Frontonasal Dysplasia Syndrome (Median Cleft Face Syndrome)
General: Congenital disorder without genetic background; condition may present a vari 232g67c ety of facial malformations, depending on the stage of embryonic development at which interference occurs.
Ocular: Hypertelorism; anophthalmia or microphthalmia; significant refractive errors; strabismus; nystagmus; eyelid ptosis; optic nerve hypoplasia; optic nerve colobomas; cataract; corneal dermoid; inflammatory retinopathy.
Clinical: Broad nasal root may be associated with median nasal groove and cleft of nose and/or upper lip; cleft of ala nasi (unilateral or bilateral); V-shaped hair prolongation into forehead.
Kinsey JA, Streeten BW: Ocular abnormalities in the medial cleft face syndrome. Am J Ophthalmol 1977; 83:261.
Roarty JD, et al. Ocular manifestations of frontonasal dysplasia. Plast Reconstr Surg
Sedano HO, et al. Frontonasal dysplasia. J Pediatr 1970; 76:906.
Weaver D, Bellinger D. Bifid nose associated with midline cleft of the upper lip: case report. Arch Otolaryngol 1946; 44:480.
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