OCULAR MANIFESTATIONS OF FRONTONASAL DYSPLASIA

The ophthalmologic findings associated with frontonasal dysplasia have not been defined previously in a large series of untreated children. We reviewed the ophthalmic manifestations of a series of patients with frontonasal dysplasia who were seen as part of their craniofacial eva luation. All had undergone a complete ophthalmologic examination befor e any manipulation of either the orbits or the soft tissues of the orb ital contents. From 1986 to 1991, 23 patients with frontonasal dysplas ia were seen; ophthalmologic abnormalities were found in 20 (87 percen t). Abnormalities included significant refractive errors, strabismus, nystagmus, and eyelid ptosis. Three patients had amblyopia, a treatabl e cause of visual loss, from strabismus or anisometropia. Ten eyes in seven patients (30 percent) had severe structural anomalies, such as o ptic nerve hypoplasia, optic nerve colobomas, microphthalmia, cataract , corneal dermoid, or inflammatory retinopathy, that resulted in an ac uity of 20/100 or worse. The high incidence of ocular abnormalities in dicates that early assessment by an ophthalmologist should be part of the initial evaluation of patients with frontonasal dysplasia to detec t treatable visual or ocular problems.