Background/purpose: The aim of the study was to assess anal sphincter performance in relation to clinical fecal continence in adult patients who have Hirschsprung's disease.
Methods: Fifty-four adult patients (mean age, 29 +/- 7.2 years; 46 men; 8 women) who had undergone surgery for Hirschsprung's disease during their childhood underwent anorectal manometry and clinical examination. Fecal continence was evaluated with a quantitative scoring method (scoring, 0-14; 14, normal bowel function; 10-13, good continence, no social problems; 5-9, fair continence, marked social limitations; 0-4, total incontinence). Thirty healthy adults were used as controls.
Results: Fourteen patients had normal bowel habits according to the quantitative scoring. The median anal resting pressure of these patients was 25 cm H2O (range, 15-37.5). The median resting pressure of patients with good continence (n = 30; median, 20 cm/H2O; range, 5-27.5) and with fair continence (n = 6; median, 15 cm/H2O; range, 5-27.5) was significantly lower (P < .01) than in patients who had normal continence. There was no statistical difference in maximal squeeze pressure between the patient groups (median normal, 52.5; good, 45; fair, 52.5). In the controls, the median resting pressure (61.5 cm H2O; range, 34-105) and maximal squeeze pressure (86 cm H2O; range, 55-148) were significantly higher than in all patient groups (P < .0001). The voluntary sphincter force (maximal squeeze pressure minus resting pressure) was similar in patients and controls (patients median, 27 cm H2O; controls median, 16 cm H2O, NS).
Conclusions: There is a positive correlation between functional outcome and anal resting pressure in adults who have repaired Hirschsprung's disease. The overall low resting pressure reflects internal sphincter dysfunction, which may be caused by operative trauma. Despite this, most patients have a satisfactory functional outcome, which is probably related to normal voluntary sphincter performance.