Case Summary: In less than 400 words, please summarize the case. Briefly describe the case and any particular complexities and/or complications encountered.: A 10-year-old boy presented with new onset dizziness, gait abnormality, and emesis 20 hours prior to admission. Parents denied infectious symptoms or preceding head or neck trauma. Medical history was notable for daily morning emesis and intermittent dysphagia since 2 years of age with negative extensive workup.
Neurologic exam showed subtle left-sided ataxia on heel-to-shin testing; finger-to-nose testing was intact. He had unstable, wide-based gait requiring assistance to ambulate, and was unable to tandem walk. Romberg was positive with sway. The remainder of the exam was normal. Initial MRI demonstrated an acute/subacute left cerebellar hemisphere infarct in the posterior inferior cerebellar artery territory. Laboratory evaluation was unremarkable and included CBC, CMP, CRP, ESR, coagulation studies and hypercoagulability labs. Transthoracic echocardiogram was without evidence of left-to-right shunt.
CTA demonstrated subtle narrowing of the left vertebral artery (VA) at the atlantooccipital joint with asymmetry in the position of the dens relative to the lateral masses of C1, raising concern for dynamic vessel compression. TCD with and without neck rotation and flexion/extension radiographs of the cervical spine were non-diagnostic. Given ongoing concern for rotational vasculopathy, the patient was started on aspirin 81 mg and was placed in a soft cervical collar with plans for further imaging once he had recovered from his acute stroke. Eight weeks later, he underwent contrast-enhanced MRI/MRA of the head and neck with dynamic positioning, which re-demonstrated left VA narrowing at the occipital condyle-axis joint with focal narrowing when piercing the dura. MRA in 8 positions demonstrated loss of flow-related enhancement with left head tilt and neck extension. A new area of T2 hyperintensity was visualized on MRI in the left cerebellum, likely representing an interval infarction. He subsequently underwent a C1 laminectomy without complication fourteen weeks after his initial presentation.
Since his procedure, he remained asymptomatic without recurrent episodes of dizziness or gait instability. Repeat MRI/MRA 6 months after his operation showed improved caliber of the left V3-V4 segment and preserved flow related enhancement in the V4 segment. Dynamic contrasted-enhanced MRA imaging can serve as a non-invasive alternative to DSA in the diagnosis of rotational VA syndrome and should be considered in suspected cases of pediatric rotational arteriopathy.