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Outcomes of penetrating keratoplasty in congenital hereditary endothelial dystrophy
  1. Hamed AlArrayedh1,
  2. Louis Collum1,
  3. Conor C Murphy1,2
  1. 1 Department of Ophthalmology, Royal Victoria Eye and Ear Hospital, Dublin, Ireland
  2. 2 Department of Ophthalmology, Royal College of Surgeons in Ireland, Dublin, Ireland
  1. Correspondence to Dr Hamed AlArrayedh, Apartment 05, Killashee House, Spencerdock, Northwall, Dublin 1, Ireland; hamed_alarrayedh{at}


Background/aim To report the outcome of penetrating keratoplasty (PKP) in Irish patients with congenital hereditary endothelial dystrophy (CHED).

Methods A retrospective case series review of patients with CHED who underwent PKP was conducted. The outcomes of PKP in 14 patients with CHED at the Royal Victoria Eye and Ear Hospital in Dublin from 1978 to 2013 were described following case note review. The main outcome measures were best-corrected visual acuity (BCVA) and graft survival.

Results Thirty-three corneal transplants were performed, which included 32 PKPs and one Descemet’s stripping endothelial keratoplasty. Twenty-four eyes underwent primary corneal grafts and nine eyes had regrafts. The graft survival rates at final follow-up were 37.5% and 33% in the primary graft and regraft groups, respectively. Preoperative BCVA was 20/200 or worse in all patients. At the final postoperative visit, the BCVA was 20/80 or better in four eyes following primary PKP, 20/160 in one eye following regrafting and was 20/200 or worse in all other eyes. The mean time to graft failure was 16 months (range 0–37 months). The mean follow-up time was 101 months (range 12–252 months). Fifty per cent of the patients continue to attend for follow-up.

Conclusions This study has demonstrated a poor outcome from PKP for CHED in this Irish cohort. This arises from a combination of dense amblyopia and a high risk of graft failure in the long term.

  • Treatment Surgery
  • Cornea
  • Dystrophy

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  • Contributors All persons who meet authorship criteria are listed as authors, and all authors certify that they have participated sufficiently in the work to take public responsibility for the content, including participation in the concept, design, analysis, writing or revision of the manuscript. Authors contributions are as follows:

    HA, LC and CCM contributed to the conception and design of the study.

    HA contributed in acquisition of the data and analysis and interpretation of the data was performed by all authors.

    HA, LC and CCM contributed to the drafting and revising the manuscript.

    Both LC and CCM contributed to performing the corneal transplants throughout the years.

    All authors approved the final version of the manuscript to be published.

    This is also to certify that HA and CCM take responsibility as guarantors of the article.

  • Competing interests None declared.

  • Patient consent Anonymisation of data was conducted. No identifiable patient information was used.

  • Ethics approval Clinical Audit Committee, Royal Victoria Eye and Ear Hospital, Dublin, Ireland.

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