Progressive idiopathic unilateral corneal endothelial failure of unknown aetiology in phakic eyes

To describe a cohort of patients with irreversible unilateral bullous keratopathy (BK) of undetermined aetiology.

Retrospective, single-centre case series in a tertiary corneal referral centre.

Eleven consecutive patients (nine females; mean age 71.7 years) presented from 1999 to 2009 with acute onset unilateral visual loss. At presentation, the best-corrected visual acuity of the affected eyes was 6/9 or worse with mean central corneal thickness (CCT) of 684 (SD 66) μm. Specular microscopy was not possible in the affected eyes. There was no other ocular pathology in the affected eye. The fellow eye remained normal throughout the study (mean endothelial cell density (ECD) of 1980 (SD 736) cells/mm² and CCT of 536 (SD 34) μm). Topical steroid, antiviral treatments (both topical and systemic) or a combination of both did not yield any improvement. After a mean follow-up of 82.2 months, eight eyes had penetrating keratoplasty (PK). One required two regrafts. Histology showed typical BK features, with endothelial cell (EC) loss and thickened Descemet's membrane (DM). Transmission electron microscopy revealed DM abnormalities in a non-consistent pattern, featuring variable collagen deposition posterior to the non-banded zone. The ECs were degenerated, reduced or absent. Neither viruses nor pseudoexfoliation material was identified.

While medical treatment is not beneficial, PK appears to offer good results. Non-guttate Fuchs' corneal endothelial dystrophy merits consideration but it would be unusual to see an exclusively unilateral presentation. DM thickening is reflective of a chronic EC loss but the cause of this loss remains elusive.