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.