Hirschsprung's Disease

Abstract

Abstract Experience with 38 cases of Hirschsprung's disease seen over a 20-year period is described. Only 24 per cent of cases presented with a combination of symptoms and signs such as to suggest the correct diagnosis on clinical grounds. Barium-enema examination carries a high ‘true-positive’ rate, but cases reported as negative must be viewed critically. A radiological diagnosis of idiopathic megacolon should not be accepted without supplementary rectal biopsy and/or anorectal pressure studies. Survival without surgery is compatible with Hirschsprung's disease, but the dangers of enterocolitis must be acknowledged. Rectosigmoidectomy, which includes resection to an area of normal innervation proximally and excision of part of the internal sphincter of the rectum distally, produces a satisfactory late functional result at an acceptable mortality-rate. A colostomy prior to rectosigmoidectomy is advisable when there is a history of repeated attacks of subacute obstruction and one or more episodes of enterocolitis. A colostomy after rectosigmoidectomy is advisable in those cases which are submitted to laparotomy for intestinal obstruction in the period following operation, even when no mechanical cause for obstruction is demonstrable. Patients with problems relating to coprostasis following treatment of congenital anorectal disorders should be submitted to rectal biopsy, since a proportion of these patients reveal a deficiency of innervation of the distal large bowek which approximate to that seen in Hirschsprung's disease.