Functional Outcomes in Hirschsprung Disease: A Single Institution's 12-year Experience

Abstract 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. Post-operative 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 6.5 days (2–2466 days) and median follow-up was 6 years (1–12 years). All patients except two underwent a Duhamel pull-through procedure. The median age at surgery was 4 months (6 days – 90 months). 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