Plexiform neurofibromatosis of the eyelid and the region about the orbit and temple is a well recognized though rare manifestation of Recklinghausen's disease, usually appearing in early childhood. Other peripheral changes, such as cutaneous coffee-colored spots, molluscum fibrosum and multiple tumors of the peripheral nerves, are frequently present.
The disease is recognized by the presence of a thickened, pendulous upper lid, which is often accompanied by a tumor-like mass in the temple, orbit and side of the face. These masses are soft and when palpated have the feel of knotted cords. When the nerves of the orbit are involved there is usually some degree of proptosis and at times pulsation of the globe. Intraocular involvement also occurs, though it is rare.
I have been unable to find a record of a case of plexiform neurofibromatosis of the orbit and globe in which there was an associated tumor of the optic
DAVIS FA. PLEXIFORM NEUROFIBROMATOSIS (RECKLINGHAUSEN'S DISEASE) OF ORBIT AND GLOBE: WITH ASSOCIATED GLIOMA OF THE OPTIC NERVE AND BRAIN REPORT OF A CASE. Arch Ophthalmol. 1939;22(5):761–791. doi:10.1001/archopht.1939.00860110047004
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