eMedicine Specialties > Ophthalmology > Cornea

Dystrophy, Lattice

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

Introduction

Background

Lattice dystrophy is usually an autosomal dominant condition and is the most common of stromal dystrophies. Like granular and Avellino dystrophy, the genetic defect of lattice dystrophy has been mapped to the BIGH3 gene on chromosome 5q.1 Onset of the corneal changes usually occurs in the first decade of life, although patients may remain asymptomatic for years. Examination of the cornea in the second to third decade of life reveals branching, refractile lattice lines with intervening haze, which are observed best in retroillumination. An example of lattice corneal dystrophy is shown in the image below.

Lattice corneal dystrophy. Image courtesy of Jame...

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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Lattice corneal dystrophy. Image courtesy of Jame...

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.


Pathophysiology

The cornea is the clear outer coat of the front of the eye. A dystrophy of the cornea is defined as a bilateral noninflammatory clouding of the cornea. The corneal dystrophies can be placed into 3 categories based on their location within the cornea as follows: (1) the anterior corneal dystrophies affect the corneal epithelium and may involve the Bowman membrane, (2) stromal corneal dystrophies affect the central layer of the cornea (the stroma), and (3) the posterior corneal dystrophies involve the Descemet membrane and the endothelium.

Most corneal dystrophies have onset when younger than 20 years (exceptions include map-dot-fingerprint dystrophy and Fuchs corneal dystrophy). Most corneal dystrophies are dominantly inherited, exceptions are macular dystrophy, type 3 lattice dystrophy, and the autosomal-recessive form of congenital hereditary endothelial dystrophy.

Frequency

United States

Although lattice dystrophy is the most common of the stromal dystrophies, it is relatively rare.

Mortality/Morbidity

Excessive corneal erosions can lead to decreased visual acuity, which may require a corneal transplant or phototherapeutic keratectomy (PTK).

Sex

No sexual predilection is noted.

Age

Onset of the corneal changes usually occurs in the first decade of life, although patients may remain asymptomatic for years. Examination of the cornea in the second to third decade of life reveals branching, refractile lattice lines, which are observed best in retroillumination. Over time, the lattice lines and other opacities coalesce, forming anterior stromal haze that decreases the visual acuity.

Clinical

History

Patients with lattice corneal dystrophy may have decreased vision, photosensitivity, and/or eye pain (from recurrent corneal erosions). This dystrophy is usually autosomal dominant, so one of the parents likely has lattice corneal dystrophy as well.

Physical

Lattice corneal dystrophy is characterized by branching, refractile lattice lines, which are observed best in retroillumination. In the most common form, type 1 lattice dystrophy, 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.

Causes

The genetic defect of lattice dystrophy has been mapped to the BIGH3 gene of chromosome 5q. Other corneal dystrophies also have genetic defects of the BIGH3 gene, including granular dystrophy, Avellino dystrophy, and Reis-Bückler dystrophy.

More on Dystrophy, Lattice

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

  1. 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].

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

  3. 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. Aug 2009;247(8):1081-8. [Medline].

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

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

  6. 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. Feb 2005;109(2):93-100. [Medline].

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

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

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Further Reading

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Keywords

lattice dystrophy, corneal dystrophy, stromal dystrophy, stromal dystrophies, corneal erosion, corneal transplant, phototherapeutic keratectomy, PTK, treatment, diagnosis, symptoms

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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: 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

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

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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