Journal of the Formosan Medical Association
Volume 109, Issue 2 , Pages 167-171, February 2010

Blood-aqueous Barrier Function in a Patient With Choroideremia

  • Muh-Shy Chen

      Affiliations

    • Department of Ophthalmology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan
    • Corresponding Author InformationCorrespondence to: Dr Muh-Shy Chen, Department of Ophthalmology, National Taiwan University Hospital, College of Medicine, National Taiwan University, 7 Chung-Shan South Road, Taipei, Taiwan
    • ,
  • Ching-Chung Chang

      Affiliations

    • Department of Internal Medicine, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan
    • ,
  • Tzyy-Chang Ho

      Affiliations

    • Department of Ophthalmology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan
    • ,
  • Tzu-Hsun Tsai

      Affiliations

    • Department of Ophthalmology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan
    • ,
  • I-More Fan

      Affiliations

    • Department of Ophthalmology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan
    • ,
  • Ping-Kang Hou

      Affiliations

    • Department of Ophthalmology, National Taiwan University Hospital, College of Medicine, National Taiwan University, Taipei, Taiwan

Received 27 March 2008; received in revised form 30 April 2008; accepted 19 May 2008.

The purpose was to determine whether there was a breakdown of the blood-aqueous barrier in a patient with choroideremia. A 27-year-old man with typical choroideremia underwent standardized ophthalmo-logical evaluation, including quantitative measurement of aqueous flare intensity, by a laser flare-cell meter. The results showed areas of atrophy of the choriocapillaries and retinal pigment epithelium in the mid-periphery and posterior pole, although not in the macula. Fluorescein angiography showed areas of loss of the choriocapillaries and retinal pigment epithelium. The fovea was spared with a surrounding zone of hy-perfluorescence. Electroretinography showed a subnormal photopic amplitude and extinguished scotopic response. Electrooculography revealed that the light peak/dark trough ratio was reduced. Goldmann perimetry showed constricted peripheral fields. Laser photometry showed an increase in the aqueous flare intensity in both eyes, as compared with normal subjects. We conclude that the function of the blood-aqueous barrier might be affected in patients with choroideremia.

Key Words:  blood-aqueous barrier , choroideremia

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PII: S0929-6646(10)60038-1

doi:10.1016/S0929-6646(10)60038-1

Journal of the Formosan Medical Association
Volume 109, Issue 2 , Pages 167-171, February 2010

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