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A ten-year-old boy with a history of neurofibromatosis type 1 presented with visual loss and proptosis in his left eye. Neuroimaging revealed a fusiform, enhancing mass of the left optic nerve extending through the optic canal and involving the intracranial portion of the nerve. Five months later, he experienced acute onset of severe pain and a dilated left pupil. Examination disclosed an increase in proptosis from 4-9 mm, neovascular glaucoma, and a central retinal vein occlusion. Repeat imaging showed a marked increase in the size of the mass. The left eye and the intraorbital portion of the optic nerve tumor were resected en bloc. Histological examination showed a pilocytic astrocytoma of the optic nerve with a prominent angiogenic response, iris neovascularization, and angle closure. This ease illustrates the rare occurrence of prominent angiogenic response and neovascular glaucoma complicating a pilocytic astrocytoma of the optic nerve.
Dr. G.K. Klintworth, Duke University Eye Center, Box 3802, Durham, NC 27710, USA
9.4.5.1 Neovascular glaucoma (Part of: 9 Clinical forms of glaucomas > 9.4 Glaucomas associated with other ocular and systemic disorders > 9.4.5 Glaucomas associated with disorders of the retina, choroid and vitreous)