Angioid Streaks: Background, Pathophysiology, Epidemiology

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Overview

Background

In 1889, Doyne first described angioid streaks in a patient with retinal hemorrhages secondary to trauma.^[1]Later in 1892, Knapp named them angioid streaks because of their resemblance to blood vessels.^[2]Angioid streaks result from pathological changes at the level of the Bruch membrane, which was confirmed histologically in the late 1930s. See the images below.

Progression of angioid streaks. Large subretinal hemorrhage.

View Media Gallery

Same eye as in previous image, 11 months later. Partial resolution of subretinal blood. Notice the old subretinal hemorrhage under the fovea and color change to white-yellow.

View Media Gallery

Late complication of choroidal neovascularization in angioid streaks. Same eye as in previous images, 5 years later. Notice the extensive scarring and subretinal exudates and dehemoglobinized blood.

View Media Gallery

Pathophysiology

Controversy about the pathophysiology of angioid streaks exists. In some diseases, including pseudoxanthoma elasticum (PXE) and Paget disease, the Bruch membrane may become calcified and brittle with subsequent development of cracks. However, cytoimmunochemistry and x-ray analysis had shown that the earliest abnormality in PXE was abnormal accumulation and metabolism of polyanions (ie, glycosaminoglycans, glycoproteins) within the Bruch membrane.

The lines of force within the eye resulting from the pull of intrinsic and extrinsic ocular muscles on the relatively fixed site of the optic nerve have been studied. Those lines of forces had the same configuration as the peripapillary interlacement and radial extensions of angioid streaks. Such forces acting on the Bruch membrane undoubtedly account for the configuration of the breaks. However, in sickle cell disease, Bruch membrane calcification is not a common part of the pathology.

It is believed that the pathology may be a combination of diffuse elastic degeneration of the Bruch membrane, iron deposition in elastic fibers from hemolysis with secondary mineralization, and impairment of nutrition because of sickling, stasis, and small vessel occlusion. Klien proposed a dual mechanism as a cause of these cracks in general, as follows: a primary abnormality of fibers of the Bruch membrane, and an increase in availability of metal salts or a tendency for their deposition, resulting in a secondary brittleness of the membranes.^[3]

Frequency

United States

Not known

International

Not known

Mortality/Morbidity

Moderate-to-severe central visual loss is mainly related to foveal involvement with a dehiscence of the underlying Bruch membrane or a neovascular membrane formation under the retina. Choroidal neovascularization (CNV) is the major cause of vision loss and affects 70-86% of patients with angioid streaks.

Race

White people are affected most. Two studies showed similar results: of all patients with angioid streaks, 66.2% of patients were white, compared with 29% of Asian origin and 3.7% of black people.^{[4, 5]}

Sex

No sexual predilection exists.

Age

The age of onset is variable with the underlying etiology. In one study, the age of onset of 50 patients with angioid streaks was reported as follows:

Patients with PXE present in the third decade with a mean age of 51.7 years.
Patients with sickle cell disease tend to be in their second and third decades with a mean age of 41.7 years.
Patients with Paget disease tend to be older at the time of diagnosis with a mean age of 67 years.
Angioid streaks in patients with no systemic disease or with rare etiologies tend to present late in life with a mean age of 65.7 years. Rare etiologies include patients with peptic ulcer, diabetes, hypertension, arthritis, breast cancer, metastatic cancer, rheumatoid spondylitis, and heart disease.

Clinical Presentation

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Knapp H. On the formation of dark angioid streaks as an unusual metamorphosis of retinal hemorrhage. Arch Ophthalmol. 1892. 21:289-292.
Klien BA. Angioid Streaks: A clinical and histopathologic study. American Journal of Ophthalmology. 1947. 30:955-68.
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Media Gallery

Progression of angioid streaks. Large subretinal hemorrhage.
Same eye as in previous image, 11 months later. Partial resolution of subretinal blood. Notice the old subretinal hemorrhage under the fovea and color change to white-yellow.
Late complication of choroidal neovascularization in angioid streaks. Same eye as in previous images, 5 years later. Notice the extensive scarring and subretinal exudates and dehemoglobinized blood.
Red-free photograph of the optic nerve and posterior pole showing the cracks in the Bruch membrane. Notice the retinal arteries and veins crossing over the dark red streaks.
Early fluorescein angiography showing the early hyperfluorescence, window defect, of the angioid streaks.
Late fluorescein angiography of the same eye as in Media file 2. Notice the staining of the edges of the streaks. Also, staining in the center of the macula is present due to extension of the Bruch membrane crack. When compared to early fluorescein angiography, no active leakage is present.
Right eye, midphase arteriovenous, showing choriocapillaris atrophic changes. This 45-year-old patient underwent 3 injections of Avastin and one session of half-time photodynamic therapy.
Same patient as in previous image, a few months before the Avastin injection and half-time photodynamic therapy.
A 50-year-old man with a 2-month history of blurring vision in the left eye. The color photograph showed subretinal blood and large membrane, extrafoveal in location.
Early fundus fluorescein angiography showing the hyperfluorescence of the choroidal neovascular membrane of the left eye of the same patient in the previous image.
Late fundus fluorescein angiography confirming the active choroidal neovascular membrane of the left eye.

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Author

Mohammad Abusamak, MD Chief of Retina Division, Jordan University Hospital; Assistant Professor, Division of Ophthalmology, University of Jordan Faculty of Medicine; Ophthalmologist, Amman Eye Clinic, Jordan

Mohammad Abusamak, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists

Disclosure: Nothing to disclose.

Coauthor(s)

Shauna Berry, DO Resident Physician, Department of Ophthalmology, Larkin Community Hospital, Nova Southeastern University College of Osteopathic Medicine

Shauna Berry, DO is a member of the following medical societies: American Academy of Ophthalmology, American Osteopathic Association, American Osteopathic Colleges of Ophthalmology and Otolaryngology-Head and Neck Surgery, Florida Osteopathic Medical Association, Women in Ophthalmology, Inc

Disclosure: Nothing to disclose.

Ama Sadaka, MD Resident Physician, Department of Ophthalmology, University of Cincinnati Hospital

Ama Sadaka, MD is a member of the following medical societies: Alpha Omega Alpha

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

Andrew G Lee, MD Chair, Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital; Clinical Professor, Associate Program Director, Department of Ophthalmology and Visual Sciences, University of Texas Medical Branch School of Medicine; Clinical Professor, Department of Surgery, Division of Head and Neck Surgery, University of Texas MD Anderson Cancer Center; Professor of Ophthalmology, Neurology, and Neurological Surgery, Weill Medical College of Cornell University; Clinical Associate Professor, University of Buffalo, State University of New York School of Medicine

Andrew G Lee, MD is a member of the following medical societies: American Academy of Ophthalmology, American Geriatrics Society, Houston Neurological Society, Houston Ophthalmological Society, International Council of Ophthalmology, North American Neuro-Ophthalmology Society, Texas Ophthalmological Association

Disclosure: Received ownership interest from Credential Protection for other.

Additional Contributors

Russell P Jayne, MD Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas

Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, American Society of Retina Specialists

Disclosure: Nothing to disclose.

Sections Angioid Streaks
Overview
Presentation
- History
- Physical
- Causes
- Show All
DDx
Workup
Treatment
Medication
Follow-up
Media Gallery
References

Angioid Streaks

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