Spontaneous rupture of an aneurysm of the right subclavian artery as a first presentation of Ehlers Danlos syndrome in a 15-year old boy.

AbstractWe report a case of a spontaneous rupture of a right subclavian aneurysm in a 15 year-old patient. This ruptured aneurysm was successfully treated in an endovascular manner by placing a covered stent-graft in the right subclavian artery via right brachial access. Subsequent work-up by skin biopsy and fibroblast culture and by DNA- screening revealed the diagnosis of Ehlers Danlos type IV. Meanwhile, the patient developed twice a spontaneaous pneumothorax, treated with thoracoscopic pleurodesis.This article provides a clear overview of the clinical and genetic characteristics of a case of Ehlers Danlos type IV and illustrates the importance of avoiding surgery in patients with connective tissue disease because of the high risk of perioperative complications.