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Inherited condition characterized by recurrent stromal keratitis and vascularization.

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Autosomal dominant. Probably caused by a mutation in the PAX6 gene, which is also involved in the development of aniridia and Peters anomaly (anterior segment malformations of the eye). Gene map locus is 11p13.

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Characterized by the presence of a circumferential band of opacification and vascularization at the level of Bowman membrane adjacent to the limbus. Histopathologic studies confirm the inflammatory nature and the anterior stomal localization of the keratitis.

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Hereditary childhood corneal clouding. Recurrent episodes of “keratoendothelitis” associated with mild iritis and stromal edema. Propensity for early recurrence after keratoplasty.

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No specific precautions associated with this medical condition. Only the presence of underlying medical problems affects preparation for anesthesia.

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No specific anesthesia considerations.

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No known pharmacological implications with this syndrome. However, some patients may be on chronic corticosteroid treatment, which indicates administration of intravenous steroids preoperatively might be necessary according to the surgical stress.

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Other hereditary syndromes with keratitis, especially the following:

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Keratitis Ichthyosis Deafness (KID) Syndrome: Polymalformative syndrome characterized by inflammation of the corneas (keratitis), skin scales, and deafness; both autosomal dominant and autosomal recessive forms have been reported.

Pearce WG, Mielke BW, et al: Autosomal dominant keratitis: A possible aniridia variant. Can J Ophthalmol 30:131, 1995.  [PubMed: 7627897]
Prosser J, van Heyningen V: PAX6 mutations reviewed. Hum Mutat 11:93, 1998.  [PubMed: 9482572]

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