Acute Spontaneous Rupture of the Superior Ophthalmic Vein

To describe patients presenting with sudden onset of localized hemorrhage from a presumed acute spontaneous rupture of the superior ophthalmic vein.

A retrospective review of the pattern of presentation, clinical implications and outcomes, and the characteristic imaging and histologic features.

Six patients (5 men; 84%) presented at a mean age of 45 years (range 15-72), with the commonest symptoms and signs being acute onset of Valsalva-negative proptosis (mean 3.3 mm; range 0-7 mm), orbital pain, and diplopia; none had a known precipitating factor (such as severe Valsalva maneuver or coagulopathy). Two patients developed a mild optic neuropathy. Imaging revealed a well-defined, ovoid, homogenous soft-tissue mass above (2 cases), superomedially to (3 cases), or below the superior ophthalmic vein; the masses were typically echogenic on B-mode ultrasonography, and there was no detectable internal blood-flow. Resolution of signs and symptoms was noted in 5 patients over an average of 4.6 months (range 3-7 months), while 1 patient-the youngest-required excision of a persistent mass with increasing exophthalmos and early optic neuropathy.

Well-localized hemorrhage from the mid-part of the superior ophthalmic vein is very rare and appears to occur spontaneously in the absence of a detectable venous-lymphatic anomaly. This event might imply a spontaneous "blowout" from an area of mural susceptibility in this part of the superior ophthalmic vein, thus forming a pseudoaneurysm. In most cases, the clinical signs slowly improve over several months, surgical intervention is not required, and recurrence appears very infrequent.