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Angioid Streaks Clinical Presentation

  • Author: Mohammad Abusamak, MD; Chief Editor: Hampton Roy, Sr, MD  more...
Updated: Feb 20, 2014


Visual acuity is normal unless a leakage, bleeding, or Bruch membrane dehiscence involves the central macula. Distorted central vision (metamorphopsia) and micropsia can be early signs of macular involvement.



Ophthalmoscopic findings are as follows:

  • At times, angioid streaks can be difficult to identify unless a careful examination of the posterior pole is performed.
  • Angioid streaks usually present as linear gray or dark red lines with irregular serrated edges lying beneath normal retinal blood vessels. The streaks intercommunicate in a ringlike fashion around the optic disc in 27% of cases and radiate outward in a tapering fashion from the peripapillary area in 73% of patients. The streaks run a convoluted course and tend to terminate abruptly. Angioid streaks usually do not extend past the equator.
  • Associated findings in patients with angioid streaks are as follows:
    • Peau d'orange or leopard skin spotting, consisting of speckled yellowish mottling of the posterior pole mostly apparent in the temporal macula. This finding may antedate the appearance of angioid streaks. It is seen more frequently in patients with PXE and is caused by alterations at the level of the Bruch membrane as demonstrated by the diffuse pattern of hyperfluorescence on indocyanine green angiography (ICGA).
    • Peripapillary chorioretinal atrophy, focal peripheral chorioretinal scars (salmon spots), and reticular pigment dystrophy of the macula may be present at the time of diagnosis.
    • Optic nerve head drusen (hyaline bodies) may antedate the appearance of angioid streaks, and they may be the earliest clinical manifestations of PXE. As many as 25% of patients with angioid streaks have clinical or echographic evidence of disk drusen. These hyaline bodies were associated with neovascularization in the peripapillary area, and, in some patients, they were associated with acute visual loss due to the pressure effect on the optic nerve head.
    • Crystalline bodies typically are seen in the midperipheral fundus or inferior to the optic nerve. They are multiple, round, small, and subretinal lesions. Usually, these crystalline bodies cause some atrophy of the retinal pigment epithelium (RPE). In older patients, peripheral retinal scars and calcifications can be seen.[6]
    • Optic atrophy may be seen in patients with Paget disease of the bone. It cannot be explained solely on the basis of bony compression.
  • Severe visual impairment is caused by one of the following conditions in 70% of cases:
    • CNV with subsequent serous and hemorrhagic detachment of the fovea (most serious complication)
    • CNV development occurs in 72-86% of all patients over time.[7] Once a patient develops CNV in one eye, it becomes bilateral in 50% within 18 months of the initial diagnosis.[8]
    • Choroidal rupture secondary to trivial trauma with secondary hemorrhage involving the fovea
    • Foveal involvement by a streak with damage to RPE and choriocapillaris (may result in permanent loss of central visual acuity)


See the list below:

  • Idiopathic: In 50% of patients with angioid streaks, no associated systemic disease is present.
  • Systemic association
    • PXE is an uncommon inherited disorder of connective tissue. It has generalized effects on the elastin fibrils in the dermis, arterial walls, heart, gastrointestinal (GI) tract, and Bruch membrane, resulting in mineralization and deposition of phosphorus. The 4 types of inheritance of PXE include 2 of which are autosomal dominant and 2 of which are autosomal recessive. It is the most common systemic disorder associated with angioid streaks. Diagnosing PXE is important because 85% of patients develop ocular involvement, usually after the second decade of life. The combination of PXE and ocular involvement is referred to as Grönblad-Strandberg syndrome.
    • Histologically, degenerative changes and calcifications of the elastic tissues in the skin and arteries are present. The following systemic findings may help the ophthalmologist to confirm the diagnosis of angioid streaks secondary to PXE, as well as to take care of complications.
    • Dermatologic findings include yellow papules, "chicken skin" arranged in a linear or reticulate pattern, in plaques, over the side of the neck, antecubital fossae, axillae, groin, and paraumbilical area.
    • Cardiovascular manifestations include accelerated hypertension at an earlier age due to atherosclerosis, which may be related to renovascular disease, premature coronary artery disease, peripheral vascular disease, and mitral incompetence.
    • Some patients develop genitourinary bleeding as part of PXE.
    • Neurologic findings may include cerebrovascular accidents, intracranial aneurysms, and cerebral ischemia.
  • Paget disease is a chronic, progressive, and in some cases inherited disease, characterized by bone deformity. It may be confined to a few bones, or in some patients, it represents a generalized abnormality that gives rise to enlargement of the skull, kyphoscoliosis, deafness, and deformities of long bones. However, angioid streaks occur in fewer than 2% of patients. Osteoclastic activity with an osteoblastic reaction occurs. Although the etiology is unknown, some clinicians believe it is related to a slow virus infection, measles, or respiratory syncytial virus. Both males and females are affected equally.
  • Ehlers-Danlos syndrome is a rare autosomal dominant disorder of collagen resulting from a deficiency of hydroxylysine. Ocular findings include epicanthal folds, keratoconus, high myopia, retinal detachment, blue sclera, ectopia lentis, and angioid streaks. Systemic associations include the following:
    • Skin and musculoskeletal - Thin hyperplastic skin that heals poorly, hyperextensible joints that may predispose to recurrent falls, hydrarthrosis, and pseudotumor formation over elbows and knees
    • Cardiovascular disease consists of bleeding diathesis, dissecting aneurysms, spontaneous rupture of large blood vessels, and mitral prolapse.
    • Others - Diaphragmatic hernias and diverticulum of the GI and respiratory tracts
  • Hemoglobinopathies that are occasionally associated with angioid streaks include the following:
    • Homozygous sickle cell disease (Hb-SS)
    • Sickle cell trait (Hb-AS)
    • Sickle cell thalassemia (Hb-thal)
    • Sickle cell hemoglobin (Hb-SC)
    • Hemoglobin H (Hb-H)
    • Homozygous B-thalassemia major
    • Intermedia
    • Minor and hereditary spherocytosis
  • The frequency of angioid streaks increases with age; it is about 1.5% in younger patients and increases to 22% in older patients. Complications, such as macular degeneration and choroidal neovascular membranes, are uncommon in this subgroup of patients with angioid streaks. In general, choroidal neovascular membranes (CNVM) and serous detachments of the macula are less common in black patients.
  • Other systemic diseases associated with angioid streaks include the following:
    • Abetalipoproteinemia
    • Acromegaly
    • Dwarfism
    • Diabetes mellitus
    • Hemochromatosis
    • Facial angiomatosis
    • Idiopathic thrombocytic purpura
    • Chronic familial hyperphosphatemia
    • Hypercalcinosis
    • Diffuse lipomatosis
    • Acquired hemolytic anemia
    • Myopia
    • Neurofibromatosis
    • Epilepsy
    • Senile elastosis
    • Sturge-Weber syndrome
    • Trauma
    • Tuberous sclerosis
Contributor Information and Disclosures

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.

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

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.

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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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