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Ophthalmologic Manifestations of Pseudoxanthoma Elasticum Treatment & Management

  • Author: Andrew A Dahl, MD, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
Updated: May 09, 2016

Medical Care

Evaluation for patients with pseudoxanthoma elasticum usually can be conducted on an outpatient basis. The most visually devastating complication of angioid streaks in the eye is neovascular membrane formation in the macula at the level of the pigment epithelium-Bruch membrane. This is similar to the neovascularization seen in age-related macular degeneration (ARMD).

If choroidal neovascularization (CNV) is found on clinical examination, fluorescein angiography and optical coherence tomography (OCT) may be helpful in delineating and documenting the location and size of a neovascular membrane.[4] Therapy with intravitreal antivascular endothelial growth factor (anti-VEGF) agents should be considered.[5] Neovascular membranes away from the fovea have also been treated via laser. Photodynamic therapy has had limited success in the treatment of CNV. Although repeated intravitreal injection of the fusion protein aflibercept or the monoclonal antibody ranibizumab or the off-label use of bevacizumab seems to maintain visual acuity, CNV frequently recurs or new areas of CNV may develop.[6]

In a small case series of patients with pseudoxanthoma elasticum, 20% of eyes had geographic atrophy independent of choroidal neovascularization. Linear pigmented pattern dystrophy appeared to predate geographic atrophy.[7] Recognition of geographic atrophy is important as therapies to slow or reverse geographic atrophy, including ciliary neurotrophic factor delivered by an encapsulated cell, stem cell therapy, fenretinide, doxycycline, sirolimus, and fluocinolone acetonide intravitreal implants, are under investigation.

If keratoconus is found in association with pseudoxanthoma elasticum, management with contact lenses, riboflavin-assisted cross-linking, or penetrating keratoplasty can be used, similarly to patients without pseudoxanthoma elasticum.



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Dietary restriction of calcium and phosphorus to minimum daily requirement levels has shown arrest in progression of the disease.



Eye protection for contact sports or physical activity is indicated.

Patients with pseudoxanthoma elasticum who are exposed to ocular trauma are predisposed to the development of angioid streaks and subsequent visual loss.

Contributor Information and Disclosures

Andrew A Dahl, MD, FACS Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center

Andrew A Dahl, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Intraocular Lens Society, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Medical Society of the State of New York, New York State Ophthalmological Society, Outpatient Ophthalmic Surgery Society

Disclosure: Nothing to disclose.


Sherif M El-Harazi, MD, MPH Private Practice in Ophthalmology

Sherif M El-Harazi, MD, MPH is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Cataract and Refractive Surgery, International Society of Refractive Surgery

Disclosure: Nothing to disclose.

Diego Calonje, MD Consulting Staff, Department of Ophthalmology, Private Practice

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.

Steve Charles, MD Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Macula Society, Retina Society, Club Jules Gonin

Disclosure: Received royalty and consulting fees for: Alcon Laboratories.

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

V Al Pakalnis, MD, PhD Professor of Ophthalmology, University of South Carolina School of Medicine; Chief of Ophthalmology, Dorn Veterans Affairs Medical Center

V Al Pakalnis, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, South Carolina Medical Association

Disclosure: Nothing to disclose.

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