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This case demonstrates a rare pathology of basilar artery pial arteriovenous shunting presenting with acutely progressive symptoms of brainstem compression, in which we applied a polytetrafluoroethylene-coated embolic device to achieve short-segment upper mid-basilar artery sacrifice. In addition to presenting a rare form of basilar arteriovenous fistula (AVF) (likely related to hereditary hemorrhagic telangiectasia) and discussing the extreme management challenges, this case demonstrates the unusual but successful application of a novel neurovascular device. Unanswered questions include the mechanism of development of this pathology and the optimum post-embolization anticoagulation plan.
Reports of pial arteriovenous fistulas (AVFs) that occur in the posterior fossa are quite rare, occasionally appearing in general pial AVF case series or as individual case reports;1–6 in particular, direct AVFs originating off the basilar artery have not previously been reported. We present the case of a child with a basilar pial AVF with mass effect and progressively accelerating neurological decline and report on its management and outcome.
A 3-year-old child with a history of ocular albinism and normal neurological milestones (walking at 10 months, speaking in full sentences) was noted to have several months of progressive gait instability, bilateral hand tremor, dysarthria, drooling, and intermittent esotropia, with accelerating worsening over the most recent 3 weeks. Her parents and three siblings were all healthy.
On examination she had left eye intorsion and limitation of upgaze. She ambulated with a hyper-plantarflexed gait and became progressively unsteady on her feet, with axial ataxia. She had intention tremors bilaterally in the upper extremities. Over the course of observation of several days she developed swallowing difficulties and, as a result, had her oral intake progressively restricted, and showed progressive loss of motor and speech milestones.
On MRI brain she was found to have an AVF that appeared to originate directly off the upper mid-basilar artery, with …
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