Bowel perforation in neonates with Hirschsprung disease: a case series and literature review

Topic overview

Key takeaways

• Bowel perforation occurs in 6% of neonatal Hirschsprung disease cases, most commonly in short-segment and long-segment disease.
• Perforations typically occur in proximal ganglionic bowel segments rather than aganglionic zones.
• Immediate surgical intervention with stoma creation (94% of cases) is the standard approach for neonatal HD with perforation.
• Despite high complication rates (stoma issues, enterocolitis, obstruction), prompt management achieved zero mortality in this series.
• Early recognition and aggressive surgical management are critical to prevent mortality in neonatal HD complicated by perforation.

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