++
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]