2016 Volume 30 Issue 1 Pages 80-86
A 60-year-old man presented with sudden right chest pain while sleeping. He had past medical histories of left pneumothorax, colon perforation, and 3 episodes of abdominal arterial rupture. Computed tomography (CT) revealed right intercostal arterial aneurysms, some of which had ruptured and created a large hematoma in the right chest wall. He gradually developed massive hemoptysis. We successfully controlled the active bleeding with transarterial embolization for intercostal arteries. However, minor bleeding in the right bronchus intermedius continued as oozing, caused by a fistula connected with the lung and airway with hematoma. We performed thoracotomy to relieve the hematoma, repair the fistula, and arrest the airway bleeding. Although we successfully controlled the airway bleeding, this patient died from cardiac failure on the 29th hospital day. We finally diagnosed the patient with vascular type Ehlers-Danlos syndrome postmortem. Ehlers-Danlos syndrome (EDS) is an uncommon inherited disorder of connective tissue. Vascular-type EDS results from mutations in the gene of type III procollagen and is the most serous type of EDS. Affected patients are at risk of rupture of the arteries, bowel, and uterus. When we encounter patients with arterial rupture, gastrointestinal perforation, and pneumothorax, we should be aware of the possible presentations of EDS and need to perform urgent and appropriate investigations and treatments.
