Surgical pitfalls in patients with Ehlers-Danlos type IV: a case of spontaneous sigmoid perforation in a 17-year-old male

Asian J Surg. 2011 Jul;34(3):143-5. doi: 10.1016/j.asjsur.2011.08.001. Epub 2011 Oct 22.

Abstract

Ehlers-Danlos syndrome (EDS) is a group of well described connective tissue disorders in which collagen production is impaired. The surgical management of affected individuals remains challenging, with no general consensus. We report a case of spontaneous sigmoid perforation in a 17-year-old Eurasian male, in whom we subsequently established the diagnosis of EDS type IV (EDS-IV). We review the literature to discuss the clinical features and diagnosis, and the recommended therapeutic management.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Colectomy
  • Colostomy
  • Ehlers-Danlos Syndrome / complications*
  • Ehlers-Danlos Syndrome / diagnosis
  • Ehlers-Danlos Syndrome / surgery
  • Humans
  • Intestinal Perforation / diagnosis
  • Intestinal Perforation / etiology*
  • Intestinal Perforation / surgery
  • Male
  • Sigmoid Diseases / diagnosis
  • Sigmoid Diseases / etiology*
  • Sigmoid Diseases / surgery