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Specular microscopic findings of corneal deposits in patients with Bietti’s crystalline corneal retinal dystrophy
  1. YUKO WADA
  1. Department of Ophthalmology, Tohoku University School of Medicine, Sendai Japan
  2. Department of Ophthalmology, Hirosaki University School of Medicine, Hirosaki, Aomori, Japan
  3. Department of Ophthalmology, Tohoku University School of Medicine, Sendai Japan
  1. MITSURU NAKAZAWA
  1. Department of Ophthalmology, Tohoku University School of Medicine, Sendai Japan
  2. Department of Ophthalmology, Hirosaki University School of Medicine, Hirosaki, Aomori, Japan
  3. Department of Ophthalmology, Tohoku University School of Medicine, Sendai Japan
  1. TOSHIAKI ABE,
  2. TAKASHI SHIONO,
  3. MAKOTO TAMAI
  1. Department of Ophthalmology, Tohoku University School of Medicine, Sendai Japan
  2. Department of Ophthalmology, Hirosaki University School of Medicine, Hirosaki, Aomori, Japan
  3. Department of Ophthalmology, Tohoku University School of Medicine, Sendai Japan
  1. Yuko Wada, MD, Department of Ophthalmology, Tohoku University School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8754, Japan.

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Editor,—In 1937, Bietti1 first described three cases of tapetoretinal degeneration characterised by yellowish glistening retinal crystals, tapetoretinal degeneration with choroidal sclerosis, and marginal crystalline deposits of the cornea. Although more than 100 cases of crystalline retinopathy have been reported, crystalline deposits of the corneal limbus have been observed in only four out of 52 Japanese patients with crystalline retinopathy. Recently, observation using specular microscopy2 3 has been reported to be useful in detecting crystalline deposits at the limbus of patients with crystalline corneal retinal dystrophy. Therefore, in this study, we examined four patients with crystalline retinopathy using specular microscopy under a “con-surface” mode, which is used for the observation of the corneal surface, and we detected the deposits at the limbus.

Twelve months after the initial specular microscopic examination, we reinspected the crystalline deposits of two cases. Interestingly, the changes in the locations and forms of the crystalline deposits in the corneal limbus were exposed over time (Fig 1). More crystalline deposits were found in the patients with more advanced retinopathy. It is supposed that corneal deposits and fundus deposits are essentially the same and it is suggested that crystalline retinopathy is caused by systemic abnormality. Although the exact pathogenesis of crystalline deposits is still uncertain, it is possible that destroyed fibroblasts appear to glitter or fibroblasts with crystalline-like deposits look glittering during breakdown. Further biochemical or cellular biological studies are needed to clarify these possibilities.

Figure 1

Specular microscopic findings. (A) Many crystalline deposits are observed at the limbus. (B) Specular microscopic findings re-examined after 1 year. The change of the locations and forms of crystalline deposit from the same vessels can be seen.

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