TWELVE years ago, at the 93rd Annual Session of the American Medical Association, the subject of pulsating exophthalmos was reviewed.1 That survey encompassed 812 cases. In practically all those cases an arteriovenous shunt was apparently the basic factor responsible for the condition. It would seem that the term "pulsating exophthalmos" automatically implied the association of an arteriovenous communication in or immediately behind the orbit.
No mention was made at that time of a small group of cases of congenital unilateral pulsating exophthalmos in which there is no associated arteriovenous shunt and in which the roentgenologic findings are indicative of defective development of the sphenoid wings and of the orbital plate of the frontal bone. A number of such cases had been placed on the record by 1954, and some authors had found a curious relationship to neurofibromatosis, thus adding further to the mystery of that disease. Also, cases of
BRUWER AJ, KIERLAND RR. NEUROFIBROMATOSIS AND CONGENITAL UNILATERAL PULSATING AND NONPULSATING EXOPHTHALMOS. AMA Arch Ophthalmol. 1955;53(1):2–12. doi:10.1001/archopht.1955.00930010004002