eMedicine Specialties > Ophthalmology > Choroid

Angioid Streaks: Differential Diagnoses & Workup

Author: Mohammad Abusamak, MD, Chief Retina Division, Assistant Professor, Division of Ophthalmology, Jordan University Hospital
Contributor Information and Disclosures

Updated: Oct 16, 2008

Differential Diagnoses

ARMD, Exudative
Choroidal Rupture
Toxoplasmosis

Other Problems to Be Considered

Choroidal sclerosis
Myopic lacquer cracks-pseudoangioid streaks
Histoplasmosis
Retinal vasculitis and papillitis
Traumatic hemorrhage

Workup

Laboratory Studies

  • Only one half of patients with angioid streaks have a systemic association. General workup is important to diagnose and treat other aspects of the disease that may be life threatening, such as GI hemorrhage, heart disease, anemia, and pathological fractures.
  • Biochemical survey: Serum calcium, phosphorous, and alkaline phosphatase levels may be abnormal in untreated cases of Paget disease. Urinary excretion of pyridinoline crosslinks is a more specific and sensitive marker. In untreated patients, a close correlation between serum activity of alkaline phosphatase and urinary excretion of hydroxyproline exists. However, 10% of patients with Paget disease who are symptomatic have serum levels of alkaline phosphatase within the reference range.

Imaging Studies

  • Fluorescein angiography
    • Red-free photographs show radiating irregular curvilinear lines of variable width and configurations.
    • Early fluorescein angiography (FA) reveals either hyperfluorescence due to window transmission defects of atrophic RPE or uncommonly hypofluorescence due to atrophy or separation of underlying choriocapillaries, which results in nonfilling window defects.
    • In late FA, some leakage at the margins of the streaks occurs from adjacent healthy choriocapillaries and from late staining of the sclera and deep choroidal vessels. The classic appearance of CNVM, RPE detachments, and serous or hemorrhagic detachments also may be noted on FA.
  • Indocyanine green angiography
    • ICGA is superior to FA in defining occult choroidal neovascularizations. It shows angioid streaks in all eyes studies. Hyperfluorescent lines are visible in most cases. However, some patients exhibit hypofluorescence and tracklike fluorescence.
    • Peau d'orange appearance of the temporal macula can be seen on ICGA as a speckled pattern in the midperiphery. Hyperfluorescent lines look larger and more numerous than those seen on FA or red-free photographs.
  • Radiology studies: X-ray films of the head, abdomen, and lower extremities are helpful to show bone involvement in Paget disease of the bone and premature calcification of arteries in PXE.

Other Tests

  • Retinal function tests
    • Visual acuity is normal, as long as no damage to the foveal RPE and no leakage from the choroid through the Bruch membrane and RPE occur.
    • Visual fields are normal unless the central macula is affected by the angioid cracks and RPE detachments.
    • Color vision is affected only when vision loss occurs and is similar to color vision in acquired macular diseases.
    • Electroretinography (ERG) findings are normal.
    • Electro-oculography (EOG) findings are normal in most cases. Findings may be subnormal in advanced cases.
    • Dark adaptation is normal.
  • GI studies: GI hemorrhage is common in patients with PXE.

Procedures

  • Dermatologic: Skin biopsy may provide important diagnostic clues in cases of PXE with angioid streaks.

More on Angioid Streaks

Overview: Angioid Streaks
Differential Diagnoses & Workup: Angioid Streaks
Treatment & Medication: Angioid Streaks
Follow-up: Angioid Streaks
Multimedia: Angioid Streaks
References
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References

  1. Doyne RW. Choroidal and retinal changes. The result of blows on the eyes. Trans Ophthal. 1889;9:128.

  2. Knapp H. On the formation of dark angioid streaks as an unusual metamorphosis of retinal hemorrhage. Arch Ophthalmol. 1892;21:289-292.

  3. Klien BA. Angioid Streaks: A clinical and histopathologic study. American Journal of Ophthalmology. 1947;30:955-68.

  4. Clarkson JG, Altman RD. Angioid streaks. Surv Ophthalmol. Mar-Apr 1982;26(5):235-46. [Medline].

  5. Karacorlu M, Karacorlu S, Ozdemir H, et al. Photodynamic therapy with verteporfin for choroidal neovascularization in patients with angioid streaks. Am J Ophthalmol. Sep 2002;134(3):360-6. [Medline].

  6. Eckstein M, Wells JA, Aylward B, Gregor Z, et al. Surgical removal of non-age-related subfoveal choroidal neovascular membranes. Eye. 1998;12 (Pt 5):775-80. [Medline].

  7. Adelung JC. Zur Geneseder angioid streaks (Knapp). Klin Monatsbl Augenheilkd. 1951;119:241.

  8. Aveline B, Hasan T, Redmond RW. Photophysical and photosensitizing properties of benzoporphyrin derivative monoacid ring A (BPD-MA). Photochem Photobiol. Mar 1994;59(3):328-35. [Medline].

  9. Bock J. Zur Klinik und Anatomic der gefaessehnlichen Streifen in Augenhintergrund. Z Augenheilkd. 1938;95:1-50.

  10. Eretto P, Krohel GB, Shihab ZM, et al. Optic neuropathy in Paget's disease. Am J Ophthalmol. Apr 1984;97(4):505-10. [Medline].

  11. Francois J, De Laey JJ, Cambie E, et al. Neovascularization after argon laser photocoagulation of macular lesions. Am J Ophthalmol. Feb 1975;79(2):206-10. [Medline].

  12. Gass J, Donald M. Stereoscopic Atlas of Macular Diseases: Diagnosis and Treatment. Vol. 1. 4th ed. CV Mosby: 1997:120.

  13. Gelisken O, Hendrikse F, Deutman AF. A long-term follow-up study of laser coagulation of neovascular membranes in angioid streaks. Am J Ophthalmol. Mar 15 1988;105(3):299-303. [Medline].

  14. Guyer DR, Gragoudas ES, D'Amico DJ. Chapter 66: Angioid Streaks. In: Principles and Practice of Ophthalmology. Vol. 2. 1993:852-60.

  15. Hagedoorn A. Angioid streaks. Arch Ophthalmol. 1939;21:746-74.

  16. Heimann H, Gelisken F, Wachtlin J, et al. Photodynamic therapy with verteporfin for choroidal neovascularization associated with angioid streaks. Graefes Arch Clin Exp Ophthalmol. Nov 2005;243(11):1115-23. [Medline].

  17. Ladas ID, Georgalas I, Rouvas AA, et al. Photodynamic therapy with verteporfin of choroidal neovascularization in angioid streaks: conventional versus early retreatment. Eur J Ophthalmol. Jan-Feb 2005;15(1):69-73. [Medline].

  18. Lafaut BA, Leys AM, Scassellati-Sforzolini B, et al. Comparison of fluorescein and indocyanine green angiography in angioid streaks. Graefes Arch Clin Exp Ophthalmol. May 1998;236(5):346-53. [Medline].

  19. Macular Photocoagulation Study. Persistent and recurrent neovascularization after krypton laser photocoagulation for neovascular lesions of age-related macular degeneration. Macular Photocoagulation Study Group. Arch Ophthalmol. Jun 1990;108(6):825-31. [Medline].

  20. Macular Photocoagulation Study Group. Persistent and recurrent neovascularization after krypton laser photocoagulation for neovascular lesions of ocular histoplasmosis. Macular Photocoagulation Study Group. Arch Ophthalmol. Mar 1989;107(3):344-52. [Medline].

  21. Meislik J, Neldner K, Reeve EB, et al. Laser treatment in maculopathy of pseudoxanthoma elasticum. Can J Ophthalmol. Jul 1978;13(3):210-12. [Medline].

  22. Miller H, Miller B, Ryan SJ. Correlation of choroidal subretinal neovascularization with fluorescein angiography. Am J Ophthalmol. Mar 15 1985;99(3):263-71. [Medline].

  23. Pece A, Avanza P, Introini U, et al. Indocyanine green angiography in angioid streaks. Acta Ophthalmol Scand. Jun 1997;75(3):261-5. [Medline].

  24. Photodynamic therapy of subfoveal choroidal neovascularization in age-related macular degeneration with verteporfin: one-year results of 2 randomized clinical trials--TAP report. Treatment of age-related macular degeneration with photodynamic therapy (TAP) Study Group. Arch Ophthalmol. Oct 1999;117(10):1329-45. [Medline].

  25. Pierro L, Brancato R, Minicucci M, et al. Echographic diagnosis of Drusen of the optic nerve head in patients with angioid streaks. Ophthalmologica. 1994;208(5):239-42. [Medline].

  26. Piro PA, Scheraga D, Fine S. Angioid Streaks: Natural history and visual prognosis. In: Management of Retinal Vascular and Macular Disorders. Williams and Wilkins; 1983:136-9.

  27. Reinke MH, Canakis C, Husain D, et al. Verteporfin photodynamic therapy retreatment of normal retina and choroid in the cynomolgus monkey. Ophthalmology. Oct 1999;106(10):1915-23. [Medline].

  28. Schmidt-Erfurth U, Hasan T, Gragoudas E, et al. Vascular targeting in photodynamic occlusion of subretinal vessels. Ophthalmology. Dec 1994;101(12):1953-61. [Medline].

  29. Scott LJ, Goa KL. Verteporfin. Drugs Aging. Feb 2000;16(2):139-46; discussion 147-8. [Medline].

  30. Shields JA, Federman JL, Tomer TL, Annesley WH Jr. Angioid streaks. I. Ophthalmoscopic variations and diagnostic problems. Br J Ophthalmol. May 1975;59(5):257-66. [Medline].

  31. Sickenberg M, Schmidt-Erfurth U, Miller JW, et al. A preliminary study of photodynamic therapy using verteporfin for choroidal neovascularization in pathologic myopia, ocular histoplasmosis syndrome, angioid streaks, and idiopathic causes. Arch Ophthalmol. Mar 2000;118(3):327-36. [Medline].

  32. Singerman LJ, Hatem G. Laser treatment of choroidal neovascular membranes in angioid streaks. Retina. 1981;1(2):75-83. [Medline].

  33. Walker ER, Frederickson RG, Mayes MD. The mineralization of elastic fibers and alterations of extracellular matrix in pseudoxanthoma elasticum. Ultrastructure, immunocytochemistry, and X-ray analysis. Arch Dermatol. Jan 1989;125(1):70-6. [Medline].

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Further Reading

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Keywords

angioid streaks, retinal hemorrhage, Bruch membrane, Bruch's membrane, choroidal neovascularization, CNV, choroidal neovascular membrane, CNVM

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Contributor Information and Disclosures

Author

Mohammad Abusamak, MD, Chief Retina Division, Assistant Professor, Division of Ophthalmology, Jordan University Hospital
Mohammad Abusamak, MD is a member of the following medical societies: American Academy of Ophthalmology and American Society of Retina Specialists
Disclosure: Nothing to disclose.

Medical Editor

Russell P Jayne, MD, Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas
Russell P Jayne, MD is a member of the following medical societies: American Medical Association, American Society of Cataract and Refractive Surgery, and American Society of Retina Specialists
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine
Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society
Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

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