Maurice F. Rabb » Lymphomatoid Granulomatosis

A 55 year old man developed respiratory symptoms which progressed rapidly over a period of several weeks to severe respiratory distreee with leukocytosis and severe anemia. Chest X-ray showed miliary infiltrates. While hospitalized, the patient developed rapid deterioration of central vision in both eyes. An ophthalmologist who saw the patient found multiple irregularly shaped yellow-white slightly elevated lesions in the fundi of both eyes. The ony available record is a non-stereo photograph of the posterior pole of the left eye.

Exploratory thoractomy was performed, and lung biopsy showed tissue compatible with the diagnosis of lymphomatoid granulomatosis. The patient was treated with high doses of oral corticosteroids with dramatic improvement in systemic symptoms. He has been maintained on Cytoxan and low dose Prednisone for the past year and has been nearly asymptomatic, but vision has not improved.

The patient was examined 6 months after the onset of symptoms. Visual acuity was limited to 20/400 in the right eye and 20/50 in the left eye. Biomicroscopy showed nuclear cataracts in both eyes estimated to reduce visual acuity to 20/30. The vitreous was clear. Fundus examination showed large geographic areas of apparent atrophy of outer retinal layers and choroid in the posterior pole of each eye.

Photographs and fluorescein angiogram are less than optimal quality because of cataracts, but show the changes described above. Fluorescein angiogram shows near absence of choroidal circulation in the early phases and hyperfluoresceence surrounding the involved areas.