Immature colonic ganglion cells as a cause of megacolon in infancy: case report

Abstract

Congenital megacolon or Hirschsprung’s disease was fi rst described by Harold Hirschsprung. It is a congenital intestinal aganglionosis as a result of arrested fetal development of the myenteric nervous system. Normal intestinal motility depends on a coordinated segmental contraction waves followed immediately by smooth muscle relaxation as it propagates caudally. Patients with Hirschsprung’s disease lack functional myenteric nervous system in the aff ected distal intestine and have ineff ective distal peristalsis. The clinical outcomes are failure to pass meconium shortly after birth, constipation, abdominal distension, palpable loops of bowel, vomiting, watery diarrhea in the newborn, poor weight gain, slow growth and malabsorption. The aganglionic distal segment of the bowel is the reason for dilatation of the proximal part of the colon or opening debility of the anal sphincter system. Hirschsprung’s disease is aff ecting 1:5000 to 1:8000 live births. Most cases are diagnosed before the patient is 10 years of age. Occasionally, patients present with this problem at a later age. Recently we operated on a 12-year-old patient with this condition. First we performed surgical resection of the rectosigmoid colon with closure of the rectal stump and formation of an end colostomy, also known as Hartmann’s procedure. Three months later, the rectum was extirpated and coloanal anastomosis formed. Based on the uneventful postoperative course, good patient’s condition and clinical tests, the treatment of congenital megacolon was successful.