Recurrent massive hemothorax in Rendu-Osler-Weber syndrome

We report a case of Rendu-Osler-Weber syndrome, occurring as sudden death after two episodes of massive hemothorax. Autopsy revealed massive hemothorax resulting from spontaneous rupture of one of three subpleural arteriovenous malformations. Review of the patient's hospital records showed that she had had a massive spontaneous hemothorax 13 years earlier that was managed conservatively. This case emphasizes the importance of early therapeutic (surgical or radiologic) intervention in the treatment of pulmonary arteriovenous malformations.