Corneal Ectasia and Hydrops in Ocular Hypotony: The Corneal Crease

To report the association of chronic ocular hypotony with the development of progressive corneal ectasia and hydrops.

Retrospective case series.

Three patients with ocular hypotony were referred for corneal evaluation and found to have ectasia and acute corneal hydrops in their hypotonous eye(s). Clinically, the globes were easily deformable with either external digital palpation and/or simple blinking. All 3 patients had a history of chronic iridocyclitis, including one with juvenile idiopathic arthritis. In each case, the area of thinning was narrow and arcuate in configuration, distinctive from other ectatic disorders. Also uncharacteristically, the acute hydrops resolved rapidly within 2 to 3 weeks without surgical intervention. In 1 case, severe thinning with perforation occurred requiring urgent penetrating keratoplasty.

This case series demonstrates a unique clinical entity in which corneal ectasia and hydrops developed in the setting of ocular hypotony and easily deformable corneas, in a pattern unlike previously described forms of ectasia. Acute hydrops, even with associated corneal perforation, demonstrated a short and self-limited course. Corneal ectasia and irregular astigmatism should be suspected as a cause of unexplained visual loss in the ever-increasing number of patients with chronic, stable ocular hypotony. Further study is warranted to determine the pathophysiology of corneal ectasia in this setting, which may include mechanical and inflammatory factors.