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Angioid Streaks Clinical Presentation

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

History

Visual acuity is normal unless a leakage, bleeding, or Bruch membrane dehiscence involves the central macula. Distorted central vision (metamorphopsia) and micropsia can be early signs of macular involvement.

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Physical

Ophthalmoscopic findings are as follows:

  • At times, angioid streaks can be difficult to identify unless a careful examination of the posterior pole is performed.
  • Angioid streaks usually present as linear gray or dark red lines with irregular serrated edges lying beneath normal retinal blood vessels. The streaks intercommunicate in a ringlike fashion around the optic disc in 27% of cases and radiate outward in a tapering fashion from the peripapillary area in 73% of patients. The streaks run a convoluted course and tend to terminate abruptly. Angioid streaks usually do not extend past the equator.
  • Associated findings in patients with angioid streaks are as follows:
    • Peau d'orange or leopard skin spotting, consisting of speckled yellowish mottling of the posterior pole mostly apparent in the temporal macula. This finding may antedate the appearance of angioid streaks. It is seen more frequently in patients with PXE and is caused by alterations at the level of the Bruch membrane as demonstrated by the diffuse pattern of hyperfluorescence on indocyanine green angiography (ICGA).
    • Peripapillary chorioretinal atrophy, focal peripheral chorioretinal scars (salmon spots), and reticular pigment dystrophy of the macula may be present at the time of diagnosis.
    • Optic nerve head drusen (hyaline bodies) may antedate the appearance of angioid streaks, and they may be the earliest clinical manifestations of PXE. As many as 25% of patients with angioid streaks have clinical or echographic evidence of disk drusen. These hyaline bodies were associated with neovascularization in the peripapillary area, and, in some patients, they were associated with acute visual loss due to the pressure effect on the optic nerve head.
    • Crystalline bodies typically are seen in the midperipheral fundus or inferior to the optic nerve. They are multiple, round, small, and subretinal lesions. Usually, these crystalline bodies cause some atrophy of the retinal pigment epithelium (RPE). In older patients, peripheral retinal scars and calcifications can be seen.[6]
    • Optic atrophy may be seen in patients with Paget disease of the bone. It cannot be explained solely on the basis of bony compression.
  • Severe visual impairment is caused by one of the following conditions in 70% of cases:
    • CNV with subsequent serous and hemorrhagic detachment of the fovea (most serious complication)
    • CNV development occurs in 72-86% of all patients over time.[7] Once a patient develops CNV in one eye, it becomes bilateral in 50% within 18 months of the initial diagnosis.[8]
    • Choroidal rupture secondary to trivial trauma with secondary hemorrhage involving the fovea
    • Foveal involvement by a streak with damage to RPE and choriocapillaris (may result in permanent loss of central visual acuity)
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Causes

See the list below:

  • Idiopathic: In 50% of patients with angioid streaks, no associated systemic disease is present.
  • Systemic association
    • PXE is an uncommon inherited disorder of connective tissue. It has generalized effects on the elastin fibrils in the dermis, arterial walls, heart, gastrointestinal (GI) tract, and Bruch membrane, resulting in mineralization and deposition of phosphorus. The 4 types of inheritance of PXE include 2 of which are autosomal dominant and 2 of which are autosomal recessive. It is the most common systemic disorder associated with angioid streaks. Diagnosing PXE is important because 85% of patients develop ocular involvement, usually after the second decade of life. The combination of PXE and ocular involvement is referred to as Grönblad-Strandberg syndrome.
    • Histologically, degenerative changes and calcifications of the elastic tissues in the skin and arteries are present. The following systemic findings may help the ophthalmologist to confirm the diagnosis of angioid streaks secondary to PXE, as well as to take care of complications.
    • Dermatologic findings include yellow papules, "chicken skin" arranged in a linear or reticulate pattern, in plaques, over the side of the neck, antecubital fossae, axillae, groin, and paraumbilical area.
    • Cardiovascular manifestations include accelerated hypertension at an earlier age due to atherosclerosis, which may be related to renovascular disease, premature coronary artery disease, peripheral vascular disease, and mitral incompetence.
    • Some patients develop genitourinary bleeding as part of PXE.
    • Neurologic findings may include cerebrovascular accidents, intracranial aneurysms, and cerebral ischemia.
  • Paget disease is a chronic, progressive, and in some cases inherited disease, characterized by bone deformity. It may be confined to a few bones, or in some patients, it represents a generalized abnormality that gives rise to enlargement of the skull, kyphoscoliosis, deafness, and deformities of long bones. However, angioid streaks occur in fewer than 2% of patients. Osteoclastic activity with an osteoblastic reaction occurs. Although the etiology is unknown, some clinicians believe it is related to a slow virus infection, measles, or respiratory syncytial virus. Both males and females are affected equally.
  • Ehlers-Danlos syndrome is a rare autosomal dominant disorder of collagen resulting from a deficiency of hydroxylysine. Ocular findings include epicanthal folds, keratoconus, high myopia, retinal detachment, blue sclera, ectopia lentis, and angioid streaks. Systemic associations include the following:
    • Skin and musculoskeletal - Thin hyperplastic skin that heals poorly, hyperextensible joints that may predispose to recurrent falls, hydrarthrosis, and pseudotumor formation over elbows and knees
    • Cardiovascular disease consists of bleeding diathesis, dissecting aneurysms, spontaneous rupture of large blood vessels, and mitral prolapse.
    • Others - Diaphragmatic hernias and diverticulum of the GI and respiratory tracts
  • Hemoglobinopathies that are occasionally associated with angioid streaks include the following:
    • Homozygous sickle cell disease (Hb-SS)
    • Sickle cell trait (Hb-AS)
    • Sickle cell thalassemia (Hb-thal)
    • Sickle cell hemoglobin (Hb-SC)
    • Hemoglobin H (Hb-H)
    • Homozygous B-thalassemia major
    • Intermedia
    • Minor and hereditary spherocytosis
  • The frequency of angioid streaks increases with age; it is about 1.5% in younger patients and increases to 22% in older patients. Complications, such as macular degeneration and choroidal neovascular membranes, are uncommon in this subgroup of patients with angioid streaks. In general, choroidal neovascular membranes (CNVM) and serous detachments of the macula are less common in black patients.
  • Other systemic diseases associated with angioid streaks include the following:
    • Abetalipoproteinemia
    • Acromegaly
    • Dwarfism
    • Diabetes mellitus
    • Hemochromatosis
    • Facial angiomatosis
    • Idiopathic thrombocytic purpura
    • Chronic familial hyperphosphatemia
    • Hypercalcinosis
    • Diffuse lipomatosis
    • Acquired hemolytic anemia
    • Myopia
    • Neurofibromatosis
    • Epilepsy
    • Senile elastosis
    • Sturge-Weber syndrome
    • Trauma
    • Tuberous sclerosis
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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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