Evaluation of repeat colostomy biopsy in Hirschsprung’s disease patients with absent ganglion cells at the colostomy site

Objectives Hirschsprung’s disease (HD) is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. Many Indian centers prefer a two-stage procedure. In the first stage, biopsies are taken from the peritoneal reflection, transition zone (TZ), and colostomy site. There are instances of ganglion cells being absent at the colostomy site after the first surgery. This paper deals with the management of such cases. Material and Methods It was a retrospective observational study from April 2015 to 2023. All patients with HD confirmed by histopathological examination (HPE) based on hematoxylin and eosin (HE) staining and not having ganglion cells at the colostomy site were included. Results During the study period, there were 311 patients. Of these, 25 (8%) had the first report of absent ganglion cells at the affected and colostomy site. The mean age of the patients was 2.16 years. Of these, two patients had abdominal distension, which were managed by colostomy dilation and laxatives. The remaining patients had no such issues. Repeat HPE of the 23 asymptomatic patients showed the presence of ganglion cells. The remaining two patients underwent revision colostomy. Conclusion Repeat colostomy site biopsy in HD patients with absent ganglion cells at TZ and colostomy sites may be beneficial in places where facilities for frozen section and acetylcholinesterase staining are limited. We can also avoid laparotomies for multiple colonic biopsies.