Diagnosis of Cleidocranial Dysplasia: Two Sisters Case Report

Abstract

Two sisters, ages 8 and 12 years old, were referred to our Orthodontics Department because of delayed eruption and retention of primary dentition. They presented specific physical and oral findings in common, which strongly suggest the idea of an undiagnosed syndrome. Cleidocranial dysplasia (CCD) is a dominant inherited condition with high penetrance and wide variation in clinical expressivity. Some CCD characteristics include maxillary hypoplasia and some dental abnormalities such as retention of primary dentition, multiple supernumerary teeth and delayed or failing eruption of permanents dentition. Dental alterations in CCD frequently result in malocclusion with long and complex dental treatment. CCD is usually under diagnosed because of the relative lack of medical complications in comparison to other syndromes. Early diagnosis of the condition is essential, for approaching to a multidisciplinary treatment. Multiple supernumerary teeth have to be an alert, where family group medical history is essential for inhered condition evaluation. The goal of an accurate diagnosis in patients with multiple syndromic characteristics is the main subject of this paper.