Macular Dystrophy Workup

  • Author: Natalie Afshari, MD, MA, FACS; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Jan 26, 2010
 

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.
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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.
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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: Allergan Consulting fee Consulting; Alcon Consulting fee Consulting; Inspire Consulting fee Consulting; Abbott Medical Optics Consulting fee Consulting

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.

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. 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].

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Macular dystrophy. Image courtesy of James J. Reidy, MD, FACS, Associate Professor of Ophthalmology, State University of New York, School of Medicine & Biomedical Sciences, Buffalo, New York.
Macular dystrophy.
 
 
 
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