Epidemiology, prevention, diagnosis, treatment, and outcomes for psychosocial problems in patients and families affected by non-intellectually impairing craniofacial malformation conditions: a systematic review protocol of qualitative data

Mikaela I Poling, Craig R Dufresne, Mikaela I Poling, Craig R Dufresne

Abstract

Background: Physical attractiveness or unattractiveness wields a tremendous impact on the social and psychological components of life. Many individuals with facial deformities are treated more negatively than normal individuals, which may affect their self-image, quality of life, self-esteem, interpersonal encounters, and ultimately, success in life. Malformations that do not create physiological problems and whose major health impact is to degrade physical attractiveness and engender psychosocial consequences are insufficiently understood and not considered functional problems by medical insurance companies.

Methods/design: As part of a clinical practice guideline development process for psychosocial concerns in Freeman-Burian syndrome, manuscripts describing psychosocial considerations related to the presence of non-intellectually impairing craniofacial malformation conditions or associated clinical activities are sought, especially focusing on epidemiology, prevention, symptoms, diagnoses, severity, timing, treatment, consequences, and outcomes. All published papers on this topic are considered in searching PubMed, OVID MEDLINE, and CINAHL Complete and again before final analyses. The results will be written descriptively to be practically useful and structured around the type or timing of psychosocial problems or consequences described or target population characteristics. No meta-analysis is planned.

Discussion: Because the quality of research on psychosocial problems in craniofacial malformation conditions is known to be fraught with methodological problems, inconsistencies, and considerable knowledge gaps, we anticipate difficulties, which may limit the review questions able to be answered. We hope to produce a survey relevant to all non-intellectually impaired craniofacially deformed patients and their families and outline knowledge gaps and prioritise areas for clinical investigation.

Systematic review registration: PROSPERO CRD42018093021: UNIVERSAL TRIAL NUMBER: U1111-1211-8153.

Keywords: Body physical appearance; Craniofacial abnormalities; Craniofacial deformities; Freeman-Sheldon syndrome; Interpersonal relations; Reconstructive surgical procedures; Self-concept; Social adjustment; Social support; Whistling face syndrome.

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Child aged 1 year and 8 months with a typical presentation of Freeman-Burian syndrome (FBS). In addition to required features of microstomia, whistling-face appearance (pursed lips), H-shaped chin defect, prominent nasolabial folds, bilateral camptodactyly, ulnar deviation, metatarsus varus, and equinovarus, the patient exhibits numerous other craniofacial stigmata of FBS, including blepharophimosis and blepharoptosis, small nose, alar naris hypoplasia, lengthened philtrum, symmetrical midface hypoplasia, and micrognathia

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Source: PubMed

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