HIRSCHSPRUNGS-DISEASE - ONE SURGEONS EXPERIENCE IN ONE INSTITUTION

The clinical experience of 137 patients treated by one pediatric surge on at HanYang University Hospital over 12 years was analyzed. The sex ratio was 3.6:1 with male predominance. Neonatal Hirschsprung's diseas e (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, o f which 4 cases were total colon aganglionosis. Symptoms were abdomina l distension, vomiting, constipation, and delayed passage of meconium. Family history was noted in 4 cases: 3 involved siblings and 1 involv ed relatives. All patients were male and had short-segment diseases. T here were 3 pairs of monozygotic twins discordant for HD. Seventy-seve n two-stage Duhamel operations were performed at the age of 121 +/- 41 .3 days. Primary Duhamel operations were performed on 30 children. Ano rectal myectomies were performed on 12 cases of short rectal aganglion osis. The incidence of enterocolitis was 25.5% preoperatively and 10.1 % postoperatively. Complications after the Duhamel operation were inte stinal obstruction (3), wound disruption (4), and septum reformation ( 3) with fecaloma formation in the blind rectal pouch. Rowel function w as normalized within 3 months in 77.3% and within 1 year after operati on in 91.6%. Duhamel operation with a GIA stapler after preliminary co lostomy could be done at the age of 104 days on average with good func tional results and with acceptable morbidity. Primary Duhamel operatio n 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. Copyright (C) 1995 by W.B. Saunders Company