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

Abstract and Introduction

The white dot syndromes are a heterogeneous group of rare inflammatory disorders affecting the retina, the retinal pigment epithelium, and the choroid. Not all of these diseases actually cause white dots, but they all have unique lesions in the fundus. We describe acute posterior multifocal placoid pigment epitheliopathy, serpiginous choroiditis, birdshot chorioretinopathy, multifocal choroiditis with panuveitis, diffuse subretinal fibrosis syndrome, punctate inner choroidopathy, multiple evanescent white dot syndrome, and diffuse unilateral subacute neuroretinitis as the white dot syndromes in this review. Some of these conditions share an association with systemic infectious diseases. In addition, treatment of these diseases is similar. Some can be treated with immunosuppressive therapy. Other treatment options include laser photocoagulation, topical or systemic steroid therapy, photodynamic therapy, and, most recently, anti-vascular endothelial growth factor agents. The new development in treatment may alter the visual prognosis of the patients, leading to a better outcome in visual acuity. (Comp Ophthalmol Update 8: 179-200, 2007)

The white dot syndromes are a heterogeneous group of uncommon inflammatory disorders affecting the retina, retinal pigment epithelium (RPE), and choroid. All of these disorders are relatively uncommon, share some similarities in appearance, and may be difficult to accurately diagnose. Most of the diseases subsumed under the white dot syndromes share an association with systemic infectious diseases, such as viral infections, toxoplasmosis, or tuberculosis. In many cases, an intensive medical and infectious workup is indicated.[1]

While multiple evanescent white dot syndrome (MEWDS) actually causes white dots, most of the white dot syndromes either do not, strictly speaking, cause white dots or white dots alone. The fundus lesions can be focal chorioretinal scars, as seen in multifocal choroiditis with panuveitis (MCP) or punctate inner choroidopathy (PIC). Round, yellowish-white lesions can be seen in birdshot chorioretinopathy; these lesions are usually one-fourth to 1 disk diameter in size. Yellowish amoeboid lesions can be seen in acute posterior multifocal placoid pigment epitheliopathy (APMPPE), and these lesions are usually 1 disk diameter or confluences of multiple 1-diskdiameter lesions. Serpiginous choroiditis causes crops of polygonal scars, often 1 disk diameter or larger, that fit together like pieces of a jigsaw puzzle. Acute serpiginous choroiditis lesions are grayish-white and lead to full-thickness scarring and atrophy with resolution. Diffuse unilateral subacute neuroretinitis (DUSN) causes a mixture of abnormalities, including white dots, optic atrophy, diffuse chorioretinal atrophy and scarring, and involvement of the entire fundus.

Thus, it is apparent that the term white dot syndrome is a misnomer, but the grouping does retain usefulness. White dot syndromes have the potential for causing severe visual loss, but many of the diseases comprising white dot syndromes can be treated. Some are treated by attacking inflammatory pathways, while DUSN is treated by lasering the intraocular nematode. The treatment paradigms are rapidly changing for both the specific white dot syndrome and, in particular, for associated sequelae, such as choroidal neovascularization (CNV). At one time laser photocoagulation was used for CNV, but with the advent of photodynamic therapy (PDT), agents that bind vascular endothelial growth factor (VEGF) and aggressive control of inflammation, CNV can often be treated without inducing the additional destruction caused by thermal laser photocoagulation. In this review, we will discuss each of the diseases commonly considered to be one of the white dot syndromes. The demographics and epidemiology, clinical findings, testing, treatment, and outcome of each disease will be discussed in detail.

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