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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.
Insights
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.
Aims:
Hirschsprung disease (HD) is a chronic condition associated with long-term morbidity. We assessed the short and long-term functional outcomes of operated patients in a single institution over a 12-year period.
Materials And Methods:
We conducted a retrospective review of all children operated for HD between 2002 and 2014. Postoperative functional outcomes were assessed using the Rintala Bowel Function Score (BFS, 0-20, 20=best score). We assessed hospital admissions, complications including Hirschsprung associated enterocolitis (HAEC) and the need for further surgical procedures.
Results:
72 (52 male) patients were studied, of whom, 6 (8%) had a positive family history, 5 (7%) had Trisomy 21 and 5 (7%) had total colonic HD. The median age at diagnosis was 6.5days (2 days-6.7 years) and median follow-up was 6years (1-12years). All patients except two underwent a Duhamel pull-through procedure. The median age at surgery was 4months (6days-90months). 37 (51%) procedures were performed single-stage and 7 (10%) were laparoscopically assisted. Our early complication rate was 15%; 11 (15%) patients were treated for HAEC and 43 (60%) did not require any further surgery. 12 (17%) underwent injection of botulinum toxin, 7 (10%) needed residual spur division and 4 (5%) required an unplanned, post pull-through enterostomy for obstructive defecation symptoms and HAEC. Two (3%) patients underwent an Antegrade Colonic Enema (ACE) stoma. The median BFS was 17 (5-20). There were two deaths both out of hospital.
Conclusions:
Long-term functional outcomes following Duhamel Pull-Through surgery are satisfactory although 40% of patients needed some form of further surgical intervention. The management of anal sphincter achalasia has improved with the use of botulinum toxin and we advocate aggressive and early management of this condition for symptoms of obstructive defecation and HAEC.
Level Of Evidence:
III.
