Unusual Cause of Spontaneous Hemothorax

Abstract

INTRODUCTION: Hemothorax is mostly related to closed or open chest trauma or procedures in relation to chest wall. Spontaneous hemothorax is much less common. Here we describe a patient who presented with spontaneous massive hemothorax, a methodical search for etiology led to the diagnosis. CASE PRESENTATION: A 56 year old Hispanic female, with history significant for recurrent epistaxis since the age of 10 was electively admitted by her gastroenterologist for evaluation of iron deficiency anemia. She had received multiple blood transfusions and embolization of the left maxillary artery for the control of epistaxis. During her current admission, while undergoing upper esophagogastroduodenoscopy she developed massive epistaxis requiring immediate tracheal intubation for protecting her airways, resuscitation with blood products and nasal tamponade for controlling the bleed. She remained intubated in the medical intensive care unit and on day 5 of her hospital stay she developed acute respiratory distress. Repeat chest roentgenogram showed complete opacification of the right hemithorax associated with a significant drop in hemoglobin from 11.9 to 8.5 gm/dl. Bedside ultrasound showed a large pleural effusion and a diagnostic thoracentesis revealed frank blood. A large bore chest tube was inserted and 850 cc of blood was drained. CT angiogram of the chest revealed a bilobed 2 x 1 cm lesion in the right lower lobe with an afferent vessel, highly suspicious for pulmonary arterio-venous malformation (PAVM). Comparison made to the prior scans from 2005 revealed the same abnormality. She underwent a transcatheter coil embolization of PAVM. A definite diagnosis of Hereditary hemorrhagic telangiectasia is made given by the presence of nasal telangiectasia, recurrent epistaxis and identification of the pulmonary arterio-venous malformation. She recovered during the hospital stay and discharged with plans to screen the relatives for this condition. DISCUSSION: Hereditary hemorrhagic telangiectasia (HHT) also called Osler Weber Rendu syndrome is an autosomal dominant disease and occurs in 1 in 5000 people. Gene mutations involving endoglin, ALK-1 and Smad-4 which play a role in TNF-beta superfamily signaling have been described1. Common pulmonary complications of HHT include PAVM and pulmonary hypertension. PAVM can manifest as dyspnea, cyanosis, cough, palpitation, chest pain or hemoptysis. Rupture of PAVM causing hemothorax is rare manifestation and can be fatal. Embolotherapy is the mainstay of treatment which has a success rate of 75%. CONCLUSIONS: Spontaneous hemothorax is a rare manifestation of Hereditary hemorrhagic telangiectasia which could be fatal. Rapid identification and appropriate treatment can be life saving.