Hirschsprung's disease: one surgeon's experience in one institution

The clinical experience of 137 patients treated by one pediatric surgeon at HanYang University Hospital over 12 years was analyzed. The sex ratio was 3.6:1 with male predominance. Neonatal Hirschsprung's disease (HD) occurred in 70 cases (51.1%). One hundred fourteen cases (83.2%) were short-segment and 23 cases (16.8%) were long-segment disease, of which 4 cases were total colon aganglionosis. Symptoms were abdominal distension, vomiting, constipation, and delayed passage of meconium. Family history was noted in 4 cases: 3 involved siblings and 1 involved relatives. All patients were male and had short-segment diseases. There were 3 pairs of monozygotic twins discordant for HD. Seventy-seven two-stage Duhamel operations were performed at the age of 121 +/- 41.3 days. Primary Duhamel operations were performed on 30 children. Anorectal myectomies were performed on 12 cases of short rectal aganglionosis. The incidence of enterocolitis was 25.5% preoperatively and 10.1% postoperatively. Complications after the Duhamel operation were intestinal obstruction (3), wound disruption (4), and septum reformation (3) with fecaloma formation in the blind rectal pouch. Bowel function was normalized within 3 months in 77.3% and within 1 year after operation in 91.6%. Duhamel operation with a GIA stapler after preliminary colostomy could be done at the age of 104 days on average with good functional results and with acceptable morbidity. Primary Duhamel operation could be done safely on the patients whose conditions were diagnosed after 90 days of life with good results. There was no mortality after Duhamel operation.