Massive Hemothorax by Ruptured Arteriovenous Malformation

Introduction and objectives: Osler Weber Rendu disease is a rare genetic disorder characterized by multiple telangiectasias and arteriovenous malformations involving parenchymatous organs, leading to hemorrhagic, sometimes life-threatening vascular complications. The most often involved regions are the central nervous system and lungs, but the disease can also manifest at hepatic, intestinal, splenic or urinary level. The objective of this article is to raise the awareness over an extremely severe complication of Osler Weber Rendu and the importance of an early diagnosis. Materials and methods: We present the case of a 65 years old woman with Osler Weber Rendu with many complications, who was hospitalized and surgically treated for massive hemothorax and hemorrhagic shock by rupture of a pulmonary vascular malformation. The patient presented for latero-thoracic and anterior chest pain with a sudden onset in the last 24 hours, which was followed by progressive dyspnea, being further diagnosed with massive right hemothorax. Results: After the emergency surgical treatment which consisted of inferior bi-lobectomy, the evolution was a difficult one, the recovery of the patient being slowed down by her altered neurological status and the presence of a persistent bronchial stump fistula. Her disease was complicated by a post transfusion syndrome which aggravated the respiratory failure and necessitated the initiation of cortico-therapy and also by the colonization and subsequent infection of the respiratory tract with multi resistant microorganisms. The patient died on the 32nd postoperative day. Conclusions: Our patient had in her personal medical history few complications of Osler Weber Rendu disease, but these manifested as non-specific symptoms which were not further investigated in order to early establish the diagnosis of the disease. Therefore, the importance of the disease was overridden as time passed and the diagnosis was delayed, which prevented any therapeutic measures of the associated life-threatening complications, as is in this case the massive hemothorax.