eMedicine Specialties > Ophthalmology > Choroid

Neovascularization, Choroidal

Author: Lihteh Wu, MD, Consulting Surgeon, Department of Ophthalmology, Vitreo-Retinal Section, Instituto De Cirugia Ocular, Costa Rica
Coauthor(s): Teodoro Evans, MD, Retina Fellow, Vitreo-Retinal Section, Instituto De Cirugia Ocular, Costa Rica
Contributor Information and Disclosures

Updated: Jul 25, 2007

Introduction

Background

This disorder describes the growth of new blood vessels that originate from the choroid through a break in the Bruch membrane into the sub–retinal pigment epithelium (sub-RPE) or subretinal space. Choroidal neovascularization (CNV) is a major cause of visual loss.

Pathophysiology

Mechanisms of CNV are not understood. Virtually any pathologic process that involves the RPE and damages the Bruch membrane can be complicated by CNV. Recently, a protein derived from the RPE, pigment epithelium derived factor (PEDF), was found to have an inhibitory effect on ocular neovascularization. Another peptide, vascular endothelium growth factor (VEGF), is a well-known ocular angiogenic factor.

The balance between antiangiogenic factors (eg, PEDF) and angiogenic factors (eg, VEGF) is speculated to determine the growth of CNV. VEGF has been temporally and spatially correlated with the development of CNV. Histopathologic specimens obtained from submacular surgery reveal the presence of VEGF in CNV. In addition, several researchers have induced CNV formation in animal models by overexpressing VEGF. Once secreted, VEGF binds to its receptors in endothelial cells activating several signal transduction pathways that end with the formation of a network of new vessels. As new choroidal blood vessels grow, they may extend into the sub-RPE space (Gass type 1) or into the subretinal space (Gass type 2). The location, growth pattern, and type (1 or 2) of CNV depend on the patient's age and the underlying disease. Bleeding and exudation occur with further growth, accounting for the visual symptoms.

Frequency

United States

In the Wisconsin Beaver Dam Study, prevalence of CNV associated with age-related macular degeneration (ARMD) was 1.2% in adults aged 43-86 years. Myopia is the second most common cause of CNV in the United States and Europe. CNV is estimated to occur in 5-10% of myopes; 60-75% of these are subfoveal.

Disciform scars secondary to CNV from presumed ocular histoplasmosis syndrome (POHS) were present in 0.1% of people living in endemic areas. In multiple evanescent white dot syndrome (MEWDS), development of CNV is rare. In multifocal choroiditis, estimates of CNV range from 25-40% of patients. In punctate inner choroidopathy (PIC), 33% of patients develop CNV. Of these, 50% are subfoveal and result in visual acuities between 20/80 and 20/200.

CNV occurs in 5% of patients with birdshot chorioretinopathy. CNV occurs in virtually all choroidal ruptures during the healing phase; most involute spontaneously. In 15-30% of patients, CNV may recur and lead to a hemorrhagic or serous macular detachment with concomitant visual loss.

Mortality/Morbidity

  • ARMD is the most common cause of visual loss in people older than 50 years in the developed world. Up to 90% of visual loss in ARMD is secondary to CNV.
  • Myopia is the seventh greatest cause of registered blindness in the United States and Europe. CNV is responsible for most of this visual loss.
  • POHS is an uncommon cause of visual loss. Incidence and prevalence in the blind of Tennessee, an area endemic for histoplasmosis, were reported to be 2.8% and 0.5%, respectively.

Sex

No gender predilection exists.

Certain diseases (ie, choroidal ruptures, angioid streaks, myopic macular degeneration, multifocal choroiditis, PIC, MEWDS) that may be complicated by CNV have gender proclivity.

Age

CNV is associated with multiple ocular conditions, so the age distribution of CNV reflects the underlying condition.  

  • For instance, younger patients are affected with POHS, multifocal choroiditis, MEWDS, and PIC.
  • Older patients will be affected by CNV secondary to ARMD.

Clinical

History

  • Painless loss of vision
  • Metamorphopsia
  • Paracentral or central scotoma
  • Apparent change in image size

Physical

  • Subretinal blood
  • Subretinal fluid
  • Lipid exudation
  • Retinal pigment epithelial detachment
  • Subretinal fibrosis (disciform scar)

Causes

Virtually any pathologic process that involves the RPE and damages the Bruch membrane can be complicated by CNV.

  • Degenerative conditions

    • ARMD
    • Myopia
    • Angioid streaks
  • Inflammatory or infectious conditions

    • Histoplasmosis
    • Sarcoidosis
    • Multifocal choroiditis
    • PIC
  • Choroidal tumors

    • Nevi
    • Melanoma
    • Hemangioma
    • Osteoma
  • Trauma

    • Choroidal rupture
    • Laser photocoagulation
  • Idiopathic

More on Neovascularization, Choroidal

Overview: Neovascularization, Choroidal
Differential Diagnoses & Workup: Neovascularization, Choroidal
Treatment & Medication: Neovascularization, Choroidal
Follow-up: Neovascularization, Choroidal
References
[ CLOSE WINDOW ]

References

  1. Blinder KJ, Blumenkranz MS, Bressler NM, Bressler SB, Donato G, Lewis H, et al. Verteporfin therapy of subfoveal choroidal neovascularization in pathologic myopia: 2-year results of a randomized clinical trial--VIP report no. 3. Ophthalmology. Apr 2003;110(4):667-73. [Medline].

  2. Blinder KJ, Bradley S, Bressler NM, Bressler SB, Donati G, Hao Y. Effect of lesion size, visual acuity, and lesion composition on visual acuity change with and without verteporfin therapy for choroidal neovascularization secondary to age-related macular degeneration: TAP and VIP report no. 1. Am J Ophthalmol. Sep 2003;136(3):407-18. [Medline].

  3. Bressler NM, Bressler SB, Childs AL, Haller JA, Hawkins BS, Lewis H, et al. Surgery for hemorrhagic choroidal neovascular lesions of age-related macular degeneration: ophthalmic findings: SST report no. 13. Ophthalmology. Nov 2004;111(11):1993-2006. [Medline].

  4. Bressler NM, Bressler SB, Gragoudas ES. Clinical characteristics of choroidal neovascular membranes. Arch Ophthalmol. Feb 1987;105(2):209-13. [Medline].

  5. Bressler SB, Bressler NM, Fine SL, Hillis A, Murphy RP, Olk RJ, et al. Natural course of choroidal neovascular membranes within the foveal avascular zone in senile macular degeneration. Am J Ophthalmol. Feb 1982;93(2):157-63. [Medline].

  6. Chakravarthy U, Houston RF, Archer DB. Treatment of age-related subfoveal neovascular membranes by teletherapy: a pilot study. Br J Ophthalmol. May 1993;77(5):265-73. [Medline].

  7. Dawson DW, Volpert OV, Gillis P, Crawford SE, Xu H, Benedict W, et al. Pigment epithelium-derived factor: a potent inhibitor of angiogenesis. Science. Jul 9 1999;285(5425):245-8. [Medline].

  8. de Juan E Jr, Loewenstein A, Bressler NM, Alexander J. Translocation of the retina for management of subfoveal choroidal neovascularization II: a preliminary report in humans. Am J Ophthalmol. May 1998;125(5):635-46. [Medline].

  9. Emerson MV, Lauer AK, Flaxel CJ, Wilson DJ, Francis PJ, Stout JT, et al. Intravitreal bevacizumab (Avastin) treatment of neovascular age-related macular degeneration. Retina. Apr-May 2007;27(4):439-44. [Medline].

  10. Eyetech Study Group. Anti-vascular endothelial growth factor therapy for subfoveal choroidal neovascularization secondary to age-related macular degeneration: phase II study results. Ophthalmology. May 2003;110(5):979-86. [Medline].

  11. Ferris FL 3rd, Fine SL, Hyman L. Age-related macular degeneration and blindness due to neovascular maculopathy. Arch Ophthalmol. Nov 1984;102(11):1640-2. [Medline].

  12. Folk JC, Reddy CV. White dot chorioretinal inflammatory syndromes. In: Medical and Surgical Retina Advances, Controversies, and Management. 1994;385-399.

  13. Fuller B, Gitter KA. Traumatic choroidal rupture with late serous detachment of macula. Report of successful argon laser treatment. Arch Ophthalmol. Apr 1973;89(4):354-5. [Medline].

  14. Goff MJ, Johnson RN, McDonald HR, Ai E, Jumper JM, Fu A. Intravitreal bevacizumab for previously treated choroidal neovascularization from age-related macular degeneration. Retina. Apr-May 2007;27(4):432-8. [Medline].

  15. Gragoudas ES, Adamis AP, Cunningham ET, Feinsod M, Guyer DR. Pegaptanib for neovascular age-related macular degeneration. N Engl J Med. Dec 30 2004;351(27):2805-16. [Medline].

  16. Gross JG, King LP, de Juan E Jr, Powers T. Subfoveal neovascular membrane removal in patients with traumatic choroidal rupture. Ophthalmology. Apr 1996;103(4):579-85. [Medline].

  17. Grossniklaus HE, Green WR. Histopathologic and ultrastructural findings of surgically excised choroidal neovascularization. Submacular Surgery Trials Research Group. Arch Ophthalmol. Jun 1998;116(6):745-9. [Medline].

  18. Hawkins BS, Bressler NM, Bressler SB, Davidorf FH, Hoskins JC, Marsh MJ, et al. Surgical removal vs observation for subfoveal choroidal neovascularization, either associated with the ocular histoplasmosis syndrome or idiopathic: I. Ophthalmic findings from a randomized clinical trial: Submacular Surgery Trials (SST) Group H Trial: SST Report No. 9. Arch Ophthalmol. Nov 2004;122(11):1597-611. [Medline].

  19. Hawkins BS, Bressler NM, Miskala PH, Bressler SB, Holekamp NM, Marsh MJ, et al. Surgery for subfoveal choroidal neovascularization in age-related macular degeneration: ophthalmic findings: SST report no. 11. Ophthalmology. Nov 2004;111(11):1967-80. [Medline].

  20. Ho AC, Yannuzzi LA, Pisicano K, DeRosa J. The natural history of idiopathic subfoveal choroidal neovascularization. Ophthalmology. May 1995;102(5):782-9. [Medline].

  21. Klein R, Klein BE, Linton KL. Prevalence of age-related maculopathy. The Beaver Dam Eye Study. Ophthalmology. Jun 1992;99(6):933-43. [Medline].

  22. Lambert HM, Capone A Jr, Aaberg TM, Sternberg P Jr, Mandell BA, Lopez PF. Surgical excision of subfoveal neovascular membranes in age-related macular degeneration. Am J Ophthalmol. Mar 15 1992;113(3):257-62. [Medline].

  23. Laud K, Spaide RF, Freund KB, Slakter J, Klancnik JM Jr. Treatment of choroidal neovascularization in pathologic myopia with intravitreal bevacizumab. Retina. Oct 2006;26(8):960-3. [Medline].

  24. Lazic R, Gabric N. Verteporfin therapy and intravitreal bevacizumab combined and alone in choroidal neovascularization due to age-related macular degeneration. Ophthalmology. Jun 2007;114(6):1179-85. [Medline].

  25. Machemer R, Steinhorst UH. Retinal separation, retinotomy, and macular relocation: II. A surgical approach for age-related macular degeneration?. Graefes Arch Clin Exp Ophthalmol. Nov 1993;231(11):635-41. [Medline].

  26. Macular Photocoagulation Study Group. Argon laser photocoagulation for neovascular maculopathy. Five-year results from randomized clinical trials. Arch Ophthalmol. Aug 1991;109(8):1109-14. [Medline].

  27. Macular Photocoagulation Study Group. Laser photocoagulation for juxtafoveal choroidal neovascularization. Five-year results from randomized clinical trials. Arch Ophthalmol. Apr 1994;112(4):500-9. [Medline].

  28. Pharmacological Therapy for Macular Degeneration Study Group. Interferon alfa-2a is ineffective for patients with choroidal neovascularization secondary to age-related macular degeneration. Results of a prospective randomized placebo-controlled clinical trial. Pharmacological Therapy for Macular Degeneration Study Group. Arch Ophthalmol. Jul 1997;115(7):865-72. [Medline].

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

  30. Rogers AH, Martidis A, Greenberg PB, Puliafito CA. Optical coherence tomography findings following photodynamic therapy of choroidal neovascularization. Am J Ophthalmol. Oct 2002;134(4):566-76. [Medline].

  31. Rosenfeld PJ. Intravitreal avastin: the low cost alternative to lucentis?. Am J Ophthalmol. Jul 2006;142(1):141-3. [Medline].

  32. [Best Evidence] Rosenfeld PJ, Brown DM, Heier JS, Boyer DS, Kaiser PK, Chung CY, et al. Ranibizumab for neovascular age-related macular degeneration. N Engl J Med. Oct 5 2006;355(14):1419-31. [Medline].

  33. Rosenfeld PJ, Moshfeghi AA, Puliafito CA. Optical coherence tomography findings after an intravitreal injection of bevacizumab (avastin) for neovascular age-related macular degeneration. Ophthalmic Surg Lasers Imaging. Jul-Aug 2005;36(4):331-5. [Medline].

  34. Rosenfeld PJ, Rich RM, Lalwani GA. Ranibizumab: Phase III clinical trial results. Ophthalmol Clin North Am. Sep 2006;19(3):361-72. [Medline].

  35. Roth DB, Downie AA, Charles ST. Visual results after submacular surgery for neovascularization in age-related macular degeneration. Ophthalmic Surg Lasers. Nov 1997;28(11):920-5. [Medline].

  36. Schmidt-Erfurth U, Miller J, Sickenberg M, Bunse A, Laqua H, Gragoudas E, et al. Photodynamic therapy of subfoveal choroidal neovascularization: clinical and angiographic examples. Graefes Arch Clin Exp Ophthalmol. May 1998;236(5):365-74. [Medline].

  37. Sears J, Capone A Jr, Aaberg T Sr, Lewis H, Grossniklaus H, Sternberg P Jr, et al. Surgical management of subfoveal neovascularization in children. Ophthalmology. May 1999;106(5):920-4. [Medline].

  38. Shiraga F, Ojima Y, Matsuo T, Takasu I, Matsuo N. Feeder vessel photocoagulation of subfoveal choroidal neovascularization secondary to age-related macular degeneration. Ophthalmology. Apr 1998;105(4):662-9. [Medline].

  39. Sivagnanavel V, Evans JR, Ockrim Z, Chong V. Radiotherapy for neovascular age-related macular degeneration. Cochrane Database Syst Rev. 2004;(4):CD004004. [Medline].

  40. Spaide RF, Guyer DR, McCormick B, Yannuzzi LA, Burke K, Mendelsohn M, et al. External beam radiation therapy for choroidal neovascularization. Ophthalmology. Jan 1998;105(1):24-30. [Medline].

  41. Spaide RF, Laud K, Fine HF, Klancnik JM, Meyerle CB, Yannuzzi LA, et al. Intravitreal bevacizumab treatment of choroidal neovascularization secondary to age-related macular degeneration. Retina. Apr 2006;26(4):383-90. [Medline].

  42. Spaide RF, Sorenson J, Maranan L. Photodynamic therapy with verteporfin combined with intravitreal injection of triamcinolone acetonide for choroidal neovascularization. Ophthalmology. Feb 2005;112(2):301-4. [Medline].

  43. Staurenghi G, Orzalesi N, La Capria A, Aschero M. Laser treatment of feeder vessels in subfoveal choroidal neovascular membranes: a revisitation using dynamic indocyanine green angiography. Ophthalmology. Dec 1998;105(12):2297-305. [Medline].

  44. Steen B, Sejersen S, Berglin L, Seregard S, Kvanta A. Matrix metalloproteinases and metalloproteinase inhibitors in choroidal neovascular membranes. Invest Ophthalmol Vis Sci. Oct 1998;39(11):2194-200. [Medline].

  45. Sternberg P Jr, Lewis H. Photodynamic therapy for age-related macular degeneration: a candid appraisal. Am J Ophthalmol. Mar 2004;137(3):483-5. [Medline].

  46. Teixeira A, Moraes N, Farah ME, Bonomo PP. Choroidal neovascularization treated with intravitreal injection of bevacizumab (Avastin) in angioid streaks. Acta Ophthalmol Scand. Dec 2006;84(6):835-6. [Medline].

  47. Tewari A, Dhalla MS, Apte RS. Intravitreal bevacizumab for treatment of choroidal neovascularization in pathologic myopia. Retina. Nov-Dec 2006;26(9):1093-4. [Medline].

  48. Thomas MA, Dickinson JD, Melberg NS, Ibanez HE, Dhaliwal RS. Visual results after surgical removal of subfoveal choroidal neovascular membranes. Ophthalmology. Aug 1994;101(8):1384-96. [Medline].

  49. Verteporfin Roundtable Participants. Guidelines for using verteporfin (visudyne) in photodynamic therapy for choroidal neovascularization due to age-related macular degeneration and other causes: update. Retina. February/March 2005;25(2):119-134. [Medline].

  50. Wood CM, Richardson J. Chorioretinal neovascular membranes complicating contusional eye injuries with indirect choroidal ruptures. Br J Ophthalmol. Feb 1990;74(2):93-6. [Medline].

  51. Wormald R, Evans J, Smeeth L, Henshaw K. Photodynamic therapy for neovascular age-related macular degeneration. Cochrane Database Syst Rev. 2003;(2):CD002030. [Medline].

  52. Wu L, Murphy RP. Photodynamic therapy: a new approach to the treatment of choroidal neovascularization secondary to age-related macular degeneration. Curr Opin Ophthalmol. Jun 1999;10(3):217-20. [Medline].

  53. Wyhinny GJ, Jackson JL, Jampol LM, Caro NC. Subretinal neovascularization following multiple evanescent white-dot syndrome. Arch Ophthalmol. Oct 1990;108(10):1384-5. [Medline].

  54. Yamamoto I, Rogers AH, Reichel E, Yates PA, Duker JS. Intravitreal bevacizumab (Avastin) as treatment for subfoveal choroidal neovascularisation secondary to pathological myopia. Br J Ophthalmol. Feb 2007;91(2):157-60. Epub 2006 Jul 26. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

choroidal neovascularization, choroidal NV, CNV, subretinal neovascularization, Bruch's membrane, subretinal space, retinal pigment epithelium, RPE, visual loss, vision loss, vascular endothelium growth factor, VEGF, pigment epithelium derived factor, PEDF

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Lihteh Wu, MD, Consulting Surgeon, Department of Ophthalmology, Vitreo-Retinal Section, Instituto De Cirugia Ocular, Costa Rica
Lihteh Wu, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Association for Research in Vision and Ophthalmology, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

Coauthor(s)

Teodoro Evans, MD, Retina Fellow, Vitreo-Retinal Section, Instituto De Cirugia Ocular, Costa Rica
Disclosure: Nothing to disclose.

Medical Editor

Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine
Brian A Phillpotts, MD is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, and National Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
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, Macula Society, and Retina Society
Disclosure: Alcon Laboratories Consulting fee 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.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.