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Introduction Dural arteriovenous fistulas (dAVFs) of the craniocervical junction (CCJ) are rare but clinically significant vascular lesions characterized by complex anatomy, heterogeneous angioarchitecture, and variable presentation. Located at the interface of cranial and spinal vascular systems, the CCJ represents a unique anatomic transition zone where the endosteal dural layer is reflected between the foramen magnum and the upper cervical lamina. The distal V3 segment, epidural venous plexus, meningeal arteries and spinal nerve roots thus exist here in a common plane. These features may permit a spectrum of arteriovenous shunts with variable clinical behavior. We present four illustrative cases to highlight relationships between angioarchitecture, venous drainage, and clinical presentation.Methods Four patients with CCJ arteriovenous fistulas were retrospectively reviewed. Clinical presentation, imaging findings, angiographic characteristics, treatment approach, and neurological outcomes were analyzed.Results Four patients with CCJ dAVFs were identified (mean age 69 years, range 58-74; 3 female). Lesions were located at the C1-C2 level or ventral medullary-cervical transition and were most commonly supplied by vertebral artery dural or muscular branches, with one case demonstrating an anterior spinal artery-adjacent feeder. Venous drainage was predominantly intradural with perimedullary reflux. Clinical presentation correlated with venous drainage patterns, with two patients presenting with hemorrhage (subarachnoid or intraventricular hemorrhage) and two with venous congestive myelopathy characterized by long-segment cervical cord edema and progressive motor deficits. In one patient, downstream arterialized venous drainage mimicked a lower cervical vascular lesion before repeat angiography localized the fistula to the CCJ. Three cases were treated with microsurgical ligation with stabilization or neurological improvement, and one case was managed conservatively.Conclusion CCJ arteriovenous fistulas represent a heterogeneous group of lesions arising from the unique anatomy of the craniospinal dural transition zone. The transition from dual-layer cranial dura to single-layer spinal dura may contribute to variability in venous drainage patterns and clinical presentation. In this series, lesions at the C1/C2 nerve root sleeves were associated with hemorrhagic presentations and intradural reflux, while more cranially located lesions demonstrated perimedullary drainage with venous congestive myelopathy. These observations suggest that anatomic location within the CCJ may influence clinical phenotype and support a location and drainage-based framework for diagnosis and management.Disclosures C. Chang: None. R. Tahir: None. D. Felbaum: None. S. Sur: None.Abstract E-058 Table 1Clinical and angioarchitectural characteristics of four CCJ dAVF casesCaseAgeSexLocationFeeding ArteriesDraining VeinsHemorrhageClinical Presentation158MC1 nerve root at foramen magnum, V3/V4 junctionVA (V3-V4 junction)Intradural/perimedullaryNon-HemorrhagicRapidly progressive quadriplegia, long cord edema273FVentral medullary-cervical transitionASA-adjacent vessel at medullaPerimedullaryNon-HemorrhagicProgressive quadriparesis, long cord edema374FC1/C2VA and muscular branch of left VAIntradural/perimedullaryHemorrhagic (SAH, IVH)Headache, neck pain471FC2 left nerve rootMusculoskeletal branch from V3 segment, left VAReflux into medullary vein, intradural midlineHemorrhagic (IVH)Headaches, AMS, balance issues