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Quality of life and neuropsychological development at school age in Hirschsprung's disease
Eva Mille1, Anne Dariel1, Claude Louis-Borrione1
1Pediatric Surgery Department, Hôpital La Timone-Enfants, 264 rue Saint Pierre, 13005 Marseille, France.
Insights
Children with Hirschsprung's disease show a higher quality of life but subtle deficits in intelligence. Early neuropsychological assessment is recommended to identify learning disabilities in these children.
Area of Science:
- Pediatric Surgery
- Neurodevelopmental Pediatrics
- Quality of Life Research
Background:
- Hirschsprung's disease is a congenital condition affecting the large intestine.
- Long-term outcomes, including quality of life and neurodevelopment, require further investigation.
- Understanding these outcomes is crucial for comprehensive patient care.
Purpose of the Study:
- To evaluate the quality of life in school-aged children with Hirschsprung's disease.
- To assess the neuropsychological development of these children.
- To determine the bowel functional outcomes in the study population.
Main Methods:
- An observational, monocentric study involving a multidisciplinary team.
- Prospective assessment of quality of life (Kidscreen, VSP-A) and neurodevelopment (Wechsler Intelligence Scale, NEuroPSYchological assessment, Rey figure test).
- Bowel function was evaluated using the Krickenbeck score; data compared to Eurostat reference population.
Main Results:
- Fifteen children (mean age 10.25 years) were included.
- Quality of life scores were higher than the reference population (p=0.01).
- Dissociated Full-Scale Intelligent Quotient (64%), lower Perceptual Reasoning and Processing Speed Indices; no executive function disturbances. Satisfactory bowel function in 46.7%.
Conclusions:
- Children with Hirschsprung's disease exhibit subtle intellectual deficits.
- Early neuropsychological screening can aid in identifying specific learning disabilities.
- These findings highlight the need for tailored developmental support in managing Hirschsprung's disease.
Purpose:
To determine the quality of life and neuropsychological development of school-aged children with Hirschsprung's disease.
Methods:
In this observational monocentric study, a multidisciplinary team prospectively assessed quality of life, neuropsychometric development and bowel functional outcomes. This study was registered on ClinicalTrial.gov (NCT03406741). Kidscreen and VSP-A questionnaires assessed the quality of life and were compared to the reference population (Eurostat database). Intelligence, attention and executive functions, perceptual organization and memory were evaluated using the Wechsler Children's Intelligence Scale, the NEuroPSYchological assessment, and the Rey figure test. Bowel functional outcomes were obtained using the Krickenbeck score.
Results:
Fifteen patients were included, with a mean age of 10.25 years. The children's Kidscreen-assessed quality of life index was higher than the reference population (p = 0.01). The Full-Scale Intelligent Quotient was dissociated in 64% of children. The Perceptional Reasoning Index and the Processing Speed Index were observed at lower levels. There were no disturbances in executive functions. A satisfactory bowel functional outcome was noted in 46.7% of children.
Conclusion:
Children with Hirschsprung's disease have been shown to have subtle decreased performances in some areas of intelligence. Performing a neuropsychological assessment upon entering elementary school could help to detect these specific learning disabilities.
Levels Of Evidence:
Level II, prognosis study.
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