disease progressed, new lesions appeared in the right eye, and the central vision seemed to be threatened. Ciclosporin was added to the therapeutic regimen for 3 months. Although there appeared to be resolution of the lesions, the disease now resembled serpiginous choroiditis (see Fig. 29-6). The patient has been followed for 10 years and his vision has been maintained at 20/32 in the right eye and 20/63 in the left eye. He has had no recurrences. The fundus in both eyes shows marked RPE clumping extending out to the retinal periphery.

Comment 29-2.  Those who have followed this patient coined the term ‘ampiginous’ chorioretinopathy to describe his retinopathy. Did our therapy play any role in maintaining his vision? We do not know. He did not suffer a recurrence, typical of APMPPE. The retinal appearance was not typical of this disease, however; the extensive changes and scarring were more typical of serpiginous chorioretinopathy.

Case 29-3

A 53-year-old myopic Japanese-American woman had a small symptomatic retinal hole noted on a routine ocular examination. This was treated with laser therapy. Some months later, she noticed floaters and decreased visual acuity. She was found to have in the right eye 1+ vitreal cells and multiple creamy-white lesions strewn throughout the posterior pole and extending toward the equator. Despite prednisone therapy her vision continued to decline in that eye. After a macular hemorrhage her vision dropped to 20/400 and fibrosis ensued. Some months later the same problem developed in her left eye. Her vision decreased somewhat to 20/30, with multiple creamywhite lesions being seen in the fundus. She underwent an extensive evaluation to uncover an underlying problem for what was diagnosed as a multifocal choroiditis. Results of a lumbar puncture were normal, as were a gallium scan and conjunctival biopsy. Of interest was the finding of elevated

serum titers for herpes simplex 1 (1 : 2560), herpes simplex 2 (1 : 320), and herpes zoster (1 : 640). Because of this finding, the patient was given a short course of aciclovir, but the lesions continued to evolve and new ones were seen. The progression in the left eye continued along the same course as that seen in the right, and neither antiviral nor immunosuppressive therapy was effective in altering the course of the disease.

Thus the ophthalmologist was presented with a diagnostic dilemma, and because the macula in the left eye was threatened it was decided to perform a chorioretinal biopsy. There was mild atrophy of the inner retinal layers. The choroid was filled with B cells and plasma cells. There were nodules at the level of the RPE that resembled Dalen–Fuchs nodules but were made up predominantly of B cells. Virus could not be detected using immunofluorescence, electron microscopy

or culture. It was decided to treat the patient with cyclophosphamide. The reasons for this decision were that (1) there was no evidence of active infection and (2) with a predominantly B-cell infiltrate, ciclosporin – with its effect being essentially on the T-cell arm of the immune response – would not be indicated. The patient’s ocular disease responded rapidly to cyclophosphamide (initial dose 2 mg/kg, then lowered) combined with 17.5 mg of prednisone. The lesions involuted, and cytotoxic medication was stopped after about 1 year. Her eyes have shown no inflammatory activity for more than 3 years after therapy was stopped. The patient required a cataract extraction in the right eye, from which a biopsy specimen was obtained, but visual acuity of 20/400 in the right eye and 20/30 in the left eye has been maintained.

Comment 29-3.  The white-dot syndromes still remain problematic. The chorioretinal biopsy helped enormously in determining a therapeutic approach. In addition, the finding of B cells in this disease was quite unexpected. We have seen a similar finding in a biopsy specimen of another patient with this disorder. It is interesting to note that T-cell infiltrates at this level of the eye can look clinically essentially the same.31

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