Visceroptosis of the bowel in the hypermobility type of Ehlers-Danlos syndrome: presentation of a rare manifestation and review of the literature

Eur J Med Genet. 2012 Oct;55(10):548-51. doi: 10.1016/j.ejmg.2012.06.012. Epub 2012 Jul 7.

Abstract

Gastrointestinal complications are common in patients with Ehlers-Danlos syndrome, affecting up to 50% of individuals depending on the subtype. The spectrum of gastrointestinal manifestations is broad and ranges from life threatening spontaneous perforation of the visceral organs to a more benign functional symptoms. Here we describe the clinical and radiographic manifestations of visceroptosis of the bowel, a rare complication of Ehlers-Danlos syndrome that is characterized by prolapse of abdominal organs below their natural position. We further review the literature on gastrointestinal complications in the different forms of Ehlers-Danlos syndrome.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Adult
  • Ehlers-Danlos Syndrome / complications*
  • Female
  • Humans
  • Lower Gastrointestinal Tract / diagnostic imaging
  • Radiography
  • Upper Gastrointestinal Tract / diagnostic imaging
  • Visceral Prolapse / diagnosis*
  • Visceral Prolapse / diagnostic imaging
  • Visceral Prolapse / etiology*

Supplementary concepts

  • Ehlers-Danlos syndrome type 3