except for a palpable, non-tender pelvic mass. ESR was elevated. PFTs revealed mild restriction and normal diffusion. The patient's chest radiograph and subsequent CT demonstrated innumerable tiny nodules in bilateral lung fields. Bronchoscopy with transbronchial biopsy was non-diagnostic. The patient underwent VATS lung biopsy with pathologic findings of benign metastasizing leiomyoma (BML). Stains for smooth-muscle actin, estrogen and progesterone receptors were positive. The patient underwent a trial of GnRH analog and responded well with significant improvement in her symptoms as well as objective improvement of her radiographic findings on CT. Subsequently, the patient elected to have a total hysterectomy with bilateral salpingoopherectomy which confirmed the absence of malignant cells.
DISCUSSIONS: BML is a rare disorder reported in women ages 23–64 with a history of fibroids. It typically presents after myomectomy by incidental findings on chest x-ray. Patients are generally asymptomatic and PFTs are normal or restrictive. The clinical course ranges from chronically-indolent to rapidly-progressive depending on hormonal status. BML in menopausal or pregnant women tends to be slowly-progressive and may even resolve. Pre-menopausal women are more likely to have progressive pulmonary insufficiency due to expanding nodules. Radiographically, BML presents as multiple, bilateral pulmonary nodules. Other patterns reported include unilateral nodules, miliary and cystic. The nodules are well circumscribed and non-calcified. Sites of metastasis include the lung, pelvic and retroperitoneal lymph nodes, omentum and muscle. The mechanism of metastasis is not well understood, but is presumed hematogenous. Histological stains for smooth-muscle actin, estrogen and progesterone receptors demonstrate the uterine smooth-muscle origin of the cells. The primary treatment for patients with BML has been TAH-BSO. The role for hormonal treatment has yet to be determined.
CONCLUSION: The pathogenesis of BML is not clearly understood. Some authors believe that it may represent a low-grade leiomyosarcoma. Also, because this disease entity is so rare, there is no established standard of care. For these reasons, identification and close follow-up of these patients is neccessary.
DISCLOSURE: Kaye Hale, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Patients with Pulmonary Hyalinizing Granuloma (PHG) usually present with lung masses diagnosed incidentally on chest radiographs (CXR). However, their appearance on a 18-FDG Positron Emission Tomography (PET) scan in adults is not known and is reported here along with the association with deep venous thrombosis (DVT) in the absence of any pro-coagulant risk factors.
CASE PRESENTATION: An 83 year old obese female with a sixty pack-year smoking history was referred for evaluation of an incidental finding on chest radiograph. Her only respiratory complaint was an occasional non-productive cough. Her past medical history was significant for recurrent deep venous thrombosis (DVT) without any predisposing factors. She retired from an office job and denied any hazardous environmental exposure.Physical examination did not reveal any evidence of clubbing, pallor, cyanosis or any pulmonary abnormality. Pulmonary function testing revealed mild restriction, explained by her obesity.Chest x-ray (CXR) showed a large mass in the right mid lung and another mass at the right apex highly suspicious for a neoplastic process. These were not present on a CXR from two years ago. Computed Tomography (CT) scan of the chest showed a large pulmonary mass within the anterior segment of the right upper lobe measuring 6.3 × 3.8 × 5.3 cm with peripheral spiculation and extension to the pleura. Two additional spiculated nodules in the right upper lobe and one in the left upper lobe were also found. A PET scan revealed that all lung masses were strongly FDG-avid, consistent with malignancy and probably multi-centric pulmonary malignancy. The patient underwent two non-diagnostic flexible bronchoscopy procedures along with multiple transbronchial biopsies and transbronchial needle aspiration of the two right sided masses. Six CT guided core-needle lung biopsies were then obtained and all contained dense, lamellar or "ropy" keloid-like collagen bundles arranged in a haphazard pattern. The biopsies lacked significant necrosis, necrotizing granulomas or epithelioid granulomas. Congo red stain with polarization was also negative for amyloid. The diagnosis of PHG was made. A complete hypercoagualable workup was done but no underlying abnormalities were found including a negative lupus anticoagulant. The patient was maintained on warfarin and followed clinically and with serial CT scans for one year after the initial presentation. She remained totally asymptomatic and had no change in the lung masses.
DISCUSSIONS: PHG was coined by Engleman et al. to describe the specific histological appearances observed in a series of pulmonary nodules with unknown etiology.1 Nodules with spiculated margins and cavitation have been reported, but their appearance on a PET scan has not been described in adults.1 The strongly avid appearance of PHG on a PET scan may lead to multiple and un-necessary invasive procedures in the pursuit of a diagnosis of malignancy. Although a case of PHG associated with DVT in the presence of lupus anticoagulant has been reported,2 our patient developed DVT without any underlying coagulation abnormalities. This may point towards a direct association of DVT with PHG, something that needs to be confirmed in future reports.
CONCLUSION: PHG may present as strongly avid (hot) lesions on a PET scan. It can be associated with DVT even in the absence of lupus anticoagulant and other coagulation abnormalities.
DISCLOSURE: Hamed Mataria, No Financial Disclosure Information; No Product/Research Disclosure Information
INTRODUCTION: Invasive aspergillosis is a severe infection that usually affects immunocompromised patients. We report a case of invasive pulmonary aspergillosis in a young, otherwise seemingly healthy woman.
CASE PRESENTATION: A 35 year old woman with mild intermittent asthma presented with a 3 day history of fever to 103.7°F, non-productive cough and dyspnea. There was no sore throat, rash,arthralgia or diarrhea. She is an avid runner and lifetime nonsmoker. She enjoys gardening and recently deposited some cut grass in a nearby compost. There was no recent travel and her only medication was an albuterol inhaler.Her physical examination was remarkable for bilateral crackles at the bases.CXR revealed diffuse reticulonodular infiltrates (see figure 1) and CBC was significant for 7% eosinophils (see table2 initial presentation). She was given an outpatient course of azithromycin.Without improvement, she returned 3 days later with persistent fever and dyspnea. Her oxygen saturation on room air was 86% prompting hospital admission. Bronchoscopy with bronchoalveolar lavage (BAL) revealed 69% eosinophils. Bacterial, viral and mycobacterial cultures were negative (table 1st admission). Workup for autoimmune disease and stool for ova and parasites were negative. Her IgE level was elevated at 2440 (table 1st admission). A diagnosis of acute eosinophilic pneumonia was made and systemic corticosteroids were started with remarkable clinical and radiological improvement. She was discharged on a prednisone taper and TMP-SMX for pneumocystis jiroveci pneumonia prophylaxis (PJP). The patient returned 5 days later with worsening dyspnea and recurrent fever. Her PaO2 was 66 mmHg on room air and CXR revealed worsening bilateral infiltrates. She was admitted to the intensive care unit. Bronchoscopy was repeated and transbronchial biopsy revealed evidence of hyphae branching at 45° angle with possible vasculitis. Video assisted thoracoscopic surgery with lung biopsy, fungal stain and culture confirmed the presence of invasive aspergillosis without vasculitis. Immunoglobulin subclasses, HIV and screening for cystic fibrosis were negative. Nitroblue tetrazolium test was positive. Genetic testing confirmed the presence of autosomal recessive chronic granulomatous disease (CGD). Final diagnosis: fulminant invasive pulmonary aspergillosis and autosomal recessive CGD. Antifungals, gamma interferon and systemic steroids were started and after a prolonged hospital course, she was discharged to a rehabilitation facility where she completed her recovery and returned home. CXR revealed remarkable resolution of the infiltrates.
DISCUSSIONS: CGD is a rare group of inherited diseases with annual incidence of 1/255,000 live births and characterized by recurrent infections with catalase positive microorganisms including many bacteria and fungi. The gene mutation in the reduced nicotinamide adenine dinucleotide phosphate (NADPH) oxidase enzyme complex renders the leukocyte unable to kill the ingested microorganisms. The X-linked form of the disease (gp91phox) is more common and carries a worse prognosis. It affects females more often and manifests with recurrent infections early in life. The autosomal recessive form (P22phox, P47phox, and P67phox) is less common,affects males more than females and carries a better prognosis. Patients with this form may present with infections in late adulthood. Pulmonary disease is most common however, cellulitis, osteomyelitis and abscesses were reported. Mortality rate is 17.6% and pneumonia due to aspergillus or Burkholderia cepacia is the most common cause of death. Prophylaxis with TMP-SMX and interferon gamma was shown to reduce the rate of infections. Systemic corticosteroids were reported to be beneficial in reducing the exuberant inflammatory response in life threatening infections.
CONCLUSION: CGD is rare inherited disease characterized by increased susceptibility to recurrent infections. Acute invasive aspergillosis in the absence of known immunodeficiency should prompt consideration of CGD.
DISCLOSURE: Ghazwan Acash, No Financial Disclosure Information; No Product/Research Disclosure Information
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