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Symptoms ⬇

Usually asymptomatic. Decreased vision may result from CNV.

Signs ⬆ ⬇

(See Figure 11.23.1.)

Critical

Bilateral reddish-brown or gray bands located deep to the retina, due to breaks within Bruch membrane, usually radiating in an irregular or spoke-like pattern from the optic disc. CNV may occur.

Other

Mottled fundus appearance with an orange hue (peau d’orange), most common in the temporal midperiphery. Subretinal hemorrhages after mild blunt trauma. Reticular pigmentary changes in the macula; small, white, pinpoint chorioretinal scars (histo-like spots) in the midperiphery; crystalline bodies within the macula. Drusen of the optic disc (especially with pseudoxanthoma elasticum [PXE]). Granular pattern of hyperfluorescent lines on IVFA. Widespread RPE damage is more evident on FAF compared to fundus ophthalmoscopy or IVFA.

11-23.1 Angioid streaks.

Gervasio-ch011-image041

Differential Diagnosis ⬆ ⬇

Etiology ⬆ ⬇

Fifty percent of cases are associated with systemic diseases; the rest are idiopathic.

  • PXE: Most common. Loose skin folds in the neck, axillae, and on flexor aspects of joints; cardiovascular complications; increased risk of gastrointestinal bleeds.
  • Ehlers–Danlos syndrome: Hyperelasticity of skin, loose joints.
  • Paget disease of bone: Enlarged skull, bone pain, history of bone fractures, hearing loss, possible cardiovascular complications. May be asymptomatic but may develop visual loss due to optic nerve compression by enlarging bone. Increased serum alkaline phosphatase and urine calcium. Ten percent develop late angioid streaks.
  • Sickle cell disease: May be asymptomatic or have decreased vision from fundus abnormalities. May have a history of recurrent infections and painless or painful crises. See 11.20, SICKLE CELL RETINOPATHY (INCLUDING SICKLE CELL DISEASE, ANEMIA, SICKLE AND TRAIT).
  • Less common: Thalassemia, acromegaly, senile elastosis, lead poisoning, Marfan syndrome, hemolytic anemia, and others.

Work Up ⬆ ⬇

Workup
  1. History: Any known systemic disorders? Previous ocular trauma?
  2. Complete ocular examination: Look carefully at the macula with a slit lamp using a 60-, 90-diopter, or fundus contact lens to detect CNV.
  3. FAF if diagnosis uncertain or IVFA or OCTA if CNV suspected.
  4. Physical examination: Look for clinical signs of etiologic diseases.
  5. Serum alkaline phosphatase and urine calcium levels if Paget disease of bone is suspected.
  6. Sickle cell preparation and hemoglobin electrophoresis in patients of African descent.
  7. Skin or scar biopsy if PXE is suspected.
  8. CBC if hematologic etiology suspected.

Treatment ⬆ ⬇

  1. Anti-VEGF therapy is now used for angioid streak-associated CNV, as focal laser photocoagulation and PDT have discouraging outcomes. See 11.17, NEOVASCULAR OR EXUDATIVE (WET) AGE-RELATED MACULAR DEGENERATION.
  2. Management of any underlying systemic disease by an internist.
  3. Recommend wearing one-piece polycarbonate safety glasses for sports due to an increased risk of subretinal hemorrhage and choroidal rupture from minor trauma.

Follow Up ⬆

  1. Fundus examination every 6 months, monitoring for CNV.
  2. Instruct patient to check Amsler grid daily and return immediately if changes are noted. See Appendix 4, AMSLER GRID.