Sigmoid Volvulus as a Clinical Manifestation of Acquired Colonic Hypoganglionosis: A Case Report

Abstract

A reduced and limited number of myenteric ganglia and low acetylcholinesterase activity in the lamina propria characterizes an unusual form of intestinal innervation disorder known as acquired or adult-onset hypoganglionosis. Only a few cases have been observed in adults, while the majority are diagnosed in infancy or youth. We report a rare case of colonic hypoganglionosis that presented as sigmoid volvulus in a 25-year-old female brought in to the ER. She underwent endoscopic decompression but developed a massive sigmoid volvulus with proximal colon dilatation. It was decided to do a total colectomy with an end ileostomy. The diagnosis was confirmed by histopathology, which revealed acquired hypoganglionosis. In order to prevent delayed or emergency presentation and the development of a stoma, the diagnosis of hypoganglionosis must be confirmed with full-thickness biopsies from all affected parts of the large bowel. Hypoganglionosis is rarely present, so young patients with a history of chronic constipation must be treated with a high index of suspicion.

Sigmoid volvulus and concomitant clinical entities

“…[78,79] Similarly, late-onset or acquired colonic hypoganglionosis in Auerbach plexus in intramuscular layers may also cause SV or SV-like disorders. [30,80] Neuronal intestinal dysplasia, which is characterized by hyperplasia of the plexus and hyperganglionosis, is another rare cause of SV. [81] Parkinson's disease leads to neuronal destruction in myenteric plexus in addition to the appearance of Lewy bodies, which prolongs the colonic transit.…”

“…[78,79] Similarly, late-onset or acquired colonic hypoganglionosis in Auerbach plexus in intramuscular layers may also cause SV or SV-like disorders. [30,80] Neuronal intestinal dysplasia, which is characterized by hyperplasia of the plexus and hyperganglionosis, is another rare cause of SV. [81]…”