SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Lymphangiomatosis is an extremely rare condition characterized by abnormal hyperproliferation of the lymphatic system. It can ultimately result in organ failure secondary to the abnormal lymphatic proliferation and infiltration of the organ systems. We discuss a case of lymphangiomatosis which presented as a unilateral chylothorax and progressed to bilateral chylothorax.
CASE PRESENTATION: A 26 year old female with episodic bronchial cast production and chronic hidradenitis suppurativa presented with two months of dyspnea, dry cough, and chest pain. Chest x-ray revealed a right sided pleural effusion and thoracentesis diagnosed a chylothorax. Workup for malignancy via bronchoscopy, laboratory evaluation, and PET-CT scan was unrevealing. An attempted lymphangiogram was unsuccessful. An open thoracotomy discovered a multifocal and multilevel chyle leak of the pleura and the thoracic duct which required a right sided thoracic duct mass ligation and mechanical pleurodesis. Post-operative radiography demonstrated a new loculated apical left chest effusion consistent with chylothorax post thoracentesis. Chylous chest tube output averaged about a liter per day for about 3 weeks despite conservative optimal medical management. Diagnosis was made at a specialized lymphatic center via MRI lymphangiogram which revealed definitive bilateral lung lymphangiomatosis with an accessory left-sided thoracic duct which underwent blood embolization. Post-embolization radiography demonstrated resolving effusions. Follow-up laboratory evaluation was notable for a mildly elevated vascular endothelial growth factor level of 168.
DISCUSSION: Lymphangiomatosis is an exceedingly rare diagnosis given the age and presentation of our patient. A biopsy can be helpful but her radiographic abnormalities, operative findings, and clinical course are consistent with lymphangiomatosis. Treatment aims at reduction of the lymphatic leak via diet, sclerotherapy, radiation, chemotherapy, and/or embolization of lesions. Case reports suggest interferon alfa-2b, bevacizumab, and propranolol may be helpful.
CONCLUSIONS: This case of lymphangiomatosis presented as a right sided chylothorax with clinical, operative, and radiographic findings consistent with lymphangiomatosis. Bilateral chylothorax developed despite surgical and medical management. Right sided surgical thoracic duct ligation and left sided accessory duct embolization resolved her chyle leak and symptoms.
Reference #1: Satria MN, et al. Pulmonary lymphangiomatosis. Lymph Res Biol. 2011;9(4):191-3.
DISCLOSURE: The following authors have nothing to disclose: William Londeree, Erik Osborn
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