Pial Arteriovenous Fistula and Capillary Malformation-Arteriovenous Malformation Associated with RASA1 Mutation: 2 Pediatric Cases with Successful Surgical Management

2017 ◽  
Vol 52 (4) ◽  
pp. 261-267 ◽  
Author(s):  
A. Jessey Chugh ◽  
Asim Shahid ◽  
Sunil Manjila ◽  
Deepak Gulati ◽  
Nicholas C. Bambakidis
2020 ◽  
pp. 159101992094051
Author(s):  
Bikei Ryu ◽  
Shinsuke Sato ◽  
Tatsuki Mochizuki ◽  
Tatsuya Inoue ◽  
Yoshikazu Okada ◽  
...  

Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is a newly described entity characterized by autosomal dominantly inherited multifocal capillary malformations caused by RASA1 mutations (CM-AVM1) or EPHB4 mutations (CM-AVM2). Concurrent high-flow vascular anomalies in the brain are often present in the form of intracranial AVM or arteriovenous fistula (AVF). These high-flow lesions are often identified at or soon after birth because of the characteristic unique capillary malformations or a systemic disorder due to a high-flow shunt, such as respiratory distress or heart failure. However, de novo intracranial AVMs have not been reported in patients with CM-AVM syndrome. Herein, we report the case of a six-year-old boy with CM-AVM1 who had been treated for an intracranial pial arteriovenous fistula approximately five years previously, in whom a de novo intracranial AVM was identified on a follow-up angiographic study. To the best of our knowledge, this report is the first to document a de novo intracranial AVM in a patient with CM-AVM. We recommend careful neuroimaging follow-up even if initial neuroimaging screening is negative because of the risk of de novo AVM development.


2020 ◽  
Vol 81 (02) ◽  
pp. 185-187
Author(s):  
Gianpaolo Jannelli ◽  
Holger Joswig ◽  
Gianmarco Bernava ◽  
Torstein R. Meling ◽  
Philippe Bijlenga

Abstract Background A pial arteriovenous fistula (PAVF) is a rare neurovascular pathology consisting of a direct connection between one or more arterial feeders and a single venous channel. A PAVF was previously considered a subtype of an arteriovenous malformation (AVM). In the current literature these are distinguished as two independent entities with different clinical, physiopathologic, and structural characteristics. We report a case of a surgically treated 37-year-old woman with an extremely rare combination of an unruptured PAVF and AVM. Patient The AVM was mainly supplied by feeders of the middle cerebral artery and drained into the sigmoid sinus via an abnormally dilated and tortuous vein. Following its resection, intraoperative digital subtraction angiography (DSA) in the hybrid operating room revealed the presence of a PAVF that had not been noted during the preoperative planning. Hence the PAVF was completely disconnected as confirmed by DSA again. Conclusion This is the second reported case of an AVM-associated PAVF. Without the intraoperative DSA in the hybrid operating room, this unusual complex cerebrovascular entity would likely have been missed.


2021 ◽  
pp. 1-6
Author(s):  
Ryan R.L. Phelps ◽  
Kunal P. Raygor ◽  
Matthew R. Amans ◽  
Nalin Gupta ◽  
Adib A. Abla

<b><i>Introduction:</i></b> Pial arteriovenous fistulas are characterized by an abnormal connection between an intracranial artery and vein without an intervening nidus. Their predominant symptoms largely arise from mass effect, shunting, or hemorrhage. Most conservatively managed cases progress to death, but endovascular and/or surgical intervention is often successful. <b><i>Case Presentation:</i></b> We present the unique case of a 15-year-old girl with spontaneous intracranial hemorrhage from a single-vessel arteriovenous fistula. Although preoperative imaging failed to show a distinct nidus, intraoperative indocyanine green angiography performed after successful clipping of the primary fistulous site revealed residual shunting from a peri-lesional arteriovenous malformation. <b><i>Discussion/Conclusion:</i></b> This case demonstrates the importance of intraoperative imaging and meticulous circumferential inspection of these lesions to detect residual vascular shunting.


2021 ◽  
pp. 1
Author(s):  
Kanneganti Vidyasagar ◽  
Narayanam Kiran ◽  
Laxminadh Sivaraju ◽  
Vivek Raj ◽  
Alangar Hegde

2021 ◽  
Vol 14 (1) ◽  
pp. e240483
Author(s):  
Rashid Ahmed ◽  
Carlos Lopez ◽  
Karan Philip ◽  
Grahame Gould

2021 ◽  
pp. 197140092110059
Author(s):  
Marco Colasurdo ◽  
Joseph Domenico Gabrieli ◽  
Giacomo Cester ◽  
Davide Simonato ◽  
Mariagiulia Anglani ◽  
...  

The pressure cooker technique was originally ideated to obtain wedge-flow conditions during arteriovenous malformation or arteriovenous fistula embolisation. The anti-reflux plug created with coils or glue around the tip of a detachable microcatheter enables a continuous injection with a more in-depth penetration. Here we describe two illustrative cases performed with a variation of the technique that we describe as the hand-compression pressure cooker technique.


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