Abstract
Acquired isolated hypoganglionosis is a rare intestinal neurological disease, which presents in adulthood with the clinical symptoms of chronic constipation. A 39-year-old man underwent laparoscopic low anterior resection and covering ileostomy for locally advanced-rectal cancer. A 6-month course of postoperative adjuvant chemotherapy was completed, followed by closure of the ileostoma. After the closure, he developed severe colitis which required 1-month of hospitalization. Mucosal erosions and pseudo-membrane formation were evident on colonoscopy and severe mucosal damage characterized by infiltration of inflammatory cells and crypt degeneration were pathologically confirmed. Even after the remission of the colitis, he suffered from severe constipation and distention. At 4 years after the stoma closure, he decided to undergo laparoscopic total colectomy. Histopathologically, the nerve fibers and ganglion cells became gradually scarcer from the non-dilated to dilated regions. Immunohistochemical staining examination confirmed that the ganglion cells gradually decreased and became degenerated from the normal to dilated region, thereby arriving at the final diagnosis of isolated hypoganglionosis. The patient recovered without any complications and there has been no evidence of any relapse of the symptoms. We present a case of acquired isolated hypoganglionosis-related megacolon, which required laparoscopic total colectomy, due to severe enterocolitis following stoma closure.
Highlights
Isolated hypoganglionosis is a hypogenetic variant of intestinal dysganglionosis characterized by the decrease and degeneration of ganglion cells in the lamina propria in the region of the colon and rectum [1]
A few adults who present with the clinical symptoms of chronic constipation, pseudoobstruction, and acute abdomen are diagnosed with an adult onset of acquired IHG (AIHG) [8, 9]
A nationwide survey over 10 years in Japan showed that only 8 (7.1%) of 112 patients with isolated hypoganglionosis were diagnosed with AIHG [3]
Summary
Isolated hypoganglionosis is a hypogenetic variant of intestinal dysganglionosis characterized by the decrease and degeneration of ganglion cells in the lamina propria in the region of the colon and rectum [1]. This rare disease represents about 3–5% of all intestinal neurological diseases, which presents with the clinical symptoms of chronic constipation and pseudo-obstruction [2]. A 39-year-old man was diagnosed with locally advanced lower rectal cancer He had no family history of bowel disease. Histopathological examination of the resected specimen revealed that the nerve fibers and ganglion cells were intact in non-dilated area and they gradually decreased from non-dilated to dilated regions (Fig. 5a–i). Immunohistochemical analysis of CD56 and S-100 showed a significant decrease in the number of neural cells in the Auerbach’s plexus
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