Management of Hirschsprung disease: A clinical review

Abstract

A retrospective analysis was made of 45 patients admitted from January 1984 to 1996. 34 patients were male and 11 were female, yielding an overall sex ratio of 3:1. The age at diagnosis was younger than 30 days in 14 neonates (31 %), 1 month to 1 year in 11 infants (24%), and older than 1 year in 20 children (45%). Associated abnormalities were seen in 6 cases (13%) including Down syndrome in four, VSD+PS+situs inversus and VSD+ASD in one case. Clinical presentation in HD included constipation, abdominal distantion, failure to thrive, billious vomitting, fecal soiling and encopresis. Delayed passage ofmeconium in 48 hours of life was the cardinal symptom in neonates. Hirschsprung enterocolitis occurred in four cases. Definitive operations were performed in 41 infants, and children. Modified Duhamel procedure was used in 21 patients. Eight patients underwent anorectal myectomy, seven patients underwent Swenson operation and five patients underwent Soave-Boley operation. Follow up evaluation was performed monthly for the first 3 months, every 3 months for 1 year and every 6 months thereafter until normal bowel function was achieved. Normal bowel function was defined as bowel movement 2 to 3 times a day without soling.