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Article
October 1948

RETINAL TUMOR ASSOCIATED WITH NEUROFIBROMATOSIS (VON RECKLINGHAUSEN'S DISEASE)Report of a Case

Author Affiliations

NEW YORK
From the service of Dr. E. B. Gresser, Department of Ophthalmology, Beth Israel Hospital.

Arch Ophthalmol. 1948;40(4):433-437. doi:10.1001/archopht.1948.00900030445004
Abstract

IN 1882 von Recklinghausen1 described 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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