Posterior Polymorphous Corneal Dystrophy

Author: Dustin J Coupal, MD, FRCSC; Chief Editor: Hampton Roy Sr, MD more...

Updated: Feb 23, 2010

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Background
Pathophysiology
Epidemiology
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Background

First described by Koeppe, posterior polymorphous corneal dystrophy (PPMD) is a dominantly inherited condition characterized by particular alterations of the Descemet membrane and the corneal endothelium.^[1]Typically, the corneal changes are either slowly progressive or nonprogressive. In severe cases, corneal decompensation and edema can occur. Although PPMD is most often a bilateral condition, marked asymmetry in the degree of involvement may be seen. Most persons with PPMD are asymptomatic.

Slit lamp image demonstrates posterior corneal vesicles and opacities in linear bands and other polymorphous configurations typical of posterior polymorphous corneal dystrophy.

Pathophysiology

Three main abnormalities are described: vesicular changes, endothelial band lesions, and irregular opacities of the posterior corneal surface. The corneal endothelium undergoes a transformation and demonstrates many epithelial characteristics on examination with electron microscopy and immunohistochemical analysis. Often, the endothelium is found to be multilayered.

Frequency

United States

The frequency of PPMD is not well documented. Although it is considered to be uncommon, PPMD may be recognized and diagnosed more often in recent years than in the past.

Mortality/Morbidity

The effect of PPMD on patients is highly variable, with a broad clinical spectrum of findings, ranging from nonprogressive asymptomatic disease to progressive or advanced debilitating corneal disease with corneal decompensation and glaucoma.

Race

No racial predilection exists.

Sex

No sexual predilection exists.

Age

Although PPMD is an inherited corneal dystrophy, the age at diagnosis is highly variable because of the broad spectrum of disease severity. Findings may be present at birth. Most patients are first identified at age 30-50 years; however, this is likely only indicative of a more common age for ocular examinations.

Some patients may present at birth with congenital disease, exhibiting advanced disease with corneal edema.
Presentation in adulthood is indicative of a more stable disease state with a decreased probability of progression to corneal decompensation.
Most patients with significant symptoms present at age 25-50 years.

Proceed to Clinical Presentation

Contributor Information and Disclosures

Author

Dustin J Coupal, MD, FRCSC Eye Specialist and Surgeon, Private Practice

Dustin J Coupal, MD, FRCSC is a member of the following medical societies: American Academy of Ophthalmology, Canadian Medical Association, Canadian Medical Protective Association, and Canadian Ophthalmological Society

Disclosure: Nothing to disclose.

Coauthor(s)

W Keith Hamilton, MD Clinical Assistant Professor, Department of Ophthalmology, Saskatoon City Hospital Eye Centre

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.

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

Disclosure: Medscape Salary Employment

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

Ralph Garzia, OD Assistant Dean for Clinical and Academic Programs, Associate Professor, College of Optometry, University of Missouri at St Louis

Ralph Garzia, OD is a member of the following medical societies: American Academy of Optometry and American Optometric Association

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

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