Kabuki make-up (Niikawa-Kuroki) syndrome: dental and craniofacial findings in a Brazilian child

Brazilian Dental Journal

Bianca Mota Dos Santos¹  Roberta Rezende Ribeiro¹  Adriana Sasso Stuani¹  Francisco Wanderley Garcia De Paula E Silva¹  Alexandra Mussolino De Queiroz¹ 

[1] ,University of São Paulo School of Dentistry of Ribeirão Preto Department of Pediatric Clinics, Preventive and Social DentistryRibeirão Preto SP ,Brazil

关键词: Kabuki make-up syndrome;    Kabuki syndrome;    special needs patients;    dental anomalies;   

DOI  :  10.1590/S0103-64402006000300014

来源: SciELO

【 摘 要 】

This article reports the case of a Brazilian child diagnosed with Kabuki make-up syndrome (KMS), addressing the clinical features observed, with emphasis on the disease-specific oral and craniofacial manifestations. The patient had the distinctive KMS craniofacial appearance, mild delayed mental development, fingers with prominent fingertip pads and visual deficit. The dental findings included fusion of the left mandibular incisors (central and lateral), gemination of the right mandibular central incisor and congenital agenesis of the right mandibular lateral incisor, in the primary dentition, as well as absence of both permanent mandibular lateral incisors. Fusion and gemination have not been previously referred to as typical dental features in KMS. The detection of unique dental findings, such as missing teeth and dental anomalies of form in the primary dentition by means of clinical and radiographic examinations, might consist of a helpful diagnosis parameter in identifying children who may have milder forms of Kabuki syndrome.

【 授权许可】

CC BY   
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