Naiem Nassiri, MD, Nolan C. Cirillo-Penn, BA, Jones Thomas, BA

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Evaluation and management of congenital peripheral arteriovenous malformations Naiem Nassiri, MD, Nolan C. Cirillo-Penn, BA, Jones Thomas, BA Journal of Vascular Surgery Volume 62, Issue 6, Pages 1667-1676 (December 2015) DOI: 10.1016/j.jvs.2015.08.052 Copyright © 2015 Society for Vascular Surgery Terms and Conditions

Fig 1 A, Isolated high-flow arteriovenous malformation (AVM) of the right hypothenar region. B, Arteriogram reveals prominent palmar arch nidus fed by the radial artery with decreased distal digital flow. C, Transvenous coil embolization of venous drainage was performed (note guidewire in distal cephalic vein and anchored detachable coil (white arrow) deposited in nidus venous drainage tract. This was followed by transarterial and direct percutaneous nidus embolization using Onyx. Note Onyx deposit cast in completion angiogram (white arrowhead). There is significant improvement in distal digital flow with no evidence of nontarget embolization. D, Asymmetric, compressible soft tissue masses along anterolateral leg in Cowden syndrome suspicious for AVMs. E, Note moderate T2 enhancement on magnetic resonance imaging with scattered flow-void foci. Journal of Vascular Surgery 2015 62, 1667-1676DOI: (10.1016/j.jvs.2015.08.052) Copyright © 2015 Society for Vascular Surgery Terms and Conditions

Fig 2 A and B, Scattered erythematous macules (telangiectasias) along volar digital and tongue distribution. C, Computed tomography angiography reveals a pelvic arteriovenous malformation (AVM) fed by the left ovarian artery. Findings strongly suggestive of Osler-Weber-Rendu (OWR) syndrome. D, Transarterial access into the AVM nidus was not possible given the extreme tortuosity of the feeding artery. Therefore, retrograde transvenous access was attempted. Note (D) extremely tortuous, elongated left ovarian artery feeding an AVM nidus with (E) subsequent shunting into an aneurysmal draining ovarian vein on delayed arterial phase runs. F, Retrograde, transvenous access is achieved via coaxial microcatheter system via the left renal vein with (G) nidus embolization using detachable coils and Onyx. H, Completion angiogram shows complete obliteration of the AVM with no evidence of nontarget embolization. Journal of Vascular Surgery 2015 62, 1667-1676DOI: (10.1016/j.jvs.2015.08.052) Copyright © 2015 Society for Vascular Surgery Terms and Conditions

Fig 3 Contrast-enhanced, cross sectional fat suppressed T2 magnetic resonance imaging demonstrating characteristic features differentiating (A) slow-flow venous malformations from (B) high-flow arteriovenous malformations (AVMs). A, Note T2-hyperintensity without flow voids. B, Note mild to moderate peripheral T2 enhancement with interspersed flow voids suggestive of high-flow shunts. (A, Courtesy of Dr Orhan Konez, MD; available at: www.birthmarks.us/MRI.htm.) Journal of Vascular Surgery 2015 62, 1667-1676DOI: (10.1016/j.jvs.2015.08.052) Copyright © 2015 Society for Vascular Surgery Terms and Conditions

Fig 4 High-flow AVM in the distal left leg. A and C, Note arterial feeders from the distal peroneal artery and posterior tibial artery feeding the nidus of the AVM with premature shunting into draining veins. B and D, Microcatheter access into the nidus of the AVM was performed with embolization of the nidus using Onyx. Note preservation and augmentation of distal outflow and elimination of the nidus. Journal of Vascular Surgery 2015 62, 1667-1676DOI: (10.1016/j.jvs.2015.08.052) Copyright © 2015 Society for Vascular Surgery Terms and Conditions

Fig 5 Isolated high-flow AVM of the right foot fed by numerous branches of the dorsalis pedis artery (A). Given the extensive network of arterial feeders that would make catheterization of each and every one extremely arduous and time consuming, direct stick embolization of the nidus was performed using a micropuncture needle and injection of Onyx. Onyx cast at multiple nidus foci can be appreciated (B). Completion angiogram shows significant reduction of flow through the nidus while preserving distal digital flow (C). Journal of Vascular Surgery 2015 62, 1667-1676DOI: (10.1016/j.jvs.2015.08.052) Copyright © 2015 Society for Vascular Surgery Terms and Conditions