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Case Presentation

A 48-year-old Hispanic male presented to the emergency department with blurred vision in his left eye for about one month. His visual acuity was 20/30 in the right eye and 20/70 in the left eye. Intraocular pressures and pupillary reactions were normal. The dilated fundus exam revealed a faint grayish lesion with adjacent hemorrhage in the left macula (Figure 1a). On closer examination, subtle angioid streaks could be visualized around the right optic nerve (Figure 1b). The remainder of the ophthalmic exam was largely unremarkable. OCT imaging was completed and revealed a subretinal hyperreflective lesion with associated RPE changes and subretinal fluid in the left eye (Figure 2). OCTA was utilized to further characterize the lesion, and a choroidal neovascular membrane (CNVM) was visualized in the choriocapillaris slab (Figure 3). 

His past medical history was noncontributory, but he did endorse smoking about a fourth of a pack of cigarettes daily for the last 20 years. He did not have any history of hypertension, diabetes, or other cardiovascular diseases, and he took no medications regularly. He also denied any family history of systemic conditions, including any known connective tissue disorders.

Given the active choroidal neovascularization, the patient received two serial anti-VEGF intravitreal injections in the left eye. At the last follow-up, the subretinal fluid had resolved and the patient’s visual acuity was 20/30. The patient was advised to monitor his vision with an Amsler grid each week and seek workup for any potential related systemic conditions with his primary care physician.

Angioid Streaks

Angioid streaks generally appear within the second to fifth decades of live and appear clinically as deep red or brownish “crack-like” bands in the posterior pole[1]. Most often, they are seen emanating from the optic nerve, and they may extend through the macula. Angioid streaks occur as a result of a break in Bruchs membrane due to the deposition of minerals within the typically elastic tissue. Mineralization of this membrane may also lead to a diffuse, mottled retinal appearance called “peau d’orange”.

Angioid streaks are caused by conditions such as pseudoxanthoma elasticum (PXE), Paget disease, sickle cell anemia, Ehlers-Danlos syndrome, and others in over 50% of cases[1]. They also may present in the absence of any systemic association, but it is thought they still likely have a genetic component[2]. Some associated diseases may be life-threatening, which highlights the importance of early recognition. For example, PXE, the most common systemic association of angioid streaks, causes calcification of elastic membranes throughout the body. It eventually affects the skin (leading to waxy-yellow deposits called “plucked-chicken skin”), gastrointestinal (GI) tract (resulting in GI hemorrhage), and cardiovascular system (causing atherosclerosis and heart disease)[3]. As optometrists, we can easily evaluate the flexural skin (neck, back of knees, armpits) for any visible changes that could suggest PXE. If suspected, they should be referred to their primary care physician who can coordinate with dermatology, cardiology, and gastroenterology as indicated.

Ophthalmic Management

Treatment for the ophthalmic manifestations of angioid streaks centers on the presence and activity of CNVMs, as they are typically the cause of vision loss. As is similar in CNVMs from other causes like neovascular macular degeneration and choroidal ruptures, intravitreal anti-VEGF agents are the mainstay of treatment. Treatment is typically administered monthly until subretinal fluid resolves. After the CNVM appears quiescent, regular monitoring is still very important as the rate of bilateral involvement is approximately 70% and lesions frequently become active again[4,5].

Angioid streaks are uncommon retinal findings that can be associated with multiple systemic diseases and have the potential to cause significant visual acuity loss. Eye care providers should always look closely for signs of this disorder, especially in middle-aged patients with choroidal neovascular membranes.

Figure 1a – left fundus with small macular heme and grayish lesion
Figure 1b – right nerve with angioid streak at superonasal border
Figure 2 – OCT of macula OS with SRF and subretinal lesion
Figure 3 – OCTA of CNVM (blue arrow)

 

  1. Chatziralli, I., et al., ANGIOID STREAKS: A Comprehensive Review From Pathophysiology to Treatment. Retina, 2019. 39(1): p. 1-11.
  2. Kumudhan, D., E.J. Wallace, and S.T. Roxburgh, Angioid streaks in identical twins. Br J Ophthalmol, 2004. 88(6): p. 837-8.
  3. Germain, D.P., Pseudoxanthoma elasticum. Orphanet J Rare Dis, 2017. 12(1): p. 85.
  4. Lekha, T., et al., Intravitreal Bevacizumab for Choroidal Neovascularization Associated with Angioid Streaks: Long-term Results. Middle East Afr J Ophthalmol, 2017. 24(3): p. 136-142.
  5. Sawa, M., et al., Long-term results of intravitreal bevacizumab injection for choroidal neovascularization secondary to angioid streaks. Am J Ophthalmol, 2009. 148(4): p. 584-590 e2.
Alison Bozung
Practicing at Bascom Palmer Eye Institute, primarily seeing patients in the ophthalmic emergency department.

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