Gastrointestinal Vascular Malformations in Patients with Turner's Syndrome: A Systematic Review of Case Reports

Horm Res Paediatr. 2018;90(1):39-43. doi: 10.1159/000490425. Epub 2018 Jul 24.


Background/aim: There is a paucity of data on gastrointestinal (GI) vascular abnormalities in patients with Turner's syndrome (TS). Therefore, this literature review aimed to characterize the nature of GI vascular malformations in patients with TS, their localization in the GI tract, and their clinical and laboratory manifestations.

Methods: A systematic search for articles was conducted using Medline and Embase (until August 2017). A manual search of the references of the included papers was performed to identify any potential additional references. We also conducted a retrospective chart review of all patients with TS and GI bleeding who were hospitalized at our institution.

Results: A total of 29 articles published between 1947 and 2015 that described 39 cases were reviewed. Additionally, we included 2 patients who were hospitalized at our institution. The median age of patients with TS was 15 years (range: 0.1-57 years). Iron deficiency anaemia (35/40), haematochezia (15/37), and melaena (14/36) were the most common symptoms. Abnormal GI vessels occurred throughout the entire bowel, with a predilection for the small intestine (72%, 29/40). The most common abnormal vessels (65%, 24/37) were telangiectasias and dilated veins.

Conclusion: Telangiectasias and dilated veins of the small intestine were the most commonly seen abnormal GI vessels in patients with TS.

Keywords: Abnormal vessels; Gastrointestinal bleeding; Telangiectasia; Vascular abnormalities.

Publication types

  • Systematic Review

MeSH terms

  • Adolescent
  • Adult
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Dilatation, Pathologic
  • Female
  • Gastrointestinal Tract* / abnormalities
  • Gastrointestinal Tract* / blood supply
  • Gastrointestinal Tract* / pathology
  • Gastrointestinal Tract* / physiopathology
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Telangiectasis* / pathology
  • Telangiectasis* / physiopathology
  • Turner Syndrome* / pathology
  • Turner Syndrome* / physiopathology