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Progressive idiopathic unilateral corneal endothelial failure of unknown aetiology in phakic eyes
  1. Weng Hon Lee1,
  2. Adrian Tey2,
  3. Kevin Gales2,
  4. Hardeep Singh Mudhar3,
  5. Francisco C Figueiredo2
  1. 1Suite 17 Eye Clinic, Hermitage Medical Clinic, Dublin, Ireland
  2. 2Department of Ophthalmology, Royal Victoria Infirmary, Newcastle University, Newcastle upon Tyne, UK
  3. 3National Specialist Ophthalmic Pathology Service, Royal Hallamshire Hospital, Sheffield, UK
  1. Correspondence to Mr Weng Hon Lee, Suite 17, Hermitage Medical Clinic, Dublin 20, Ireland; whlee1{at}


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

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

Results 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.

Conclusion 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.

  • cornea
  • infection
  • pathology
  • yreatment medical
  • treatment surgery

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  • Contributors WHL and FCF designed the study and are responsible for data acquisition, analysis and interpretation. They also prepared the manuscript and approved its final version. AT, KG and HSM made substantial contribution in data collection, data interpretation, clinical imaging and histopathological expertise. In addition, they critically reviewed the manuscript and approved its final version for submission.

  • Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Detail has been removed from this case description/these case descriptions to ensure anonymity. The editors and reviewers have seen the detailed information available and are satisfied that the information backs up the case the authors are making.

  • Ethics approval Governance and Audit Department of Newcastle upon Tyne Hospitals NHS Foundation Trust.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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