April 2011
Volume 52, Issue 14
Free
ARVO Annual Meeting Abstract  |   April 2011
Unilateral Retinitis Pigmentosa? A Retrospective Case Series Of Unilateral Pigmentary Retinopathy
Author Affiliations & Notes
  • Marie-Helene Errera
    Moorfields Eye Hospital, London, United Kingdom
  • Andrew R. Webster
    Moorfields Eye Hospital, London, United Kingdom
  • Anthony . G. Robson
    Moorfields Eye Hospital, London, United Kingdom
    University College London, London, United Kingdom
  • Tracey Wong
    Moorfields Eye Hospital, London, United Kingdom
  • Philip G. Hykin
    Moorfields Eye Hospital, London, United Kingdom
  • Bishwanath Pal
    Moorfields Eye Hospital, London, United Kingdom
  • Anthony T. Moore
    Moorfields Eye Hospital, London, United Kingdom
  • Graham E. Holder
    Moorfields Eye Hospital, London, United Kingdom
    University College London, London, United Kingdom
  • Footnotes
    Commercial Relationships  Marie-Helene Errera, None; Andrew R. Webster, None; Anthony . G. Robson, None; Tracey Wong, None; Philip G. Hykin, None; Bishwanath Pal, None; Anthony T. Moore, None; Graham E. Holder, None
  • Footnotes
    Support  Mr Anthony G. Robson is supported by the Foundation Fighting Blindness.
Investigative Ophthalmology & Visual Science April 2011, Vol.52, 4995. doi:
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      Marie-Helene Errera, Andrew R. Webster, Anthony . G. Robson, Tracey Wong, Philip G. Hykin, Bishwanath Pal, Anthony T. Moore, Graham E. Holder; Unilateral Retinitis Pigmentosa? A Retrospective Case Series Of Unilateral Pigmentary Retinopathy. Invest. Ophthalmol. Vis. Sci. 2011;52(14):4995.

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Abstract

Purpose: : To survey the clinical characteristics of patients with unilateral pigmented retinopathy.

Methods: : Thirty seven patients were ascertained retrospectively following pattern and full field electroretinography (PERG;ERG) that incorporated the International standards. Clinical findings, fundus photographs and fundus autofuorescence were reviewed.

Results: : Of 37 patients, 29 women and 8 men between 13 and 67 years of age were ascertained. Associated conditions, that may explain aetiology were noted in 13 cases (35%) and included direct ocular trauma, prior ocular inflammation, systemic carcinoma, pregnancy induced hypotension and meningitis. An inherited disorder was definitely identified in 2 patients; one had a RP1 germline mutation and dominant family history and one was a female carrier of X-linked RP. Peripheral field loss and photopsias were common symptoms. Some were asymptomatic and were found to have UPR as part of a routine spectacle check-up. Reduced visual acuity was a later feature of the disorder. Fundoscopy revealed a range of abnormalities and varying degrees of intraretinal pigmentary deposition with 12.5% of patient not having frank bone-spicule pigmentation. Two patients had cystoid macular oedema (5%). On electrophysiologic findings, all patients presented a generalised loss of retinal function affecting both rod and cone systems and a dysfunction at the level of the retinal pigment epithelium/ photoreceptor complex. Pattern ERG was abnormal in 29 patients (78.4%) indicating macular involvement. There was subclinical involvement in the better seeing eye in 6 cases (16%) as assessed by electrophysiology with borderline or marginally subnormal full field ERGs, multifocal ERG and fundus autofluorescence imaging.

Conclusions: : True unilateral RP is an uncommon cause of unilateral pigmentary retinopathy. Post traumatic and post-inflammatory disorders are more common, but the aetiology of most cases is unknown. Nevertheless, mosaisism for a mutant gene during development cannot be excluded.

Keywords: retina • electrophysiology: clinical • retinal degenerations: hereditary 
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