Alagille syndrome: facial characteristics, rare oral manifestations, and management of the case

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 agreenish 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.

Introdução: A síndrome de Alagille (AGS) é uma doença autossômica dominante com expressividade variável que podem afetar o fígado, coração, rins, vasos sanguíneos, olhos, rosto e esqueleto. Objetivo: Descrever um caso de um paciente pediátrico com síndrome de Alagille. Relato do caso: A história familiar era negativa até mesmo para as manifestações mais leves de AGS. Clinicamente, o paciente apresentava face triangular, hipertelorismo, filtro curto e face média plana. O exame intraoral revelou ausência dos incisivos laterais superiores permanentes, hipoplasia de esmalte e aspecto esverdeado em alguns dentes, hiperplasia gengival, retenção de dois incisivos inferiores decíduos, presença de um dente supranumerário, e um nódulo de tecido mole pediculado na face lingual do primeiro molar inferior permanente esquerdo. Resultados: O tratamento odontológico exigiu a extração dos dentes decíduos retidos e do dente supranumerário, biópsia excisional e exame histopatológico da lesão além da aplicação de fluoreto tópico. Também foi realizada instrução dietética e de higiene oral. Conclusão: Atualmente, o paciente faz visitas de acompanhamento frequentes para monitorar o desenvolvimento dentário.

Profile of intrusive luxation and healing complications in deciduous and permanent teeth - a retrospective study.

OBJECTIVE: This retrospective study analyse the profile of intrusion in deciduous (DT) and permanent teeth (PT), the occurrence of healing complications (HC), type of treatments and predisposing factors. METHODS: The records of patients attended from 2005 to 2011 were analysed. Records of patients who had one or more intruded DT or PT were included in the study. Data collected from dental records included age, gender, attendance (immediate/mediate), healing complications and type of treatment. A Chi-square test and Logistic regression (p ≤ .05) were performed to evaluate the associations between the type of teeth and presence of HC, as well as to explore the interactions between predisposing factors. RESULTS: The intrusion prevalence was 9.98% in DT and 2.45% in PT. Partial intrusion (57.3%) with palatal direction of the crown (61.8%) was most prevalent in DT, while complete intrusion (56.7%) with buccal direction of the crown (58.1%) was predominant for PT. Mobility was the most common HC in DT compared to root resorption in PT. Minimally invasive treatments were the most common therapy for DT compared to invasive treatments for PT. PT demonstrated increased HC when compared to DT (p = .004). A logistic regression illustrated that the level of intrusion and crown direction during the trauma did not influence the occurrence of HC (p > .05). CONCLUSION: Intrusion's profile in DT and PT are different. PT showed more HC than DT, however the level of intrusion and crown direction were not associated with the presence of HC.

Uncommon true isolated macrodontia of a maxillary tooth.

Macrodontia is a rare dental anomaly which consists on the presence of any tooth or teeth larger than the average. In the present report the case of a child with an uncommon true isolated macrodontia of a maxillary central incisor is presented. The maxillary left central incisor was considered a macrodontic tooth since its mesiodistal crown dimension was 12.41 mm while the mesiodistal crown dimension of the maxillary right central incisor was 10.06 mm. The radiographic examination confirmed an enlarged maxillary left central incisor and a normal developing dentition. The patient did not report esthetic complaints, however, due the presence of anterior open bite and crowding, he was referred to orthodontic clinic. Thus, it must be point out the importance of the radiographic diagnosis to support the clinical findings, helping to plan and provide better treatment for the patient.