Experience in the surgical management of Hirschsprung's disease.

ABSTRACT

The clinical presentation, surgical treatment and results in a group of 126 patients with histologically proven Hirschsprung's disease are presented. This study has enabled the authors to identify various problems and complications associated with the management of this condition. The typical clinical picture in the neonate has been observed in only 45.2% of our cases. In the long segment Hirschsprung's, especially those presenting with bowel perforation, the precise localisation of the aganglionic segment has been difficult. The standard surgical procedure in our department has been the mucosal-stripping endorectal pull-through operation. The problems of prolapse, stricture and cuff abscess associated with this procedure are, related to specific technical aspects of the operation which we believe have not been emphasised previously. Our current experience with this type of procedure has enabled us to carry out definitive surgery in early infancy without preliminary colostomy. The results justify our confidence that this approach to the surgical management of neonatal Hirschsprung's disease will become the standard procedure in our practice. An unusual feature has been the relatively common occurrence of the short segment type of Hirschsprung's disease--30.2% with rectal aganglionosis and 7.1% with ultrashort segment disease. This group has posed a dilemma as to the selection of the most appropriate surgical procedure. Rectal myectomy, which was the operation of choice, has given excellent results in some cases and yet in other cases it has been unsatisfactory, leading to the development of very gross megacolon.