Aganglionosis of the entire colon

Abstract

<h2>Summary</h2> Four patients with aganglionosis of the entire colon, one with extensive small bowel involvement, are presented. The patient in case 1 lived to age fourteen before a definitive diagnosis and surgical correction was achieved. This represents the oldest case reported to date. The incidence, sexual distribution, and familial occurrence are discussed. The usual clinical presentation is incomplete small bowel obstruction without evidence of megacolon. Radiographically, the diagnosis may be suspected but not proved. The diagnosis can only be made when pathologic examination of the bowel confirms the absence of ganglion cells. Operations for this disease should be staged, an ileostomy being performed with normal bowel at one operation and reconstitution being carried out by one of the several procedures at a later date. The over-all mortality is a dismal 67 per cent, but can be improved by suspecting and treating adequately any child first explored for obstruction or its complications in which no obvious mechanical cause is found.