Cornea plana is a pathognomonic phenotype of flat cornea, indistinct limbus, shallow anterior chamber, hyperopia, and associated accommodative esotropia (Fig 21-4); it is specific for biallelic KERA mutations. Refractive correction and monitoring for glaucoma, which may develop later in life, are the mainstays of treatment.
Figure 21-3 Posterior embryotoxon (arrow) in Axenfeld-Rieger syndrome.
Figure 21-4 An extremely flat cornea is apparent in this child with cornea plana. Also note the indistinct limbus and irregular pupil.
(Reproduced with permission from Khan AO. Ocular genetic disease in the Middle East. Curr Opin Ophthalmol. 2013;24(5):369–378.)
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.