Overview
Type II arteriovenous malformations of the spinal cord, specifically intradural perimedullary arteriovenous fistulas, involve abnormal connections between arteries and veins within the spinal cord, often presenting with neurological deficits and potentially hemorrhagic complications. 4Diagnosis
Clinical Presentation: Sudden onset of neurological deficits such as weakness, sensory loss, and pain in the lower extremities. 14
Imaging Studies: MRI showing intramedullary mass lesions with flow voids; angiography crucial for visualizing the vascular malformation and varix. 14
Angiographic Features: Identification of fistulous connections and varix formation within the spinal cord. 14Management
Endovascular Embolization: Primary treatment approach, targeting fistulous points while carefully preserving drainage routes to avoid complications like varix occlusion. 1
Surgical Intervention: Reserved for cases where endovascular treatment is insufficient or contraindicated, involving resection of the fistula and hematoma. 24
Preoperative Assessment: Utilize fusion images from cone-beam CT and volumetric MRI to plan safe embolization and surgical approaches. 1Special Populations
Pediatrics: Early presentation in infants and toddlers is noted, with spinal arteriovenous malformations presenting before 2 years of age being relatively rare but requiring prompt intervention. 34
Comorbidities: Hemorrhagic complications, including hematomyelia and varix formation, can occur and necessitate careful management to prevent further neurological damage. 2Key Recommendations
Utilize advanced imaging techniques (MRI, angiography with fusion images) for accurate diagnosis and planning of endovascular procedures. (Evidence: Moderate 1)
Perform targeted embolization cautiously to avoid occlusion of shared drainage routes and to minimize risk of varix complications. (Evidence: Moderate 1)
Consider surgical intervention when endovascular methods are inadequate or contraindicated, especially in cases with significant intramedullary hematoma. (Evidence: Weak 24)References
1 Yoshida K, Niimi Y, Kamamoto D, Fukumura M, Imai R, Nagoshi N et al.. Targeted embolisation for coexisting conus medullaris arteriovenous malformation and cauda equina arteriovenous fistulas with a varix on a shared drainer. British journal of neurosurgery 2023. link
2 Minami M, Hanakita J, Takahashi T, Kitahama Y, Onoue S, Kino T et al.. Spinal dural arteriovenous fistula with hematomyelia caused by intraparenchymal varix of draining vein. The spine journal : official journal of the North American Spine Society 2009. link
3 Cullen S, Alvarez H, Rodesch G, Lasjaunias P. Spinal arteriovenous shunts presenting before 2 years of age: analysis of 13 cases. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 2006. link
4 Ikezaki K, Miyoshi K, Muratani H, Ogata N, Goto K, Fukui M. Spinal intradural perimedullary arteriovenous fistula with varix in infant. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2000. link