Crystalline Dystrophy Treatment & Management

  • Author: Jayne S Weiss, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Nov 28, 2011
 

Medical Care

  • No local or systemic medical treatment is available to stop the progression of corneal lipid deposition or the alteration of serum cholesterol levels.
  • Penetrating keratoplasty can be performed successfully in those patients with advanced disease, but dystrophy can recur in the graft.
  • Phototherapeutic keratectomy can remove subepithelial crystals if they are causing decreased vision and glare.
Proceed to Follow-up
 
 
Contributor Information and Disclosures
Author

Jayne S Weiss, MD  Professor of Ophthalmology, Director of Refractive Surgery, Kresge Eye Institute, Wayne State University

Jayne S Weiss, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American Ophthalmological Society, Association for Research in Vision and Ophthalmology, Eye Bank Association of America, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Brad Spagnolo, MD  Ophthalmology, Baltimore-Washington Eye Center

Brad Spagnolo, MD is a member of the following medical societies: American Academy of Ophthalmology and American Society of Cataract and Refractive Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

Fernando H Murillo-Lopez, MD  Senior Surgeon, Unidad Privada de Oftalmologia CEMES

Fernando H Murillo-Lopez, MD is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Simon K Law, MD, PharmD  Associate Professor of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Christopher J Rapuano, MD  Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Director of the Cornea Service, Co-Director of Refractive Surgery Department, Wills Eye Institute

Christopher J Rapuano, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Cornea Society, Eye Bank Association of America, International Society of Refractive Surgery, and Pan-American Association of Ophthalmology

Disclosure: Allergan Honoraria Speaking and teaching; Allergan Consulting fee Consulting; Alcon Honoraria Speaking and teaching; Inspire Honoraria Speaking and teaching; RPS Ownership interest Other; Vistakon Honoraria Speaking and teaching; EyeGate Pharma Consulting; Inspire Consulting fee Consulting; Bausch & Lomb Honoraria Speaking and teaching; Bausch & Lomb Consulting fee Consulting

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

References

  1. Van Went JM, Wibaut F. Een zyeldzame erfelijke hoornvliesaandoening. Ned Tijdschr Geneeskd. 1924;68(B):2996-2997.

  2. Schnyder WF. Mitteilung uber einen neuen Typus von familiarer hornhauterkrankung. Schweiz Med Wochenschr. 1929;59:559-571.

  3. Weiss JS, Khemichian AJ. Differential diagnosis of Schnyder corneal dystrophy. Dev Ophthalmol. 2011;48:67-96. [Medline].

  4. Bron AJ. Corneal changes in the dislipoproteinaemias. Cornea. 1989;8(2):135-40. [Medline].

  5. Bron AJ, Williams HP, Carruthers ME. Hereditary crystalline stromal dystrophy of Schnyder. I. Clinical features of a family with hyperlipoproteinaemia. Br J Ophthalmol. May 1972;56(5):383-99. [Medline].

  6. Delleman JW, Winkelman JE. Degeneratio corneae cristallinea hereditaria. A clinical, genetical and histological study. Ophthalmologica. 1968;155(5):409-26. [Medline].

  7. Freddo TF, Polack FM, Leibowitz HM. Ultrastructural changes in the posterior layers of the cornea in Schnyder's crystalline dystrophy. Cornea. Sep 1989;8(3):170-7. [Medline].

  8. Gaynor PM, Zhang WY, Weiss JS, et al. Accumulation of HDL apolipoproteins accompanies abnormal cholesterol accumulation in Schnyder's corneal dystrophy. Arterioscler Thromb Vasc Biol. Aug 1996;16(8):992-9. [Medline].

  9. Gibbels E, Schaefer HE, Runee U, et al. Severe polyneuropathy in Tangier disease mimicking syringomyelia or leprosy. Clinical, biochemical, electrophysiological, and morphological evaluation, including electron microscopy of nerve, muscle, and skin biopsies. J Neurology. 1985;232(5):283-294. [Medline].

  10. Gjone E. Familial lecithin cholesterol acyltransferase (LCAT) deficiency. Birth Defects Orig Artic Ser. 1982;18(6):423-31. [Medline].

  11. Hoang-Xuan T, Pouliquen Y, Gasteau J. [Schnyder's crystalline dystrophy. II. Association with genu valgum]. J Fr Ophtalmol. 1985;8(11):743-7. [Medline].

  12. McCarthy M, Innis S, Dubord P, et al. Panstromal Schnyder corneal dystrophy. A clinical pathologic report with quantitative analysis of corneal lipid composition. Ophthalmology. May 1994;101(5):895-901. [Medline].

  13. Orr A, Dube MP, Marcadier J, et al. Mutations in the UBIAD1 gene, encoding a potential prenyltransferase, are causal for Schnyder crystalline corneal dystrophy. PLoS ONE. Aug 1 2007;2(1):e685. [Medline].

  14. Philipson BT. Fish eye disease. Birth Defects Orig Artic Ser. 1982;18(6):441-8. [Medline].

  15. Rodrigues MM, Kruth HS, Krachmer JH, et al. Unesterified cholesterol in Schnyder's corneal crystalline dystrophy. Am J Ophthalmol. Aug 15 1987;104(2):157-63. [Medline].

  16. Theendakara V, Tromp G, Kuivaniemi H, et al. Fine mapping of the Schnyder's crystalline corneal dystrophy locus. Hum Genet. May 2004;114(6):594-600. [Medline].

  17. Vesaluoma MH, Linna TU, Sankila EM, et al. In vivo confocal microscopy of a family with Schnyder crystalline corneal dystrophy. Ophthalmology. May 1999;106(5):944-51. [Medline].

  18. Weiss JS. Schnyder crystalline dystrophy sine crystals. Recommendation for a revision of nomenclature. Ophthalmology. Mar 1996;103(3):465-73. [Medline].

  19. Weiss JS. Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea. Mar 1992;11(2):93-101. [Medline].

  20. Weiss JS. Visual morbidity in thirty three families with Schnyder's crystalline corneal dystrophy. Transactions of the American Ophthalmology Society. In press.

  21. Weiss JS, Kruth HS, Kuivaniemi H, et al. Mutations in the UBIAD1 gene on chromosome short arm 1, region 36, cause Schnyder crystalline corneal dystrophy. Invest Ophthalmol Vis Sci. Nov 2007;48(11):5007-12. [Medline].

  22. Weiss JS, Rodrigues MM, Kruth HS, et al. Panstromal Schnyder's corneal dystrophy. Ultrastructural and histochemical studies. Ophthalmology. Jul 1992;99(7):1072-81. [Medline].

  23. Weller RO, Rodger FC. Crystalline stromal dystrophy: histochemistry and ultrastructure of the cornea. Br J Ophthalmol. Jan 1980;64(1):46-52. [Medline].

  24. Wu CW, Lin PY, Liu YF, et al. Central corneal mosaic opacities in Schnyder's crystalline dystrophy. Ophthalmology. Apr 2005;112(4):650-3. [Medline].

 
Previous
Next
 
A 22-year-old woman with circular corneal opacity best seen in retroillumination. Weiss JS: Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea 1992; 11(2): 93-101.
A 20-year-old woman with ringlike deposition of anterior stromal cholesterol crystals. Weiss JS: Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea 1992; 11(2): 93-101.
A 37-year-old man with central disclike opacity, affecting the entire stromal thickness, anterior stromal cholesterol crystals, and peripheral arcus lipoides. Weiss JS: Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea 1992; 11(2): 93-101.
A 78-year-old woman with dense arcus lipoides in the corneal periphery, sparing the corneal scleral limbus. Weiss JS: Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea 1992; 11(2): 93-101.
The natural history of Schnyder dystrophy with age. Weiss JS: Schnyder's dystrophy of the cornea: a Swede-Finn connection. Cornea 1992; 11(2): 93-101.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.