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Severe Pediatric Takayasu Arteritis Presenting With Cerebral Ischemia and Supra-Aortic Trunk Occlusion
Roy Ferrufino Mejia1, Alicia Alejandra Peña-García1, Adacrid Colunga-Duran1
1Neurological Surgery, Juarez Hospital of Mexico, Mexico City, MEX.
Abstract:
Takayasu arteritis (TAK) is a rare, chronic, autoimmune, and granulomatous vasculitis that predominantly affects large vessels, especially the aorta and its main branches, often leading to stenosis, occlusion, or aneurysm formation. Although TAK primarily affects young adult females, its occurrence in pediatric patients is uncommon and frequently associated with a more aggressive clinical course. We report a severe neurovascular presentation of TAK in a 16-year-old Mexican female patient who developed sudden-onset right hemiparesis, global aphasia, and multiple syncopal episodes. Marked interlimb blood pressure discrepancies were recorded, with upper limbs showing 63/30 mmHg and 66/35 mmHg compared with 127/47 mmHg and 135/52 mmHg in the lower limbs. Initial computed tomography revealed extensive cerebral ischemia involving the right posterior and left middle cerebral arteries. Diagnostic digital subtraction angiography (DSA) performed via a femoral 7-Fr introducer and JB2/pigtail catheters demonstrated complete occlusion of the supra-aortic trunks with collateral filling from intercostal arteries at T2-T4, consistent with type IIB TAK according to the Numano classification. Intra-arterial pressure measurements confirmed a systolic gradient exceeding 20 mmHg across the aortic arch. The patient required vasopressor support to maintain cerebral perfusion and received corticosteroids, anticoagulation, and neurocritical care. Clinical improvement was achieved with partial recovery of right-sided strength and language function. The absence of renal involvement and the coexistence of hidradenitis suppurativa raise potential questions about overlapping autoimmune mechanisms. This report emphasizes the neuroendovascular diagnostic approach and the importance of early vascular imaging and hemodynamic assessment in pediatric TAK presenting with cerebral ischemia.
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