Aspectos do manejo odontológico na síndrome de Bloch-Sulzberger: uma revisão integrativa

Abstract

ncontinentia Pigmenti or Bloch-Sulzberger syndrome (SBS) is a rare inherited genodermatosis linked to the X chromosome that mainly affects women. The diagnosis of SBS is mainly based on clinical characteristics, especially cutaneous findings in addition to genetic studies. The most frequent oral manifestations are hypodontia, shape anomalies, delays in tooth eruption, impacted teeth and malocclusions. The aim of this study was to carry out an integrative review on the relevant aspects of dental management in people with SBS. It is an integrative literature review, based on bibliographic evidence found in Pubmed, Scopus, Virtual Health Library (VHL) and Google Scholar databases, using the keywords: Bloch-Sulzberger syndrome, dental management, oral rehabilitation. Articles published in English, Portuguese or Spanish that reported dental treatment and its outcomes in individuals with SBS were included, resulting in 11 studies. Orofacial manifestations were the most recurrent in all reports. Agenesis and conoid teeth were the most prevalent characteristics. For the dental treatment, the most used protocol was the permanent prosthetic treatment (61%), followed by the orthodontic (46 %), the restorative (38 %) and the temporary prosthetic treatment (30%). The patient with SBS needs the follow-up with a multidisciplinary dental team and the treatment plan must be based on age, masticatory function and missing teeth. Multidisciplinary dental management in SBS is essential for effective treatment focusing on oral rehabilitation. In addition, the patient with SBS should receive periodic dental appointments to maintain general and oral health.