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Angioid Streaks Workup

  • Author: Mohammad Abusamak, MD; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Feb 20, 2014
 

Laboratory Studies

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  • 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.
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Imaging Studies

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  • Fluorescein angiography
    • Red-free photographs (see image below) show radiating irregular curvilinear lines of variable width and configurations.
      Red-free photograph of the optic nerve and posteriRed-free photograph of the optic nerve and posterior pole showing the cracks in the Bruch membrane. Notice the retinal arteries and veins crossing over the dark red streaks.
    • Early fluorescein angiography (FA) (see image below) 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.
      Early fluorescein angiography showing the early hyEarly fluorescein angiography showing the early hyperfluorescence, window defect, of the angioid streaks.
    • In late FA (see image below), 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.
      Late fluorescein angiography of the same eye as inLate fluorescein angiography of the same eye as in Media file 2. Notice the staining of the edges of the streaks. Also, staining in the center of the macula is present due to extension of the Bruch membrane crack. When compared to early fluorescein angiography, no active leakage is present.
  • 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: Radiographs 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.
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Other Tests

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  • 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.
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Procedures

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  • Dermatologic: Skin biopsy may provide important diagnostic clues in cases of PXE with angioid streaks.
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Contributor Information and Disclosures
Author

Mohammad Abusamak, MD Chief of Retina Division, Jordan University Hospital; Assistant Professor, Division of Ophthalmology, University of Jordan Faculty of Medicine; Ophthalmologist, Amman Eye Clinic, Jordan

Mohammad Abusamak, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, Macula Society, Retina Society, Club Jules Gonin

Disclosure: Received royalty and consulting fees for: Alcon Laboratories.

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, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

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, American Society of Retina Specialists

Disclosure: Nothing to disclose.

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. Abujamra S, Negretto AD, Saraceno JJ, Oliveira TL, Gomes AM. [Angioid streaks: fundoscopic analysis of 317 cases]. Arq Bras Oftalmol. 2008 Nov-Dec. 71(6):819-21. [Medline].

  5. Mansour AM, Ansari NH, Shields JA, Annesley WH Jr, Cronin CM, Stock EL. Evolution of angioid streaks. Ophthalmologica. 1993. 207(2):57-61. [Medline].

  6. Dhermy P. Histologie Angioid Streaks. Cosca G, Soubane G, eds. Neovasseaux Sous-Retiniens et Laser. Paris, France: 1987. 210-1.

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

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

  9. Kubota M, Hayashi T, Arai K, Tsuneoka H. Choroidal neovascularization after blunt ocular trauma in angioid streaks. Clin Ophthalmol. 2013. 7:1347-51. [Medline]. [Full Text].

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

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

  12. Sawa M, Gomi F, Tsujikawa M, Sakaguchi H, Tano Y. Long-term results of intravitreal bevacizumab injection for choroidal neovascularization secondary to angioid streaks. Am J Ophthalmol. 2009 Oct. 148(4):584-590.e2. [Medline].

  13. Neri P, Salvolini S, Mariotti C, Mercanti L, Celani S, Giovannini A. Long-term control of choroidal neovascularisation secondary to angioid streaks treated with intravitreal bevacizumab (Avastin). Br J Ophthalmol. 2009 Feb. 93(2):155-8. [Medline].

  14. Finger RP, Charbel Issa P, Hendig D, Scholl HP, Holz FG. Monthly Ranibizumab for Choroidal Neovascularizations Secondary to Angioid Streaks in Pseudoxanthoma Elasticum: A One-Year Prospective Study. Am J Ophthalmol. 2011 Jun 24. [Medline].

  15. Gliem M, Finger RP, Fimmers R, Brinkmann CK, Holz FG, Charbel Issa P. Treatment of choroidal neovascularization due to angioid streaks: a comprehensive review. Retina. 2013 Jul-Aug. 33(7):1300-14. [Medline].

  16. Prabhu VV, Morris RJ, Shah PK, Narendran V. Combination treatment of low fluence photodynamic therapy and intravitreal ranibizumab for choroidal neovascular membrane secondary to angioid streaks in Paget's disease - 12 month results. Indian J Ophthalmol. 2011 Jul-Aug. 59(4):306-8. [Medline]. [Full Text].

  17. Artunay O, Yuzbasioglu E, Rasier R, Sengul A, Senel A, Bahcecioglu H. Combination treatment with intravitreal injection of ranibizumab and reduced-fluence photodynamic therapy for choroidal neovascularization secondary to angioid streaks: Preliminary Clinical Results of 12-Month Follow-Up. Retina. 2011 Jul-Aug. 31(7):1279-86. [Medline].

  18. 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].

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

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

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

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

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

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

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

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

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

  28. 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. 2005 Jan-Feb. 15(1):69-73. [Medline].

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

  30. 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. 1990 Jun. 108(6):825-31. [Medline].

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

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

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

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

  35. 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. 1999 Oct. 117(10):1329-45. [Medline].

  36. 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].

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

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

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

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

  41. 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. 2000 Mar. 118(3):327-36. [Medline].

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

  43. 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. 1989 Jan. 125(1):70-6. [Medline].

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Progression of angioid streaks. Large subretinal hemorrhage.
Same eye as in previous image, 11 months later. Partial resolution of subretinal blood. Notice the old subretinal hemorrhage under the fovea and color change to white-yellow.
Late complication of choroidal neovascularization in angioid streaks. Same eye as in previous images, 5 years later. Notice the extensive scarring and subretinal exudates and dehemoglobinized blood.
Red-free photograph of the optic nerve and posterior pole showing the cracks in the Bruch membrane. Notice the retinal arteries and veins crossing over the dark red streaks.
Early fluorescein angiography showing the early hyperfluorescence, window defect, of the angioid streaks.
Late fluorescein angiography of the same eye as in Media file 2. Notice the staining of the edges of the streaks. Also, staining in the center of the macula is present due to extension of the Bruch membrane crack. When compared to early fluorescein angiography, no active leakage is present.
Right eye, midphase arteriovenous, showing choriocapillaris atrophic changes. This 45-year-old patient underwent 3 injections of Avastin and one session of half-time photodynamic therapy.
Same patient as in previous image, a few months before the Avastin injection and half-time photodynamic therapy.
A 50-year-old man with a 2-month history of blurring vision in the left eye. The color photograph showed subretinal blood and large membrane, extrafoveal in location.
Early fundus fluorescein angiography showing the hyperfluorescence of the choroidal neovascular membrane of the left eye of the same patient in the previous image.
Late fundus fluorescein angiography confirming the active choroidal neovascular membrane of the left eye.
 
 
 
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