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Anterior subcapsular plaque cataract in hyperornithinaemia gyrate atrophy--a case report.
  1. D. Steel,
  2. C. M. Wood,
  3. J. Richardson and
  4. J. McCarthy
  1. Sunderland Eye Infirmary, Tyne and Wear.

    Abstract

    Hyperornithinaemia gyrate atrophy (HOGA) is a rare autosomal recessive disorder in which chorioretinal degeneration occurs with cataracts, myopia, and hyperornithinaemia. We report the case of an 18-year-old female who presented with the typical features of HOGA, including posterior subcapsular cataracts and elevated plasma ornithine. She later developed distorted vision in both eyes owing to wrinkling of the anterior lens capsules. Histological examination following lens extraction showed the wrinkling was caused by focal distortion from capsular fibrosis (anterior subcapsular plaque cataract). This specific lens change has not been linked previously with HOGA.

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