22 - Prevalence of Dental Anomalies in Patients with Wolf–Hirschhorn Syndrome: A Case-Control Study

Abstract:

Purpose: Wolf–Hirschhorn syndrome (WHS, OMIM:194190) is a rare syndrome with a prevalence of 1:60000 births and characterized by distinctive facial features. This retrospective case-control study evaluated developmental dental anomalies and pathologies in pediatric patients with Wolf–Hirschhorn syndrome (WHS).

Methods: The study screened 1.5 million children (1-18 years) reported at the Children’s Hospital Colorado for a diagnosis of WHS. Twenty-six children were identified with WHS. Medical and dental findings from the WHS cohort were evaluated and compared with age- and sex-matched healthy controls. Clinical findings and radiographs were assessed for anomalies of shape, number, position, structure, and other pathologies. Data was compared between WHS and healthy controls using two-sample t-tests and chi-squared or Fisher’s exact tests.

Results: Children with WHS demonstrated a significantly higher prevalence of medical findings (P < .05). A significantly higher proportion ( >80%) of WHS subjects demonstrated less than 5^th percentile on weight, height, and body mass index (P < .05). Although there was a higher prevalence of dental anomalies in subjects with WHS, very few anomalies reached significance due to low sample size. Hypodontia, delayed tooth eruption, and hypoplasia were significantly higher in the WHS cohort compared to healthy controls. There was a significantly higher prevalence of parafunctional habits in the WHS cohort (P=.001).

Conclusions: Wolf–Hirschhorn syndrome is associated with distinct patterns of dental developmental anomalies and delayed dental maturation. Recognition of these findings underscores the importance of early dental surveillance and interdisciplinary management to optimize oral health outcomes in children with WHS.

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