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Functional outcomes in Hirschsprung disease: A single institution's 12-year experience
Hemanshoo S Thakkar1, Christopher Bassett1, Andy Hsu1
1Department of Paediatric Surgery, Evelina London Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, Westminster Bridge Road, London, SE1 7EH.
Journal of Pediatric Surgery
|December 4, 2016
Summary
This study evaluated outcomes for Hirschsprung disease (HD) patients after surgery. While the Duhamel Pull-Through procedure shows satisfactory long-term results, many patients require additional interventions for complications like Hirschsprung associated enterocolitis (HAEC).
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Hirschsprung disease (HD) is a congenital disorder causing chronic morbidity.
- Effective management strategies are crucial for improving long-term outcomes in operated patients.
Purpose of the Study:
- To assess the short and long-term functional outcomes of patients operated for Hirschsprung disease.
- To evaluate complications, hospital admissions, and the need for further surgical procedures post-operatively.
Main Methods:
- Retrospective review of 72 children operated for HD between 2002 and 2014.
- Assessment of postoperative functional outcomes using the Rintala Bowel Function Score (BFS).
- Analysis of hospital admissions, complications (including Hirschsprung associated enterocolitis - HAEC), and subsequent surgical interventions.
Main Results:
- The median Rintala Bowel Function Score (BFS) was 17 (range 5-20), indicating generally satisfactory function.
- 51% of procedures were single-stage, and 10% were laparoscopically assisted.
- 15% early complication rate; 15% treated for HAEC; 40% required further surgical intervention (e.g., botulinum toxin, spur division, enterostomy).
Conclusions:
- The Duhamel Pull-Through procedure offers satisfactory long-term functional outcomes for Hirschsprung disease.
- Aggressive and early management of anal sphincter achalasia, including botulinum toxin, is recommended for obstructive defecation and HAEC symptoms.