TY - JOUR
T1 - Oral Health-Related Quality of Life in Rare Disorders of Congenital Facial Weakness
AU - Liberton, Denise K.
AU - Almpani, Konstantinia
AU - Mishra, Rashmi
AU - Bassim, Carol
AU - Van Ryzin, Carol
AU - Webb, Bryn D.
AU - Jabs, Ethylin Wang
AU - Engle, Elizabeth C.
AU - Collins, Francis S.
AU - Manoli, Irini
AU - Lee, Janice S.
N1 - Publisher Copyright:
© 2024 by the authors.
PY - 2024/5
Y1 - 2024/5
N2 - 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 (Formula presented.) SD: 13.11 (Formula presented.) 8.11 vs. 4.46 (Formula presented.) 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.
AB - 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 (Formula presented.) SD: 13.11 (Formula presented.) 8.11 vs. 4.46 (Formula presented.) 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.
KW - Carey Fineman Ziter syndrome
KW - OHIP-14
KW - OHRQoL
KW - Oral Health Impact Profile
KW - congenital fibrosis of the extraocular muscles
KW - facial weakness disorders
KW - hereditary congenital facial palsy
KW - moebius syndrome
KW - smile surgery
UR - http://www.scopus.com/inward/record.url?scp=85194219195&partnerID=8YFLogxK
U2 - 10.3390/ijerph21050615
DO - 10.3390/ijerph21050615
M3 - Article
C2 - 38791829
AN - SCOPUS:85194219195
SN - 1661-7827
VL - 21
JO - International Journal of Environmental Research and Public Health
JF - International Journal of Environmental Research and Public Health
IS - 5
M1 - 615
ER -