engleski

Lobodontia: genetic entity with specific pattern of dental dysmorphology

The condition of multiple dental anomalies with trituberculate and pointed crowns of canine and premolars was first described by Robbins and Keene in 1964. Characteristic pattern of dental anomalies includes cone-shaped premolars, multitubercular molar crowns, pyramidal molar roots with single root canals, shovel-shaped incisors with palatal invaginations and hypodontia. Very few family reports on this condition have been published since now. The prevalence of the condition is estimated to be less than 1:1, 000.000. In the present work we want to delineate and clarify some additional aspects of this rare genetic entity in three families with 17 affected members. This represents the largest number of cases analysed since now. Clinical, radiographic, and genetic evaluation of affected subjects was performed in three families. Analysis of dental morphology, crown-size profile patterns, pedigree analyses, and analysis of digitopalmar dermatoglyphics was performed in all patients. Crown-size profile pattern was calculated for all patients and compared with standard for Croatian population. Most striking features of the condition are conical premolars, trirubercular canines, single pyramidal molar roots, multitubercular molar crowns and invaginated upper incisors. Significant reduction of crown-size was observed for all premolars, particularly in mandible. Alveolar processus in the premolar region was hypoplastic and thin in all probands. Sex ratio of affected individuals was approximately M1:F1. Our data suggest that the prevalence of this condition is less than 1:300.000 in Croatian population what is considerably higher than previously reported in the literature. The analysis of the anomaly in all families showed slight variability of clinical picture and autosomal dominant (AD) mode of inheritance. It could be concluded that this rare condition described as lobodontia represents a true genetic entity which follows AD mode of inheritance and displays variability in its expression.

lobodontia ; dental anomalies ; pyramidal roots ; prevalence

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