Hirschsprung's disease: functional and psychological follow up comparing total colonic and rectosigmoid aganglionosis

To compare the long term functional and psychosocial outcomes following surgical treatment for total colonic aganglionosis (TCA) with those in an age and gender matched group of patients with rectosigmoid aganglionosis (RSA).

Fifteen patients with TCA matched for age and gender with 15 patients with RSA were studied 7-17 years after the definitive operation. The internal and external sphincters were examined using anal endosonography. Functional outcome (faecal continence) was assessed by a surgeon not involved in the patients' care, and by a research psychologist in separate assessment sessions. The behavioural and emotional status of the patients was also assessed.

Based on the surgeon's assessment, 6/15 TCA and 7/15 RSA patients were continent. In comparison, based on the psychological interview, 2/15 TCA and 6/15 RSA patients were continent. The TCA patients reported significantly more behavioural/emotional problems and lower levels of self esteem than the RSA patients. The parent and teacher assessments of psychosocial status revealed no differences between the groups. There was no association between incontinence and psychosocial adjustment in either group. There was no association between the assessments of functional outcome and the endosonographic appearance of the anal sphincters.

The proportion of patients with faecal incontinence 7-17 years after definitive surgery was high in both groups, but no association was found between incontinence and the psychosocial outcome measures. TCA patients perceived themselves as less well adjusted than their matched pairs. Differences between the groups in length of hospitalisation and severity of illness, especially in infancy and early childhood, may account for these differences.