Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + At a Glance Download Section PDF Listen ++ Inherited condition characterized by recurrent stromal keratitis and vascularization. + Genetic Inheritance Download Section PDF Listen ++ 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. + Pathophysiology Download Section PDF Listen ++ 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. + Clinical Aspects Download Section PDF Listen ++ Hereditary childhood corneal clouding. Recurrent episodes of “keratoendothelitis” associated with mild iritis and stromal edema. Propensity for early recurrence after keratoplasty. + Precautions before Anesthesia Download Section PDF Listen ++ No specific precautions associated with this medical condition. Only the presence of underlying medical problems affects preparation for anesthesia. + Anesthetic Considerations Download Section PDF Listen ++ No specific anesthesia considerations. + Pharmacological Implications Download Section PDF Listen ++ 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 Condition to Be Considered Download Section PDF Listen ++ 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. + References Download Section PDF Listen ++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]