Oligodontia in the Clinical Spectrum of Syndromes: A Systematic Review

The aim of this systematic review was to describe clinical and genetic features of syndromes showing oligodontia as a sign. The review was performed according to the PRISMA 2020 checklist guidelines, and the search was conducted using PubMed, Scopus, Lilacs, Web of science, Livivo and EMBASE and supplemented by a gray literature search on Google Scholar and ProQuest, applying key terms relevant to the research questions. The systematic review identified 49 types of syndromes in 91 studies, and the most common was hypohidrotic ectodermal dysplasia, which was reported in 24 patients in 22 studies. Other commonest syndromes that reported oligodontia included Axenfeld-Rieger syndrome, Witkop’s syndrome, Ellis-van Creveld syndrome, blepharocheilodontic syndrome and oculo-facio-cardio-dental syndrome. The X-linked mode of inheritance was the most reported (n=14 studies), followed by the autosomal dominant (n=11 studies). The review describes the main syndromes that may have oligodontia as a clinical sign and reinforce the need of oro-dental-facial examining for adequate diagnosis and treatment of the affected patients. Molecular analysis in order to better understand the occurrence of oligodontia is imperative.