Oral Manifestations of Non Vascular Ehlers-Danlos Syndrome Cross-Sectional Study

Ehlers-Danlos syndromes are rare hereditary connective tissue disorders; however, their oral manifestations remain poorly characterized in molecularly confirmed individuals. The aim of this study was to describe the oral phenotype of patients with non-vascular Ehlers-Danlos syndromes compared to healthy controls.

In this single-center, cross-sectional study, 14 patients molecularly diagnosed with non-vascular Ehlers-Danlos syndromes and 30 matched healthy controls underwent a complete clinical and radiological oral examination. Data were compared using chi-squared, Fisher's exact, Student's t-tests, or Wilcoxon tests as appropriate.

Compared to controls, patients with non-vascular Ehlers-Danlos syndromes had a significantly higher prevalence of temporomandibular disorders (64.3% vs. 16.7%; p = 0.004), gingivitis (85.7% vs. 56.7%; p = 0.017), and lingual parafunction (57.1% vs. 16.7%; p = 0.012). Anatomical variations were also more frequent, including atrophy of the lingual (85.7% vs. 10.0%; p < 0.001) and inferior labial frenula (78.6% vs. 20.0%; p < 0.001), pulpal retractions (61.5% vs. 27.6%; p = 0.047), and dental root hypoplasia (57.1% vs. 20.0%; p = 0.034).

Our findings suggest that patients with non-vascular Ehlers-Danlos syndromes present a distinct pattern with oral manifestations. These findings, including notably frenula atrophy and specific dental anomalies. These oral findings commonly observed in non-vascular Ehlers-Danlos syndromes patients can aid in diagnosis and underscore the need for specialized dental care.