Anaesthetic Implications of Vascular Type Ehlers-Danlos Syndrome

Abstract

Vascular type Ehlers-Danlos syndrome is an inherited connective tissue disease, which is typified by tissue fragility, joint hypermobility, a tendency to bleed excessively and rupture of the uterus, the bowel and arteries. Two case reports are presented which describe the anaesthetic management of patients with spontaneous bowel perforations due to vascular type Ehlers-Danlos syndrome. Both cases were associated with relatively minor problems. The implications of this condition for anaesthesia include difficulty (and risk of complications) with vascular access, a risk of spontaneous arterial rupture in the perioperative period, possible cervical spine instability and airway trauma on intubation. There is also a risk of excessive bleeding, potentially difficult neuraxial anaesthesia and an increased risk of epidural haematoma. There also may be associated cardiac or pulmonary disease. However, these two cases and the limited previously published literature suggest that such potential complications are actually rare and that anaesthesia may be conducted in a relatively standard manner in most circumstances.