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

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.

Study Overview

• Status: Completed
• Conditions: Pierre Robin Syndrome
• Intervention / Treatment: Other: Pierre Robin sequence patients

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 Type: Observational
• Enrollment (Estimated): 100

Contacts and Locations

Study Locations

• France
  • Paris, France
    • Necker - Enfants Malades Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 12 years to 18 years (Child, Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Patient included will be patients affected with Pierre Robin sequence, and coming at the study reference center in the frame of a routine follow-up visit.

Description

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 Plan

How is the study designed?

Design Details

• Observational Models: Case-Only
• Time Perspectives: Prospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
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.	Other: Pierre Robin sequence patients; 4 questionnaires will be completed by the patient : "Kidscreen 52" (generic quality of life questionnaire); "Voice Handicap Index" : VHI-9i (specific quality of life questionnaire); "Child Oral Health Impact Profile" : COHIP (specific quality of life questionnaire); "MDI-C" (composite depression scale for minors)

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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	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.	3 years

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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	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)	3 years
Comparison of the results of the 2 surgical protocols on the maxillo-mandibular growth	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.	3 years
Comparison of the results of the 2 surgical protocols on the phonatory aftereffects	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.	3 years
Need of secondary surgery	The investigators will assess the need to realize a secondary surgery, according the initial surgery protocol used.	3 years

Collaborators and Investigators

• Sponsor: Imagine Institute
• Investigators: Principal Investigator: Véronique Abadie, Pr, Necker - Enfants Malades Hospital

Study record dates

Study Major Dates

• Study Start (Actual): July 1, 2016
• Primary Completion (Actual): July 1, 2019
• Study Completion (Actual): July 1, 2019

Study Registration Dates

• First Submitted: June 9, 2017
• First Submitted That Met QC Criteria: June 20, 2017
• First Posted (Actual): June 21, 2017

Study Record Updates

• Last Update Posted (Actual): May 6, 2025
• Last Update Submitted That Met QC Criteria: May 2, 2025
• Last Verified: May 1, 2025

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Musculoskeletal Diseases; Stomatognathic Diseases; Jaw Diseases; Jaw Abnormalities; Maxillofacial Abnormalities; Craniofacial Abnormalities; Musculoskeletal Abnormalities; Stomatognathic System Abnormalities; Congenital Abnormalities; Pierre Robin Syndrome
• Other Study ID Numbers: IMIS2016-01

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
• product manufactured in and exported from the U.S.: No