Bietti's Crystalline Retinopathy

Prominent yellow-white crystalline deposits in the posterior pole. 

  • 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.