Children with congenital colorectal malformations often require special education or remedial teaching, despite normal intelligence

Aim
This study prospectively evaluated neuropsychological functioning in eight-year-old patients with anorectal malformation (ARM) and Hirschsprung's disease (HD).

Methods
School functioning and behaviour were assessed in a standardised interview. Intelligence, attention, self-esteem and quality of life were evaluated with validated tests and questionnaires. The following predictors were assessed: social economic status, number of episodes of general anaesthesia, laxative treatment and premature birth. Severely intellectually disabled patients were excluded.

Results
Twelve of the 23 (52%) patients with ARM and 11 (55%) of the 20 patients with HD received special education or remedial teaching. The intelligence quotient was normal: mean (standard deviation or SD) 98 (17) and 96 (17), respectively. However sustained attention was below the norm: mean (SD) Z-score -1.90 (1.94) and -1.43 (1.98) for ARM and HD patients; both p<0.01. Self-esteem was normal: mean (SD) Z-score 0.10 (1.29) and -0.20 (1.11) for ARM and HD patients. Quality of life was normal in ARM patients and slightly impaired in HD patients. No predictors for neuropsychological outcome were identified.

Conclusion
Despite normal intelligence, more than half of these patients received special education or remedial teaching. In addition, problems with sustained attention were found. These findings are important for long-term care.