Which nonproliferative retinal changes are characteristic of hemoglobinopathy retinopathy?

Updated: Sep 03, 2019
  • Author: Brian A Phillpotts, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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While abnormal, nonproliferative sickle retinal changes generally are asymptomatic and do not require treatment.

Venous tortuosity

Although common, it is not pathognomonic of sickle cell disease.

Venous tortuosity is due to decreased perfusion/circulation (ie, venous stasis, arteriolar-venous shunting).

Salmon patch hemorrhage

An intraretinal hematoma develops when sickled erythrocytes suddenly occlude the arterioles with subsequent blowout of the vessel wall.

Often found in the mid periphery, it varies in size and is round or ovoid in shape.

The hemorrhage often is confined to the neural retina, but it occasionally leaks through the internal limiting membrane or into the subretinal space.

The hemorrhage immediately appears bright red; over several days, it becomes an orange-red, salmon color for which it is named.

Black sunburst

This pigmented chorioretinal scar usually is found in the peripheral retina.

On ophthalmoscopy, these scars appear round or ovoid, measuring 1.5-2 disk diameters, have stellate or spiculate borders, and often are associated with iridescent spots.

Hemorrhage from retinal arteriolar occlusion can dissect between the neural retina and the retinal pigment epithelium (RPE), resulting in irritation and hypertrophy of the pigment epithelium and migration of pigmented cells into the area.

Angioid streaks

In 1959, Lieb and coworkers associated angioid streaks with sickle cell disease.

Appearing as pigmented striae that lie under the retinal vessels, angioid streaks are breaks in the Bruch membrane. They usually surround the disc, extending radially.

Angioid streaks are not specific to hemoglobinopathies but are seen in patients with other hemolytic anemia, Paget disease, Ehlers-Danlos syndrome, and pseudoxanthoma elasticum.

In hemoglobinopathies, incidence ranges from 1-6%.

The frequency of angioid streaks increases with age.

The pathogenesis of angioid streaks is generally controversial but that of sickle cell disease is even more confusing. The 4 most commonly proposed and accepted mechanisms of angioid streak development include the following:

  • Diffuse elastic degeneration

  • Iron deposition in the Bruch membrane secondary to hemolysis of retinal and subretinal hemorrhage

  • Impaired nutrition caused by sickling and stasis

  • Calcification of the Bruch membrane

Fibrovascular ingrowth can occur at the breaks.

Secondary changes may include the following:

  • Thickening of the retinal pigment epithelium basement membrane

  • Retinal pigment epithelium atrophy, hypertrophy, or hyperplasia

  • Choriocapillaris damage

  • Photoreceptor loss

  • Serous retinal detachment

  • Disciform scar formation

These lesions are usually asymptomatic; however, in the presence of choroidal neovascularization (CNVM) and macular degeneration, these lesions can lead to visual loss.

Treatment of the CNVM is laser photocoagulation. Recurrence rates are high.

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