Disseminated intestinal hypoganglionosis treated by colectomy and tapering of the small intestine. A case report
Abstract
A girl suffering from chronic constipation and abdominal distension from her first year of life underwent internal anal sphincter myectomies at 5 and 7 years of age without resolution of her symptoms. At the age of 8, an ileostomy was performed because of excessive colonic dilation and hypomotility.
Biopsies from the colon and distal ileum showed intestinal neuronal dysplasia type B (INDB) with hypoganglionic areas. Colectomy and ileorectal anastomosis were done at the age of 10.
Three years later, however, an ileostomy was re-established because of recurrent episodes of pseudo-obstruction. In the hope of improving intestinal motility, the dilated small intestine was tapered over its entire length of 3.6 meters.
Histological findings still demonstrated oligoneuronal hypoganglionosis and INDB all along the resected strip of bowel wall. After 6 months, the stoma was closed.
At the age of 15 years, tapering of the distal 80 cm of the ileum was repeated in combination with cholecystectomy for cholecystolithiasis. Intestinal transit time decreased from 55 hours before the first to 18 hours after the second tapering procedure.
Now, 7 years after the last operation, the patient passes 3 - 4 soft stools daily, is physically active, on a normal diet and not on any regular medication.