Skip to main content
Log in

The differential diagnosis of crystals in the retina

  • Published:
International Ophthalmology Aims and scope Submit manuscript

Abstract

Crystalline deposits in the retina may be associated with a wide variety of systemicdisorders such as oxalosis,cystinosis, hyperornithinaemia and Sjögren-Larssonsyndrome. Refractile crystalline deposits may also be a manifestation of drug toxicity like the antineoplastic agent tamoxifen, the anesthetic methoxyflurane and the oral tanning agent canthaxanthine. Crystals may also occur in drug abusers who inject multiple crushed tablets of methadone or meperidine intravenously (talc retinopathy). The differential diagnosis of retinal crystals also includes primary ocular diseases like Bietti's crystalline retinopathy, calcified macular drusen, idiopathic parafoveal telangiectasis and longstanding retinal detachment .This article will review the most common causes of crystalline retinopathies, their etiologies, pathologies and clinical characteristics.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Bullock JD, Albert DM. Flecked retina appearance secondary to oxalate crystals from methoxyflurane anesthesia. Archives of Ophth 1975; 93: 26–31.

    Google Scholar 

  2. Farrel J, Shoemaker JD, Otti T, Jordan W, Schoch L, Neu LT, Bastani B. Primary hyperoxaluria in an adult with renal failure, livedo reticularis, retinopathy and peripheral neuropathy. American Journal of Kidney Diseases 1997; 29: 947–52.

    PubMed  Google Scholar 

  3. Wells C, Johnson R, Luo Qingli, Bunt-Milam A, Kalina RE. Retinal oxalosis; a clinicopathologic report. Arch Ophthalmol 1989; 107: 1638–43.

    PubMed  Google Scholar 

  4. Bullock JD, Albert DM, Skinner CW, Miller WH, Galla JH. Calcium oxalate retinopathy associated with generalized oxalosis: X-ray diffraction and electron microscopic studies of deposits. Investigative Ophthalmology 1974; 13: 256–65.

    PubMed  Google Scholar 

  5. Town M, Jean G, Cherqui S, Attard M, Forestier L, Whitmore SA, Callen DF et al. A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis. Nat Genet 1998 April; 18(4): 319–24.

    PubMed  Google Scholar 

  6. Adamson MD, Andersson HC, Gahl WA. Cystinosis. Semin Nephrol 1989; 9: 147–61.

    PubMed  Google Scholar 

  7. Offner G, Latta K, Hoyer PF, Baum H, Ehrich JH, Pichlmayr R, Brodehl J. Kidney transplanted children come of age. Kidney International 1999; 55: 1509–17.

    PubMed  Google Scholar 

  8. Demetrios S, Theodoropoulos, Krasnewich D, Kaiser-Kupfer MI, Gahl W. Classic Nephropathic Cystinosis as an adult disease. JAMA 1993; 270: 2200–4.

    PubMed  Google Scholar 

  9. Read J, Goldberg MF, Fishman G, Rosenthal I. Nephropathic cystinosis. American J Ophth 1973; 76: 791–96.

    Google Scholar 

  10. Fellers FX, Cogan DG. Cystinosis with extensive choroidal involvement. Arch Ophth 1965; 74: 868–9.

    Google Scholar 

  11. Sanderson PO, Kuwabara T, Stark WJ, Wong VG, Collins EM. Cystinosis, a clinical, histopathologic and ultrastructural study. Arch Ophth 1974; 91: 270–4.

    Google Scholar 

  12. Kaiser-Kupfer MI, Fujikawa L, Kuwabara T, Jain S, Gahl WA. Removal of corneal crystals by topical cysteamine in nephropathic cystinosis. N Engl J Med 1987; 316: 775–9.

    PubMed  Google Scholar 

  13. Takki D. Gyrate atrophy of the choroid and retina associated with hyperornithenemia. British J Ophth 1974; 58: 3–23.

    Google Scholar 

  14. McKusick VA. Mendelian inheritance in man. Catalogs of human genes and genetic disorders. Baltimore: Johns Hopkins University Press, 1998.

    Google Scholar 

  15. Rizzo WB, Craft DA. Sjogren-Larsson syndrome. Semin Dermatol 1993; 12: 210–18

    PubMed  Google Scholar 

  16. Willemsen MAAP, Cruysberg JRM, Rotteveel JJ, Aandekerk AL, Van Domburg PHMF, Deutman AF. Juvenile macular dystrophy associated with deficient activity of fatty aldehyde dehydrogenase in Sjogren-Larsson syndrome. Am J Ophthalmol 2000; 130: 782–9.

    PubMed  Google Scholar 

  17. Gilbert WR jr, Smith JL, Nyhan WL. The Sjogren Larsson Syndrome. Arch Ophth 1968; 80: 308–16.

    Google Scholar 

  18. Van Domburg PHMF, Willemsen MAAP, Rotteveel JJ et al. Clinical and MRI/MRS findings in FALDH deficient patients. Neurology 1999; 52: 1345–52.

    PubMed  Google Scholar 

  19. Reinboth JJ, Gautschi K, Clausen M, Reme CE. Lipid mediators in the rat retina:light exposure and trauma elicit leukotriene B4 release in vitro. Curr Eye Res 1995; 14: 1001–8.

    PubMed  Google Scholar 

  20. Willemsen MAAP, De Jong JGN, Van Domburg PHMF, Rotteveel JJ, Wanders RJA, Mayatepek E. Defective inactivation of leukotriene B4 in patients with Sjogren-Larsson syndrome. J Pediatr 2000; 136: 258–60.

    PubMed  Google Scholar 

  21. Kaiser-Kupfer MI, Lippman ME. Tamoxifen retinopathy. Cancer Treat Rep 1978; 62: 315–20.

    PubMed  Google Scholar 

  22. Kaiser-Kupfer MI, Kupfer C, Rodrigues MM. Tamoxifen retinopathy, a clinicopathologic report. Ophthalmology 1981; 88: 89–93.

    PubMed  Google Scholar 

  23. McKeown CA, Swartz M, Blom J, Maggiano JM. Tamoxifen retinopathy. British Journal Ophth 1981; 65: 177–9.

    Google Scholar 

  24. Chang T, Gonder JR, Ventresca MR. Low-doze tamoxifen retinopathy. Can J Ophthalmol 1992; 27: 148–9.

    PubMed  Google Scholar 

  25. Lonn LI. Canthaxanthine retinopathy, photo essay. Arch Ophth 1987; 105: 1590–1.

    Google Scholar 

  26. Cortin P, Corriveau LA, Rousseau A, Tardif Y, Malenfant M, Boudreault G. Maculopathie en paillettes d'or. Can J Ophth 1982; 17: 103–6.

    Google Scholar 

  27. Boudreault G, Cortin P, Corriveau LA, Rousseau AP, Tardif Y, Malenfant M. La retinopathie à la canthaxanthine: 1. Etude clinique de 51 consommateurs. Can J Ophth 1983; 18: 325–8.

    Google Scholar 

  28. Cortin P, Boudreault G, Rousseau A, Tardif Y, Malenfant M. La retinopathie à la canthaxanthine: 2. Facteurs predisposants Can J Ophth 1984; 19: 215–9.

    Google Scholar 

  29. Friberg TR, Gragoudas ES, Regan CD. Talc emboli and macular ischemia in intravenous drug abuse. Arch Ophth 1979; 97: 1089–91.

    Google Scholar 

  30. Atlee WE. Talc and cornstarch emboli in eyes of drug abusers. JAMA 1972; 219: 49–51.

    PubMed  Google Scholar 

  31. Wise GN, Dollery CT, Henkind P. The retinal circulation. New York, Harper & Row Publishers Inc. 1971, p. 39.

    Google Scholar 

  32. Murphy SB, Jackson WB, Pare JA. Talc retinopathy. Can J Ophth 1978; 13: 152–6.

    Google Scholar 

  33. Ballantyne AJ, Michaelson IC. Textbook of the Fundus of the Eye. E&S, Livingston, Edinburgh, 1970, p. 62.

    Google Scholar 

  34. Arnett EN, Battle WE, Roberts WC. Intravenous injection of talc containing drugs intended for oral use. Am J Med 1976; 60: 711–718.

    PubMed  Google Scholar 

  35. Hopkins GB, Taylor DG. Pulmonary talc granulomatosis. Am Rev Resp Dis 1970; 101: 101–4.

    PubMed  Google Scholar 

  36. Douglas FG, Kafilmout KH, Patt NL. Foreign particle embolism in drug addicts: Respiratory pathophysiology. Ann Int Med 1971; 75: 865–72.

    PubMed  Google Scholar 

  37. Ibanez HE, Williams DF, Boniuk I, Mo SL. Crystalline therapy associated with long term nitrofurantoin therapy. Arch Ophth 1994; 112: 304–5.

    Google Scholar 

  38. Maguire JI, Annesly WH. Age related macular degeneration. In Tasman WS, ed. Clinical decisions in medical retinal diseases. St. Louis. Mosby 1994: 80–1.

    Google Scholar 

  39. Farkas TG. Drusen of the retinal pigment epithelium. Surv Ophthalmol 1971; 16: 75.

    Google Scholar 

  40. Farkas TG, Sylvester V, Archer D, Altona M. The histochemistry of Drusen. Am J Ophthalmol 1971; 71: 1206.

    PubMed  Google Scholar 

  41. Sarks SH, Sarks JP. Age related macular degeneration: atrophic form. In Ryan S, ed. Retina Vol 2. St. Louis Mosby 1991; 161.

    Google Scholar 

  42. Gass JD, Oyakawa RI. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophth 1982; 100: 769–80.

    Google Scholar 

  43. Kleiner RC. Idiopathic parafoveal telangiectasis. In Tasman WS, ed. Clinical decisions in medical retinal diseases. St. Louis. Mosby 1994; 198–203.

    Google Scholar 

  44. Moiseiev J, Leevis H, Bartov E, Fine SL, Murphy RP. Superficial retinal refractile deposits in juxtafoveal telangiectasis. Am J Ophth 1990; 109; 604–5.

    PubMed  Google Scholar 

  45. Brucker A, Robinson F. Primary retinal vascular abnormalities. In Yanuzzi L ed. Laser photocoagulation of the macula. Philadelphia, JB Lippincot 1989.

    Google Scholar 

  46. Welch RB. Bietti's tapetoretinal degeneration with marginal corneal dystrophy, Crystalline retinopathy. Trans Am Ophthalm Soc 1977; 75: 164–75.

    Google Scholar 

  47. Grizzard WS, Deutman AF, De Kerk AA. Crystalline retinopathy. Am J Ophth 1978; 86: 81–88.

    PubMed  Google Scholar 

  48. Mauldin MW, O'Connor AS. Crystalline retinopathy, Bietti's tapetoretinal degeneration without marginal corneal dystrophy. Am J Ophth 1981; 92: 640–6.

    PubMed  Google Scholar 

  49. Yuzawa M, Mae U, Masteri M. Bietti's retinopathy. Ophthalmic Pediatric Genetics 1986; 7: 9–20.

    Google Scholar 

  50. Wilson DJ, Weleber RG et at. Bietti's crystalline dystrophy, a clinicopathologic correlative study. Arch Ophth 1989; 107: 213–21.

    Google Scholar 

  51. Carr R. Hereditary choroidal disease. In Ryan SJ ed. Retina vol 1. St Louis Mosby 1991: 455–6.

    Google Scholar 

  52. Bagolini B, Ioli Spada G. Bietti's tapetoretinal degeneration with marginal corneal dystrophy Am J Ophth 1968; 65: 53–9.

    PubMed  Google Scholar 

  53. Hayasaka S, Okuyawa S. Crystalline retinopathy. Retina 1984; 4:177–81.

    PubMed  Google Scholar 

  54. Bernauer W, Daieker B. Bietti's corneal retinal dystrophy. Retina 1992; 12: 18–20.

    PubMed  Google Scholar 

  55. Richards BW, Brodstein DE, Nussbaum JJ, Ferencz JR, Maeda K, Weiss L. Autosoinal dominant crystalline dystrophy. Ophthalmology 1991; 98: 658–65.

    PubMed  Google Scholar 

  56. Kaiser Kupfer MI, Charr CC, Markello TC, Crawford MA, Caruso RC, Csaky KG et al. Clinical, biochemical and pathologic correlations in Bietti's crystalline dystrophy. Am J Ophth 1994; 118: 569–82.

    PubMed  Google Scholar 

  57. Cogan DG, Kuwabara T, Silbert J et al. Calcium oxalate and calcium phosphate in detached retinas. Arch Ophth 1958; 60: 366–71.

    Google Scholar 

  58. Ahmed I, McDonald R, Schatz H, Johnson R, Ali E, Cruess A et al. Crystalline Retinopathy Associated with Chronic Retinal Detachment. Arch Ophthalmol 1998; 116: 1449–53.

    PubMed  Google Scholar 

  59. Anderson B. Discussion. Trans Am Ophth Soc 1977; 75; 175–9.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Nadim, F., Walid, H. & Adib, J. The differential diagnosis of crystals in the retina. Int Ophthalmol 24, 113–121 (2001). https://doi.org/10.1023/A:1021189215498

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1023/A:1021189215498

Keywords

Navigation