Binder's phenotype with ankyloglossia: Report of a rare inherited association in an Indian female

摘要

© 2022 Journal of Oral and Maxillofacial Pathology.Binder's syndrome, a rare congenital malformation of nasomaxillary complex, first described in 1962, has a hexad of characteristic clinical and radiographic features consisting of arhinoid face, intermaxillary hypoplasia with malocclusion, abnormal position of nasal bones, atrophy of nasal mucosa, reduced or absent anterior nasal spine and hypoplastic/absent frontal sinus. The typical facies due to mid-face hypoplasia may also be accompanied by other midline malformations such as cleft palate, spinal, skeletal and cardiac abnormalities. It is usually sporadic, of unknown etiology although various environmental and genetic mechanisms are implicated due to few familial cases predominantly in the Swedish population. A case of inherited Binder's syndrome is presented in an Indian female patient with an unusual finding of ankyloglossia (AG). The development of the anterior nasal spine and AG are chronologically related as they both occur during the 5th-6th weeks of gestation. The possible etiopathogenetic mechanisms for this rare association are reviewed.