Hirschsprung's Disease (HD)HirschsprungNCT03406741
Neuropsychological Development and Functional Outcome Sin Children With Hirschsprung Disease at School Age
A study testing psychometric evaluation and neuropsychological evaluation in people with Hirschsprung's Disease (HD), run by Assistance Publique Hopitaux De Marseille at 1 site in France.
Trial summary
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. Read more about this study
- Who is it for?
- Ages 8 to 13 years
- Conditions the study is for
- Hirschsprung's Disease (HD)
- What is being tested?
- psychometric evaluation and neuropsychological evaluation
- Type of treatment
- Lifestyle or therapy programme
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 2 participants
- Where
- 1 siteFrance
- Study dates
- 17 Jan 2018to 26 May 2023
- Phase
- Interventional
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 8 to 13 years
- All sexes
- No healthy volunteers
- Child
Who may be able to join
- 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,
Who cannot join
- 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
Trial locations and contacts
1 site in 1 country.
About this study
Official title: 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.
Read more
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.
- Conditions
- Hirschsprung's Disease (HD)
- Being tested
- psychometric evaluation, neuropsychological evaluation
- Medical subject headings
- Hirschsprung Disease
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
psychometric evaluationBehavioural
Wechsler type composite scales (WISC)
neuropsychological evaluationBehavioural
Evaluation of child behavior by the Goodman questionnaire: Strengths and Difficulties Questionnaire
| Study group | Role | What they receive |
|---|---|---|
| Child with Hirschsprung's disease | Experimental | Neuropsychological assessment at elementary school Receives: Behavioral: psychometric evaluation; Behavioral: neuropsychological evaluation |
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: 12 months
Secondary outcomes (1)
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.
Measured: 12 months
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted23 Jan 2018
- Study start17 Jan 2018
- Primary completion17 Jan 2019
- Study completion26 May 2023
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for Hirschsprung's Disease (HD)Studies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase N/A
- Primary purpose
- Supportive care
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Enrolment
- 2 participants (actual)
Sponsor and investigators
- Lead sponsor
- Assistance Publique Hopitaux De Marseille
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Jean-Olivier ARNAUD, Director, Study director, Assistance Publique Hôpitaux de Marseille
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT03406741
- Sponsor's protocol ID
- 2017-13
- Other IDs
- 2017-A01224-49
- Acronym
- Hirschsprung
- Status last verified
- May 2023
- First submitted
- 16 Jan 2018
- Last updated
- 8 May 2026
- Results posted
- No
- Data monitoring committee
- No
- FDA-regulated drug
- No
- FDA-regulated device
- No
- Individual data sharing
- No
Publications
Mille E, Dariel A, Louis-Borrione C, Merrot T, Loundou A, Tosello B, Gire C. Quality of life and neuropsychological development at school age in Hirschsprung's disease. J Pediatr Surg. 2020 Aug;55(8):1481-1487. doi: 10.1016/j.jpedsurg.2020.01.005. Epub 2020 Jan 30.
PubMed 32063371
Study at a glance
From the registry record, last updated by the sponsor on 8 May 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Neuropsychological Development and Functional Outcome Sin Children With Hirschsprung Disease at School Age - Hirschsprung
- ClinicalTrials.gov ID
- NCT03406741
- Acronym / sponsor ID
- Hirschsprung / 2017-13
- Conditions
- Hirschsprung's Disease (HD)
- Study type
- Interventional
- Recruitment status
- Completed
- Enrolment
- 2 participants (actual)
- Lead sponsor
- Assistance Publique Hopitaux De MarseilleUniversity, hospital or other
- Study start
- 17 Jan 2018 (actual)
- Primary completion
- 17 Jan 2019 (actual)
- Study completion
- 26 May 2023 (actual)
- First posted
- 23 Jan 2018 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
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 design
- Primary purpose
- Supportive care
- Allocation
- Not applicable
- Intervention model
- Single group
- Masking
- None (open label)
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Child with Hirschsprung's disease | Experimental | Behavioral: psychometric evaluation, Behavioral: neuropsychological evaluationNeuropsychological assessment at elementary school |
Who may qualify
- Ages
- 8 to 13 years
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child
Key inclusion criteria (8 listed)
- 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
Key exclusion criteria (3 listed)
- 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
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| psychometric evaluationWechsler type composite scales (WISC) | Behavioural | Child with Hirschsprung's disease |
| neuropsychological evaluationEvaluation of child behavior by the Goodman questionnaire: Strengths and Difficulties Questionnaire | Behavioural | Child with Hirschsprung's disease |
Primary outcome
Evaluation of a sensory deficit
Time frame: 12 months
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.
1 secondary outcome
Evaluation of child behavior by the Goodman questionnaire: Strengths and Difficulties Questionnaire (SDQ).
Time frame: 12 months
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 8 May 2026.
- Results publication: PubMed 32063371Mille E, Dariel A, Louis-Borrione C, Merrot T, Loundou A, Tosello B, Gire C. Quality of life and neuropsychological development at school age in Hirschsprung's disease. J Pediatr Surg. 2020 Aug;55(8):1481-1487. doi: 10.1016/j.jpedsurg.2020.01.005. Epub 2020 Jan 30.
- Individual participant dataSharing planned: No
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Jean-Olivier ARNAUD, Director study director
Assistance Publique Hôpitaux de Marseille
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A study testing psychometric evaluation and neuropsychological evaluation in people with Hirschsprung's Disease (HD), run by Assistance Publique Hopitaux De Marseille at 1 site in France.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 8 inclusion and 3 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 8 May 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in France. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Evaluation of a sensory deficit (time frame: 12 months). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT03406741, which also lists 1 linked publication.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 8 May 2026; AzTrials last refreshed its copy on 1 Nov 2026.