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Summary
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.
Background
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.
Case presentation
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.
Investigations
On MRI brain she was found to have an AVF that appeared to originate directly off the upper mid-basilar artery, with …
Footnotes
Contributors APS made substantial contributions to the conception and design of the work, acquisition, analysis, and interpretation of data for the work; drafting and critically revising the work for important intellectual content; final approval of the version to be published; and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. MAK made substantial contributions to the design of the work and interpretation of data for the work; drafting the work; final approval of the version to be published; and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. DBO made substantial contributions to the conception and design of the work and analysis and interpretation of data for the work; critically revising the work for important intellectual content; final approval of the version to be published; and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.