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Bowel perforation in neonates with Hirschsprung disease: a case series and literature review
Zhaozhou Liu1, Yanan Zhang1, Dayan Sun1
1Department of Neonatal Surgery, National Center for Children's Health, Beijing Children Hospital, Capital Medical University, 56 Nanlishi Road, Beijing, 100045, China.
Insights
Bowel perforation complicates 6% of neonatal Hirschsprung disease (HD) cases, often in the ganglionic bowel. Prompt surgery, including stoma creation, leads to favorable outcomes without mortality in these severe HD complications.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Neonatal Care
Background:
- Bowel perforation is a critical complication of Hirschsprung disease (HD), demanding urgent diagnosis and management.
- Neonatal HD with perforation presents significant mortality and morbidity risks.
Purpose of the Study:
- To investigate the clinical management strategies for neonatal Hirschsprung disease complicated by bowel perforation.
- To analyze the incidence, characteristics, and outcomes of bowel perforation in neonates with HD.
Main Methods:
- A retrospective review of 300 neonates with HD was conducted between January 2007 and January 2024.
- Included neonates were ≤28 days old with confirmed HD diagnosis via postoperative histology.
- Data encompassed perinatal history, patient demographics, HD classification, clinical presentation, surgical interventions, and outcomes.
Main Results:
- Eighteen neonates (6.0%) experienced preoperative bowel perforation, predominantly in short-segment and long-segment HD.
- Perforations most commonly occurred in the proximal ganglionic bowel segments.
- All patients underwent timely surgery, with 94.4% requiring stoma creation; no mortality was reported.
Conclusions:
- The incidence of preoperative bowel perforation in neonatal HD is 6.0%, frequently associated with short and long-segment disease.
- Perforations are typically located in the ganglionic bowel proximal to the aganglionic segment.
- Early surgical intervention, especially stoma creation, is crucial for achieving positive outcomes in these cases.
Background:
Bowel perforation is a severe complication of Hirschsprung disease (HD) that necessitates prompt diagnosis and aggressive management to reduce mortality and morbidity. This study aimed to provide insights into the clinical management of neonatal HD cases complicated by bowel perforation.
Methods:
A retrospective review was conducted on neonates diagnosed with HD and bowel perforation at Beijing Children's Hospital from January 2007 to January 2024. Inclusion criteria included neonates ≤ 28 days old with confirmed HD diagnosis based on postoperative histology. Data collected included perinatal history, patient characteristics, HD classification, clinical presentations, surgical interventions, and outcomes. Statistical analysis was performed using SPSS 26.0.
Results:
Among 300 neonates diagnosed with HD, 18 (6.0%) developed preoperative bowel perforation. Most perforations were in the proximal ganglionic bowel, with short-segment HD (7/18, 38.9%) and long-segment HD (9/18, 50.0%) being most common. All patients received timely surgical intervention upon the discovery of bowel perforation, with 94.4% (17/18) requiring stoma creation. Postoperative complications included stoma retraction, Hirschsprung disease-associated enterocolitis, and adhesive bowel obstruction, but no mortality was reported.
Conclusion:
The incidence of preoperative bowel perforation in neonates with HD was 6.0%, primarily in cases of short-segment and long-segment HD. Perforations were mainly located in the proximal ganglionic segments. Prompt surgical intervention, particularly stoma creation, resulted in favorable outcomes for most patients.
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