eMedicine Specialties > Ophthalmology > Cornea

Dystrophy, Macular: Differential Diagnoses & Workup

Author: Natalie Afshari, MD, MA, FACS, Associate Professor of Ophthalmology, Duke University Eye Center, Duke University Medical Center
Coauthor(s): William B Trattler, MD, Ophthalmologist, The Center for Excellence in Eye Care; Volunteer Assistant Professor of Ophthalmology, Bascom Palmer Eye Institute; William Lloyd Clark, MD, Consulting Staff, Palmetto Retina
Contributor Information and Disclosures

Updated: Jan 26, 2010

Differential Diagnoses

Dystrophy, Crystalline
Dystrophy, Granular
Dystrophy, Lattice

Other Problems to Be Considered

Corneal opacities

Workup

Procedures

  • A corneal biopsy sample, which is not clinically indicated, reveals deposits of glycosaminoglycans, which stain with Alcian blue and colloidal iron.
  • Light microscopy reveals glycosaminoglycan deposits within stromal keratocytes.

Histologic Findings

  • Deposits in macular dystrophy are composed of glycosaminoglycans, which stain with Alcian blue and colloidal iron.
  • Light microscopy demonstrates glycosaminoglycan deposits within stromal keratocytes.
  • Two types of macular dystrophy are noted based on the absence or presence of keratan sulfate within the corneal stroma. Type I macular dystrophy has an absence of keratan sulfate, whereas in type II, keratan sulfate is present.

More on Dystrophy, Macular

Overview: Dystrophy, Macular
Differential Diagnoses & Workup: Dystrophy, Macular
Treatment & Medication: Dystrophy, Macular
Follow-up: Dystrophy, Macular
Multimedia: Dystrophy, Macular
References

References

  1. Kannabiran C. Genetics of corneal endothelial dystrophies. J Genet. Dec 2009;88(4):487-94. [Medline].

  2. Quantock AJ, Young RD, Akama TO. Structural and biochemical aspects of keratan sulphate in the cornea. Cell Mol Life Sci. Dec 27 2009;[Medline].

  3. Al-Swailem SA, Al-Rajhi AA, Wagoner MD. Penetrating keratoplasty for macular corneal dystrophy. Ophthalmology. Feb 2005;112(2):220-4. [Medline].

  4. Albert D, Jakobiec F. Principles and Practice of Ophthalmology. Vol 1. 1996:26-49.

  5. Edward DP, Yue BY, Sugar J, et al. Heterogeneity in macular corneal dystrophy. Arch Ophthalmol. Nov 1988;106(11):1579-83. [Medline].

  6. Hafner A, Langenbucher A, Seitz B. Long-term results of phototherapeutic keratectomy with 193-nm excimer laser for macular corneal dystrophy. Am J Ophthalmol. Sep 2005;140(3):392-6. [Medline].

  7. Klintworth GK, Meyer R, Dennis R, et al. Macular corneal dystrophy. Lack of keratan sulfate in serum and cornea. Ophthalmic Paediatr Genet. Dec 1986;7(3):139-43. [Medline].

  8. Krachmer J. Cornea. Vol 2. 1996.

  9. Wirtitsch MG, Ergun E, Hermann B. Ultrahigh resolution optical coherence tomography in macular dystrophy. Am J Ophthalmol. Dec 2005;140(6):976-983. [Medline].

Further Reading

Keywords

corneal dystrophy, corneal stroma, anterior corneal dystrophy, stromal corneal dystrophy, posterior corneal dystrophy, corneal dystrophies

Contributor Information and Disclosures

Author

Natalie Afshari, MD, MA, FACS, Associate Professor of Ophthalmology, Duke University Eye Center, Duke University Medical Center
Natalie Afshari, MD, MA, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Coauthor(s)

William B Trattler, MD, Ophthalmologist, The Center for Excellence in Eye Care; Volunteer Assistant Professor of Ophthalmology, Bascom Palmer Eye Institute
William B Trattler, 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.

William Lloyd Clark, MD, Consulting Staff, Palmetto Retina
William Lloyd Clark, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
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.

Managing Editor

Christopher J Rapuano, MD, Professor, Department of Ophthalmology, Jefferson Medical College of Thomas Jefferson University; Co-Chairman of the Cornea Service, Co-Chairman 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

CME Editor

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.

 
 
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