OcuLearning › Signs
Angioid Streaks
ROUTINE — Signs
Symptoms
- Frequently asymptomatic in early stages.
- Decreased central vision or metamorphopsia if choroidal neovascularization (CNV) develops.
- Sudden, profound vision loss following minor blunt ocular trauma.
Clinical Findings
Pathophysiology
- Crack-like dehiscences in a thickened, calcified, and brittle Bruch membrane.
- Linear breaks radiating from the peripapillary region caused by normal ocular pulsations.
Clinical appearance
- Bilateral, jagged, reddish-brown to gray linear bands radiating from a peripapillary ring (spoke-wheel pattern).
- Peau d'orange: Orange-peel mottling of the temporal mid-periphery (indicates diffuse Bruch thickening).
- Salmon spots: Peripheral chorioretinal scars from prior small subretinal hemorrhages.
- Optic disc drusen (commonly associated with pseudoxanthoma elasticum).
Systemic associations (pepsi mnemonic)
- P: Pseudoxanthoma elasticum (most common; skin "plucked-chicken" appearance, GI bleeding, atherosclerosis).
- E: Ehlers-Danlos syndrome (joint hypermobility, skin hyperextensibility).
- P: Paget disease of bone (enlarged skull, elevated alkaline phosphatase).
- S: Sickle cell disease and other hemoglobinopathies (thalassemia, spherocytosis).
- I: Idiopathic (approximately 50% of cases).
Complications
- Choroidal neovascularization (CNV): Develops in 70–80% of patients; typically Type 2 with high risk of disciform scarring.
- Traumatic Bruch rupture: Minor trauma can cause catastrophic subretinal hemorrhage.
Imaging
- Fundus Autofluorescence (FAF): More sensitive than ophthalmoscopy; shows diffuse damage beyond visible streaks.
- Fluorescein Angiography (FA): Early hyperfluorescence (window defects) and late staining; detects active CNV.
- OCT / OCTA: Essential for monitoring subretinal fluid and detecting non-exudative CNV.
Etiology
Genetic & metabolic
- Pseudoxanthoma elasticum (PXE): ABCC6 gene mutation; involves skin flexures and vascular calcification.
- Paget disease of bone: Late-stage manifestation; associated with bone remodeling and hearing loss.
- Hemoglobinopathies: Sickle cell, thalassemia, and hereditary spherocytosis.
Connective tissue
- Ehlers-Danlos syndrome: Collagen abnormality leading to fragile tissues.
Other
- Idiopathic: No identifiable systemic cause in half of the population.
- Rare: Acromegaly, hemochromatosis, lead poisoning, and senile elastosis.
Red Flags
- Central distortion or new scotoma: Suggests active maculopathy or CNV.
- History of blunt trauma: High risk for extensive Bruch rupture even with trivial force.
- Systemic symptoms: GI bleeding or cardiovascular disease (especially in PXE).
- Peripheral neovascularization: May require panretinal photocoagulation.
Investigations
Ocular evaluation
- Dilated fundus exam with macular biomicroscopy.
- OCT: Perform at every visit to screen for subretinal/intraretinal fluid.
- FAF and OCTA: Preferred for diagnosis and non-invasive surveillance.
Systemic workup
- Skin biopsy (Verhoeff stain) if PXE is suspected.
- Hemoglobin electrophoresis (especially in patients of African descent).
- Serum alkaline phosphatase and urinary calcium (rule out Paget disease).
- Cardiology and GI referral for patients confirmed with PXE.
Treatment
CNV management
- Intravitreal anti-VEGF (aflibercept, ranibizumab, or bevacizumab) is first-line therapy.
- Avoid PDT or focal laser due to poor outcomes and increased scarring.
Trauma prevention
- Mandatory use of polycarbonate safety eyewear.
- Strict avoidance of contact sports and high-impact activities.
Systemic management
- Referral to internist or specialist (genetics, cardiology, dermatology) for underlying conditions.
Clinical Pearl
Angioid streaks are a "biopsy of the body" found in the eye. Remember the PEPSI mnemonic: pseudoxanthoma elasticum, Ehlers-Danlos, Paget disease, sickle cell, and idiopathic.
Differential Diagnosis
- Lacquer cracks (pathologic myopia; radiate from staphyloma)
- Traumatic choroidal rupture (concentric to optic disc)
- Choroidal folds
- Peripapillary atrophy