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Inherited condition characterized by recurrent stromal keratitis and vascularization.

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.

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.

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

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

No specific anesthesia considerations.

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.

Other hereditary syndromes with keratitis, especially the following:

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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