RESUMEN

OBJECTIVE: The purpose of this study was to evaluate the dentomaxillofacial imaging features of one family affected by the gingival fibromatosis (GF) and dental abnormalities (DA) syndrome. METHODS: Conventional radiographs (periapical and panoramic) and cone beam CT (CBCT) were performed in nine members of this family: four were affected by the syndrome and five were not. RESULTS: The four affected members demonstrated mild generalized GF in association with DA, including hypoplastic amelogenesis imperfecta, intrapulpal calcifications, delay on tooth eruption and pericoronal radiolucencies in unerupted teeth. None of these oral changes were identified in the five unaffected members. All nine members presented alterations in the paranasal sinuses and mucosal thickening of the maxillary sinus was the most common finding. CONCLUSION: Family members not affected by the syndrome showed similar alterations in the paranasal sinuses and CBCT was useful to characterize the dentomaxillofacial features of this new syndrome associating GF and DA.

Asunto(s)

Fibromatosis Gingival/diagnóstico por imagen , Adolescente , Adulto , Amelogénesis Imperfecta/diagnóstico por imagen , Amelogénesis Imperfecta/genética , Tomografía Computarizada de Haz Cónico , Consanguinidad , Calcificaciones de la Pulpa Dental/diagnóstico por imagen , Calcificaciones de la Pulpa Dental/genética , Femenino , Fibromatosis Gingival/genética , Genes Recesivos , Humanos , Masculino , Persona de Mediana Edad , Senos Paranasales/diagnóstico por imagen , Linaje , Radiografía Dental/métodos , Síndrome , Diente no Erupcionado/diagnóstico por imagen , Diente no Erupcionado/genética , Adulto Joven