Hirschsprung's disease in the newborn

Area of Science:

• Pediatric Surgery
• Gastroenterology
• Neonatology

Background:

• Hirschsprung's disease is an increasingly recognized cause of intestinal obstruction in neonates.
• Necrotizing enterocolitis is the most common cause, but Hirschsprung's disease is the second most frequent.
• This study reviews a cohort of infants diagnosed with Hirschsprung's disease.

Purpose of the Study:

• To analyze the clinical presentation, diagnostic methods, and surgical outcomes of infants with Hirschsprung's disease.
• To evaluate the effectiveness of early surgical management in this patient group.

Main Methods:

• Retrospective review of 26 infants diagnosed with Hirschsprung's disease (aganglionosis) between 1972 and 1978.
• Analysis of patient demographics, clinical presentation, diagnostic procedures (barium enema, rectal biopsy), and surgical interventions (colostomy, ileostomy, endorectal pullthrough).

Main Results:

• The average age at presentation was 8.3 days, with a male predominance (21 males, 5 females).
• Associated congenital anomalies were present in 35% of infants, including Down's syndrome in four cases.
• Diagnostic accuracy was high: 92% for barium enema and 100% for suction rectal biopsies.
• All 26 patients underwent initial ostomy followed by successful endorectal pullthrough at a mean age of 11.8 months, with no mortality or major complications.

Conclusions:

• Hirschsprung's disease requires prompt diagnosis and surgical treatment in newborns.
• Early surgical intervention, including ostomy and subsequent pullthrough, is associated with excellent outcomes and no mortality.
• Associated anomalies, particularly in infants with Down's syndrome, should be carefully evaluated.