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Background  Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder whose oral manifestations and dental phenotypes have not been well-characterized. The aim of this study was to explore the influence of oral manifestations on oral health-related quality of life (OHRQoL) in LDS patients.    Material and methods  LDS subjects were assessed by the craniofacial team at the National Institutes of Health Clinical Center Dental Clinic between June 2015 and January 2018. Oral Health Impact Profile (OHIP-14) questionnaire, oral health self-care behavior questionnaire and a comprehensive dental examination were completed for each subject. OHRQoL was assessed using the OHIP-14 questionnaire with higher scores corresponding to worse OHRQoL. Regression models were used to determine the relationship between each oral manifestation and the OHIP-14 scores using a level of significance of  p  ≤ 0.05.    Results  A total of 33 LDS subjects (51.5% female) aged 3–57 years (19.6 ± 15.1 years) were included in the study. The OHIP-14 scores ( n  = 33) were significantly higher in LDS subjects (6.30 [SD 6.37]) when compared to unaffected family member subjects (1.50 [SD 2.28],  p  < 0.01), and higher than the previously reported scores of the general U.S. population (2.81 [SD 0.12]). Regarding oral health self-care behavior ( n  = 32), the majority of LDS subjects reported receiving regular dental care (81%) and maintaining good-to-excellent daily oral hygiene (75%). Using a crude regression model, worse OHRQoL was found to be associated with dental hypersensitivity (β = 5.24;  p  < 0.05), temporomandibular joints (TMJ) abnormalities (β = 5.92;  p  < 0.01), self-reported poor-to-fair oral health status (β = 6.77;  p  < 0.01), and cumulation of four or more oral manifestations (β = 7.23;  p  < 0.001). Finally, using a parsimonious model, self-reported poor-to-fair oral health status (β = 5.87;  p  < 0.01) and TMJ abnormalities (β = 4.95;  p  < 0.01) remained significant.    Conclusions  The dental hypersensitivity, TMJ abnormalities, self-reported poor-to-fair oral health status and cumulation of four-or-more oral manifestations had significant influence on worse OHRQoL. Specific dental treatment guidelines are necessary to ensure optimal quality of life in patients diagnosed with LDS.

Oral health-related quality of life in Loeys-Dietz syndrome, a rare connective tissue disorder: an observational cohort study