Choroiditis, Serpiginous

Disease Overview

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.

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Synonyms

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

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Signs & 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.

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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.

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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.

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Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Serpiginous Choroiditis. Comparisons may be useful for a differential diagnosis:

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) is a rare acquired eye disorder characterized by the sudden onset of vision loss and inflammation of the outer retina and the pigmented layer of the retina (retinal pigment epithelium). Multiple yellow-white plaque-like lesions appear in the posterior pole of the retina in each eye. The rapid but temporary vision loss often subsides without treatment. Pigment alterations usually remain after resolution of the lesions. Vision loss may be permanent if the lesions affect a specific area on the retina (subfoveally), but more than 90 percent of affected individuals recover visual clarity within a few months of onset. (For more information on this disorder, choose “APMPPE” as your search term in the Rare Disease Database.)

Posterior Uveitis is an eye disorder characterized by inflammation of the vascular layer of the eye (the uvea, which includes the iris, ciliary body and choroid). The other layers of the eye (sclera, cornea, and retina) may also be affected. Posterior Uveitis affects the rear hemisphere of the eye, while Anterior and Intermediate Uveitis affect areas of the front hemisphere. Scars left by Posterior Uveitis on the eye membranes may cause impaired vision. Major symptoms include blurred vision, distortion of the size or shape of objects (metamorphopsia), and floating black spots in the visual field. If the foveal area is affected central vision loss may occur. Onset may be sudden or gradual, depending on the cause of the disorder. In many cases, this disorder occurs as a complication of Toxoplasmosis or other infections. In other cases, the cause cannot be determined. (For more information on this disorder, choose “Posterior Uveitis” as your search term in the Rare Disease Database.)

Tuberculous Uveitis is a very rare type of inflammation of the vascular layer of the eye (uveitis), and may be very difficult to differentiate from other types of uveitis. The vascular layer (uvea) includes the iris, ciliary body and choroid. Tuberculous Uveitis is characterized by a long chronic course and involvement of the choroid and the retina of the eye. Symptoms may appear suddenly, and may include blurred vision, distortion of the size or shape of objects, floating black spots in the visual field, and/or impaired central vision. Diagnosis may be made by indirect evidence, which may include a lack of response to steroid therapy, positive test results for tuberculosis, and/or the ruling out of other causes for the uveitis. In some cases, tentative therapy for tuberculosis (i.e., the antimycobacterial drug isoniazid) may be administered to determine if the uveitis responds. If the treatment is effective Tuberculous Uveitis is the probable diagnosis.

Macular Degeneration is the descriptive term for many forms of deterioration of the central area of vision (macula) from a previous state of normality. There are genetic, age-related, and environmental (including toxic) causes. Subtypes of the disorder include Stargardt’s Disease and fundus flavimaculatus (occuring in children), and disciform macular degeneration (termed senile macular degeneration). Macular Degeneration caused by environmental elements may occur at any age. Major symptoms may include impaired or absent central vision with normal peripheral vision, a disturbance of vision in which shapes are distorted (metamorphopsia), and/or impaired central vision (central scotoma). (For more information on this disorder, choose “Macular Degeneration” as your search term in the Rare Disease Database.)

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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.

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Clinical Trials and Studies

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

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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.

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