Choroiditis, Serpiginous

Choroiditis, Serpiginous

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Choroiditis, Serpiginous is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Geographic Choroiditis
  • Geographic Helicoid Peripapillary Choroidopathy (GHPC)
  • Geographic Serpiginous Choroiditis
  • Peripapillary Choroidopathy
  • Serpiginous Choroidopathy
  • Geographic Choroidopathy

Disorder Subdivisions

  • None

General Discussion

Serpiginous Choroiditis is one of the conditions in a group termed the white dot syndromes which all involve inflammation of the retina and choroid and are defined by the appearance of white dots in the posterior inner part of the eye (fundus). Serpiginous Choroiditis is a rare recurrent eye disorder characterized by irregularly shaped (serpiginous) lesions involving two layers of the eye surface (the retinal pigment epithelium and the choriocapillaris). No symptoms are apparent unless a specific area of the retina (macula) is damaged. A sudden, painless decrease in vision in one or both eyes may be the first sign of Serpiginous Choroiditis. Patients may also notice blind gaps in the visual field (scotomata) or a sensation of flashes of light (photopsia). Both eyes are commonly affected, although the second eye may not develop lesions for weeks to years after the first eye. The exact cause of Serpiginous Choroiditis is not known.

Symptoms

Serpiginous Choroiditis is a rare disorder characterized by recurrent lesions affecting two layers of the eye (the retinal pigment epithelium and the choriocapillaris). The retina is the light-sensitive internal coat of the eyeball, consisting of eight layers. Seven of these layers contain nerves, and one layer contains pigmentation (retinal pigment epithelium). The choroid is a vascular membrane located between the retina inside the eye and the "white" section on the outside of the eye (sclera). The choriocapillaris is the layer of the choroid next to the retina.



Lesions usually begin at the back of the eye near the connection to the optic nerve (optic disk) and may then extend in any direction along the eye layers. Lesions may also begin at other locations and spread toward the optic disk. The disorder has two stages: an acute stage during which lesions develop and/or spread, and a chronic stage during which the disease does not progress but inactive lesions lead to scarring, wasting of tissue (atrophy), and/or clumping of pigment. Recurrences usually start in scar tissue left by old lesions.



Symptoms of Serpiginous Choroiditis appear only if a specific area of the retina (macula) is damaged. A sudden, painless decrease in central vision in one eye may be the first noticeable sign of disease. Blurred central vision and/or the sensation that something is obstructing central vision (positive scotoma) are characteristic symptoms. Both eyes are commonly affected, although the second eye may not develop lesions for weeks to years after the first eye. Vision outside center vision (peripheral) remains normal in most cases. Recurrences are common for this condition.



A membrane composed of tiny fibers and blood vessels (subretinal neovascular membrane) may grow from the choriocapillaris and cause vision distortion and/or blurring in some people with Serpiginous Choroiditis. If a neovascular membrane affects the macular region of the retina, central vision loss may occur.

Causes

The exact cause of Serpiginous Choroiditis is not known. Symptoms develop due to lesions that damage the part of the retina that absorbs short wavelengths of light (macular region). Vision loss may also occur if a membrane composed of fibers and blood vessels (subretinal neovascular membrane) develops and then moves into the macular region. It has been suggested in the medical literature that an abnormal immune response may cause inflammation of the blood vessels (localized vasculitis) of the eye, leading to the development of Serpiginous Choroiditis. Some scientists suggest that the disorder is one of impaired blood circulation in the eye membranes. A few affected individuals have been reported to have had chronic exposure to an unusual variety of chemicals, but the relationship between this exposure and the development of Serpiginous Choroiditis is not clear at this time.

Affected Populations

Serpiginous Choroiditis is a rare disorder that affects males more often than females. Symptoms usually appear during the early to middle adult years.

Standard Therapies

Diagnosis of Serpiginous Choroiditis may be made by clinical evaluation and specialized ophthalmologic examinations such as electroretinography, electro-oculography, and/or fluorescein angiography. Widespread wasting (atrophy) of the choroid and retinal pigment epithelium membranes of the eye, pigment clumping, and/or deposits of fibrous tissue under the retina are characteristic findings.



Periodic clinical examinations are recommended for people with Serpiginous Choroiditis to monitor the status of the disease and allow early detection of neovascular membrane development. Krypton or argon laser treatments may arrest the progress of neovascular membranes associated with Serpiginous Choroiditis.



Treatment is not indicated unless the fovea of the retina is affected. When the fovea is involved, anti-inflammatory medication is recommended.



Vision aids and/or specialized equipment (i.e., closed circuit television) may help to offset vision impairment associated with Serpiginous Choroiditis. Other treatment is symptomatic and supportive.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Kanski, JJ, Ed. Clinical Opthalmology, 2nd Edition. Butterworth-Heineman, 1990. Pp.171-172.



Laatikainen L, et al. A follow-up study on serpiginous choroiditis. Opthalmol. 1981;59(5)707-718.



JOURNAL ARTICLES

Lampariello DA. Geographic (serpiginous) choroiditis. J Am Optom Assoc. 1992;63(2):112-116.



Hooper PL, et al. Triple agent Immunosuppression in serpiginous choroiditis Opthalmology 1991;98(6):944-51; discussion 951-2.



Secchi AG, et al. Cyclosporin-A in the treatment of serpiginous choroiditis. Int Opthalmol. 1990;14(5-6):395-399.



Ustinva EI, et al, Experience in the differential diagnosis of peripapillary "geographic" choroid disease and tuberculous chorioretinitis. Vestn Ofthlmol. 1990;106(6):43-46.



Wu JS, Lewis H, Fine SL, et al. Clinicopathologic findings in a patient with serpiginous choroiditis and treated choroidal neovascularization. Retina. 1989;9(4):292-301.



Mansour AM, Jampol LM, Packo KH, et al. Macular serpiginous choriditis. Retina. 1988;8(2):125-31.



Hardy RA, et al. Macular geographic helicoid choroidopathy. Opthalmol 1987;105(9);1237-42.



Erkkila H, et al. Immunologic studies on serpiginous choroiditis. Graefs Arch Clin Exp Opthalmol. 1982;219(3):131-134.

Resources

Lighthouse International

111 E 59th St

New York, NY 10022-1202

Tel: (800)829-0500

Email: info@lighthouse.org

Internet: http://www.lighthouse.org



Association for Macular Diseases, Inc.

210 E. 64th St.

8th Floor

New York, NY 10065

Tel: (212)605-3719

Fax: (212)605-3795

Email: association@retinal-research.org

Internet: http://www.macula.org/



NIH/National Eye Institute

31 Center Dr

MSC 2510

Bethesda, MD 20892-2510

United States

Tel: (301)496-5248

Fax: (301)402-1065

Email: 2020@nei.nih.gov

Internet: http://www.nei.nih.gov/



Eye Cancer Foundation

115 East 61st Street, Suite 5B

New York, NY 10021

Tel: (212)832-7297

Fax: (212)888-4030

Email: contactus@eyecarefoundation.org

Internet: http://www.eyecarefoundation.org



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

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