Septic Superior Ophthalmic Vein Thrombosis in a Prothrombotic Adolescent Patient

A 14-year-old boy presented to the emergency room with 5 days of fever and periorbital edema and erythema refractory to 4 days of augmentin and clindamycin. Examination revealed normal visual acuity, mild left periorbital edema and erythema, and a (-)1 supraduction deficit, concerning for orbital cellulitis. CT imaging revealed pansinusitis, orbital cellulitis with subtle orbital fat stranding, and a dilated superior ophthalmic vein with concern for thrombosis. This was confirmed on subsequent magnetic resonance venography. He was treated with intravenous antibiotics and enoxaparin with significant improvement in 24 hours. Standard hypercoagulable workup revealed positive lupus anticoagulant and cardiolipin of unclear significance; on genetic sequencing, he was found to have a homozygous mutation of the 4G variant of plasminogen activator inhibitor-1. Plasminogen activator inhibitor-1 homozygosity is a rare predisposing hypercoagulable state. This case highlights the importance of hematologic and genetic studies in adolescent patients with superior ophthalmic vein thrombosis, particularly those with mild presentation.