Management and outcome Of Hirschsprung’s Disease, in Soba University Hospital

Abstract

Introduction: Hirschprung's disease (HD) is a congenital disease resulting from failure of caudal migration of ganglion cells in Auerbach’s and Meissner’s plexuses, resulting in intestinal obstruction. Objectives: To evaluate Sudanese patients with HD, their presentation, diagnosis and treatment. Methods: A descriptive retrospective and prospective study was performed in 40 patients with HD, at Soba University Hospital, from January 1999 to March 2003. Data tabulated included personal data, presentation, family and drug history, abdominal examination, investigations, treatment and outcome. Results: The main symptoms are failure to pass meconium in first 24 hours of life in 36 patients (90%), constipation in 36 patients (90%) and abdominal distension in 38 patients (95%). Swenson’s procedure was carried in all patients, preceded by a loop colostomy, which was transverse in 7 patients (17.5%), and sigmoid in 33 patients (82.5%). In 13 patients (32.5%) postoperative complications occurred. Enterocolitis occurred in 5 patients (12.5%) post-operatively, anastomotic stricture in 2 patients (5%), wound infection in 7 patients (17.5%) and stool incontinence in 2 patients (5%). Death occurred in 2 patients (5%), both developed postoperative enterocolitis, one of them had Down's syndrome. Conclusion: The treatment of HD is surgery and can be done early.