SESSION TITLE: Cardiothoracic Surgery Student/Resident Case Report Posters
SESSION TYPE: Student/Resident Case Report Poster
PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM
INTRODUCTION: Neurofibromatosis is a genetic condition of neurofibromas with only 32 cases of spontaneous aneurysmal ruptures from intrathoracic neurofibromatosis tumors managed by thoracotomy, video-assisted thoracoscopic surgery or coil embolization. We discuss a case of a 35-year-old female presenting with hemothorax from a ruptured right subclavian artery secondary to tumor involvement.
CASE PRESENTATION: We present a case of a previously healthy 35-year-old female with neurofibromatosis presenting with worsening chest pain and shortness of breath over one day. On examination, there was decreased breath sound on the right chest with dullness to percussion. An initial chest x-ray showed a large right pleural effusion and a chest tube was placed which drained one liter of sanguineous fluid. A CT chest with IV contrast raised suspicion for an aneurysmal right subclavian artery with active contrast extravasation. The patient’s hemodynamic condition deteriorated with worsening respiratory distress. The decision was made to operate emergently. An extended right posterolateral thoracotomy approach revealed a large hemothorax. After temporary control of active bleeding, she was found to have large rupture of distal right subclavian aneurysm. The aneurismal bleeding was controlled by pressure and the ruptured artery was oversewn incorporating pleural tissue followed by pledgeted sutures. Postoperative CT chest demonstrated complete resolution of the hemothorax and no evidence of bleeding. The patient was discharged home in stable condition on postoperative day 7.
DISCUSSION: Neurofibromatosis (NF) is a tumor disorder that rarely presents in the thoracic cavity with a suggesed incidence of 3.6%. In the literature, there are 32 cases of spontaneous hemothorax involving the subclavian, intercostal, thyrocervical trunk, and internal thoracic artery. Studies have attributed mesodermal dysplasia as the case for NF vascular weakness, while others suggest that direct tumor invasion causes ischemic wall weakness and aneurysmal dilatation. There have been cases of a subclavian artery rupture repaired with collagen fleece and direct ligation with video-assisted thoracoscopic surgery and ruptured costo-cervical arteries in more stable patient successful coil embolized. In our case, we used open thoracotomy to successfully control the aneurismal bleed and emergently save the patient’s life.
CONCLUSIONS: There are various ways to manage an intrathoracic arterial rupture from a NF tumor. Depending on the patient’s hemodynamic stability and the surgeon’s expertise, the ruptured aneurysm can be repair primarily via open thoracotomy and VATS or coil embolized.
Reference #1: Miyazaki T, Tsuchiya T, Tagawa T, Yamasaki N, Nagayasu T. Spontaneous hemothorax associated with von Recklinghausen's disease. Ann Thorac Cardiovasc Surg. 2011;17(3).
Reference #2: Hoonjan B, Thayur N, Abu-Own A. Aneurysmal rupture of the costo-cervical trunk in a patient with neurofibromatosis type 1. Int J Surg Case Rep. 2014;5(2).
DISCLOSURE: The following authors have nothing to disclose: Bo Gu, Sean Kwon
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