It represents a clinical analysis of 123 patients of Hirschsprung's disease whose surgical management was performed by one pediatric surgeon at the Division of Pediatric Surgery of Hanayang University Hospital during 11 years from January 1980 to
December 1990.
@ES Ther results were as follows;
@EN 1) Sex ratio was 3.6 : 1 with male preponderance.
2) Neonatal Hirschsprung's disease was 70 cases(56.9%), and 99 cases(80.5%) were diagnosed within 1 year of age.
3) The presenting symptoms were delayed or no passage of meconium(65%), abdominal distension and constipation. On the contrary, normal passage of meconium within 24 hours of age was also noted in 24 cases, and diarrhea was noted in 20 cases.
4) The associated anomalies were detected in 21 cases(17%) among all cases and chromosomal aberrations were detected in 6 cases(14.6%) among 41 study cases.
5) The aganglionic segment was rectum and sigmoid colon in 108 cases(86.2%), extended above sigmoid colon in 17 cases(13.8%) among which 4 cases extended into ileum.
6) Diagnosis was made by history taking, physical examination, simple abdominal lateral X-ray, barium colon study, and rectal wall biopsy.
7) Neonatal Hirschsprung's disease cases underwent preliminary colostomy and ileostomy as first stage management, and definitive corrective surgery was done around the age of 4 months.
8) One hundred and five cases were treated by Duhamel operation(using GIA autosuture in 103 cases and Kocher clamps in 2 cases). Among those, 26 cases presented mostly after 3 months of age were treated by one stage Duhamel operation. Two cases
of
total colonic aganglionosis were treated by modified Duhamel operation(Martin's operation), 3 cases by Soave operation. 1 case by Swenson operation, and 6 cases by myomectomy. Three reoperation cases after failed Duhamel operation were managed by
Duhamel operation, one reoperation case was managed by Soave operation.
9) The average interval between preliminary colostomy or ilecostomy and definitive operation was 112 days.
10) The incidnce of enterocolitis was 26% in preoperative period and 19.7% after definitive operation.
11) The complications after definitive operation were septum adhesion(8 cases), intestinal adhesion(3 cases) and fecaloma in blind rectal pouch(2 cases).
12) Among all study cases, only one total aganglionosis coli case was died after ileostomy due to electrolyte imbalance.
13) The frequency of bowel movement was normalized within 1 month after operation in 88 cases(75.2%), but 5 cases(4.2%) required more than 6 months after definitive operation. None had fecal incontinence problem.
The average age and body weight at the time of definitive Duhamel operation usins GIA autosuture after preliminary colostomy in cases of neonatal Hirschsprung's disease were 4 months 7 days and 7.2 kg. According to the results, therefore, we
suggest
that the definitive operation for neonatal Hirschsprung's disease could be done during 3 months of age without any problem. On the contrary, Hirschsprung's disease patients after 3 months of age could by treated by one stage Duhamel operation
after
colonic decompression conservative measure.
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