We report a case of spontaneous hemothorax in a 48-year-old woman with Neurofibromatosis type 1 (NF1), presumed to result from rupture of an intrathoracic meningocele. She had been hospitalized for treatment of an intracranial carotid artery aneurysm and underwent endovascular embolization. Preoperative body CT demonstrated a meningocele protruding into the left thoracic cavity at the level of the T3/T4 vertebrae. On postoperative day 6, she experienced sudden cardiopulmonary arrest and died. Postmortem CT revealed a massive left hemothorax and an acute high-density hematoma within the intrathoracic meningocele. Autopsy findings were consistent with hemothorax likely caused by rupture of the meningocele. NF1 is associated with intrathoracic meningoceles and vascular fragility. The acute hematoma seen within the meningocele on postmortem CT suggested meningocele rupture as the source of bleeding, illustrating a rare but characteristic complication of NF1.
Ikebe et al. (Wed,) studied this question.