Corneal Ectasia and Hydrops in a Patient with Autosomal Recessive Cornea Plana

Abstract

Purpose: To report the development of corneal ectasia and hydrops in a patient with autosomal recessive cornea plana. Methods: Retrospective observational case report. Results: A 16-year-old male with a prior diagnosis of autosomal recessive cornea plana who complained of unilateral visual loss of one month's duration was found to have corneal edema consistent with resolving hydrops in the affected eye. The edema resolved over time, and keratometry revealed high astigmatism in both eyes despite documentation of no significant corneal astigmatism 11 years before. Slit-lamp examination confirmed corneal thinning in both eyes corresponding to the meridian of the astigmatism. The prior diagnosis of cornea plana was confirmed by molecular genetic testing. Conclusions: Although not a characteristic finding of cornea plana, corneal ectasia can rarely occur and be associated with corneal hydrops.