[Hirschprung's disease in the Negev].
We treated 65 children with proven Hirschprung's disease between 1970-1992. After definitive surgery, 35 were over 10 years of age and 13 were over 18. The male:female ratio was 4:1. All but 3 were born full-term. 44% were of Bedouin origin, with a higher prevalence in 3 families of 2 tribes. 38 (58%) were diagnosed in the neonatal period: by barium enema and rectal muscle biopsy in 42 (65%), and by barium enema alone in 23 (35%). In the latter the diagnosis was verified by intra-operative biopsy. Severe constipation, intestinal obstruction or enterocolitis were the presenting features. 19 associated anomalies were found in 12 children, but none was life-threatening; 5 (8%) had cardiac anomalies; none had Down's syndrome. The rectosigmoid colon was the most common aganglionic segment involved (only 1 had total colonic aganglionosis). 7 of the 8 with short segment involvement responded well to posterior rectal myectomy. 55 patients had an abdominoperineal pull-through: 48 by Swenson's procedure and 5 by the Soave and 2 by the Duhamel modifications. In 43 a protective colostomy was performed at the end of the procedure. 53 had complete diversion colostomy at the time of initial diagnosis (neonatal and early infancy). There was no intra- or immediate post-operative death. 1 patient died 2 months after operation of complications following enterocolitis and total parenteral nutrition. 2 died a few hours after admission of severe sepsis due to enterocolitis before operation was possible. There were early postoperative complications in 11% of the 151 operations, mostly minor wound infections.(ABSTRACT TRUNCATED AT 250 WORDS)
Halevy
H
H
Dept. of Pediatric Surgery and Epidemiology, Soroka Medical Center, Ben-Gurion University, Negev, Beer Sheba.
Mares
A
A
Cohen
Z
Z
Finaly
R
R
Freud
E
E
Pilpel
D
D
D000293:Adolescent; D002648:Child; D003125:Colostomy; D005006:Ethnicity; D005260:Female; D005500:Follow-Up Studies; D006627:Hirschsprung Disease / Q000175:diagnosis / Q000208:ethnology / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D007557:Israel / Q000453:epidemiology; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology