An unusual presentation of isolated optic nerve sarcoidosis.

A 32-year old diabetic female had unilateral reduced vision (20/30) in an eye having the ophthalmoscopic appearance of papillophlebitis of the optic nerve head. After an apparently benign course, with recovery, the optic disc became edematous again and a central retinal vein occlusion blinded the eye. Thereafter, an avascular bilobed mass progressively protruded from the optic nerve head. Computerized tomography demonstrated uniform thickening of the optic nerve from the globe through the optic canal. The radiographic appearance was consistent with an optic nerve glioma or meningioma. Spinal fluid, chest x-ray, and serum angiotensin converting enzyme activity were normal. Tuberculin skin test was positive. Optic nerve biopsy demonstrated a noncaseating granuloma and a subsequent Kveim test was positive. Isolated optic nerve sarcoidosis may exist without other systemic manifestations. The appearance on computerized tomography may be similar to that of an optic nerve glioma or meningioma.