Maurice F. Rabb » Intraocular Inflammation

This white man first presented to Doheny in May 1984, with a long ocular history dating to 1965 when at age six he presented with best corrected vision of 20/40 in the right and 20/50 in the left. Although no retinal abnormalities were described at that time, he was treated in childhood for intraocular inflammation with steroids. He did satisfactorily until February of 1984 when he noticed symptoms of decreased vision.

On his initial Doheny presentation in May 1984, the best corrected vision in the right eye was 20/30 and in the left eye was 20/70. There was no abnormality on the external or slit lamp examination except that the left eye demonstrated numerous cells in the vitreous cavity and a proteinaceous ray as well.

On fundus examination of the right eye, there was pigmentary alteration affecting the macula. An inactive peripheral perivasculitis affected the veins more than the arteries, particularly superiorly. There was vitreous debris with an appearance that suggested that the inflammatory process in the right eye seemed older and inactive with the exception of one vein at 12 o'clock which suggested active periphlebitis. On fundus examination of the left eye, the vitreous haze was quite prominent with extensive chorioretinal scarring. There was a great deal of lipid, particularly inferiorly, and especially from 6 to 9 o'clock. There was a very prominent angioma temporally, from approximately 2:30 to 5 o'clock with a localized peripheral exudative retinal detachment. This was of the variety that has previously been described by Shields and by Laqua among others.

His past medical history is noteworthy for a hereditary pontocerebellar syndrome. However, CT scan and EEG did not reveal abnormalities or specific lesions. No CNS manifestations of phacomatoses or other diseases were present.

The patient had a thorough workup for intraocular inflammation. No definite diagnosis or etiology could be made despite a battery of tests for intraocular inflammation including HLA typing. The patient was treated with steroids, without a positive response to either systemic steroids or to treatment with Depo-Medrol injections. The visual acuity in the left eye continued to decrease to 20/200 as the exudative detachment extended to involve the macula in the area from 2 to 7 o'clock. Despite the risk of aggravating the exudative detachment, extensive cryotherapy in the manner of freeze-refreeze was carried out to the right eye in April and again in June and has been repeated for a third time. There has been no significant change.