Impact of Phonatory and Facial Morphology Disorders, on the Quality of Life of Adolescents With Pierre Robin Sequence

Completed

• Conditions: Pierre Robin Syndrome
• Interventions: Other: Pierre Robin sequence patients

• Registration Number: NCT03194178
• Lead Sponsor: Imagine Institute

Brief Summary

The study team has made the hypothesis that the intensity of the phonatory disorders (rhinolalia), and of the maxillo-mandibular growth anomalies (facial morphology), may have negative effects on the quality of life of adolescents with Pierre Robin sequence.

The investigators also want to assess the impact of 2 different surgical protocols of closure of the cleft palate (1 or 2 step(s)), on the current phonatory and morphology aspects. These 2 protocols were performed, by 2 parisian clinical teams, that have now been merged at Necker hospital.

Detailed Description

In this protocol, the investigator will include patients who were treated, via maxillo-facial surgery, in their early childhood for a Pierre Robin sequence.

In the frame of a follow-up visit, if they accept to participate in the study, they will fulfill with 2 psychologists, 3 quality of life questionnaires, and 1 depression assessment questionnaire.

This will aim at evaluate their quality of life and their social integration, at the time of adolescence.

Study Timeline

• Study Start: 2016-07
• Study First Submitted: 2017-06-09
• Study First Submitted QC: 2017-06-20
• Study First Posted: 2017-06-21
• Primary Completion: 2019-07
• Study Completion: 2019-07
• Status Verified: 2025-05
• Last Update Submitted: 2025-05-02
• Last Update Posted: 2025-05-06

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 100

Inclusion Criteria

• Having a Pierre Robin sequence, either isolated, either integrated to a collagenopathy, or associated to any other malformation, but without any mental retardation
• Being schooled in normal environment, with a maximum of 2 years of academic delay
• Having being treated, in the early childhood, for a maxillo-facial surgery, either in Paris-Necker or Paris-Trousseau hospitals (1 or 2 step(s) protocols)

Exclusion Criteria

• Having any other form of Pierre Robin sequence (syndromic or associated to a mental retardation)
• Having more than 2 years of academic delay, or being schooled in a specialized environment
• Having an organic severe intercurrent disease, that could have an impact on the quality of life of the patients.

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Pierre Robin sequence patients	Pierre Robin sequence patients	Patients presenting a Pierre Robin sequence, and who have been cared in their early childhood by either the Necker or Trousseau hospitals teams, and who are between 12 and 18 years old at the beginning of the study.

Primary Outcome Measures

Name	Time	Method
Correlation between generic and specific quality of life of the patients, scored via the questionnaires, and the severity of the troubles (facial and phonatory), assessed by the physicians	3 years	The data obtained via the 4 quality of life questionnaires will be assessed on regards of the severity of the facial dysmorphy and phonatory disorders.; The investigators will aggregate these results, and will be able to check if there is a clear correlation between them. They will be able to say if it can be concluded that the physical troubles due to the Pierre Robin sequence affect the quality of life of the patients.

Secondary Outcome Measures

Name	Time	Method
Comparison of the generic quality of life of patients with Pierre Robin sequence, scored via the "Kidscreen 52" questionnaire, with the data of the general population	3 years	The investigator will assess the generic quality of life of the study patients, scored via the "Kidscreen 52" questionnaire, on regards of general population (data of the literature)
Comparison of the results of the 2 surgical protocols on the maxillo-mandibular growth	3 years	The investigator will compare the long-term maxillo-mandibular growth of the "Pierre Robin" patients, compared to the general population, and compared between the 2 surgical protocols used.
Comparison of the results of the 2 surgical protocols on the phonatory aftereffects	3 years	The investigator will compare the phonatory aftereffects of the "Pierre Robin" patients, compared to the general population, and compared between the 2 surgical protocols used.
Need of secondary surgery	3 years	The investigators will assess the need to realize a secondary surgery, according the initial surgery protocol used.

Trial Locations

Locations (1)

Necker - Enfants Malades hospital; 🇫🇷 Paris, France