A case of intercostal aneurysm in association with von Recklinghausen's disease

Abstract

A 58-year-old female with von Recklinghausen's disease consulted her home doctor, complaining of sudden chest pain. Chest x-ray revealed pleural effusion in her left thoracic cavity and she was referred to our hospital. Bloody effusion was obtained by thoracocentesis, and enhanced chest CT and selective angiography demonstrated an aneurysm in the 8th intercostal artery. Rupture of the aneurysm was diagnosed and aneurysmectomy was performed. Fifty-seven cases of arterial aneurysm or laceration in association with von Recklinghausen's disease including our case have been reported in Japanese literature. Twenty-two patients had the lesions in the thoracic cavity and 8 of them died of bleeding. The mortality rate was the highest in lesions of the thorax. We should know that patients with von Recklinghausen's disease sometimes have vessel disease, which is rarely associated with life-threatening bleeding.