ALAGILLE SYNDROME: FACIAL CHARACTERISTICS, RARE ORAL MANIFESTATIONS, AND MANAGEMENT OF THE CASE

Abstract

Introduction: Alagille syndrome (AGS) is an autosomal dominant disease with variable expressiveness that can affect the liver, heart, kidneys, blood vessels, eyes, face and skeleton. Objective: To describe a case of a pediatric patient with Alagille syndrome. Case report: The family history was negative for even the mildest manifestations of AGS. Clinically, the patient had a triangular face, hypertelorism, short philtrum and flat midface. Intraoral examination revealed the absence of the permanent upper lateral incisors, enamel hypoplasia and a greenish color in some teeth, gingival hyperplasia, retention of two primary lower incisors, presence of a supernumerary tooth, and a pediculated nodule of soft tissue on the lingual aspect of the left permanent mandibular first molar. Results: The dental treatment required the extraction of the retained primary teeth and the supernumerary tooth, excisional biopsy and histopathological examination of the lesion were performed and also application of topical fluoride. Also dietary and oral hygiene instructions were given. Conclusion: Currently, the patient makes frequent follow-up visits to monitor the dental development.