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

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.

Study Overview

• Status: Completed
• Conditions: Hirschsprung's Disease (HD)
• Intervention / Treatment: Behavioral: psychometric evaluation; Behavioral: neuropsychological evaluation

• Study Type: Interventional
• Enrollment (Actual): 2
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Catherine GIRE, PH; Phone Number: +33 491968750; Email: catherine.gire@ap-hm.fr
• Study Contact Backup: Name: Jean DHORNE, Manager; Phone Number: +33 491381475; Email: jean.dhorne@ap-hm.fr

Study Locations

• France
  • Marseille, France, 13354
    • Assistance Publique Hopitaux de Marseille

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 8 years to 13 years (Child)
• Accepts Healthy Volunteers: No

Description

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

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Supportive Care
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Child with Hirschsprung's disease; Neuropsychological assessment at elementary school	Behavioral: psychometric evaluation; Wechsler type composite scales (WISC); Behavioral: neuropsychological evaluation; Evaluation of child behavior by the Goodman questionnaire: Strengths and Difficulties Questionnaire

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Evaluation of a sensory deficit	Administration d'un test psychometric de type Wechsler. The Wechsler Intelligence Scale for Children (WISC). The WISC-IV includes 15 subtests exploring four major components assessing the different intellectual skills essential to learning processes. The minimum score is 70, the normality between 90 ant 110, the maximum score is 130.	12 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Evaluation of child behavior by the Goodman questionnaire: Strengths and Difficulties Questionnaire (SDQ).	It includes 25 questions that are asked to parents. It is used within research, evaluating treatment outcome and as part of clinical assessment in order to examine a child's mental well-being. A total difficulties score is calculated using the SDQ, which ranges from 0-40. Each 1 point increase in the total difficulties score corresponds with an increase in the risk of developing a mental health disorder. Categories have been proposed by Youth in Mind authors in order to assess whether a child's score is close to average, slightly above average, high or very high.	12 months

Collaborators and Investigators

• Sponsor: Assistance Publique Hopitaux De Marseille
• Investigators: Study Director: Jean-Olivier ARNAUD, Director, Assistance Publique Hopitaux de Marseille

Study record dates

Study Major Dates

• Study Start (Actual): January 17, 2018
• Primary Completion (Actual): January 17, 2019
• Study Completion (Actual): May 26, 2023

Study Registration Dates

• First Submitted: January 16, 2018
• First Submitted That Met QC Criteria: January 16, 2018
• First Posted (Actual): January 23, 2018

Study Record Updates

• Last Update Posted (Actual): May 30, 2023
• Last Update Submitted That Met QC Criteria: May 26, 2023
• Last Verified: May 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Digestive System Diseases; Congenital Abnormalities; Gastrointestinal Diseases; Colonic Diseases; Intestinal Diseases; Digestive System Abnormalities; Megacolon; Hirschsprung Disease
• Other Study ID Numbers: 2017-13; 2017-A01224-49 (Registry Identifier: ID RCB)

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No