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The Korean Journal of Pathology 1987;21(1): 62-65.
A Case of Ocular Neurofibromatosis.
Je G Chi, In Ae Park
Department of Pathology, College of Medicine, Seoul National University, Seoul, Korea.
ABSTRACT
Neurofibromatosis is one of the neurocristopathies that involve many system or tissues forming various types of lesion. Almost every tissue or organ can be involved by this disease. However, the eyeball itself is very rarely affected by this process. The findings seen in our case indicate the diversity of lesions in neurofibromatosis, and also suggest hamartomatous nature. Heterotopic ganglion cells and glial cells in uveal tract are not easily understood. We reported here a case of intra-occular neurofibromatosis with its characteristic involvement of the uveal tract, in a 21 year old female. Her ocular symptoms began at her age of 11 as poor vision and were slowly progressive together with multiple facial neurofibromas. The involved left eyeball showed many ganglioneuroglial cell nests in iris, ciliary body and retina. Minute plexiform neurofibromas were also seen in small nerve twigs around the eyeball.
Key Words: neurofibromatosis; eyeball; hamartoma; uveal tractanomaly
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