Rare case of adult intestinal hypoganglionosis and review of the literature

Clin J Gastroenterol. 2021 Apr;14(2):599-607. doi: 10.1007/s12328-021-01342-5. Epub 2021 Jan 27.

Abstract

Intestinal hypoganglionosis is a rare condition in adults. We report a case of intestinal hypoganglionosis in the mid-distal transverse colon to splenic flexure in a 65-year-old female patient presenting with altered bowel habit and abdominal distension, and reviewed the current literature on this topic. Our patient had a medical history of neurofibromatosis type 1. A preoperative computed tomography (CT) scan demonstrated a grossly dilated transverse colon without obstruction. A laparotomy for subtotal colectomy was performed, with histopathology demonstrating intestinal hypoganglionosis.

Keywords: Colonic pseudo-obstruction; Hypoganglionosis; Intestinal hypoganglionosis; Intestinal innervation disorder.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Aged
  • Colectomy*
  • Dilatation, Pathologic
  • Female
  • Humans
  • Tomography, X-Ray Computed*