A 35-year-old man with Ehlers-Danlos syndrome type IV (EDS IV) underwent surgical repair of an enteroatmospheric fistula. Despite the substantially increased operative risk, repair was undertaken in view of his poor quality of life and severe nutritional deficits. Dense adhesions and extremely fragile bowel and vasculature characteristic of EDS IV were encountered intraoperatively. Multiple traction enterotomies and faecal matter leaking from suture holes necessitated leaving the abdomen open for a prolonged period. An Abdominal Reapproximation Anchor device was applied to prevent lateral retraction of the abdominal wall during this time. At relook on day 6, no leak was found, and the abdomen was closed. Two years postoperatively, the patient has an intact abdominal wall, with a vastly improved quality of life. This case illustrates the challenges of operating on patients with EDS IV, and presents a novel technique in managing fistulas in these patients.
- general surgery
- connective tissue disease
- gastrointestinal surgery
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Contributors KS was the treating Surgeon for the case described. She obtained the clinical photos used in the figures. She was involved in editing, proof-reading and supervising the case report. OZ was involved in writing and editing the report, obtaining patient consent and submission to the BMJ.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.