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Lattice Corneal Dystrophy Clinical Presentation

  • Author: Natalie A Afshari, MD, MA, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Sep 22, 2014
 

History

Patients with lattice corneal dystrophy may have decreased vision, photosensitivity, ocular discomfort, diminished corneal sensation, and/or eye pain (from recurrent corneal erosions). Recurrent corneal erosions may precede corneal opacities and recognizable stromal disease.[1] As the pattern of inheritance for this dystrophy is autosomal dominant, one of the parents of the patient likely also has lattice corneal dystrophy.

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Physical

Lattice corneal dystrophy is characterized by branching, refractile lattice lines, which are observed best in retroillumination. Both corneas are usually symmetrically involved. In the most common form, lattice corneal dystrophy type I, the refractile lines are more prominent centrally than peripherally. Later in life, stromal haze can develop. Eventually, significant stromal scarring and subepithelial fibrosis may occur.

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Causes

The genetic defect of lattice corneal dystrophy type I has been mapped to the TGFBI (BIGH3) gene of chromosome 5q. Several other corneal dystrophies also have genetic defects of the TGFBI (BIGH3) gene, including granular dystrophy, Avellino dystrophy, and Reis-Bückler dystrophy. Lattice corneal dystrophy type II results from a mutation in the GSN gene.

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Contributor Information and Disclosures
Author

Natalie A Afshari, MD, MA, FACS Stuart I Brown, MD, Chair in Ophthalmology In Memory of Donald P Shiley, Professor of Ophthalmology, Chief of Cornea and Refractive Surgery, Director of Education, Fellowship Program Director in Cornea and Refractive Surgery, Shiley Eye Center, University of California, San Diego, School of Medicine

Natalie A Afshari, MD, MA, FACS is a member of the following medical societies: American Academy of Ophthalmology, American Society of Cataract and Refractive Surgery, Association for Research in Vision and Ophthalmology, Heed Ophthalmic Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

William Lloyd Clark, MD Palmetto Retina

William Lloyd Clark, MD is a member of the following medical societies: Alpha Omega Alpha, Association for Research in Vision and Ophthalmology, American Academy of Ophthalmology

Disclosure: Nothing to disclose.

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, American Society of Cataract and Refractive Surgery

Disclosure: Received consulting fee from Allergan for consulting; Received consulting fee from Alcon for consulting; Received consulting fee from Bausch & Lomb for consulting; Received consulting fee from Abbott Medical Optics for consulting; Received consulting fee from CXLUSA for none; Received consulting fee from LensAR for none.

Joanne W Ho University of California, San Diego, School of Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

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 Hospital

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

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Cornea Society, Allergan, Bausch & Lomb, Bio-Tissue, Shire, TearScience, TearLab<br/>Serve(d) as a speaker or a member of a speakers bureau for: Allergan, Bausch & Lomb, Bio-Tissue, TearScience.

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

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.

References
  1. Klintworth GK. Corneal dystrophies. Orphanet J Rare Dis. 2009 Feb 23. 4:7. [Medline]. [Full Text].

  2. Weiss JS, Møller HU, Lisch W, Kinoshita S, Aldave AJ, Belin MW, et al. The IC3D classification of the corneal dystrophies. Cornea. 2008 Dec. 27 Suppl 2:S1-83. [Medline]. [Full Text].

  3. Stone EM, Mathers WD, Rosenwasser GO, et al. Three autosomal dominant corneal dystrophies map to chromosome 5q. Nat Genet. 1994. 6(1):47-51. [Medline].

  4. Klintworth GK, Bao W, Afshari NA. Two mutations in the TGFBI (BIGH3) gene associated with lattice corneal dystrophy in an extensively studied family. Invest Ophthalmol Vis Sci. 2004 May. 45(5):1382-8. [Medline].

  5. Musch DC, Niziol LM, Stein JD, Kamyar RM, Sugar A. Prevalence of corneal dystrophies in the United States: estimates from claims data. Invest Ophthalmol Vis Sci. 2011 Aug. 52(9):6959-63. [Medline]. [Full Text].

  6. Meretoja J. Familial systemic paramyloidosis with lattice dystrophy of the cornea, progressive cranial neuropathy, skin changes and various internal symptoms. A previously unrecognized heritable syndrome. Ann Clin Res. 1969 Dec. 1(4):314-24. [Medline].

  7. Das S, Langenbucher A, Seitz B. Excimer laser phototherapeutic keratectomy for granular and lattice corneal dystrophy: a comparative study. J Refract Surg. 2005 Nov-Dec. 21(6):727-31. [Medline].

  8. Morita Y, Chikama T, Yamada N, Morishige N, Sonoda KH, Nishida T. New mode of treatment for lattice corneal dystrophy type I: corneal epithelial debridement and fibronectin eye drops. Jpn J Ophthalmol. 2012 Jan. 56(1):26-30. [Medline].

  9. Resch MD, Schlotzer-Schrehardt U, Hofmann-Rummelt C, Kruse FE, Seitz B. Alterations of epithelial adhesion molecules and basement membrane components in lattice corneal dystrophy (LCD). Graefes Arch Clin Exp Ophthalmol. 2009 Aug. 247(8):1081-8. [Medline].

  10. Albert D, Jakobiec F. Principles and Practice of Ophthalmology. 1994. Vol 1: 26-49.

  11. Das S, Langenbucher A, Seitz B. Delayed healing of corneal epithelium after phototherapeutic keratectomy for lattice dystrophy. Cornea. 2005 Apr. 24(3):283-7. [Medline].

  12. Kawashima M, Yamada M, Funayama T, et al. Six cases of late-onset lattice corneal dystrophy associated with gene mutations induced by the transforming growth factor-beta. Nippon Ganka Gakkai Zasshi. 2005 Feb. 109(2):93-100. [Medline].

  13. Krachmer J. Cornea (3 volume set). Vol 2: 1996.

  14. Mashima Y, Yamamoto S, Inoue Y, et al. Association of autosomal dominantly inherited corneal dystrophies with BIGH3 gene mutations in Japan. Am J Ophthalmol. 2000 Oct. 130(4):516-7. [Medline].

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Lattice corneal 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.
Slit lamp image of lattice corneal 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.
 
 
 
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