Hemostasis Surgery of Spontaneous Hemothorax Complicating Neurofibromatosis Type 1

Abstract

Vascular lesions in Von Recklinghausen’s disease also known as Neurofibromatosis type 1 (NF1), are rare but have a fatal and potentially life threatening complications such as spontaneous hemothorax. An emergent thoracotomy is indicated when there is an active bleeding associated unstable hemodynamic status. Despites surgery is laborious and unpredictable but it have a merit to stop hemorrhage. A conservative management with endovascular embolization or non-operative approach have also been reported in case of hemodynamic stability. We report two case report of spontaneous hemothorax in patient with Recklinghausen disease. A chest tube was immediately inserted for two patients. Due to continuous bleeding and hemodynamic instability (Patient 1), and the increase of pleural effusion volume (Patients 1 & 2), emergent surgery of thorax was done with favourable post-operative follow up.