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Prevalence of cystoid macular oedema, epiretinal membrane and cataract in retinitis pigmentosa
  1. Gerald Liew1,2,
  2. Stacey Strong2,3,
  3. Patrick Bradley2,
  4. Philip Severn2,4,
  5. Anthony T Moore2,3,5,
  6. Andrew R Webster2,3,
  7. Paul Mitchell1,
  8. Annette Kifley1,
  9. Michel Michaelides2,3
  1. 1 Centre for Vision Research, Westmead Institute for Medical Research, University of Sydney, Sydney, New South Wales, Australia
  2. 2 Department of Medical Retina, Moorfields Eye Hospital, London, UK
  3. 3 University College London, Institute of Ophthalmology, London, UK
  4. 4 Department of Ophthalmology, South Tees Hospital NHS Foundation Trust, Middlesbrough, UK
  5. 5 Ophthalmology Department UCSF School of Medicine, San Francisco, California, USA
  1. Correspondence to Michel Michaelides, University College London, Institute of Ophthalmology, London EC1V 9EL, UK; michel.michaelides{at}


Background/Aims To report the prevalence of treatable complications (cystoid macular oedema, CME; epiretinal membrane, ERM and cataract) in patients with retinitis pigmentosa (RP).

Methods Consecutive patients with RP attending a tertiary eye clinic in 2012. Spectral domain-optical coherence tomography was used to determine presence of CME and ERM. Clinic records were reviewed to identify cataract and pseudophakia. Multivariable analyses adjusted for age, gender and other confounders.

Results Data are presented for 338 eyes from 169 patients. CME was present in 58.6% of patients and 50.9% of eyes and was bilateral in 73.7%. ERM, cataract and pseudophakia were present in 22.8%, 23.4% and 11.2% eyes, respectively. In multivariable analyses, CME was associated with younger age (OR 0.81, 95% CI 0.67 to 0.98) but not with gender. Patients with ERM and cataract/pseudophakia were less likely to also have CME (OR 0.19, 95% CI 0.09 to 0.40 and OR 0.37, 95% CI 0.16 to 0.84, respectively). CME was most prevalent in patients with autosomal-dominant inheritance (71.4%), followed by autosomal recessive/sporadic inheritance (58.9%) and least likely in persons with X linked inheritance (12.5%, p<0.001).

Conclusions The prevalence of treatable RP complications is high and suggests it may be clinically beneficial to screen patients with RP to identify those who may benefit from current or future interventions.

  • macula
  • retina
  • epidemiology
  • dystrophy
  • genetics
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  • Contributors GL, ATM, ARW and MM conceived the study idea. GL, MM, SS, PB and PS designed the study and collected data. AK and GL performed statistical analyses. GL drafted the initial manuscript. GL, SS, PB, PS, ATM, ARW, PM, AK and MM all reviewed the draft and provided critical evaluations and improvements.

  • Funding The work was supported by grants from NHMRC Australia, National Institute for Health Research Biomedical Research Centre at Moorfields Eye Hospital National Health Service Foundation Trust and UCL Institute of Ophthalmology, Fight For Sight (UK) and FFSMercer Fund, Moorfields Eye Hospital Special Trustees, Macular Disease Society, the Foundation Fighting Blindness (USA) and Retinitis Pigmentosa Fighting Blindness. MM is supported by an FFB Career Development Award.

  • Disclaimer The sponsors or funding organisations had no role in the design or conduct of this research. The authors have no proprietary or commercial interest in any materials discussed in this article.

  • Competing interests None declared.

  • Patient consent Not required.

  • Ethics approval Human Research Ethics Committee of Moorfields Eye Hospital NHS Foundation Trust.

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

  • Data sharing statement Unpublished data are available on contacting the corresponding author.

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