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

NCT03194178 | Completed

• 1 other identifier: IMIS2016-01
• Study Type: observational
• Target: 100
• Locations: 1 country
• Sites: 1

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.

Conditions

Pierre Robin Syndrome

Outcome Measures

Primary Outcomes (1)

• 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 Outcomes (4)

• 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

• Comparison of the results of the 2 surgical protocols on the maxillo-mandibular growth

  3 years

• Comparison of the results of the 2 surgical protocols on the phonatory aftereffects

  3 years

• Need of secondary surgery

  3 years

Study Arms (1)

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

Interventions

Pierre Robin sequence patients OTHER

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)

Pierre Robin sequence patients

Eligibility Criteria

• Age: 12 Years - 18 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64)
• 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.

You may qualify if:

• 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)

You may not qualify if:

• 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.

Sponsors & Collaborators

• Imagine Institute: lead

Study Sites (1)

Necker - Enfants Malades hospital

Paris, France

Location

MeSH Terms

Conditions

Pierre Robin Syndrome

Condition Hierarchy (Ancestors)

Jaw Abnormalities; Jaw Diseases; Musculoskeletal Diseases; Maxillofacial Abnormalities; Craniofacial Abnormalities; Musculoskeletal Abnormalities; Stomatognathic Diseases; Stomatognathic System Abnormalities; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Véronique Abadie, Pr

  Necker - Enfants Malades Hospital

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: CASE ONLY
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: June 9, 2017
• First Posted: June 21, 2017
• Study Start: July 1, 2016
• Primary Completion: July 1, 2019
• Study Completion: July 1, 2019
• Last Updated: May 6, 2025

Record last verified: 2025-05

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)