Abstract
A 14-year-old boy was admitted to our center with sudden back pain and impending rupture of an abdominal aortic aneurysm was diagnosed by computed tomography (CT) scan. He underwent urgent replacement of the abdominal aorta. The patient had been evaluated for a cerebral aneurysm at 7 years of age and hypertension at 13 years of age. He also had a renal artery aneurysm. Post-operatively, the disease was identified as type IV Ehlers-Danlos syndrome (EDS) based on the multiple aneurysms, thin skin with visible veins, and loose joints.
In type IV EDS extreme fragility of the arteries is associated with multiple aneurysm formation, spontaneous rupture, and dissection. Most deaths result from arterial rupture. Patients with type IV EDS should probably be evaluated at regular intervals by CT and related techniques for early detection of aneurysms.
