J Cerebrovasc Endovasc Neurosurg.  2024 Mar;26(1):85-96. 10.7461/jcen.2023.E2022.07.010.

Curative transvenous embolization for congenital multi-hole pial arteriovenous fistula

• Fry L
• Brake A
• Lei C
• Stefano FAD
• Bhargav AG
• Peterson J
• Ebersole K

• KMID: 2554051
• DOI: http://doi.org/10.7461/jcen.2023.E2022.07.010

Abstract

Objective
Congenital intracranial pial arteriovenous fistula (PAVF) is a rare cerebral vascular pathology characterized by a direct shunt between one or more pial feeding arteries and a cortical draining vein. Transarterial endovascular embolization (TAE) is widely considered first line therapy. Curative TAE may not be achievable in the multihole variant due to the potential to harbor innumerable small feeding arteries. Transvenous embolization (TVE) may be considered to target the final common outlet of the lesion. Here, we present a series of four patients with complex multi-hole congenital PAVF treated with staged TAE followed by TVE.
Methods
A retrospective review was conducted on patients who underwent treatment for congenital, multi-hole PAVFs treated by a combined TAE/TVE approach at our institution since 2013.
Results
We identified four patients with multi-hole PAVF treated by a combined TAE/TVE. Median age was 5.2 (0-14.7) years. Median follow-up of 8 (1-15) months by catheter angiography and 38 (23-53) months by MRI/MRA was obtained. TVE achieved complete occlusion in three patients that proved durable on radiographic follow-up and demonstrated excellent clinical outcomes with a modified Rankin Score (mRS) of 0 or 1. Complete occlusion of the draining vein was not achieved by TVE in one case. This patient is graded as pediatric mRS=5 three years post-procedure.
Conclusions
With thorough technical considerations, our series indicates that TVE of multi-hole PAVF that are refractory to TAE is feasible and effective in arresting the consequences of chronic, high-flow AV shunting produced by this pathology.

Keyword

Pial atriovenous fistula; Transvenous embolization; Vascular malformation; Endovascular; Pediatric

Figure

• 

  Fig. 1. Schematic highlighting our approach to multi-hole PAVFs. ① In the acute setting, the goal is to temporize the lesion with strategically targeted embolization toward the suspected site of rupture, often with flow control techniques such as temporary balloon occlusion, to prevent unwanted premature venous occlusion. ② Concomitantly or in subsequent sessions, serial flow reduction is achieved via TAE of all navigable arterial feeders. When staging this phase, smaller, often tortuous feeders, previously masked by high-flow competitors, can become newly angiographically apparent. Decreasing the time interval between stages minimizes the impact of these previously occult feeders on the treatment plan. This stage is completed when the lesion is rendered to the lowest flow state achievable by trans-arterial approach. ③ The sine qua non of curative venous embolization is the identification of the proximal venous point, and confirmation that the point is accessible to microcatheter navigation. On occasion, internal strictures may compartmentalize the anatomy, and may be best addressed by targeting each segment separately, functionally defining more than one proximal venous point (as in Fig. 4). The vein is then prepared by simplifying the final venous structure by targeting morphologic features that might impede the creation of the final occlusive cast. Eccentric varices are embolized, taking care to avoid premature compromise to the final venous outflow. Final exit strategies, often utilizing detachable tip microcatheters, are devised. ④ Curative embolization is then performed, typically with liquid embolic from proximal to distal. Onyx is often the agent of choice due to its long polymerization time. The cast is considered final when follow-up angiography of all vessel distributions supplying the lesion demonstrate complete occlusion. PAVF, pial arteriovenous fistula; TAE, transarterial endovascular embolization

• 

  Fig. 2. Schematic of case 2. (A) Ruptured multi-hole PAVF involving the basal right frontal lobe in a 17-month-old. (B) Emergent and subsequent staged TAE targeting high-flow navigable feeders with concentrated NBCA (glue casts in white). Innumerable non-navigable feeders not shown. (C) Trans-venous approach to prepare the vein, targeting an eccentric varix with coils and NBCA. (D) The fistulous vein was functionally separated by an internal stricture, creating a frontal compartment draining to the SSS and a sylvian compartment draining to the transverse sinus. The compartments were treated one after the other with Onyx delivered by detachable tip microcatheters navigated to the proximal most element of each compartment at the internal stricture. (E) The final cast was created from proximal to distal in each compartment, resulting in a singular, completely occlusive cast. PAVF, pial arteriovenous fistula; TAE, transarterial endovascular embolization; NBCA, N-butyl cyanoacrylate; SSS, superior saggital sinus

• 

  Fig. 3. (A) AP vertebral artery arteriograms at baseline, (B) lowest flow state after serial TAE, and (C) 14-month follow-up after TVE demonstrating durable complete occlusion of the fistula (dashed line highlights venous cast). AP, anteroposterior; TAE, transarterial endovascular embolization; TVE, transvenous embolization

• 

  Fig. 4. (A) AP right ICA arteriograms at baseline demonstrating severe, diffuse cerebral vasospasm and extensive fistulous drainage, (B) lowest flow state after serial TAE, (C) 18-month follow-up after TVE demonstrating durable complete occlusion of the fistula (dashed line highlights venous cast). AP, anteroposterior; ICA, internal carotid artery; TAE, transarterial endovascular embolization; TVE, transvenous embolization

• 

  Fig. 5. (A) Lateral vertebral artery arteriograms at baseline, (B) lowest flow state after TAE, and (C) 8-month follow-up after TVE demonstrating durable complete occlusion of the fistula (dashed line highlights venous cast). TAE, transarterial endovascular embolization; TVE, transvenous embolization

• 

  Fig. 6. (A) Baseline axial T2 MRI demonstrating complex vascular malformation with variceal involvement of vein of Galen, hydrocephalus, and global volume loss. (B) Post-procedure axial CT demonstrating intraventricular hemorrhage and hydrocephalus. (C) Axial MRI DWI sequence demonstrating bilateral basal ganglia/internal capsule diffusion restriction secondary to thrombosis of the deep venous drainage (white arrows highlights areas of diffusion restriction). (D) 4-year follow-up axial T2 MRI demonstrating complete regression of previously dilated pathologic veins, normalization of cerebral volume, and no obvious persistent pathology. DWI, diffusion-weighted imaging

• 

  Fig. 7. (A) AP right ICA arteriograms at baseline (B) lowest flow state after serial TAE, and (C) immediate post-treatment follow-up after TVE demonstrating apparent complete occlusion. (D) AP vertebral artery arteriograms at baseline, (E) lowest flow state after serial TAE, and (F) arterial phase immediate post-treatment follow-up after TVE. Dashed line highlights venous cast. (Continued in Fig. 8). AP, anteroposterior; ICA, internal carotid artery; TAE, transarterial endovascular embolization; TVE, transvenous embolization

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  Fig. 8. Immediate post-treatment AP vertebral artery injection, early venous phase. Dashed line indicates venous cast. Arrowheads indicate hyperemia and congestion in the choroid plexus. Arrow indicates persistent, early venous drainage signifying that the fistula was not fully occluded. AP, anteroposterior

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