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Familial disorder with features limited to the eyes.


Fewer than 25 patients have been described in the literature. Autosomal dominant.


Dysplastic malformation of the anterior segment of the eye characterized by the combination of congenital cataract and microcornea (diameter <11 mm). Occasionally, other ocular anomalies such as iris coloboma, sclerocornea, myopia, or Peters anomaly (central corneal opacity associated with abnormal corneal development that may result in cataract, adhesions of the iris to the cornea, and glaucoma) may be present.


No known specific anesthetic problems or other associated abnormalities.


Nance-Horan Syndrome: A very rare congenital disorder combining cataract and dental anomalies.

Green JS, Johnson GJ: Congenital cataract with microcornea and Peters' anomaly as expressions of one autosomal dominant gene. Ophthalmic Paediatr Genet 7:187, 1986.  [PubMed: 3550563]
Salmon JF, Wallis CE, Murray AD: Variable expressivity of autosomal dominant microcornea with cataract. Arch Ophthalmol 106:505, 1988.  [PubMed: 3355418]

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