Normal Venous Phase Documented during Angiography in Patients with Spinal Vascular Malformations: Incidence and Clinical Implications

Sorte, Danielle Eckart; Obrzut, Marzanna; Wyse, Emily; Gailloud, Philippe

doi:10.3174/ajnr.a4601

Cited by 11 publications

(2 citation statements)

References 11 publications

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“…The angiographic features of spinal cord parenchymal venous drainage in AESA contrasts with those seen in cases of an AV shunt with medullary venous hypertension (ie, spinal dural AVF) in which there is an absent or delayed angiographic appearance of the veins serving the affected spinal cord segments. 14 Marked arterial ectasia, out of proportion to venous ectasia, is another diagnostic hallmark of AESA. In our series, catheter-directed DSA shows ectasia and tortuosity of the anterior spinal artery and corresponding central sulcal perforators.…”

Section: Discussionmentioning

confidence: 99%

Arterioectatic Spinal Angiopathy of Childhood: Clinical, Imaging, Laboratory, Histologic, and Genetic Description of a Novel CNS Vascular Pathology

Abruzzo

Berg

Vadivelu

et al. 2022

AJNR Am J Neuroradiol

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Pediatric patients with myelopathy expressing intradural spinal vascular ectasia without arteriovenous shunting were studied at four tertiary referral neuropediatric centers. Patients were identified by retrospective review of institutional records and excluded if spinal vascular pathology could be classified into a previously described category of spinal vascular malformation. Four patients meeting the study criteria were enrolled in the study. Clinical, magnetic resonance imaging, catheter-directed angiography, laboratory, histological and genetic data were analyzed to characterize the disease process and elucidate underlying pathomechanisms. Our study revealed a highly lethal, progressive multi-segmental myelopathy associated with a unique form of non-inflammatory spinal angiopathy featuring diffuse enlargement and tortuosity of spinal cord arteries, spinal cord hyperemia, and spinal cord edema (Arterioectatic Spinal Angiopathy of Childhood). The condition was shown to mimic venous congestive myelopathy associated with pediatric spinal cord arteriovenous shunts on MRI but to have distinct pathognomonic findings on catheter-directed angiography. Clinicopathological, genetic, and neuroimaging features, which are described in detail, closely overlap with those of mitochondrial disease. ABBREVIATIONS: AESA ¼ arterioectatic spinal angiopathy; AV ¼ arteriovenous; CPA ¼ cerebral proliferative angiopathy; RMA ¼ radiculomedullary artery; WES ¼ whole exome sequencing S pinal cord arteriovenous (AV) shunts, including AV malformations (AVM) and AV fistulae (AVF) are a rare but treatable cause of myelopathy. [1][2][3][4] Marked ectasia of the spinal cord vasculature on MR imaging is traditionally considered pathognomonic. In children with spinal cord AV shunts, subarachnoid hemorrhage is the clinical presentation in two-thirds, while venous congestive myelopathy is the presentation in one-third. 1,2 Notably, venous congestive myelopathy due to a spinal cord AV shunt is more common in children younger than 2 years of age and features cord edema that spares the cord periphery. 3 A definitive diagnosis relies on demonstration of an AV shunt by catheter-directed DSA. Children with venous congestive myelopathy due to a spinal cord AV shunt most often present with progressive episodic neurologic decline, culminating in complete loss of spinal cord function within months to years. [1][2][3][4] If the diagnosis is established early, treatment of the underlying AV shunt by embolization or microsurgical ligation leads to neurologic stabilization with varying degrees of functional recovery. 1,5 Recognized forms of spinal cord vascular malformations include AV shunt lesions and nonshunting lesions. While multiple classification systems have been proposed, none account for spinal cord vascular ectasia in the absence of an AV shunt. 6 The authors of this report have encountered pediatric patients with myelopathy presenting with striking enlargement of the spinal cord vasculature and vasogenic edema of the central spinal cord in the abse...

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Section: Discussionmentioning

confidence: 99%

Arterioectatic Spinal Angiopathy of Childhood: Clinical, Imaging, Laboratory, Histologic, and Genetic Description of a Novel CNS Vascular Pathology

Abruzzo

Berg

Vadivelu

et al. 2022

AJNR Am J Neuroradiol

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“…In a study by Eckart Sorte et al, they concluded that the mere observation of a normal venous phase cannot be used to exclude the presence of a vascular malformation or justify interrupting a diagnostic spinal angiogram. 8 However, demonstration of venous phase/perimedullary veins is very useful and improves the understanding of venous system anatomy and hemodynamics.…”

Section: Discussionmentioning

confidence: 99%

Spinal Epidural Lipomatosis Associated with Intrathecal Flow Voids: Demonstration of Engorged Veins Using Flat Panel Catheter Angiotomography

Yadav

Hedaoo

Kumar

2021

JNRP

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We present a case of a 54-year-old male with spinal epidural lipomatosis who had associated flow voids on magnetic resonance imaging with dilated intrathecal vessels. During spinal angiogram, 20s DynaCT (flat panel catheter angiotomography) was utilized to demonstrate the intrathecal engorged veins. Venous engorgement of epidural venous plexus has been previously described in epidural lipomatosis; however, dilated intrathecal perimedullary veins have not been demonstrated by imaging. We have described the utility of flat panel catheter angiotomography in understanding venous disorders in such patients.

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Spinal Angiography

Harrigan,

Deveikis

2023

Contemporary Medical Imaging

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Normal Venous Phase Documented during Angiography in Patients with Spinal Vascular Malformations: Incidence and Clinical Implications

Cited by 11 publications

References 11 publications

Arterioectatic Spinal Angiopathy of Childhood: Clinical, Imaging, Laboratory, Histologic, and Genetic Description of a Novel CNS Vascular Pathology

Arterioectatic Spinal Angiopathy of Childhood: Clinical, Imaging, Laboratory, Histologic, and Genetic Description of a Novel CNS Vascular Pathology

Spinal Epidural Lipomatosis Associated with Intrathecal Flow Voids: Demonstration of Engorged Veins Using Flat Panel Catheter Angiotomography

Spinal Angiography

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