Moderate Right-sided Pleural Effusion Research Articles

S1922 A Twisted Plot: Pancreas Divisum With Right-Sided Pancreaticopleural Fistula

Introduction: We report on a 53-year-old male with a past medical history significant for chronic pancreatitis who presented with a right-sided pleural effusion secondary to pancreaticopleural fistula with pancreatic divisum. Pleural effusion secondary to pancreaticopleural fistula (PPF) is a rare complication of acute or chronic pancreatitis and usually presents with a left-sided pleural effusion. This case also highlights the difficulty and significant delay in diagnosing pancreas divisum on cross-sectional imaging. Case Description/Methods: A 53-year-old male with a medical history significant for chronic pancreatitis complicated by pancreatic ascites, walled-off necrosis, splenic and portal vein thrombosis, alcoholic gastritis, and polysubstance use presented with five days of dyspnea with new and worsening oxygen requirements. Chest x-ray showed a new moderate right-sided pleural effusion. Pleural fluid analysis revealed elevated lipase of >3,000 U/L and amylase of 21,008 U/L concerning for a pancreaticopleural fistula. MRI of pancreas showed a disconnected duct at the tail of the pancreas with a fistula extending into the mediastinum and peritoneum. During ERCP a bulging minor papilla was noted. After failed initial attempts at cannulating ventral duct, minor papilla was approached and dorsal duct was cannulated. Pancreatogram revealed a complete pancreatic divisum with a leak in the tail of the pancreas. A temporary plastic stent was placed in the dorsal duct after minor papilla sphincterotomy. The patient's symptoms resolved and he was discharged home with outpatient follow-up (Figure). Discussion: PPF is a rare complication of pancreatitis that typically presents as large, recurrent, left-sided pleural effusion often refractory to thoracentesis management. Transpapillary stent placement in the pancreatic duct is widely used in the management of PPF. Our case shows a rare right-sided presentation of PPF in the setting of a newly diagnosed pancreas divisum missed on numerous previous cross-sectional imaging. High clinical suspicion of underlying divisum raised by endoscopic and imaging findings led to successful endoscopic management of this complex case.Figure 1.: MRCP Pancreas showing pancreas divisum and disconnected duct.

METASTATIC COLORECTAL CANCER PRESENTING AS AN OBSTRUCTING ENDOBRONCHIAL LESION

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Colorectal cancer is one of the most common malignancies worldwide with the pulmonary parenchyma a common site for distant metastasis. Colorectal endobronchial metastases, however, are exceedingly rare. We present a patient with previously resected colon cancer who was found to have an obstructing endobronchial colorectal metastasis. CASE PRESENTATION: A 66-year-old male with a 12 pack-year smoking history presented with dyspnea. On routine colonoscopy 4 years prior, he was diagnosed with moderately differentiated colorectal adenocarcinoma with negative tumor margins and without any lympho-vascular involvement. Imaging was without evidence of regional or distant metastases so he underwent hemicolectomy. Medical oncology recommended no adjuvant chemotherapy and 1 year follow-up. The patient did not follow-up. Four years later, he presented with fever, dyspnea, cough, and pleuritic chest pain. CT pulmonary angiography demonstrated a RUL mass with RML lobe collapse, a moderate right-sided pleural effusion, and no pulmonary embolism. He was started on empiric antibiotics. Thoracentesis revealed an exudative effusion with negative cultures and cytology. Bronchoscopy demonstrated a fungating endobronchial lesion completely occluding the right middle lobe for which cryobiopsy and cryo-excision were performed. Pathology was consistent with metastatic moderately differentiated colorectal adenocarcinoma, with morphology and histology identical to his prior colorectal cancer. Transbronchial needle aspiration using endobronchial ultrasound of the station 7 mediastinal lymph node was negative for malignant cells. Patient left against medical advice before further work-up was completed. DISCUSSION: About 20% of patients with colorectal cancer have distant metastases at presentation, with the lungs being a common site, usually involving the pulmonary parenchyma and very rarely as an endobronchial lesion. In fact, endobronchial metastases from extra-thoracic malignancies account for 1% of all endobronchial tumors, with the most common being colorectal, breast and renal. These patients may present with a variety of non-specific respiratory symptoms. It is also not uncommon for endobronchial metastasis to occur after resection of the primary tumor and without other signs of metastases. One should consider the possibility of endobronchial involvement of a tumor in a patient with a history of an extra-thoracic malignancy who presents with vague respiratory symptoms, as timely bronchoscopic intervention can improve quality of life and help mitigate the risk of post obstructive complications. CONCLUSIONS: Endobronchial metastasis from extra-thoracic malignancies are very rare, accounting for 1% of all endobronchial tumors. Diagnosis can be difficult due to vague respiratory complaints. Bronchoscopic intervention can assist in diagnosis, management, and it can lead to substantial improvement in pulmonary symptoms. REFERENCE #1: Akoglu S, Uçan ES, Celik G, et al. Endobronchial metastases from extrathoracic malignancies. Clin Exp Metastasis. 2005;22:587–591. REFERENCE #2: Coriat R, Diaz O, de la Fouchardiere C, et al. Endobronchial metastases from colorectal adenocarcinomas: clinical and endoscopic characteristics and patient prognosis. Oncology. 2007;73:395-400. REFERENCE #3: Lin CY, Chung FT. Central airway tumors: interventional bronchoscopy in diagnosis and management. J Thoracic Dis. 2016;8(10):E1168-E1176. DISCLOSURES: Speaker/Speaker's Bureau relationship with Boehringer-Ingelheim Pharmaceuticals Please note: $5001 - $20000 by Jaime Betancourt, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Vapotherm, Inc Please note: $5001 - $20000 by Jaime Betancourt, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Boehringer-Ingelheim Pharmaceuticals Please note: 2014-Present Added 04/21/2021 by Jaime Betancourt, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Vapotherm, Inc Please note: 2019-Present Added 04/21/2021 by Jaime Betancourt, source=Web Response, value=Honoraria Advisory Committee Member relationship with Vapotherm, Inc Please note: 2020 Added 04/21/2021 by Jaime Betancourt, source=Web Response, value=Honoraria No relevant relationships by Ken Dekitani, source=Web Response No relevant relationships by Michael Lewis, source=Web Response No relevant relationships by Roberto Mempin, source=Web Response No relevant relationships by Scott Oh, source=Web Response

ACUTE RESPIRATORY DISTRESS: A RARE PRESENTATION OF URETERAL RUPTURE

TOPIC: Signs and Symptoms of Chest Diseases TYPE: Medical Student/Resident Case Reports INTRODUCTION: Cardiorespiratory disorders usually present with acute respiratory distress; likewise, ureteral rupture usually presents as an acute abdomen [1]. We report a case of ureteral rupture presenting with acute respiratory distress leading to a delayed diagnosis. CASE PRESENTATION: An 82 y/o M with a history of congestive heart failure (CHF), chronic obstructive pulmonary disease, chronic renal failure and recently diagnosed COVID -19 pneumonia, was admitted for progressive dyspnea and abdominal discomfort for 2 days. Arterial blood gas showed hypercapnic hypoxic respiratory failure. Chest computed tomographic (CT) scan without contrast showed moderate right-sided pleural effusion but pulmonary embolism (PE) could not be ruled out. He also had a left leg deep venous thrombosis with worsening renal function and B-type natriuretic peptide (BNP) of 139pg/ml.His symptoms were thought to be due to acute decompensated CHF, COVID-19 pneumonia and possible PE. He failed to improve with intravenous furosemide, bilevel positive airway pressure ventilation and empiric anticoagulation. Due to worsening renal function and abdominal pain, an abdominal CT was done which showed right ureteral rupture with retroperitoneal collection and multiple non-obstructing renal calculi. The patient had placement of a double J stent with prompt resolution of respiratory distress. DISCUSSION: There are very few case reports of ureteral rupture occurring spontaneously and causing marked dyspnea. Respiratory distress may occur if the extravasated urine leads to an urinothorax or abdominal compartment syndrome [1]. Suspicion of non-cardiorespiratory causes of acute dyspnea in patients with underlying congestive heart failure includes respiratory distress disproportionate to the size of pleural effusion, unilateral effusions or effusions of markedly disparate size, absence of pulmonary vascular congestion and cardiomegaly, worsening renal function, ascites, urinary retention or anuria, abdominal pain, failure of the effusion to respond to management of heart failure, fever or pleuritic chest pain, and low or near-normal natriuretic peptides [2,3].When there are concerns for non-cardiorespiratory causes of respiratory distress, the diagnosis can be made with thoracentesis and imaging such as abdominal ultrasound, or CT [2]. CONCLUSIONS: This case illustrates a rare presentation of ureteral rupture causing respiratory distress and the key points that should elicit high clinical suspicion for non-cardiorespiratory causes of acute respiratory distress. When these findings are present, appropriate investigations and urgent intervention may be life-saving. REFERENCE #1: Choi SK, Lee S, Kim S, et al. A rare case of upper ureter rupture: ureteral perforation caused by urinary retention. Korean J Urol. 2012;53(2):131-133. doi:10.4111/kju.2012.53.2.131 REFERENCE #2: Leonidas Laskaridis, Spyridon Kampantais, Chrysovalantis Toutziaris, Basileios Chachopoulos, Ioannis Perdikis, Anastasios Tahmatzopoulos, Georgios Dimitriadis, "Urinothorax—An Underdiagnosed Cause of Acute Dyspnea: Report of a Bilateral and of an Ipsilateral Urinothorax Case", Case Reports in Emergency Medicine, vol. 2012, Article ID 395653, 3 pages, 2012. https://doi.org/10.1155/2012/395653 REFERENCE #3: Porcel JM. Pleural effusions from congestive heart failure. Semin Respir Crit Care Med. 2010 Dec;31(6):689-97. DOI: 10.1055/s-0030-1269828. Epub 2011 Jan 6. PMID: 21213200. DISCLOSURES: No relevant relationships by sharmela Brijmohan, source=Web Response No relevant relationships by Angelica Medina-Pena, source=Web Response No relevant relationships by Noura Semreen, source=Web Response

PRIMARY INTRATHORACIC SYNOVIAL SARCOMA

TOPIC: Lung Cancer TYPE: Medical Student/Resident Case Reports INTRODUCTION: Synovial sarcomas (SS) are a rare group of malignancies, seen in 0.17 per 100,000 persons, accounting for 7% of all soft tissue sarcomas. These malignant tumors most commonly present as a tumor of the extremities. While they have a high propensity for metastasis to bone and lung, primary intrathoracic malignancy is rare. We present a case of a patient who presented with acute onset shortness of breath, diagnosed with primary synovial sarcoma of the lung. CASE PRESENTATION: A 22-year-old male with no past significant medical history presented with a four-week history of right upper quadrant abdominal pain and acute onset chest pain radiating to his shoulder with associated dyspnea. On arrival to emergency department, patient was tachycardia, otherwise vitally stable. Labs were notable for leukocytosis. Chest x-ray showed a moderate right-sided pleural effusion with associated opacity of the lower hemithorax. A chest tube was placed however no fluid was evacuated. A CT of the chest was obtained which showed a right lower lobe hemithorax, a right lower lobe pleural-based nodule measuring 2x2 x 1.6 cm, and a lesion within the right hemithorax measuring 20x17.9x19.3 cm. The patient was taken for video-assisted thoracoscopic surgery for surgical exploration and chest tube placement. Patient was found to have large tumor invading whole right chest with compression on mediastinal structures including the heart, lung, and IVC. Biopsies were taken. Patient developed acute respiratory distress and obstructive shock and was intubated and placed on vasopressor support. Preliminary pathology was revealing of synovial sarcoma. Operative findings revealed a large right pleural and posterior mediastinal mass invading the right lower lobe through the diaphragm into the peritoneal space and liver capsule. He underwent thoracotomy with excision and resection of the thoracoabdominal and mediastinal tumor, total pulmonary decortication, wedge resection of right lower and middle lobe, and reconstruction of diaphragm. The patient was successfully extubated three days post operatively and soon after discharged home. Post-operative CT showed no definite evidence of gross residual disease. The patient received 3 cycles of doxorubicin/isofosfamide following discharge. DISCUSSION: Reports of primary thoracic SS are rare. Diagnosis is often delayed because of the benign radiographic appearance and indolent nature of these tumors; sequelae of disease often do not manifest until tumor burden is great enough to cause mass effect. Though standard of care is chemotherapy, the patient in this case required emergency palliative resection due to cardiovascular compromise. CONCLUSIONS: Though soft tissue sarcomas account for less than 0.1% of all malignant thoracic neoplasms, it should be considered in the differential for thoracic malignancies as delay in diagnosis could be consequential to patient outcome. REFERENCE #1: Javad Parvizi, Gregory K. Kim, Chapter 218 - Synovial Sarcoma, Editor(s): Javad Parvizi, Gregory K. Kim, High Yield Orthopaedics, W.B. Saunders, 2010, Pages 455-456, ISBN 9781416002369, https://doi.org/10.1016/B978-1-4160-0236-9.00230-3. REFERENCE #2: Nedra Joseph, Samantha St. Laurent, Jeanenne Joy Nelson, Shan Zheng, and Heide Stirnadel-Farrant. Journal of Clinical Oncology 2019 37:15_suppl, e22535-e22535 DISCLOSURES: No relevant relationships by Neiha Kidwai, source=Web Response

Visual Diagnosis: Rapid Deterioration of Respiratory Status and Lower Limb Hypotonia in a 6-month-old Infant.

1. Guillaume J. LimFat, BMSc* 2. Vinay Kukreti, MD†,‡ 1. *Undergraduate Medicine, Queen’s University School of Medicine, Kingston, ON, Canada 2. †Department of Pediatrics, Queen’s University School of Medicine, Kingston General Hospital, Kingston, ON, Canada 3. ‡Department of Pediatrics, Lakeridge Health Oshawa, Oshawa, ON, Canada A 6-month-old previously healthy boy presents to the local emergency department with a 1-week history of progressive lethargy, irritability, and anorexia. Two days before presentation he developed an unusual breathing pattern and worsening respiratory distress, and he had a fever of 101.3°F (38.5°C). There was no rhinorrhea, coryza, cough, vomiting, or diarrhea. He appears unwell, with respiratory distress, tachypnea, and grunting. His blood pressure is 80/45 mm Hg, heart rate is 200 beats/min, respiratory rate is 60 breaths/min, and oxygen saturation is 77% in room air. He is afebrile. There is significantly decreased air entry bilaterally, most impressively over the right lung field. His neurologic examination reveals a mild right-sided ptosis. Although he moves both arms normally, there is a noticeable paucity of movements in his lower limbs, with decreased tone in both legs. The remainder of his examination findings are normal. On further questioning the parents clarify that for the past 2 months there has been a relative decrease in leg movements, and he had stopped attempting to bear weight when made to stand with support. His respiratory status does not improve, and he requires supplemental oxygen to maintain appropriate oxygen saturation. Clinical laboratory tests reveal a white blood cell count of 18,800/ μ L (18.8×109/L) (neutrophil count, 6,200/ μ L [6.2×109/L]; lymphocyte count, 11,100/ μ L [11.1×109/L]; and monocyte count, 1,100/ μ L [1.1×109/L]) and normal electrolyte levels. A chest radiograph reveals a large right paravertebral mass with significant left mediastinal shift associated with rib and vertebral destruction and moderate right-sided pleural effusion (Fig 1). Given these findings and worsening …

SPONTANEOUS BACTERIAL EMPYEMA: HOW IS IT ACTUALLY DEFINED?

SESSION TITLE: Monday Fellow Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Hepatic hydrothorax refers to a pleural effusion in a cirrhotic without another identifiable cause. When this pleural fluid becomes infected, it is called a spontaneous bacterial empyema (SBE). SBE needs to be distinguished from an effusion secondary to a primary lung infection because chest tube drainage is avoided in SBE. We present a case of a cirrhotic patient with an infected pleural space where the diagnosis is not clear based on existing diagnostic criteria for SBE. CASE PRESENTATION: A 64 year old male with alcoholic cirrhosis presented to the Emergency Department for shortness of breath. He had a history of refractory ascites requiring paracentesis every two weeks and a small, right-sided pleural effusion that had been present for two years. Upon presentation, he was tachycardic and hypoxic requiring three liters of oxygen. Initial workup was significant for a white blood cell (WBC) count of 14,000/uL, chest x-ray showed a moderate right-sided pleural effusion, thoracic ultrasound showed a loculated pleural space, and abdominal ultrasound revealed no ascites. A thoracentesis was performed with serosanguinous fluid appearance, WBC of 6,000/uL with 98% neutrophils, pH of 6.50, and a glucose of 172 mg/dL. A chest tube was placed soon after admission. After four days of drainage and intravenous antibiotics, the patient was weaned off supplemental oxygen and his leukocytosis resolved. The chest tube was removed, and he was discharged to complete six weeks of antibiotics. DISCUSSION: Chest tube drainage has been associated with increased incidence of protein loss, kidney injury, and infection in cirrhotic patients [1], and most guidelines recommend against tube drainage for SBE [1, 2, 3]. To our knowledge, there is no consensus definition of SBE (Table 1), and none incorporate the presence of loculations on chest imaging. This patient met some definitions of SBE while not meeting others (Table 1). Because of the low pleural fluid pH and complex appearing pleural space, we proceeded with chest tube drainage despite recommendations to avoid catheter placement in SBE because of concern for inadequate source control with antibiotics alone. The patient’s clinical improvement after chest tube drainage confirmed our suspicion that he likely had a parapneumonic effusion instead of SBE. Integrating pleural loculations and standardizing the definition for SBE may help stratify patients that require chest tube drainage from those that will improve with antibiotics alone. CONCLUSIONS: The diagnostic criteria for SBE is not clear. The lack of a consensus definition can lead to treatment uncertainty and inappropriate delays in chest drainage. Standardizing the diagnostic criteria will allow for more judicious use of chest tube drainage in cirrhotic patients that are at increased risk of catheter related complications. Reference #1: 1. Xiol X, Casais L. Spontaneous Bacterial Empyema in Cirrhotic Patients: Analysis of Eleven Cases. Hepatology. 1990 Mar; 11(3): 365-7 Reference #2: 2. Tu CY, Chen CH. Spontaneous Bacterial Empyema. Curr Opin Pulm Med. 2012 Jul; 18(4): 355-8 Reference #3: 3. Cardenas A, Kelleher B, Chopra S. Hepatic Hydrothorax. UptoDate. Retrieved January 25, 2019 from https://www.uptodate.com/contents/hepatic-hydrothorax DISCLOSURES: No relevant relationships by Kevin Ho, source=Web Response No relevant relationships by Jennifer McCallister, source=Web Response

SYMPTOMATIC PLEURAL EFFUSION: TROUBLESHOOTING VENTRICULOPLEURAL SHUNT

SESSION TITLE: Disorders of the Pleura 2 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Ventriculopleural shunt (VPLS) is an alternative to the commonly used ventriculoperitoneal shunt (VPS). The pleural space has an excellent absorptive capacity. Pleural effusion is one of the most common complications following VPLS, likely due to inflammation causing an imbalance between cerebrospinal fluid (CSF) production and pleural space resorption. CASE PRESENTATION: A 55-year-old male with a PMH of VPS after surgical removal of a giant bi-frontal meningioma in 2015, complicated with enterococcus and Propionibacterium CSF infection requiring multiple shunt revision, modification of shunt valve and external drainage. He underwent placement of right VPLS in April of 2017. 10 months after placement of VPLS, he presented to ED with one week of flu-like symptoms and right-sided pleuritic chest pain. Physical examination revealed absent breath sounds over the right lung base and hypoxia needing 6 L of Oxygen via NC. Pleural ultrasound revealed moderate right-sided pleural effusion with fibrinous septations. Thoracentesis revealed un-complicated LDH discordant exudative pleural effusion. CSF culture was again positive for Propionibacterium acne, blood and pleural fluid culture were negative. He underwent chest tube placement with TPA/Dornase instillation. Neurosurgery proceeded with VPLS removal and placement of an external ventricular drain for a week followed by placement of a new antibiotic-impregnated VPS. DISCUSSION: The most common indication for using pleural cavity for drainage in a patient with a pre-existent ventriculoperitoneal shunt is due to the infection, adhesion or thrombosis of the shunt causing obliteration. Most of the complications including pleural effusion following VPLS occur within the first year of placement. Pleural fluid analysis showing clear transudative effusion resembling CSF, but exudative effusion can occur secondary to chronic inflammation. Most of the cases of mild effusion resolve spontaneously without any specific treatment but symptomatic cases with moderate effusion may require a thoracentesis, chest tube insertion and in recurrent cases even a shunt replacement. P. acnes is a part of the skin flora and most isolates in culture are considered as contaminant. On the contrary, P. acnes is frequently isolated in patients with shunt infections and evidence suggests that a P. acnes positive CSF culture should not be neglected and treated with appropriate antibiotics. The use of anti-siphon pressure control devices and acetazolamide have been proven to somewhat reduce the likelihood of developing the pleural effusion. CONCLUSIONS: The pleural cavity is the most common alternative to peritoneal cavity for drainage of CSF in a patient presenting with refractory hydrocephalus. He was also found positive for P. acnes on CSF culture. We suggest drainage of pleural cavity and revision/replacement of shunt as well as antibiotic therapy for P. acnes infection. Reference #1: Sahn SA. Pleural effusions of extravascular origin. Clin Chest Med. 2006;27:285–308. Reference #2: Nisbet M, Briggs S, Ellis-Pegler R, Thomas M, Holland D. 2007. Propionibacterium acnes: an under-appreciated cause of post-neurosurgical infection. J. Antimicrob. Chemother. 60:1097–1103. 10.1093/jac/dkm351 Reference #3: Martínez-Lage JF, Torres J, Campillo H, Sanchez-del-Rincón I, Bueno F, Zambudio G, et al. Ventriculopleural shunting with new technology valves. Childs Nerv Syst. 2000;16:867–71. DISCLOSURES: No relevant relationships by James Dean, source=Web Response No relevant relationships by Shuhong Guo, source=Web Response No relevant relationships by Brandon Murguia, source=Web Response No relevant relationships by Ali Saeed, source=Web Response No relevant relationships by Samarthkumar Thakkar, source=Web Response

PLEURAL AND CHEST WALL LYMPHANGIOMATOSIS

SESSION TITLE: Disorders of the Mediastinum 2 SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/10/2018 10:45 am - 11:45 am INTRODUCTION: Recurrent pleural effusions of unclear etiology can be a challenging clinical scenario. We present a rare case of pleural Lymphangiomatosis with no lung parenchymal involvement, presenting atypically late in adult life. CASE PRESENTATION: A 66 year old lady presented to pulmonary clinic with subacute on chronic shortness of breath. She had a history of recurrent exudative right sided pleural effusions for the last 5 years. The effusions were consistently culture and cytology negative. She had no other cardiopulmonary symptoms, past history, exposures or family history. Pertinent exam findings included absent breath sounds in the right lower lung field. Chest X-ray revealed a moderate right-sided pleural effusion with associated right lower lobe atelectasis (Image 1). CT chest (Image 2) showed a large mediastinal cystic mass, cysts along the internal mammary wall and a small right pleural effusion with areas of pleural thickening. She underwent a diagnostic and therapeutic Video-assisted Thoracoscopy/Thoracotomy. Serosanguinous pleural fluid was drained and a pleural peel excision/pleurodesis/cyst excisions were performed. Histopathology revealed lymphangiomatosis with lymphangiectasia. She was symptom free post-procedure but presented a few years later with recurrent disease confirmed on CT chest (Image 3). She declined surgery and is being managed with therapeutic thoracenteses, propranolol, anti-Vascular Endothelial Growth Factor (VEGF) therapies and Sirolimus, with good response over the last few years. DISCUSSION: Lymphangiomatosis is a rare disorder characterized by lymphatic overgrowth along normal systemic lymphatic channels(1). While it has been reported to present over diverse age groups from birth to 80 years, with no clear gender predilection, it rarely presents after 20 years of age. In the chest, it can present as Diffuse Pulmonary Lymphangiomatosis, involving only pulmonary parenchyma and is clinically/histopathologically unique from Lymphangioleiomyomatosis. It may also present purely with mediastinal, pleural and chest wall involvement. CT chest usually shows interlobular septal thickening with mediastinal involvement (2). Factor 8 related antigen and CD31 are usually positive on immunohistochemistry. Chylous effusions, chyloptysis, chylopericardium and chylous ascites are common and complications include protein wasting enteropathy, lymphedema and Disseminated Intravascular Coagulation. Surgical interventions are complex given difficulty separating pathologic from normal lymphatics /other anatomic structures. Lymphoscintigraphy can often help to guide therapy. Anti-VEGF therapies and Sirolimus(3) are being increasingly used as therapeutic and maintenance measures. CONCLUSIONS: While a rare cause of pleural effusions in the adult population, Lymphangiomatosis can be a challenging condition to manage. Reference #1: Tazelaar, H.D., et al., Diffuse pulmonary lymphangiomatosis. Human Pathology. 24(12): p. 1313-1322. Reference #2: Faul, J.L., et al., Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med, 2000. 161(3 Pt 1): p. 1037-46. Reference #3: Reinglas, J., R. Ramphal, and M. Bromwich, The successful management of diffuse lymphangiomatosis using sirolimus: a case report. Laryngoscope, 2011. 121(9): p. 1851-4. DISCLOSURES: No relevant relationships by Augustine Lee, source=Admin input No relevant relationships by Divya Padmanabhan Menon, source=Web Response

The utility of multiparametric flow cytometry in the detection of primary effusion lymphoma (PEL).

Primary effusion lymphoma (PEL) is a rare B cell lymphoproliferative disorder that arises predominantly in body cavities causing malignant effusions. The incidence of PEL is very low, accounting for approximately 4% of all HIV-associated non-Hodgkin lymphomas. PEL has also been described in elderly patients and after solid-organ transplantation. It is associated in all cases with human herpes virus 8 (HHV8). We describe a case of PEL in a 88-year-old HIV-negative woman who presented with progressive dyspnea and moderate right-sided pleural effusion without significant lymphadenopathies or other effusions. The cytological study of the pleural fluid revealed a dense proliferation of large plasmablastic cells. A six-color multiparametric flow cytometry immunophenotyping study was carried out, and revealed 45% of large in size and high cellular complexity cells positive for CD45 (dim), CD38, CD138, CD30 and HLA-DR; and negative for CD19, CD20, cytoplasmatic CD79a, surface and cytoplasmic light chains Kappa and Lambda, CD3, CD4, CD5, CD7, CD8, CD28, CD56, CD81, and CD117. In situ hybridization for EBV-encoded smalI RNA was negative and immunohistochemistry for Kaposi sarcoma herpesvirus (HHV8) confirmed the diagnosis of PEL. Our results confirm that flow cytometry bring useful data in the diagnosis of large-cell lymphomas involving body cavities. © 2018 International Clinical Cytometry Society.

The role of medical thoracoscopy in the diagnosis of exudative pleural effusion at the Chest Department of Zagazig University Hospitals

Thoracoscopy is a minimally invasive procedure that allows visualization of the pleural space and intrathoracic structures. It enables taking pleural biopsies under direct vision, therapeutic drainage of effusions, and pleurodesis in one sitting. Persistent and recurrent exudative pleural effusions become common and thoracocentesis and blind pleural biopsy procedures do not give a definitive diagnosis in many patients. Therefore, thoracoscopy today remains the gold standard for these cases. In tuberculous pleuritis, the combined yield of histology and culture for rigid thoracoscopy was nearly 100%. The aim of the present study was to examine the diagnostic yield of medical thoracoscopy in patients with undiagnosed exudative pleural effusion. This study was carried out at the chest department of Zagazig University Hospitals in the period from October 2014 to October 2015. It included 36 patients with undiagnosed pleural effusion. All participants signed a written informed consent. The included patients were subjected to full history taking, clinical examination, plain chest radiograph, ultrasonography, computed tomography of the chest, and tuberculosis assessment. Diagnostic aspiration of pleural fluid was performed by using chemical, cytological, and bacteriological analyses. Closed pleural biopsy was carried out in patients with unhelpful pleural fluid analysis. Lastly, if the etiology remained unknown, thoracoscopy was carried out. The present study included 18 men and 18 women patients, with a mean age of 54.4±16.1 years. Dyspnea was the most common presenting symptom among the studied patients. Sixteen (72.2%) patients had moderate right-sided pleural effusion. The diagnostic yield of medical thoracoscopy among the studied patients was 80.6%. The histopathological yield of thoracoscopic pleural biopsies was as follows: 25 (69.4%) malignant cases, two (5.6%) patients with tuberculous pleuritis, one (2.8%) patient diagnosed with empyema, and another one (2.8%) with collagenic disease. There were seven (19.4%) patients who were not diagnosed. The post-thoracoscopic complications in this study occurred only in nine (25%) patients, which were minor complications in the form of surgical emphysema and prolonged air leak, wound infection, dislodged drain, trapped lung, and pain during the procedure. Medical thoracoscopy is a good diagnostic procedure for pulmonologists to evaluate undiagnosed pleural effusions.

Chyliform effusion without pleural thickening in a patient with rheumatoid arthritis: A case report.

Pseudochylothorax, also known as chyliform effusion rich in cholesterol crystals, is a rare entity that sometimes occurs in long-standing rheumatoid arthritis (RA) and is usually associated with thickened pleura. There have only been a few case reports in the literature on pseudochylothorax unassociated with pleural thickening and with a short duration of articular symptoms in patients with RA. We report the case of a 70-year-old male patient with a history of RA and heart failure due to severe aortic stenosis, who presented with signs and symptoms of decompensated heart failure due to a moderate right-sided pleural effusion that was consequently proved to be pseudochylothorax unassociated with pleural thickening on chest computed tomography (CT) scan. The patient's outcome was favorable after thoracocentesis was carried out and leflunomide was added to the standard heart failure treatment.

Rare presentation of a gastrointestinal stromal tumor with spontaneous esophageal perforation: A case report

INTRODUCTIONGastrointestinal stromal tumors (GISTs) of the alimentary canal are malignant tumors with <1% of cases diagnosed in esophagus. These cases require special consideration given their close proximity to vital structures and propensity to be highly aggressive. Management of patients with GISTs has been transformed since the introduction of tyrosine kinase inhibitors. In this report, we present an unusual case of GIST with spontaneous esophageal perforation. PRESENTATION OF CASEA 39-year-old Caucasian male presented to our hospital with complaints of severe chest and abdominal pain. A diagnostic chest radiograph revealed a moderate right-sided pleural effusion. Subsequently, an esophagram demonstrated a perforation proximal to an elongated stricture in the distal esophagus. A left thoracotomy was performed whereby a large mediastinal mass firmly attached to the esophagus and gastroesophageal junction was encountered. The neoplasm involved proximal one-third of the stomach and perforated into the right hemithorax. Histopathological evaluation of the tumor led to a diagnosis of GIST. DISCUSSIONGISTs of the gastroesophageal junction are uncommon and may rarely present with esophageal perforation. The standard of care for treating GIST at present includes tyrosine kinase inhibitors. This pharmacologic agent, along with improved surgical techniques and understanding of molecular markers for accurate diagnosis, will assuredly continue to improve overall survival of patients with GISTs. CONCLUSIONWhen stricture or achalasia is detected on imaging, GIST should be considered in the differential diagnosis for individual patients. Additionally, chest and abdomen CT scans of may be performed to confirm presence of a tumor mass, thereby ruling out achalasia.

Mycophenolate mofetil inducing remission of lupus enteritis

We report the case of a young woman with a background history of discoid lupus who presented with abdominal pain, vomiting and intermittent diarrhoea. Physical examination revealed tenderness in the right upper quadrant with a palpable right inguinal lymph node without any other clinical signs of active lupus. Laboratory investigations showed normal inflammatory markers, positive ANA and Anti-Ro antibodies, persistent hypocomplementemia and lymphopenia, CT showed marked bowel oedema involving the small and large bowel (halo sign) with massive ascites and moderate right-sided pleural effusion. Mantoux test, AFB and TB cultures were negative. A diagnosis of lupus enteritis was made and treatment with high-dose steroids was commenced with little improvement. Treatment with cyclophosphamide was discussed but declined by the patient. Mycophenolate mofetil was commenced and resulted in significant clinical and radiological resolution. To the best of the authors' knowledge this is the first report of the successful use of mycophenolate mofetil in inducing and maintaining remission in lupus enteritis.

Hemangioendotelioma epitelióide da pleura – Uma apresentação rara a propósito de um caso clínico

Epithelioid Hemangioendothelioma (EHE) is a vascular tumour with rare pleural presentation. As there are a small number of cases described in the literature the authors present the case of a 65-year-old woman, who was admitted to the Emergency Department for a right-sided chest pain, which progressed over 7 months, after a thoracic trauma. The chest X-ray showed signs of a moderate right-sided pleural effusion. A pleural-pulmonary biopsy carried out by toracotomy established the histological diagnosis of EHE of the pleura.Due to the locally advanced stage of the tumour, chemotherapy with carboplatin and etoposide was prescribed and the patient died 6 months later.This case confirms that pleural EHE has an aggressivebehaviour, similar to an angiossarcoma, with a median survival of only a few months after diagnosis.Rev Port Pneumol 2010; XVI (3): 477-482

Pulmonary Artery Sarcoma Mimicking a Pulmonary Artery Aneurysm

Pulmonary artery sarcoma is an uncommon neoplasm, and its clinical and radiological presentation usually simulates chronic thromboembolic disease. We present the case of a 77-year-old woman admitted with dyspnea, chest pain, and hemoptysis. A chest computed tomographic scan showed moderate right-sided pleural effusion and a saccular dilatation of the interlobar portion of the right pulmonary artery, which was filled with contrast and surrounded by an irregular soft-tissue attenuation mass, suggesting a ruptured pulmonary artery aneurysm. The patient was operated on. Intraoperatively, a pseudoaneurysm and a solid mass were identified within the oblique fissure around the interlobar artery. Therefore, a right pneumonectomy was performed. Definitive pathologic examination was consistent with pulmonary artery sarcoma. The patient had a good outcome and is free of disease 2 years after surgery.

CALCIFIC CONSTRICTIVE PERICARDITIS WITH RIGHT PLEURAL EFFUSION

An 84-year-old man had a history of tuberculosis and was cured by antituberculosis drug therapy in his sixth decade. He presented with palpitations, gradual dyspnoea and cough. On examination, he showed marked jugular vein distension with Kussmaul’s sign, oedema of bilateral lower limbs and mild dullness in percussion with diminished breathing sound in the right lung zone. His electrocardiogram showed atrial fi brillation with rapid ventricular responds. A posterior-anterior and lateral chest x-ray (Figure 1) demonstrated dense and localized calcifi cation of pericardium at most of the right ventricular boundary. Echocardiography showed dilation of both atriums and general hypokinesia of the ventricle. Chest computed tomography scan (Figure 2) revealed calcifi ed plaques over the pericardium with compressed right ventricle and moderate right-sided pleural effusion. Due to the development of hypotension and worse clinical presentation, he underwent emergent pericardiectomy with removal of thickened, calcifi ed pericardium. Pathology of the pericardium showed caseous material and tuberculosis was considered as aetiology of constrictive pericarditis. The patient got stable haemodynamic status and improved pleural effusion after surgery.

A 13-year-old female with shortness of breath and pleuritic chest pain

A 13-yr-old Caucasian female was referred to the Dept of Paediatric Diseases (Havana, Cuba) with shortness of breath and pleuritic chest pain located in her right hemithorax. The pain had occurred the day before while doing physical activity and lasted ∼1 h. The patient reported a 2-month history of recurrent episodes of pleuritic chest pain, which were either self-limited or relieved by paracetamol, as well as growing fatigue in the previous few weeks. Her past history was remarkable for a diagnosis of pneumonia after exposure to fumigation during a dengue haemorrhagic fever epidemic 2 yrs earlier, which was treated with steroids and antibiotics. On admission, the patient had no weight loss, no fever or dyspnoea. Physical examination showed decreased breathing sounds in the inferior part of both hemithoraces. Cardiovascular, abdominal and neurological examination, as well as ear, nose and ophthalmologic investigations were unremarkable. The chest radiograph showed bilateral pneumonia with moderate right-sided pleural effusion. The leukocyte count was 9.8×109 cells·L−1 with 54% neutrophils and 32% lymphocytes. The erythrocyte sedimentation rate was 85 mm·h−1. Serum chemistry, renal and liver function tests were in the normal range. Blood cultures were negative. Antibiotic therapy with ceftriaxone and amikacin was started. During the treatment the patient reported 2 days of moderate fever and various episodes of left-sided pleuritic chest pain, which occurred especially during forceful diaphragm contractions ( i.e. sneezing, laughing and coughing). Following a short stay in the Cuban hospital, the patient was transferred to an Italian hospital (Como). Amikacin was discontinued and clarithromycin added. A new chest radiograph (fig. 1⇓), 14 days after the previous one, and a computed tomography (CT) scan of the thorax were performed. Echocardiography and abdominal ultrasound were both negative. An increased value of the tumour marker CA-125 (220 U·mL−1, versus a normal value <35 U·mL−1) was found. Microbiological …

Hydrothorax in Continuous Ambulatory Peritoneal Dialysis

CONTRERAS-PUERTAS, PABLO M.D.*; BENÍTEZ-SÁNCHEZ, MANUEL M.D.†; JIMÉNEZ-HEFFERNAN, AMELIA M.D.*; REBOLLO-AGUIRRE, ANGEL M.D.*; CRUZ-MUÑOZ, SONIA M.D.† Author Information

Rounded atelectasis caused by histoplasmosis.

A 37-year-old man had a 3-month history of intermittent hemoptysis, which for 2 days had been associated with wheezing and coughing. Results of physical examination and laboratory tests were within normal limits. He denied asbestos exposure. His medical history was remarkable for pulmonary histoplasmosis diagnosed 18 years earlier on the basis of rising serologic titers. Reportedly, the chest radiograph revealed hilan lymphadenopathy, a poorly defined panenchymal opacity abutting the pleura of the right lower lobe posteriorly, and a moderate right-sided pleural effusion. Wonkup during the initial hospitalization included thoracentesis. Results of cytologic studies were normal, and smears for acid-fast bacilli and cultures for fungi, bacteria, and acid-fast organisms performed on the fluid were all negative. No treatment was instituted, and the patient