Ines Kapferer-Seebacher, Dagmar Schnabl, Johannes Zschocke, F. Michael Pope
DOI: 10.2340/00015555-3428

Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders characterized by joint hypermobility, skin hyperextensibility, and variable tissue fragility. However, there are limited published data on the dental manifestations of EDS. This review systematically assessed the spectrum of published dental anomalies in various types of EDS. Twenty-four individual case reports/series and 3 longer case-control studies, reporting on a total of 84 individuals with a clinical diagnosis of EDS, were included in the data analysis. The main dental features listed in classical EDS were pulp calcification and localized root hypoplasia. Common dental abnormalities observed in vascular EDS were pulp shape modifications (52.2%), exceeding root length (34.8%), and molar root fusion (47.8%). Dentinogenesis imperfecta is a consistent finding in osteogenesis imperfecta/EDS overlap syndrome. Data on dental manifestations in other types of EDS are both rare and generally inconclusive.

Ehlers-Danlos syndromes are a group of rare inherited connective tissue diseases. In general, dental problems in Ehlers-Danlos syndromes are minor compared with the serious systemic manifestations, such as complications of generalized joint hypermobility or life-threatening events (e.g. vascular and organ ruptures). Nevertheless, dental problems can severely impact on patients’ quality of life. In order to clarify the range of dental manifestations in Ehlers-Danlos syndromes, and create precise disease-specific information for medical and dental practitioners, a systematic search of the medical literature for relevant reports was carried out.