Oral Health-Related Quality of Life in Rare Disorders of Congenital Facial Weakness

Denise K. Liberton, Konstantinia Almpani, Rashmi Mishra, Carol Bassim, Carol Van Ryzin, on behalf of the Moebius Syndrome Research Consortium, Bryn D. Webb, Ethylin Wang Jabs, Elizabeth C. Engle, Francis S. Collins, Irini Manoli () and Janice S. Lee ()
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Denise K. Liberton: Craniofacial Anomalies and Regeneration Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892, USA
Konstantinia Almpani: Craniofacial Anomalies and Regeneration Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892, USA
Rashmi Mishra: Department of Orofacial Sciences, School of Dentistry, University of California, San Francisco, CA 94143, USA
Carol Bassim: Craniofacial Anomalies and Regeneration Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892, USA
Carol Van Ryzin: Metabolic Medicine Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA
Bryn D. Webb: Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA
Ethylin Wang Jabs: Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY 10029, USA
Elizabeth C. Engle: Departments of Neurology and Ophthalmology, Boston Children’s Hospital, Boston, MA 02115, USA
Francis S. Collins: Center for Precision Health Research, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA
Irini Manoli: Metabolic Medicine Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA
Janice S. Lee: Craniofacial Anomalies and Regeneration Section, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, MD 20892, USA

IJERPH, 2024, vol. 21, issue 5, 1-11

Abstract: Congenital facial weakness (CFW) encompasses a heterogenous set of rare disorders presenting with decreased facial movement from birth, secondary to impaired function of the facial musculature. The aim of the present study is to provide an analysis of subject-reported oral health-related quality of life (OHRQoL) in congenital facial weakness (CFW) disorders. Forty-four subjects with CFW and age- and sex- matched controls were enrolled in an Institutional Review Board (IRB)-approved study. Demographic data, medical and surgical history, comprehensive oral examination, and the Oral Health Impact Profile (OHIP-14) were obtained. Compared to unaffected controls, subjects with CFW had higher OHIP-14 scores overall (mean ± SD: 13.11 ± 8.11 vs. 4.46 ± 4.98, p < 0.0001) and within five of seven oral health domains, indicating decreased OHRQoL. Although subjects with Moebius syndrome (MBS) were noted to have higher OHIP-14 scores than those with Hereditary Congenital Facial Paresis (HCFP), there was no significant correlation in OHIP-14 score to age, sex, or specific diagnosis. An increase in OHIP-14 scores in subjects was detected in those who had undergone reanimation surgery. In conclusion, subjects with CFW had poorer OHRQoL compared to controls, and subjects with MBS had poorer OHRQoL than subjects with HCFP. This study provides better understanding of oral health care needs and quality of life in a CFW cohort and suggests that guidelines for dental treatment are required.

Keywords: facial weakness disorders; moebius syndrome; Oral Health Impact Profile; Carey Fineman Ziter syndrome; hereditary congenital facial palsy; congenital fibrosis of the extraocular muscles; smile surgery; OHIP-14; OHRQoL (search for similar items in EconPapers)
JEL-codes: I I1 I3 Q Q5 (search for similar items in EconPapers)
Date: 2024
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