Angioid Streaks: Symptoms, Types, Causes and Treatment

Angioid streaks are an important but often overlooked finding in ophthalmology, with implications that extend far beyond the eye. These crack-like lines in the retina are not only a clue to underlying systemic disease but are also risk factors for sight-threatening complications. Understanding angioid streaks—their symptoms, types, causes, and treatment options—is essential for both clinicians and patients striving for optimal eye and systemic health.

Symptoms of Angioid Streaks

Angioid streaks often begin silently, but as they progress, they can have a profound impact on vision and quality of life. Recognizing the symptoms early can help prevent irreversible complications and prompt investigation for associated systemic disorders.

Symptom	Description	Associated Conditions	Source
Visual Changes	Blurred vision, distortion, or scotomas	Choroidal neovascularization (CNV)	6 7 9
Asymptomatic	No initial symptoms; detected on exam	Early disease	1 3 6
Metamorphopsia	Visual distortion, especially with CNV	Advanced disease	6 7 9
Peripheral Signs	Weak/absent pulses, paresthesias	Associated vascular disease	2 8

Table 1: Key Symptoms

Early and Subtle Manifestations

In many cases, angioid streaks do not cause symptoms in the early stages. They are often discovered incidentally during routine eye examinations, especially when the patient is being evaluated for unrelated issues. The streaks themselves do not initially affect vision because they most often occur outside the macula—the central part of the retina responsible for detailed sight 1 3.

Visual Disturbances and Complications

As the condition progresses, particularly when choroidal neovascularization (CNV) develops, patients may experience:

• Blurred or reduced central vision
• Metamorphopsia (visual distortion, such as straight lines appearing wavy)
• Scotomas (blind spots)

These symptoms are usually a consequence of CNV, which is the primary cause of severe vision loss in angioid streaks 6 7 9. CNV can lead to hemorrhage, exudation, and scarring, directly threatening the macula and central vision.

Systemic and Peripheral Signs

Because angioid streaks are often associated with systemic diseases affecting connective tissue and blood vessels, some patients may present with symptoms outside the eye. This can include weak or absent pulses, numbness, paresthesias, or intermittent claudication, especially if the underlying systemic disease involves vascular compromise 2 8.

Importance of Early Detection

Due to their often silent onset, routine eye exams—including fundus photography and angiography—are crucial for at-risk individuals. Early detection allows for timely monitoring and intervention, reducing the risk of severe visual impairment 6 7. Moreover, the presence of angioid streaks should prompt investigation for systemic diseases, as discussed below.

Types of Angioid Streaks

Angioid streaks are not all the same. Advances in retinal imaging have revealed distinct morphological types, each with specific characteristics and clinical implications.

Type	Key Features	Detection Method	Source
Crack	Thin, linear, “crack-like” streaks	Adaptive Optics	4
Band	Broader, linear streaks	Adaptive Optics	4
Hypopigmented	Pale, less pigmented areas	Adaptive Optics	4
Classic	Reddish/brown radiating lines	Fundoscopy	3 6 7

Table 2: Types of Angioid Streaks

Classic Angioid Streaks

Traditionally, angioid streaks are described as reddish or dark brown lines radiating outward from the optic disc. They often appear to encircle the disc partially or completely, creating a striking fundus appearance. These streaks are located beneath the retinal vessels and above the choroid, corresponding histologically to defects in Bruch’s membrane 3 7.

Imaging-Based Classification

Recent advances in retinal imaging, particularly adaptive optics (AO), have allowed for more nuanced classification:

• Crack Type: Thin, sharply demarcated lines resembling cracks.
• Band Type: Broader and more diffuse compared to crack-type streaks.
• Hypopigmented Type: Areas with less pigment, appearing paler than surrounding retina 4.

These subtypes may coexist in the same patient and can be differentiated based on their size and reflective features on AO imaging. The identification of these types is more than academic; it may have implications for monitoring disease progression and risk of complications.

Associated Lesions

Other retinal findings may be seen in conjunction with angioid streaks, especially in diseases like pseudoxanthoma elasticum. For example, “comet lesions”—small, hyper-reflective bodies—are detectable on AO imaging, and “peau d’orange” (orange peel) appearances may be noted on fundus autofluorescence 4 6. These findings can help differentiate angioid streaks related to specific systemic diseases.

Variability and Mimics

It is important to note that not all streak-like lesions in the fundus are true angioid streaks. Similar appearances can result from choroidal detachments, retinal folds, or pigmentary changes. Thus, careful examination and correlation with systemic findings are necessary for accurate diagnosis 3.

Causes of Angioid Streaks

Understanding the causes of angioid streaks is essential, as they are often a marker of underlying systemic disease—particularly those affecting connective tissue and blood vessels.

Cause/Association	Underlying Mechanism	Common Diseases	Source
Breaks in Bruch’s Membrane	Degeneration and calcification	PXE, Paget’s, Sickle cell	3 5 7 8
Genetic Mutations	ABCC6 gene mutations	Pseudoxanthoma elasticum	5
Connective Tissue Disorders	Altered collagen/elastin structure	Ehlers-Danlos, others	7 8
Vascular Disease	Elastic tissue degeneration	Generalized arterial disease	2 8

Table 3: Causes and Associations

Bruch’s Membrane Pathology

The fundamental lesion in angioid streaks is a break or dehiscence in Bruch’s membrane, a thin, elastic layer between the retina and choroid. Degeneration, calcification, and loss of elasticity in Bruch’s membrane make it prone to rupture, resulting in the characteristic streaks seen on examination 3 7.

Systemic Disease Associations

Angioid streaks are most commonly associated with the following systemic conditions:

• Pseudoxanthoma elasticum (PXE): A genetic disorder causing fragmentation and calcification of elastic fibers in the skin, eyes, and blood vessels. PXE is the most frequent systemic association, and angioid streaks are seen in a high proportion of affected patients 1 4 5 7 8.
• Paget’s Disease of Bone: A chronic bone disorder leading to abnormal bone remodeling. Angioid streaks may occur due to secondary changes in elastic tissues 1 3 8.
• Sickle Cell Disease and Other Hemoglobinopathies: Chronic vascular injury and tissue degeneration contribute to streak formation 1 3 8.
• Ehlers–Danlos Syndrome: A group of connective tissue disorders affecting collagen production 7.

Genetic and Molecular Factors

Mutations in the ABCC6 gene are strongly implicated in PXE, and by extension, the development of angioid streaks. Several specific variants have been identified in affected individuals, supporting the genetic basis of the disease 5.

Generalized Vascular and Elastic Tissue Degeneration

Some patients with angioid streaks demonstrate signs of generalized arterial disease, including reduced peripheral pulses and vascular calcifications. This supports the idea that angioid streaks are part of a broader disorder of connective tissue and elastic fiber degeneration 2 8.

Idiopathic Cases

In some instances, angioid streaks are found without any identifiable systemic disease. These cases require thorough investigation to rule out subtle or early manifestations of systemic conditions 1.

Treatment of Angioid Streaks

While there is no cure for angioid streaks themselves, modern therapies have significantly improved outcomes for the most serious complication—choroidal neovascularization (CNV). Management strategies also include regular monitoring and systemic evaluation.

Therapy Type	Main Indication	Key Outcomes/Limitations	Source
Anti-VEGF Agents	CNV secondary to angioid streaks	Best vision stabilization	7 10 11 12
Photodynamic Therapy	CNV, especially extrafoveal	Slows progression, less effective	7 9 10 13
Laser Photocoagulation	Extrafoveal CNV	Limited use, risk of scotoma	10
Systemic Management	Associated systemic disease	Reduces risk of further damage	1 8

Table 4: Treatment Approaches

Monitoring and Early Detection

All patients with angioid streaks require routine ophthalmic examinations. Advanced imaging techniques—such as autofluorescence, optical coherence tomography (OCT), fluorescein angiography, and indocyanine green angiography—are critical for detecting early CNV and monitoring progression 6 7.

Anti-VEGF Therapy

The mainstay treatment for CNV associated with angioid streaks is intravitreal injection of anti-vascular endothelial growth factor (anti-VEGF) agents such as ranibizumab and bevacizumab. These therapies:

• Stabilize or improve vision in the majority of cases
• Reduce macular edema and leakage
• Require repeated injections over time

Studies report that 85% of eyes treated with ranibizumab had stabilized or improved vision, and bevacizumab showed similar trends in smaller cohorts 11 12. Anti-VEGF therapy currently offers the best long-term functional outcomes 10.

Photodynamic Therapy (PDT)

Photodynamic therapy with verteporfin has been used for CNV, particularly for lesions not involving the fovea. While PDT can slow progression, its effects are often temporary, and many patients still experience vision loss over time 9 13. PDT may be considered when anti-VEGF agents are contraindicated or as adjunctive therapy 10.

Laser Photocoagulation

Laser photocoagulation is mainly reserved for extrafoveal CNV due to the risk of creating absolute scotomas and the high rate of recurrence. It is now less commonly used due to the superiority of anti-VEGF agents 10.

Systemic Management

Because angioid streaks frequently signify underlying systemic disease, a comprehensive medical evaluation is crucial. Addressing systemic conditions—such as controlling hypertension, managing bone disease, and preventing vascular complications—can reduce the risk of further ocular and systemic damage 1 8.

Patient Education and Lifestyle

Patients should be counseled on the importance of regular follow-up, prompt reporting of new visual symptoms, and maintaining systemic health. Avoidance of ocular trauma is recommended, as fragile Bruch’s membrane can rupture more easily in these individuals 7.

Conclusion

Angioid streaks are not just an ocular curiosity—they are key indicators of systemic disease with potentially blinding complications. Early recognition, thorough investigation, and prompt management are essential.

Key Points:

• Angioid streaks often begin asymptomatically but can lead to significant vision loss due to CNV 7 9.
• They may present in several morphological types, detectable with advanced imaging 4.
• Most cases are associated with systemic connective tissue disorders, especially pseudoxanthoma elasticum, Paget’s disease, and hemoglobinopathies 1 3 5 8.
• The mainstay of treatment for CNV is intravitreal anti-VEGF therapy, which stabilizes or improves vision in most patients 10 11 12.
• Regular monitoring and systemic evaluation are vital for optimal outcomes 6 7 8.
• Patient education and lifestyle modifications play a supporting role in long-term care 7.

By recognizing angioid streaks as both an ocular and systemic sign, clinicians and patients can work together to protect vision and overall health.