Orbital venography in the diagnosis of intermittent exophthalmos.

Abstract

Orbital venography represents the definitive diagnostic study in the evaluation of intermittent exophthalmos. The syndrome of intermittent exophthalmos consists of proptosis that occurs rapidly whenever the head is lowered or when venous pressure is temporarily increased (9, 10). The cause of this unusual form of exophthalmos is a venous abnormality behind or above the globe. Its angiographic demonstration has been reported previously in only one patient (5). Recently two patients with intermittent exophthalmos were seen, in whom the anatomic diagnosis was established by orbital venography. Case Reports Case I: A 32-year-old white man was admitted to the University of California Medical Center for evaluation of intermittent right-sided exophthalmos, present since the age of fourteen years. The patient complained of progressively severe episodes of right-sided proptosis occurring whenever the head was lowered. Severe local pain and blurring of vision limited to the involved eye were associated with the proptosis. The attacks usually lasted a few minutes and were relieved by resuming an upright position. A progressive right-sided enophthalmos developed and was noticeable, between attacks (Fig. 1, A). During hospitalization four years previously, roentgenograms gave evidence of normal symmetric orbits. No phleboliths or widening of the superior orbital fissure were noted. Right common carotid arteriograms at that time showed no abnormality. Orbital venography, employing the angular vein approach, was attempted but was not successful. A presumptive clinical diagnosis of orbital varix was made. Silicone injections into the right upper lid for cosmetic reasons had been given two years before the present admission. On physical examination the vision was 20/15 in each eye. No bruit was heard over the orbits and the fundi were normal. The right eye was slightly enophthalmic (Fig. 1, A), but when the head was lowered an immediate exophthalmos developed, associated with severe pain (Fig. 1, B). Selective right internal and external carotid angiograms demonstrated no abnormality. The ophthalmic artery was visualized and no arteriovenous malformation was noted. Orbital venography, performed by injecting contrast medium into the inferior petrosal sinus, showed excellent opacification of the orbital veins bilaterally. The right inferior ophthalmic vein communicated with a network of dilated venous channels situated in the posterior portion of the orbit (Fig. 2). The venous angioma was limited to the orbit and did not extend intracranially. Case II: A 15-year-old boy was admitted to the Karolinska Hospital in Stockholm, Sweden, for investigation of intermittent left-sided exophthalmos. The patient had had slight prominence of the left eye since early childhood. One year before admission, he bumped his left temple in a minor automobile accident, after which the exophthalmos increased progressively.