Hirschsprung's disease presenting beyond infancy: surgical options and postoperative outcome.

BACKGROUND

Hirschsprung's disease (HD) presenting late is not unusual in developing nations.

AIM

To study surgical procedures and postoperative outcome in Hirschsprung's disease (HD) presenting after infancy.

METHOD

Records of 112 cases of confirmed HD who presented after infancy were analyzed for clinical presentations, investigations, surgical procedures and postoperative outcome. A Boley Scot pull through procedure was performed in all with or without a preliminary transverse colostomy.

RESULTS

The ages varied from 1 to 35 years. The rectosigmoid region was involved in all. Five cases presented with enterocolitis. Open biopsy and a diverting colostomy was performed in 91 cases (82%) with a colotomy in 4 for removal of fecalomas due to bowel dilatation on Barium study. This was followed by Boley Scot abdominal perineal pull through in 80 and Classical Soave in 11. Only 21 cases (18%) underwent single-stage endorectal pull through. Postoperative complications were encountered in 11/112 (9.8%).

CONCLUSION

82% children with HD presenting late required staged procedures due to dilated bowel. Single-stage pull through was feasible only in 18% cases. The postoperative complications were uncommon and managed successfully.