A Patient with Cavernous Sinus Dural Arteriovenous Fistula Complicating Klippel-Trenaunay Syndrome

Objective: We report a case of cavernous sinus dural arteriovenous fistula (CSdAVF) associated with Klippel-Trenaunay syndrome (KTS). Case Presentation: A 58-year-old woman was diagnosed with KTS with port-wine stain, overgrowth of tissues and bones, and venous malformation on the left upper limb. She was admitted to our hospital with the primary complain of ptosis and diplopia due to left oculomotor palsy, and her cerebral angiography revealed CSdAVF with retrograde leptomeningeal venous drainage. The shunt point was located at the posteromedial part of the left cavernous sinus (CS) on the angiogram. An enlarged subclavian vein and giant varix was detected in the left upper limb, and abnormality of the coagulation-fibrinolysis system caused by localized intravascular coagulation was confirmed. We performed transvenous coil embolization, and the symptoms improved after a few weeks with no new neurological deficits. However, the activation of coagulation-fibrinolysis system continued even after the surgery. Conclusion: The formation of dAVF occurrence in this case is unclear. If genetic abnormalities that cause angiogenesis are involved in KTS, follow-up is important in the future.