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[Extensive Hirschsprung's disease associated with intestinal malrotation]
1Anatomie et cytologie pathologiques, site Eurasanté, CHRU, 59037 Lille cedex, France.
Summary
Hirschsprung
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Developmental Biology
Background:
- Hirschsprung's disease (HD) is a congenital condition affecting the large intestine.
- Extensive HD involving the entire colon is rare, and its association with intestinal malrotation is even rarer.
- This rare combination can complicate diagnosis and treatment in infants.
Observation:
- An infant presented with neonatal intermittent intestinal obstruction.
- Initial diagnosis and surgical treatment for intestinal malrotation did not resolve the symptoms.
- Persistent obstruction prompted further investigation, revealing aganglionic segments in the colon and terminal ileum.
Findings:
- The patient was diagnosed with extensive Hirschsprung's disease (HD) and intestinal malrotation.
- Surgical management included ileostomy, excision of the affected bowel segment, and later ileoanal anastomosis.
- A favorable outcome was achieved after definitive surgical treatment for HD.
Implications:
- Persistent intestinal occlusion after malrotation surgery in infants warrants consideration of associated Hirschsprung's disease.
- Early recognition and comprehensive surgical planning are crucial for managing complex cases of HD and malrotation.
- This case highlights the importance of considering rare congenital anomalies in pediatric surgical practice.
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