Surgical management of complicated sporadic pulmonary arterio venous malformation

The onset of a spontaneous hemothorax may represent a life threatening emergency. Several etiologies may be involved, mainly vascular abnormalities. Pulmonary Arterio Venous Malformation (PAVM) is a rare etiology, and it is often associated with Hereditary Hemorrhagic Telangiectasia HHT. We report the case of a young women who was presented to the emergency department with left basal chest pain and mild dyspnea. Hemodynamic and respiratory states were maintained. Vesicular murmuring was abolished on the left side. The chest roentgenogram showed a left pleural effusion with mild deviation of the mediastinum to the opposite side. Thoracocentesis found a serohematic liquid. While the patient was undergoing exploration by computed chest tomography, she represented a cardio respiratory arrest. Resuscitation was successful after few minutes. The chest CT showed a PAVM located on the upper lobe that was very enhanced after injection of the contrast product, with a feeding artery coming from the pulmonary artery. The patient underwent an emergent left upper lobectomy thorough a posterolateral thoracotomy. The PAVM was peripheral, large with a diameter of 5 cm, and ruptured on the pleural cavity. Elective controlling the hemorrhage was very difficult, so a total hilar clamping was done with a Satinsky clamp. The postoperative course was complicated with pulmonary embolism managed by anticoagulation treatment. The definitive histological management concluded to a PAVM. Retrospectively, there was no personal or familial sign of HHT, neither any anomaly of blood hemostasis. Isolated PAVM is a rare cause of spontaneous hemothorax. Rapid worsening of an initially maintained state makes the situation more critical. Rapid control of a massive bleeding is mandatory to better explore and plan surgical resection. For stable patient, endovascular treatment may represent an effective option.