Segmental Hypoganglionosis of the Colon: A Case Report

Abstract

A 64-year-old female was admitted to the hospital with constant abdominal pain. She had been previously hospitalized five times in 2 years with similar symptoms. Computed tomography revealed dilatation and fecal impaction from the ileum to the transverse colon. A barium enema and simultaneous ileus tube radiography demonstrated a segment of narrow descending-sigmoid colon. Colonoscopy did not show any mucosal change. These symptoms did not improve with conservative therapy when a descending and sigmoid colectomy was performed. In terms of histology, there was a disappearance of ganglion cells, and axon of Meissner's plexuses was present along with a decrease in the number of Auerbach's plexuses. The definite diagnosis given was segmental hypoganglionosis (SH) of the colon. The postoperative course was uneventful, and the functional result was positive following 1 year postoperatively. SH is extremely rare; however, surgical intervention is expected to be of benefit. Therefore, it is important to keep SH in mind when treating patients with chronic obstruction in the left side of the colon as it can cause SH.

Idiopathic Megacolon—Short Review

“…Changes in Cajal interstitial cell numbers are described in patients with idiopathic megacolon or chronic constipation by numerous studies and suggested as an etiopathogenic basis [18][19][20][21][22][23][24]. They are considered a true intestinal pacemaker ("little brain" at the enteral level); however, starting from the premise that their number decreases physiologically with age anyway and by the fact that a number of other communications do not confirm their involvement in the etiopathogenesis of idiopathic megacolon, this theory remains for the moment only a new subject of controversy [8].…”