Quality of life and neuropsychological development at school age in Hirschsprung's disease

Abstract

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.

Keywords: Hirschsprung's disease; Intellectual quotient; Kidscreen; Krickenbeck score; Neuropsychological assessment; VSP-A.