Clinical and radiologic aspects of maxillonasal dysostosis (binder syndrome)

Abstract

Maxillonasal dysostosis, described by Binder in 1962, is probably more common than one would think from the small number of cases that have been published (fewer than 40). We have seen 37 cases of this syndrome over a period of 8 years. Diagnosis of the syndrome is easy because of its characteristic anomalies of the upper lip and nose: smallness of the nose, a groove under the columella, a “half‐moon” appearance of the nasal apertures, absence of the nasal spine, and projection of the chin. In addition, we have observed several associated abnormalities: convergent strabismus (2 cases), mongolism (1 case), labiomaxillopalatine cleft (1 case), and, of particular note, numerous abnormalities of the cervical spine, frequently associated with mandibular prognathism. In view of the frequency of the latter abnormalities, Binder syndrome might well be called a “nasomaxillovertebral syndrome”.