Massive hemothorax: an exceptional complication of Rendu-Osler-Weber disease
Hereditary familial telangiectasis is a vascular dysplasia characterized by a triple syndrome: hereditary character, telangiectasias and hemorrhages. Its association with systemic and/or pulmonary arterio-venous fistulae is frequent and can condition several complications: cerebral abscess, digestive hemorrhages, epistaxis, hemoptysis, hypoxemia, polyglobulia, and rarely the appearance of hemothorax. We describe a patient who was admitted in hospital with a hypovolemic shock secondary to a massive hemothorax; during emergency surgery, which was necessary to perform, a pulmonary A-V fistula was observed in the middle lobe. The studies afterwards performed demonstrated that it was a Rendu-Osler-Weber disease with duodenal, hepatic, and cerebral involvement.