Non-operative management of diverticular perforation in a patient
with suspected Ehlers–Danlos syndrome!
M.C. Caseya, I. Robertsona,!, P.S. Watersa, J. Hanaghanb, W. Khana, K. Barrya
a Department of General Surgery, Mayo General Hospital, Ireland
b Department of Diagnostic and Interventional Radiology, Mayo General Hospital, Ireland
a r t i c l e i n f o
Article history:
Received 16 September 2013
Accepted 18 December 2013
Available online 8 January 2014
Keywords:
Ehlers–Danlos syndrome
Colonic perforation
Conservative management
a b s t r a c t
INTRODUCTION: No consensus exists regarding definitive management of colonic perforation in
Ehlers–Danlos syndrome (EDS), with various authors advocating different operative techniques. Spontaneous
colonic perforation is a recognised complication of vascular-type EDS (type IV), with many reported
cases in the literature. No such cases have been reported concerning classical-type EDS (type I/II).
PRESENTATION OF CASE: A 55-year-old male with a family history of EDS presented with acute lower
abdominal pain and signs of localised peritonitis. Following resuscitation, computerised tomography
identified perforation of a sigmoid diverticulum with localised intraperitoneal air. Considering the
potential complications associated with laparotomy in a patient with EDS, a trial of conservative management
was undertaken including image-guided drainage of a mesenteric abscess. Intensive care
monitoring, nutritional support and intravenous antibiotics also facilitated successful non-operative
management. Following discharge, molecular studies confirmed COL5A1 mutation, and a diagnosis of
classical Ehlers–Danlos syndrome was established.
DISCUSSION: This is the first reported case of successful conservative management of colonic diverticular
perforation in a patient with classical Ehlers–Danlos syndrome.
CONCLUSION: EDS is highly significant in the surgical context, with the causative genetic factors serving
to further complicate the course of surgical intervention. In the absence of consensus regarding best
surgical management, due consideration should be given to non-operative management of benign colonic
perforation.
