Multiple surgical problems in two patients with Ehlers-Danlos syndrome

Abstract

Ehlers-Danlos syndrome is a genetically determined disorder of connective tissue, the internal manifestations of which carry significant morbidity and mortality rates. During the past 14 years, we have treated multiple life-threatening surgical complications of this disease in two patients. The difficulties encountered at operation require modification of anesthetic and surgical techniques to accommodate the sometimes surrealistic situations that develop with alarming suddenness in patients with the Ehlers-Danlos syndrome. These techniques include handling the extremely friable tissues, dissecting and ligating vessels that disintegrate under the pressure of a hemostat, stemming spontaneous arterial hemorrhage, and special care in harvesting and placing split-thickness skin grafts.