Pierre Robin Syndrome
Conditions
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.
Interventions
OTHERPierre 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)
Sponsors
Imagine Institute
Study design
Observational model
CASE_ONLY
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
12 Years to 18 Years
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 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
| Measure | Time frame | Description |
|---|---|---|
| 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. |
Countries
France
Outcome results
None listed