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An Unexpected Cause of Right-Sided Facial and Periorbital Edema

Tauber, Jenna M.D.*; Joiner, Devon M.D.*; Hsu, Kevin M.D.; Barmettler, Anne M.D.*

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Ophthalmic Plastic and Reconstructive Surgery: April 13, 2022 - Volume - Issue - 10.1097/IOP.0000000000002192
doi: 10.1097/IOP.0000000000002192
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Superior vena cava (SVC) syndrome results from compression or occlusion of the SVC or brachiocephalic vein. Ensuing superior vascular congestion can present as edema of the face, neck, arm, and thorax.

A 53-year-old man with poorly controlled diabetes on hemodialysis presented with progressive atraumatic right-sided eye pain and swelling for 3 weeks. Right visual acuity had decreased to 20/800. He had a baseline left sensory exotropia and new limited right abduction. Eye pressures and fundus examination were noncontributory. He had right-sided eyelid edema and erythema with no warmth or tenderness (Fig.1A) and mild right temporal chemosis and injection. He was afebrile without leukocytosis. MRI of the orbits with intravenous contrast (Fig.1B) demonstrated normal filling of the right cavernous sinus (black arrow, Fig.1B) with right-sided proptosis and periorbital and orbital soft tissue swelling (white arrow, Fig.1B). Edema progressed despite broad-spectrum intravenous antibiotics and steroids. CT chest showed a distal right internal jugular vein thrombosis adjacent to the brachial-jugular vascular hemodialysis graft. Occlusion of the SVC on digital subtraction venogram (Fig.1C, arrow) confirmed SVC syndrome. Therapeutic intervention should be directed at underlying causes, such as removal of hardware or treatment of malignancy. In this case, SVC recanalization and stenting (Fig. 2A) resulted in improvement in visual acuity and near resolution of periorbital edema (Fig. 2B) and extraocular motility within 2 hours.

FIG. 1.:
A, External color photograph illustrating right-sided periorbital and facial edema. B, MRI of the orbits with intravenous contrast demonstrating normal filling of the right cavernous sinus (black arrow) and perioorbital and orbital soft tissue edema (white arrow). C, Digital Subtraction Venogram of the right subclavian vein accessed through the right arm AV fistula demonstrating occlusion of the right superior vena cava (SVC) (black arrow) and retrograde flow through the right internal jugular vein.
FIG. 2.:
A, Digital Subtraction Venogram of the right subclavian vein demonstrating patency of the right superior vena cava (SVC) (black arrow) after angioplasty with resolution of retrograde flow. B, External photograph obtained 2 hours after SVC recanalization and stenting, demonstrating mild periorbital and facial edema and improved eyelid opening.

Diagnostic delays of SVC syndrome can lead to serious irreversible ocular damage. SVC syndrome should be considered on the differential for periorbital edema.

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