The White Dot Syndromes

Yoko Matsumoto, MD, PHD; Sebastian P. Haen, MD; Richard F. Spaide, MD

Disclosures

Compr Ophthalmol Update. 2007;8(4):179-200. 

In This Article

Serpiginous Choroiditis

Serpiginous choroiditis is a rare, chronic, progressive, recurring inflammatory condition of unknown origin that primarily involves the choroid and the RPE. Most of the patients with this condition are middle-aged or older (in the fourth to eighth decade), but younger patients can also be affected.[42] There is slight male predominance in the only case series reported to date. No racial predilection could be observed.[43] Different associations with other diseases, such as infections,[1] viral infections,[44] or neoplasias,[45] have been discussed controversially, but no associations have been proved.

Serpiginous choroiditis is generally asymptomatic until the macula is affected. The classical symptoms are blurred vision, photopsia, metamorphopsia, paracentral scotomas, and visual field loss. Vision loss is related to direct inflammatory damage to the macula or to the advent of CNV.[46,47,48] Elevated serum level of factor VIII, von Willebrand factor antigen,[49] and an increased immune responsiveness against the retinal S-antigen (arrestin)[50] have been reported.

Fundus examination shows grayishyellow, jigsaw-puzzle-shaped lesions at the level of the RPE and choriocapillaris that are usually bilateral, although not necessarily symmetrical.[42,51] The earliest lesions usually emanate from the optic nerve head with geographic shape and distinct borders. Subsequent lesions extend outward toward the periphery. Over a few weeks, the lesions show loss of tissue and become more atrophic with variable amounts of pigmentation (Figure 2). Unlike in APMPPE, these lesions show destruction and loss of the RPE, choroidal vasculature, and a more profound alteration in pigmentation. Inflammation evidenced by cells in the anterior chamber[52] or vitreous may be seen. Recurrent crops of new lesions form over a period of years with extension of retinal and choroidal destruction from the optic nerve.[53,54] If the lesions are near or adjacent to the center of the macula, there is a risk for CNV development. Direct involvement of the center of the macula with inflammatory lesions leads to profound atrophy with severe loss of vision.

During fluorescein angiography, the early phase shows hypofluorescence in acute lesions starting from the margin of the lesion.[43] The central portion of the lesion, which has acute damage to the choriocapillaris, shows little filling. The outer portions of the lesion are bounded by choriocapillaris with relatively good perfusion. Fluorescein originating from these border vessels raises the brush fire staining pattern characteristic for this disease. Later in the angiogram, the entire lesion stains (Figure 3). There may be leakage from adjacent retinal vessels or staining of the optic nerve. Older lesions show variable staining of scar tissue and visible sclera. Indocyanine green angiography reveals hypofluorescence throughout all phases in both acute and old lesions (Figure 3).[34,55] The affected area appears larger in ICG than in fluorescein angiography. Pattern ERGs and visual field testing show abnormalities corresponding to affected areas.

Some patients present with serpiginous lesions as an incidental finding or have a past history of inflammation with no recent activity. These patients may require no treatment. In the acute phase, corticosteroids are most commonly used to control inflammation. The number, severity, and location of the lesions dictate how aggressively the lesions are treated. Often, oral administration of corticosteroids is the most commonly used approach,[56] but recent reports have shown remarkable treatment response to intravitreal triamcinolone.[57,58] Systemic or periocular corticosteroid therapy is effective in healing active lesions and in shortening the duration of the disease but does not prevent recurrences. For patients with initially widespread disease, recent lesions near the central macula, or a history of recurrent lesions appearing with a quick tempo, systemic immunosuppression is required.[59]

In 1991, Hooper and Kaplan suggested a triple-agent immunosuppressive therapy.[59] They used lowdose azathioprine, cyclosporine, and prednisone in combination and then weaned steroids, cyclosporine, and azathioprine in this order. Combination therapies can reduce the individual side effects of each drug because of the low dosage compared to the single treatment. By using this combination therapy of three different agents, the resolution of Fig. 6. Multifocal choroiditis and panuveitis. Secondary juxtafoveal choroidal neovascularization is seen with color (A), fluorescent angiogram (B), and optical coherence tomography (C). active lesions required a much shorter time span than usually seen in untreated eyes and even in sole treatment with prednisone. In 2001, Frank et al reported a case treated with a triple immunosuppressive therapy.[60]

Considering Frank et al's case and Hooper's cases, recurrence seemed to be more frequent if azathioprine was weaned too early. The case number is very limited, however, and further randomized clinical trials are needed to prove that this more aggressive therapy will have a better outcome. There is, however, an indication that this triple immunosuppressive therapy can prevent vision loss of the first affected eye.

Choroidal neovascularization complicating serpiginous choroiditis was treated in the past with laser photocoagulation with poor results for most patients. In many patients, CNV represents a complication of destructive inflammatory lesions. Laser photocoagulation even adds to the destruction of the posterior pole. Pharmacologic approaches aim to reduce neovascularization by using a combination therapy of drugs on the one hand directly targeting VEGF (VEGF-binding antibodies or antibody fragments, such as bevacizumab or ranibizumab), and on the other hand, appropriate drugs directly targeting the inflammation in serpiginous choroiditis.[58,61,62]

Approximately 50% of the affected eyes with serpiginous choroiditis have had persisting central vision loss.[63] The risk for persisting vision loss correlated with recurrences of disease.[63] Aggressive treatment using a combination of corticosteroids and other systemic immunosuppressive agents probably reduces the risk of severe central vision loss, but further investigation is required to delineate the risks and benefits. In addition, the treatment of CNV, which can occur in up to 25% of cases,[164] has improved with the expansion of our armamentarium; this should improve visual acuity prospects of patients with serpiginous choroiditis as well.

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