Intracranial aneurysm surgery in Ehlers-Danlos syndrome Type IV

Ehlers-Danlos syndrome Type IV is a heritable connective tissue disorder with frequent neurovascular manifestations, such as intracranial aneurysms. Patients with this syndrome have notoriously fragile blood vessels, and the reported mortality rate for any type of vascular surgical procedure is 40%. This syndrome is rare, however, and the complication rate of aneurysm surgery may have been overestimated.

We reviewed our experience with aneurysm surgery in a group of patients with Ehlers-Danlos syndrome Type IV.

The patient population consisted of three women and one man with a mean age of 44 years (age range, 20-57 yr). One patient, who had a ruptured anterior circulation aneurysm, died as a direct result of surgery because of marked vascular fragility. Three patients underwent successful surgery, consisting of a craniotomy and clipping of a ruptured anterior circulation aneurysm in two patients and a craniotomy and clip ligation of the parent artery in one patient with a ruptured dissecting vertebral artery aneurysm. Intraoperatively, mild vascular or connective tissue fragility was commonly observed. Postoperative complications (e.g., spontaneous pneumothorax and vertebral artery dissection) also were common but did not result in permanent morbidity.

The risk of neurovascular surgery is high in patients with Ehlers-Danlos syndrome Type IV and intra- and postoperative complications are common. However, most patients tolerate the operation without permanent morbidity.