Ophthalmologic Manifestations of Pseudoxanthoma Elasticum Treatment & Management

  • Author: Andrew A Dahl, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 30, 2012
 

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] Although 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]

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

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Consultations

  • Neurologist
  • Pediatrician
  • Internist
  • Cardiologist
  • Gastroenterologist
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Diet

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

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Activity

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.

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Contributor Information and Disclosures
Author

Andrew A Dahl, MD  Director of Ophthalmology Teaching, Mid-Hudson Family Practice Institute, The Institute for Family Health; Assistant Professor of Surgery (Ophthalmology), New York College of Medicine

Andrew A Dahl, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Ophthalmology, American College of Surgeons, American Medical Association, American Society of Cataract and Refractive Surgery, and Wilderness Medical Society

Disclosure: Nothing to disclose.

Coauthor(s)

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

Disclosure: Nothing to disclose.

Sherif M El-Harazi, MD, MPH  Consulting Staff, Department of Ophthalmology, Sherif El-Harazi, MD

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, and International Society of Refractive Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

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, and South Carolina Medical Association

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

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

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

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers 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

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