[Hirschprung's disease in the Negev]

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.