ADULT HIRSCHSPRUNGS DISEASE A SUSPECT IN REFRACTORY CONSTIPATION

Abstract

Hirschsprungs disease (HD) in adults is rare and often underdiagnosed or misdiagnosed. We report a case of HD in a 43 year old male who presented with chronic            constipation since 18 years of age. He had undergone             emergency laparotomy and sigmoid resection in the past in another centre. He developed severe abdominal pain and distention for three months duration. A plain X-ray of the  abdomen and computed tomographic scan of the abdomen showed markedly dilated colon and rectum with faecal loading and transition zone at the recto-sigmoid portion. A barium enema confirmed similar findings. A rectal biopsy showed hypertrophic nerve bundles and paucity of ganglion cells  suggestive of Hirschsprungs disease. Because of the severe constipation that was refractory to medical management and poor nutrition he initially underwent a diversion loop  transverse colostomy. Few months later he underwent a  definitive procedure of low anterior resection. The colon was grossly dilated with faecoloma, but the rectum was collapsed. Histologic examination showed excised colorectal segment with hypertrophied nerve bundles and absent ganglion cell in the distal segment. His postoperative recovery was uneventful with complete resolution of symptoms. Hirschsprungs disease should be suspected in adults with long-standing and               refractory constipation.