Vitreous & Retina

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Hereditary / Congenital
Prominent yellow-white crystalline deposits in the posterior pole.
Bietti's Crystalline Retinopathy

  • More prevalent in Asians, i.e. Japanese and Chinese or Italians.
  • Clinical features:
    • Symptoms: mild to moderate decrease in vision, nyctalopia, paracentral scotoma.
    • Signs:
      • Slowly progressive disease
      • Intraretinal glittering yellow-white crystalline deposits, which is most numerous in the posterior pole
      • Multiple areas of geographic atrophy of the RPE and sclerosis or loss of choriocapillaris
      • Paralimbal conjunctival or corneal crystalline depositions may be found
  • Fluorescein angiography demonstrates patchy areas of blocked fluorescence by the crystals, multiple areas of RPE depigmentation and choriocapillaris nonperfusion.
  • Differential diagnoses include: oxalosis, cystinosis, Sjögren-Larsson Syndrome, calcified drusen, tamoxifen intoxication, autosomal dominant crystalline dystrophy.

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