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Non-operative management of diverticular perforation in a patient with suspected Ehlers–Danlos syndrome

Authors :
J. Hanaghan
I. Robertson
Kevin Barry
Waqar Khan
M. C. Casey
Peadar S. Waters
Source :
International Journal of Surgery Case Reports. 5:135-137
Publication Year :
2014
Publisher :
Elsevier BV, 2014.

Abstract

INTRODUCTIONNo 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 CASEA 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.DISCUSSIONThis is the first reported case of successful conservative management of colonic diverticular perforation in a patient with classical Ehlers–Danlos syndrome.CONCLUSIONEDS 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.

Details

ISSN :
22102612
Volume :
5
Database :
OpenAIRE
Journal :
International Journal of Surgery Case Reports
Accession number :
edsair.doi.dedup.....1cce0276affccf601b773dd863f6803a