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[Hirschprung's disease in the Negev]
1Dept. of Pediatric Surgery and Epidemiology, Soroka Medical Center, Ben-Gurion University, Negev, Beer Sheba.
Insights
This study analyzed 65 children with Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Hirschsprung's disease is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
- It affects approximately 1 in 5,000 live births, with a higher incidence in males.
- Early diagnosis and surgical intervention are crucial for managing complications such as enterocolitis and intestinal obstruction.
Purpose of the Study:
- To review the clinical presentation, diagnostic methods, surgical management, and outcomes of Hirschsprung's disease in a cohort of children.
- To evaluate the prevalence and associated anomalies in Hirschsprung's disease.
- To assess the effectiveness of different surgical procedures for Hirschsprung's disease.
Main Methods:
- Retrospective analysis of 65 children diagnosed with Hirschsprung's disease between 1970 and 1992.
- Review of patient records including demographics, clinical features, diagnostic tests (barium enema, rectal muscle biopsy), surgical procedures, and postoperative outcomes.
- Surgical interventions included posterior rectal myectomy, Swenson's procedure, Soave modification, and Duhamel modification.
Main Results:
- The male:female ratio was 4:1, with 44% of patients of Bedouin origin.
- Common presenting features were severe constipation, intestinal obstruction, and enterocolitis.
- The rectosigmoid colon was the most frequent aganglionic segment; posterior rectal myectomy showed good results for short-segment disease.
- Swenson's procedure was the most common abdominoperineal pull-through technique used.
- Mortality was low, with no intra- or immediate postoperative deaths; 11% experienced minor early postoperative complications.
Conclusions:
- Hirschsprung's disease presents with varied clinical features, necessitating prompt diagnosis and surgical management.
- Surgical techniques like Swenson's procedure are effective in treating Hirschsprung's disease with acceptable outcomes.
- While associated anomalies were noted, they were generally not life-threatening, and Down's syndrome was absent in this cohort.
Abstract:
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)