Hirschsprung disease and fecal incontinence: diagnostic and management strategies

Ideally, fecal incontinence after operative management for Hirschsprung disease should not occur. If it does, it presents a formidable challenge. The purpose of this study was to describe the causes of fecal incontinence and present our algorithm for its treatment.

We reviewed 68 patients with Hirschsprung disease and fecal incontinence referred to us after surgery at other institutions. Patients were evaluated by contrast enema and by an examination under anesthesia to look specifically for the integrity of the anal canal. They were designated as having a dilated colon and constipation or a nondilated colon and a tendency to diarrhea based on their stooling pattern and the appearance of the contrast enema. Medical management was started that included laxatives for those patients with a dilated colon and constipation. For those with a nondilated colon and tendency to diarrhea, the management included loperamide, pectin, and a special dietary regimen (constipating diet, 3 meals per day, and no snacks). Those patients who responded to medical management were retrospectively considered to have been pseudoincontinent. Those who did not respond were considered truly incontinent. The truly incontinent group was treated with enemas alone for those with a dilated colon, or enemas, loperamide, pectin, and a constipating diet for those with a nondilated colon and tendency to diarrhea.

Fifty-six patients had true incontinence and 12 had pseudoincontinence. Of the true incontinent group, 27 had a dilated colon and 29 had a nondilated colon. Five of these patients had a damaged or absent anal canal (anastomosis at the anal skin) and all of them had true incontinence. In the dilated colon group with true incontinence, 23 (85%) patients were clean after treatment. In the nondilated colon group with true incontinence, 23 (79%) were successfully treated. All patients in the pseudoincontinent groups had no soiling after treatment. Of 55 in the truly incontinent group, 39 (70%) had had an endorectal (Soave type) pull-through.

Fecal incontinence after operative management of Hirschsprung disease represents a serious problem. Poor surgical technique may be a contributing factor in some of the cases. Successful management depends on the appropriate evaluation, which determines whether the incontinence is true or pseudo, and the type of colon the patient has. Each category can be well treated, leading most of the time to a clean child.