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Functional outcomes in Hirschsprungs disease.

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Dr Swati Bhave    20 February 2018

Hirschsprung’s disease (HD) is a congenital disorder which results in functional obstruction of the bowel.

A study published in the Journal of Pediatric Surgery assessed the short and long-term functional outcomes of operated patients in a single institution over a 12-year period.

This retrospective review enrolled all children operated for HD between 2002 and 2014, wherein hospital admissions, complications including Hirschsprung’s associated enterocolitis (HAEC) and the need for further surgical procedures were assessed.

The results revealed that among the patients evaluated, 8% had a positive family history, 7% had Trisomy 21, and 7% had total colonic HD. The median age at diagnosis was 6.5days and median follow-up was 6 years. All patients except two underwent a Duhamel pull-through procedure; the median age at surgery was 4 months. Among these, 51% procedures were performed single-stage and 10% were laparoscopically assisted. Early complication rate was 15%; of the patients who suffered complications, 15% were treated for HAEC while 60% did not require any further surgery. On the other hand, 17% underwent injection of botulinum toxin, 10% needed residual spur division, whereas 5% required an unplanned, post pull-through enterostomy for obstructive defecation symptoms and HAEC. Meanwhile, 3% patients underwent an Antegrade Colonic Enema (ACE) stoma. The median Bowel Function Score (BFS) was 17. However, two deaths were reported, both out of hospital.

From the findings, it was inferred that long-term functional outcomes following Duhamel Pull-Through surgery are satisfactory, yet 40% of patients need some form of further surgical intervention. It was acknowledged that the management of anal sphincter achalasia has improved with the use of botulinum toxin. Aggressive and early management of HD for symptoms of obstructive defecation and HAEC was recommended.

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