RETINAL TUMOR ASSOCIATED WITH NEUROFIBROMATOSIS (VON RECKLINGHAUSEN'S DISEASE)

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Abstract
IN 1882 von Recklinghausen1described a disease which he called neurofibromatosis. The disease is usually familial, showing mendelian dominance in successive generations, although occasionally skipping a generation. There is no sign of sex-linked inheritance. The disorder is characterized by cutaneous pigmentation and multiple tumors of the cranial and peripheral nerves. In some cases neurofibromatosis is associated with involvement of the skeleton and with disturbances of the glands of internal secretion and mental disorders, including epilepsy. The skin may show a uniform bronzing of various parts. The most typical cutaneous lesions are the circular or oval patches, with sharply defined edges, observed on the trunk. Nevoid formations are frequent. An ocular complication described by Michel2(1873) before the syndrome itself was known was a tumor of the optic nerve in a girl with elephantiasis neuromatodes, a form of neurofibromatosis. At present the ocular complications of neurofibromatosis are classified in

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