REVIEW   

Minerva Stomatologica 2019 February;68(1):42-56

DOI: 10.23736/S0026-4970.18.04129-8

Copyright © 2018 EDIZIONI MINERVA MEDICA

lingua: Inglese

Syndromes associated with dental agenesis

Daniele DE SANTIS ¹, Stefano SINIGAGLIA ¹ ✉, Paolo FACCIONI ¹, Paola PANCERA ¹, Umberto LUCIANO ¹, Dario BERTOSSI ¹, Alessandra LUCCHESE ^{2, 3}, Massimo ALBANESE ¹, Pier Francesco NOCINI ¹

¹ Section of Oral and Maxillofacial Surgery, Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, University of Verona, Verona, Italy; ² Department of Orthodontics, Vita Salute San Raffaele University, Milan, Italy; ³ Unit of Dentistry, Division of Orthodontics, Research Area in Dentofacial Orthopedics and Orthodontics, IRCCS San Raffaele Scientific Institute, Milan, Italy

INTRODUCTION: Dental agenesis is the congenital absence of a variable number of teeth due to the lack of formation of the corresponding tooth germ. The aim of this work was to investigate the syndromic conditions characterized by dental agenesis.
EVIDENCE ACQUISITION: Based on the research conducted through the OMIM® (Online Mendelian Inheritance in Man) and PubMed online databases, more than ninety syndromes associated with severe or moderate agenesis have been found.
EVIDENCE SYNTHESIS: The main clinical features of these syndromes are described, especially those concerning the stomatognathic apparatus, referring to the most recent literature. Among these syndromes there are three clinical conditions associated with dental agenesis that are common for the clinician: Down Syndrome, ectodermal dysplasia and labio-palatal cleft.
CONCLUSIONS: It must be kept in mind that the success of the treatment of these patients is based on the compliance of the patient as well as on the collaboration among specialists.

KEY WORDS: Abnormalities - Genes - Syndrome