Pulsating Exophthalmos Due to Neurofibromatosis.

نویسنده

P SIVARAMASUBRAHMANYAM

چکیده

CASES of pulsating exophthalmos, which are seen from time to time, are mostly vascular in origin. Rarely they are due to transmitted cerebral pulsations, as in congenital failure of the orbital roof, erosion of the walls due to inflammatory processes, and traumatic hiatus of the orbital roof. Occasionally they are due to plexiform neurofibromatosis. von Recklinghausen (1882) first described a combination of melanomata and multiple neurofibromata arising from cutaneo-us nerve filaments. The complete picture is characterized by tumours of the skin, cutaneous pigment, multiple tumours arising from the sheaths ofcranial, spinal, peripheral, and sympathetic nerves, defective development of the central nervous system, mental abnormalities, and buphthalmos. There are various incomplete forms. P'aez Allende (1945) listed the sites of ocular involvement in neurofibromatosis in the order of frequency-the lids, optic nerve, orbit, retina, iris, cornea, tarsal conjunctiva, and bulbar conjunctiva. Cockayne (1933) reported that there is a definite limitation to the male sex in isolated cases of neurofibromatosis. Hine and Wyatt (1928) reported a case of neurofibromatosis of the right orbit. Moore (1931) suggested that pulsatile exophthalmos in neurofibromatosis was caused by dehiscence of the posterior part of the orbit. Lisch (1937) and Wolter and Butler (1963) found that nodules in the iris were always bilateral in these cases. That glaucoma might not occur until adult life has been emphasized by Duke-Elder (1940) and has been illustrated by Meeker's (1936) case. A case is reported below of von Recklinghausen's disease presenting with multiple cutaneous neurofibromata, cafJ-au-lait spots, and plexiform neurofibromata of the upper lid and temporal region involving the right orbit, producing pulsatile exophthalmos, nodules in the iris, and asymmetry of the pupil of the same side.

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