Intermittent Proptosis of Eye Worsened by Coughing

Case History: 25-year-old female presented with complaints of intermittent proptosis of left eye for the past 3 years, worsened by straining, coughing, and in prone position. History of occasional pain, and minimal restriction of ocular movements. Her vision was normal. CT scan was performed.

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Figure 1. Axial NECT (without provocation) shows no proptosis. Coronal MPR shows extraconal soft tissue density in the inferior aspect of left orbit.

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Figure 2. Axial CECT shows intensely enhancing well-defined mass lesion in left orbit.

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Figure 3. Coronal and sagittal CECT show tubular, enhancing mass lesion with both intraconal and extraconal components, in the posteroinferior aspect of left orbit.

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Figure 4. Axial CECT in the same patient performed with Valsalva, shows tortuous, tubular venous varix that has distended due to increased venous pressure. Proptosis of left eye is also evident.

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Figure 5. Coronal and sagittal CECT, under Valsalva show tangled mass of venous channels.

Diagnosis: Orbital varix

Discussion: Orbital venous varix is a congenital low flow venous malformation, presumably associated with congenital weakness in the venous wall. A varix may include a single smooth contoured, dilated vein (simple varix) or a tangled mass of venous channels (complex varix). Varices within the orbit may appear to have both cystic and solid components. Orbital varices may arise secondary to intracranial vascular malformations, particularly arteriovenous shunts and carotid-cavernous fistula. Patients present with intermittent proptosis, precipitated by coughing, straining and valsalva because of the varix's connection to the venous system. There may be associated pain and restricted ocular movements. Sudden worsening of symptoms may occur due to thrombosis or hemorrhage.

Orbital varix is usually retrobulbar and extraconal in location, though it can occur in intraconal compartment. Ultrasound shows anechoic tubular/rounded lesion in retrobulbar location, with slow flow on Doppler. USG is useful as distensibility with valsalva maneuver can be checked bedside. When thrombosed, it appears circumscribed and mass-like.

Because the varix may be completely collapsed or barely visible when the patient is lying quietly supine, any time an orbital varix is suspected, it is recommended that additional CT sections be obtained with provocation - Valsalva maneuver, jugular tourniquet, head hanging, prone positions. Plain CT may show high density mass lesion, with occasional phleboliths. On CECT, there is intense enhancement, and the dynamic change in size and globe displacement may be appreciated on provocative maneuvers. MRI shows orbital varix as several round or tubular structures, with complex signal due to blood flow and thrombosis. It is usually hyperintense on both T1 and T2- weighted images, with intense enhancement on gadolinium administration. Irregular flow voids may be present due to turbulent flow.

Treatment options include transcatheter embolization and surgery.

References
Diagnostic Imaging- Head & Neck, Harnsberger, 1^st edition, 2004.
Head and neck imaging, Som & Curtin, 5^th edition, 2011.