Angioid streaks result from breaks in which ocular structure?

Bruch's membrane Angioid streaks are caused by breaks in a weakened Bruch's membrane, which is why the lines lie deep to the retina.

How are angioid streaks typically configured on fundus examination?

Radiating outward from the optic disc They appear as bilateral, narrow, irregular lines deep to the retina arranged in a radiating fashion emanating from the optic disc.

Concentric rings around the fovea

Confined to the temporal periphery

Scattered punctate dots in one eye

Which systemic disease is the commonest association of angioid streaks?

Pseudoxanthoma elasticum Numerous systemic diseases are associated with angioid streaks, and pseudoxanthoma elasticum is the most common of them.

Which complication of angioid streaks drives progressive visual loss?

Choroidal neovascularization Subretinal hemorrhage from choroidal fibrovascular ingrowth can lead to choroidal neovascularization, which is often recurrent and causes progressive visual loss.

Rhegmatogenous retinal detachment

In pseudoxanthoma elasticum, what does a longer angioid streak predict?

A lower risk of macular atrophy

Spontaneous regression of the streak

What is the mainstay of treatment for choroidal neovascularization complicating angioid streaks?

Intravitreal anti-VEGF therapy Intravitreal anti-VEGF treatment is the mainstay of therapy and gives good results; combining photodynamic therapy with bevacizumab has also regressed the neovascular membrane.

How did laser photocoagulation and photodynamic therapy perform historically in angioid streaks?

Relatively poor results with frequent recurrences Both were used in the past for associated choroidal neovascularization but gave relatively poor results with frequent recurrences.

Curative in almost every treated eye

Superior to every other available option

Contraindicated because of scleral thinning

What improves visual outcomes in choroidal neovascularization from angioid streaks?

Deferring therapy until vision worsens

Treating both eyes simultaneously

Angioid Streaks: disease entity, complications and therapeutic management

DISEASE

Angioid streaks are bilateral, narrow, irregular lines deep to the retina configured in a radiating fashion emanating from the optic disc, which result from breaks in a weakened Bruch’s membrane.

Ophthalmologists should be aware that there are numerous systemic diseases associated with angioid streaks, the most common being pseudoxanthoma elasticum. The diagnosis of angioid streaks is one that can usually be made clinically.

Most patients are asymptomatic but secondary complications can result in choroidal neovascularization, lead to vision loss, and warrant treatment.

Secondary Complications

Angioid streaks can cause subretinal hemorrhages from choroidal fibrovascular ingrowth. These hemorrhages can resolve spontaneously or can lead to choroidal neovascularization, which can be recurrent and lead to progressive visual loss.

In fact, recent studies have revealed that in patients with pseudoxanthoma elasticum, the longer the angioid streak, the higher the risk of choroidal neovascularization and macular atrophy.

Test your knowledge 10 questions from this article

MANAGEMENT

A general management strategy includes determining if any underlying systemic association is present and if any secondary ocular complication exists that warrants treatment.

Medical Workup

A patient may be sent to the ophthalmologist with a known systemic disease association but at other times may be newly diagnosed with angioid streaks and have no known underlying systemic cause.

The medical workup should be tailored to the individual after a thorough review of the systems is undertaken.

The three most common systemic associations are pseudoxanthoma elasticum, Paget’s disease of bone, and sickle cell hemoglobinopathies, and therefore any screening workup should focus on these entities.

Patients should receive a complete physical examination by an internist, a skin biopsy, and blood testing to include serum alkaline phosphatase, serum calcium and phosphate, and hemoglobin electrophoresis.

Treatment

Angioid streaks are usually asymptomatic requiring observation only. When existent treatment methods can simply restrict the pathology;

however, it cannot be perpetually eliminated. When associated choroidal neovascularization is present, laser photocoagulation and photodynamic therapy have been used in the past with relatively poor results and frequent recurrences.

Intravitreal anti-VEGF treatment is the mainstay of treatment with good results. A combination of photodynamic therapy and bevacizumab has also been shown to regress choroidal neovascularization.

Furthermore, early intervention has been shown to be associated with significantly better outcomes.

REFERENCES

Chatziralli I; Saitakis G; Dimitriou E; Chatzirallis A; Stoungioti S; Theodossiadis G; Theodossiadis P; Retina (Philadelphia, Pa.), U.S. National Library of Medicine, Jan. 2019,
Clarkson JG, Altman RD. Angoid Streaks. Surv Ophthalmol. 1982;26(5)235-46.
Mahroo OA, Hykin PG Syndrome. “Confirmation That Angioid Streaks Are Not Common in Ehlers-Danlos Syndrome.” JAMA Ophthalmology. 2019 Dec 1;137(12):1463. doi: 10.1001/jamaophthalmol.2019.2549.PMID: 31556922
Regillo C, et al. Retina and Vitreous (Basic and Clinical Science Course). San Francisco, CA: American Academy of Ophthalmology; 2008:93-4.
Risseeuw, Sara. “The Extent of Angioid Streaks Correlates With Macular Degeneration in Pseudoxanthoma Elasticum.” American Journal of Ophthalmology, 20 July 2020, www.ajo.com/article/S0002-9394(20)30379-2/fulltext#relatedArticles.
Singman EL, Doyle JJ. JAMA Ophthalmology. 2019 Mar 1;137(3):239. doi: 10.1001/jamaophthalmol.2018.5995.PMID: 30543351
Yanoff M, Duker JS. Ophthalmology. 2nd edition. St. Louis, MO: Mosby; 2004:969-72.
Yanuzzi, LA, The Retinal Atlas. Elsevier; 2010:516-8.

Test yourself

A few questions straight from this article.

1 / 10 0 correct

Angioid streaks result from breaks in which ocular structure?
Angioid streaks are caused by breaks in a weakened Bruch's membrane, which is why the lines lie deep to the retina.
How are angioid streaks typically configured on fundus examination?
They appear as bilateral, narrow, irregular lines deep to the retina arranged in a radiating fashion emanating from the optic disc.
Which systemic disease is the commonest association of angioid streaks?
Numerous systemic diseases are associated with angioid streaks, and pseudoxanthoma elasticum is the most common of them.
Which blood test belongs in the workup of newly diagnosed angioid streaks?
Screening blood work should include serum alkaline phosphatase, serum calcium and phosphate, and hemoglobin electrophoresis to detect sickle cell hemoglobinopathies.
Which bone disorder is among the three commonest systemic associations of angioid streaks?
The three commonest systemic associations are pseudoxanthoma elasticum, Paget's disease of bone, and sickle cell hemoglobinopathies.
Which complication of angioid streaks drives progressive visual loss?
Subretinal hemorrhage from choroidal fibrovascular ingrowth can lead to choroidal neovascularization, which is often recurrent and causes progressive visual loss.
In pseudoxanthoma elasticum, what does a longer angioid streak predict?
Recent studies show that in pseudoxanthoma elasticum the longer the angioid streak, the higher the risk of choroidal neovascularization and macular atrophy.
What is the mainstay of treatment for choroidal neovascularization complicating angioid streaks?
Intravitreal anti-VEGF treatment is the mainstay of therapy and gives good results; combining photodynamic therapy with bevacizumab has also regressed the neovascular membrane.
How did laser photocoagulation and photodynamic therapy perform historically in angioid streaks?
Both were used in the past for associated choroidal neovascularization but gave relatively poor results with frequent recurrences.
What improves visual outcomes in choroidal neovascularization from angioid streaks?
Early intervention has been shown to be associated with significantly better outcomes, so treatment should not be deferred once neovascularization is found.