Introduction
This case report describes the perioperative planning of a woman with Ehlers-Danlos syndrome (EDS) type IV, which increased the risk of surgical bleeding complications, requiring close medical and surgical collaboration. Paramount to success was the meticulous planning of the operation with respect to minimising the risk of anticipated complications that stem from increased tissue friability.
EDS defines a group of genetic connective tissue disorders characterised by fragile skin, easy bruising, spontaneous rupture of arteries and joint dislocation1. EDS has multiple surgical risk factors described in the literature including a high risk of visceral perforation and aneurysm rupture2,3. The overall incidence of Ehlers-Danlos is approximately 1 in 5,000 people2. EDS subtype IV (EDS4) accounts for approximately 5% of all cases4.
EDS4 refers to an autosomal dominant vascular subtype caused by mutations of the COL3A1 gene5 causing a deficit of type III collagen. Type III collagen is a constituent of arterial walls and the digestive tract predisposing EDS4 patients to vascular and digestive ruptures.
We report a case of a patient with EDS4 who required an elective cholecystectomy for gallstone disease. EDS4 patients with gallstone disease are at an increased risk of requiring emergency surgery, therefore elective cholecystectomy is indicated5. However, minimal published information is available to the surgeon to help plan such cases6,7.
Collaboration between upper gastrointestinal and vascular surgeons, cardiologists and anaesthetists was important in the planning of the surgery to reduce complication risk. The case identifies important considerations in planning elective operations in patients with EDS4 which may help guide clinicians who deal with similar cases in the future.