The Nance-Horan syndrome of dental anomalies, congenital cataracts, microphthalmia, and anteverted pinna: case report.

A case of a male patient presenting with unusual dental morphology, anterior supernumerary teeth, and agenesis of premolars associated with congenital cataracts, microphthalmia and anteverted pinna is described. Although 3 similar cases are in the medical literature, this is the first case in which the dental features are described in detail. Dental anomalies associated with many congenital and hereditary syndromes have been well documented. Hypodontia, the agenesis of one or more teeth, is observed in various ectodermal dysplasias E while hyperdontia or the presence of supernumerary teeth is a well-described feature of cleidocranial dysostosis 2 and Gardner’s syndrome.3 Disorders of tooth form also are associated with many genetic diseases. Taurodontism, an anomaly in which the furcation of molars is displaced apically, resulting in elongation of the body and pulp chamber and in shortening of the roots, has been described in the trichoonychondental (TOD) syndrome, the trichodento-osseous syndrome, the Mohr syndrome (oralfacial-digital syndrome II) as well as the Kleinfelter’s syndrome. 4 Alteration in crown morphology also is seen in Ellis-van Creveld syndromes where conically shaped lateral incisors and canines and barrel-shaped central incisors are present. In many of these inherited conditions, the dental findings are so consistent as to constitute important features of the syndrome. Thus, recognition of dental anomalies in patients with congenital medical conditions may play an important role in the diagnosis of the disease, especially in cases where dysmorphological features and metabolic changes are not well defined. This report describes a patient who presented with peg-shaped central incisors, anterior supernumerary teeth, agenesis of premolars, and taurodont molars with unusual rhomboidal crown morphology and some prominent pulp horns. These multiple dental anomalies were associated with congenital cataracts, microphthalmia, and anteverted pinna.