NUMERO: #20241015

Título: ORAL MANIFESTATIONS OF PROTEUS SYNDROME: CASE REPORT

Nome do Apresentador: Alice Souza Villar Cassimiro FONSECA

Categoria do Trabalho: Painel de caso clínico (PCC)

Área Temática: Imaginologia dentomaxilofacial

Resumo: Proteus Syndrome is a rare, congenital, and non-hereditary disorder, caused by a mutation in the AKT1 gene and with a highly variable phenotype. This condition is characterized by exacerbated, heterogeneous, and progressive growth of multiple tissues, notably bone, causing alterations and asymmetries in the oral and maxillofacial region. Caucasian 32-year-old man came to the dental service complaining of painful symptoms when chewing and generalized gingival inflammation. The extraoral and intraoral clinical examination revealed significant facial asymmetry, limited mouth opening, and unilateral macroglossia. The panoramic radiograph showed bimaxillary asymmetry, bone involvement in a mosaic pattern, midline displacement, and changes in tooth eruption and maturation, and the occlusal radiograph showed images suggesting exostoses or multiple osteomas. A planigraphy of the temporomandibular joint showed a disproportionate increase in the mandibular structures on the left. According to the clinical and imaging findings, the diagnosis of Proteus Syndrome was reached and periodic follow-up was indicated.

Autor 1:  Alice Souza Villar Cassimiro FONSECA

E-mail 1:  [email protected]

Autor 2:   Luciana MUNHOZ

E-mail 2:  [email protected]

Autor 3 :  Plauto Christopher Aranha WATANABE

E-mail 3:  [email protected]

Autor 4:  Emiko Saito ARITA

E-mail 4:  [email protected]

Autor 5:  Núbia Rafaelle Oliveira de MENESES

E-mail 5:  [email protected]

Autor 6:  Lucila Massu Yoshizaki AKINAGA Moreira

E-mail 6:  [email protected]

Autor 7:  Claudio COSTA

E-mail 7:  [email protected]