Children with congenital colorectal malformations often require special education or remedial teaching, despite normal intelligence.
OBJECTIVE
This study prospectively evaluated neuropsychological functioning in 8-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: socio-economic status, number of episodes of general anaesthesia, laxative treatment and premature birth. Severely intellectually disabled patients were excluded.


RESULTS
In total, 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) was 98 (17) and 96 (17), respectively. However, sustained attention was below the norm: mean (SD) Z-score was -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 was 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.


CONCLUSIONS
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.
Department of Paediatric Surgery, Erasmus MC - Sophia Children's Hospital, Rotterdam, the Netherlands.
Desiree
D
van den Hondel
Department of Paediatric Surgery, Erasmus MC - Sophia Children's Hospital, Rotterdam, the Netherlands.
Femke K
FK
Aarsen
Department of Paediatric Surgery, Erasmus MC - Sophia Children's Hospital, Rotterdam, the Netherlands.
Rene M H
RM
Wijnen
Department of Paediatric Surgery, Erasmus MC - Sophia Children's Hospital, Rotterdam, the Netherlands.
Cornelius E J
CE
Sloots
Department of Paediatric Surgery, Erasmus MC - Sophia Children's Hospital, Rotterdam, the Netherlands.
Hanneke
http://orcid.org/0000-0001-5824-3492
H
IJsselstijn
D001288:Attention; D002648:Child; D002657:Child Development; D003071:Cognition; D003106:Colon / Q000002:abnormalities*; D004519:Education, Special; D004644:Emotions; D005260:Female; D006627:Hirschsprung Disease / Q000503:physiopathology; D006801:Humans; D007360:Intelligence; D007407:Interviews as Topic; D008297:Male; D011446:Prospective Studies; D011788:Quality of Life; D012007:Rectum / Q000002:abnormalities*; D012073:Remedial Teaching; D012649:Self Concept
Anorectal malformations; Attention; Concentration; Hirschsprung's disease; Neuropsychology