Defecation disorders in children after surgery for Hirschsprung disease.

BACKGROUND AND

OBJECTIVE

The majority of children with Hirschsprung disease (HD) after corrective surgery (CS) develop protracted defecation disorders (DDs) such as constipation, fecal incontinence, and/or enterocolitis. The aim of this investigation was to determine the diagnoses, therapies, and long-term clinical outcomes using a systematic algorithm to address protracted DD in children with HD after CS.

METHODS

Retrospective review of children with HD after CS cared for using a systematic algorithm at a tertiary care center. Potential anatomic etiologies were evaluated for first. Clinical outcome was categorized into 4 groups based on symptom severity, time interval from last enterocolitis episode, laxative usage, and/or rectal therapies at the time of last follow-up.

RESULTS

Fifty-seven children were identified, of whom 51 (89.5%) had obstructive symptoms and/or enterocolitis and 6 (10.5%) had nonretentive fecal incontinence. Nonintractable constipation responsive to laxatives was identified in 10 (17.5%), colonic dysmotility in 4 (7.0%), nonrelaxing anal sphincter as a primary etiology in 22 (38.6%), bacterial overgrowth in 2 (3.5%), food intolerance in 2 (3.5%), and rapid transit in 2 (3.5%). Further surgical intervention was undertaken in 22 (38.6%), including 9 (15.8%) for residual aganglionosis. Mean follow-up was 41.4 ± 4.5 months. Clinical outcomes were excellent in 16 (28.1%), good in 22 (38.6%), fair in 1 (1.8%), and poor in 18 (31.6%). Children with enterocolitis were more likely to have an excellent or good clinical outcome.

CONCLUSIONS

The majority of children with HD and protracted DD after CS have a favorable long-term clinical outcome when following a systematic algorithm.