Abstract

Alagille syndrome (ALGS) is a complex multi-systemic genetic disorder which involves primarily the liver, heart, eyes, face, and skeletal structure. The purpose of the work is to report a case of Alagille syndrome in which the facial and dental characteristics of the patient were analyzed.

Methods: A clinical and radiographic examination of the 15-year-old patient was performed with orthopantomography of dental arches and teleradiography of the skull in lateral-lateral projection. A cephalometric trace was performed which presented 2 class values, deep bite.

Results: The case presents commons skeletal and dental findings of this syndrome reported in literature: maxillary hypoplasia, maxillo-mandibular retrusion, a class II molar relationship on both sides, a deep overbite, talon cusp on element 22. A further feature that this case presents is agenesis of elements 35 e 45 with infra occlusion of elements 75 and 85.

Conclusion: In clinical and radiographic analysis, the patient showed the common characteristics reported in the literature for Alagille’s syndrome. In addition, a feature not reported in the literature for this syndrome, i.e. agenesis of elements 35 and 45, has been found. These patients need orthodontic early diagnosis as they may present skeletal and dental characteristics that may be intercepted early second class, talon cusps and eventually agenesis.

Keywords: Alagille syndrome; Agenesis; Talon cusp; Premolar agenesis