Temporomandibular Joint Replacement for Ankylosis Correction in Nager Syndrome: Case Report and Review of the Literature

Abstract

p o l i a Nager acrofacial dysostosis is a mandibulofacial abnormality characterized by downward slanting of the palpebral fissures, malar hypoplasia, bilateral conductive hearing loss, cleft palate, micrognathia, absent or hypoplastic thumbs, and radial limb hypoplasia. The syndrome shares many phenotypic features with Treacher-Collins syndrome; however, it is recognized as a separate clinical entity. A comprehensive review of the published data regarding Nager syndrome was completed to better understand the syndrome itself and the multiple treatment modalities historically used for correction of the variety of clinical manifestations, including mandibular retrognathism. In addition, we present the 94th reported case of Nager syndrome and the correction of bilateral temporomandibular joint (TMJ) ankylosis, resulting from multiple failed previous mandibular surgeries, with bilateral custom total TMJ prosthetic replacement. Nager syndrome, also known as Nager acrofacial dysostosis, was first described and distinguished from other acrofacial disorders in 1948 by Nager and Derenier. It is a genetic craniofacial disorder with varying opinions regarding its etiology, thereby suggesting a possible heterogeneous pathogenesis. An