Neuropsychological Development and Functional Outcome Sin Children With Hirschsprung Disease at School Age

Neuropsychological Development and Functional Outcome Sin Children With Hirschsprung Disease at School Age - Hirschsprung

Hirschsprung's disease (HD) is a rare congenital disease (1:5000) characterized by neonatal functional low bowel obstruction that is caused by aganglionosis of the distal bowel. HD treatment consists in surgery of colonic reduction in the early childhood, requiring afterward a long-term follow-up. In the long term complications (incontinence, constipation, enterocolitis, soiling)but also iterative anesthesia in childhood and repeated hospitalizations can have negative effects on child's development.

The main aim of the study is to estimate neuropsychological development at school age (6-10 years). Of children operated of HD Secondary aims are evaluation of the neuro-driving development and global health, comparison of quality of life levels to those of French population standards as well as to study the complex relations between on one hand the quality of life of these children and on the other hand the socio-demographic data, the initial clinical elements and the surgical coverage, as well as their health and current cognitive profile.

This multicenter study included pediatric surgery departments of Marseille. All children born between 1/1/2005 and 31/12/2010 and presenting HD represent the population. All the families will be contacted resting on the networks of set up follow-up. A written agreement will be collected with the parents. For all the participating children, a consultation will be organized: quality of life data (reported by children and by their parents in validated standardized questionnaires), clinical examination of the child, cognitive profile of the child. The duration of inclusion is scheduled for 12 months.

This is the first French study using auto-reported data on children's quality of life at school age affected by a HD. The results of this study will allow developing hypotheses on predictive risk factors for neuropsychological development disorders. Besides a better knowledge of the relation existing between these children quality of life and their functional results due to the disease could help clinicians in their medical reflections.

Inclusion Criteria:

• Child aged 8 to 13
• Child with Hirschsprung's disease (diagnosis confirmed by anatomopathological analysis),
• Child with no severe cerebral palsy
• Child with no developmental psychosis
• Child not showing amblyopia
• Child not deaf hearing
• Child able to answer a questionnaire in French language,
• a child whose parents or legal representatives have accepted the principle of participation in this study, and who have signed an informed consent,

Exclusion Criteria:

• Child deceased between birth and the date of assessment,
• Child with cerebral palsy
• Child whose parents or legal representatives refused to allow their child to participate in this study