Publications by authors named "Alejandro Berenstein"

83 Publications

High Output Cardiovascular Physiology and Outcomes in Fetal Diagnosis of Vein of Galen Malformation.

Pediatr Cardiol 2021 May 8. Epub 2021 May 8.

Pediatric Heart Center, Icahn School of Medicine At Mount Sinai, New York, NY, USA.

Vein of Galen aneurysmal malformation (VGAM) is a rare anomaly associated with poor outcomes from high output cardiac failure and neurologic complications. Studies addressing fetal cardiovascular status and outcomes in this population are limited. A single-center retrospective review was conducted on patients with a prenatal diagnosis of VGAM who underwent a fetal echocardiogram between January 2015 and July 2019. Fetal echocardiographic data, brain magnetic resonance imaging (MRI) findings and outcomes were collected. Nine fetuses [median gestational age at echocardiogram 34 (1.1) weeks] were included. All patients had superior vena cava dilation and reversal of diastolic flow in the transverse aortic arch. Median cardiothoracic (CT) ratio was 0.39 (0.09). Right ventricular (RV) and left ventricular (LV) dysfunction was present in 66% and 11% fetuses, respectively. Four out of five patients that underwent postnatal endovascular neurosurgical interventions at our center were alive at follow-up (mean 2.7 years). Of the non-survivors (n = 5), 3 received comfort care because of severe brain damage and died in the neonatal period. Non-survivors more commonly had > mild tricuspid regurgitation (TR) (40% vs. 25%) and > mild RV dilation (60% vs. 25%). Combined cardiac index (CCI) was higher in non-survivors when compared to survivors (672.7 vs. 530.2 ml/kg/min, p = 0.016). Fetuses with significant parenchymal damage on brain MRI tended to have a higher CCI than those without (979.8 vs. 605.0 ml/kg/min, p = 0.047). RV dysfunction, TR and elevated CCI are more commonly seen in non-survivors with VGAM. A higher CCI is seen in those deemed untreatable due to significant parenchymal volume loss. Future multicenter studies are needed to assess for prenatal prediction of outcomes in this high-risk population.
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http://dx.doi.org/10.1007/s00246-021-02627-9DOI Listing
May 2021

Middle Meningeal Artery Embolization of a Pediatric Patient With Progressive Chronic Subdural Hematoma.

Oper Neurosurg (Hagerstown) 2021 May 6. Epub 2021 May 6.

Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York, USA.

Background And Importance: Evidence suggests middle meningeal artery (MMA) embolization benefits adult patients with chronic subdural hematoma (CSDH) at high risk for recurrence or hemorrhagic complications. Yet, there has not been any report discussing MMA embolization in the pediatric population. Thus, we present a case of an infant with CSDH successfully managed with MMA embolization without surgical management.

Clinical Presentation: A 5-mo-old girl with idiopathic dilated cardiomyopathy underwent surgical implantation of a left ventricular assist device for a bridge to heart transplantation. This was complicated by left ventricular thrombus causing stroke. She was placed on dual antiplatelet antithrombotic therapy on top of bivalirudin infusion. She sustained a left middle cerebral artery infarction, but did not have neurological deficits. Subsequent computed tomography scans of the head showed a progressively enlarging asymptomatic CSDH, and the heart transplant was repeatedly postponed. The decision was made to proceed with MMA embolization at the age of 7 mo. Bilateral modified MMA embolization, using warmed, low-concentration n-butyl-cyanoacrylate (n-BCA) from distal microcatheter positioning, allowed the embolic material to close the distal MMA and subdural membranous vasculature. The patient underwent successful heart transplant and the CSDH improved significantly. She remained neurologically asymptomatic and had normal neurological development after the MMA embolization.

Conclusion: MMA embolization may represent a safe and effective minimally invasive option for pediatric CSDH, especially for patients at high risk for surgery or hematoma recurrence.
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http://dx.doi.org/10.1093/ons/opab144DOI Listing
May 2021

Bleomycin sclerotherapy following doxycycline lavage in the treatment of ranulas: A retrospective analysis and review of the literature.

Neuroradiol J 2021 Apr 8:19714009211008790. Epub 2021 Apr 8.

Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, USA.

Objective: A ranula is a mucus-filled salivary pseudocyst that forms in the floor of the mouth, commonly arising from the sublingual or submandibular salivary glands following obstruction or trauma. Complete excision of the injured gland and removal of the cyst content is the first-choice therapy, but has the potential for complications related to injury to nearby structures. As such, minimally invasive approaches such as percutaneous sclerotherapy have been investigated. We aim to contribute to the literature by assessing the efficacy and safety of our technique through our experience with 18 patients over the last decade.

Methods: This retrospective study evaluated 18 patients with intraoral and plunging ranulas treated by percutaneous bleomycin ablation. The primary endpoint was the treatment result. Secondary endpoints included bleomycin dosage and complications.

Results: The study evaluated 12 males and six females with a median age of 23.5 years (range 13-39 years). At a final follow-up of at least 2 months (6.5±5.5 months), four patients demonstrated complete response (22%) and 14 patients demonstrated residual presence, recurrence, or regrowth of the lesion (78%). There were no statistically significant associations between outcomes and history of prior treatment, number of treatments, and size or type of ranula. No complications were noted.

Conclusions: Our findings indicate that bleomycin, while safe for use in various head and neck malformations, is of limited utility in ranula therapy when the offending gland is not addressed primarily.
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http://dx.doi.org/10.1177/19714009211008790DOI Listing
April 2021

Management of a Laryngeal Venous Malformation With Nd:YAG Laser and Bleomycin Sclerotherapy.

Laryngoscope 2020 09 11;130(9):2199-2201. Epub 2019 Nov 11.

Department of Otolaryngology, Icahn School of Medicine at Mount Sinai, New York, New York, U.S.A.

Literature describing neodymium:yttrium-aluminum-garnet (Nd:YAG) photocoagulation and sclerotherapy for laryngeal venous malformations (VMs) is sparse. Here we present a case in which an extensive laryngeal VM in a 28-year-old female was managed through a combination of four serial Nd:YAG laser photocoagulation sessions and four bleomycin injections over the course of 2 years. The treatment plan resulted in resulted in noticeable lesion ablation, mucosalization, and significant improvement in symptoms. To our knowledge, this case is the first instance of bleomycin injection specifically into a laryngeal VM reported in the English medical literature. Laryngoscope, 130:2199-2201, 2020.
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http://dx.doi.org/10.1002/lary.28392DOI Listing
September 2020

Distal microcatheter placement using the Asahi Chikai 0.008-inch micro-guidewire for liquid embolic material delivery: A technical note.

World Neurosurg 2019 Jan 4. Epub 2019 Jan 4.

Department of Neurosurgery, Icahn School of Medicine ,Mount Sinai Health Care System, New York, New York,USA.

Introduction: Management of vascular malformations requires microcatheter manipulation through distal tortuous arterial feeders less than a millimetre in diameter, to permit safe and effective embolization at the fistula or nidus location. The Asahi Chikai 0.008 inch micro-guidewire recently introduced in the United States was used to evaluate microcatheter torque, support and safety in distal microcatheter navigation.

Methods: The use of the Asahi Chikai 0.008 inch micro-guidewire in conjunction with flow-guided, and over the wire microcatheters was evaluated in 25 neuroendovascular cases over a 1 year period. It involved single operator usage for endovascular microcatheter embolization of intracranial vascular malformations listed below. The study evaluated angiographic evidence of distal microcatheter positioning, subsequent obliteration of the fistula at the location and complications if any.

Results: The micro-guidewire was used in microcatheter embolization of 10 vein of Galen malformations, 11 brain and head & neck arteriovenous malformations, 2 spinal and 2 cranial dural arteriovenous fistula. Good flow directed and over the wire microcatheter placement using the Asahi Chikai 0.008 inch micro-guidewire was possible across the spectrum of cases achieving optimum catheterization and safe embolization in all. There were no micro-guidewire or microcatheter related vessel spasm, dissection, or perforation during their combined or individual manipulation, contrast or embolic extravasation on fluoroscopy or post- embolization angiograms CONCLUSION: The Asahi Chikai 0.008 inch micro-guidewire is a safe, sturdy micro-guidewire which provides good torque in distal arterial anatomy while providing microcatheter support and maintaining tip configuration on repeated use.
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http://dx.doi.org/10.1016/j.wneu.2018.12.132DOI Listing
January 2019

Mutations in Chromatin Modifier and Ephrin Signaling Genes in Vein of Galen Malformation.

Neuron 2019 02 18;101(3):429-443.e4. Epub 2018 Dec 18.

Division of Nephrology and Center for Vascular Biology Research, Beth Israel Deaconess Medical Center, and Department of Medicine, Harvard Medical School, Boston, MA, USA.

Normal vascular development includes the formation and specification of arteries, veins, and intervening capillaries. Vein of Galen malformations (VOGMs) are among the most common and severe neonatal brain arterio-venous malformations, shunting arterial blood into the brain's deep venous system through aberrant direct connections. Exome sequencing of 55 VOGM probands, including 52 parent-offspring trios, revealed enrichment of rare damaging de novo mutations in chromatin modifier genes that play essential roles in brain and vascular development. Other VOGM probands harbored rare inherited damaging mutations in Ephrin signaling genes, including a genome-wide significant mutation burden in EPHB4. Inherited mutations showed incomplete penetrance and variable expressivity, with mutation carriers often exhibiting cutaneous vascular abnormalities, suggesting a two-hit mechanism. The identified mutations collectively account for ∼30% of studied VOGM cases. These findings provide insight into disease biology and may have clinical implications for risk assessment.
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http://dx.doi.org/10.1016/j.neuron.2018.11.041DOI Listing
February 2019

Commentary: Vein of Galen Malformations: The Texas Children's Hospital Experience in the Modern Endovascular Era.

Oper Neurosurg (Hagerstown) 2019 09;17(3):E98-E99

Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York.

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http://dx.doi.org/10.1093/ons/opy374DOI Listing
September 2019

Novel aspiration catheter design for acute stroke thrombectomy.

J Neurointerv Surg 2019 Feb 30;11(2):190-195. Epub 2018 Jul 30.

Mount Sinai Health System, New York City, New York, USA.

Background: Navigable, large diameter aspiration catheters demonstrate markedly improved recanalization rates over smaller lumen devices in suction embolectomy. We evaluated the performance of a novel aspiration catheter system designed to maximize lumen size, and compare it to other commercially available aspiration catheters.

Methods: The 6F RQ aspiration catheter system comprises a proximal pusher wire of 117 cm length connected to a distal catheter of 25 cm length. When placed through standard guide catheters and into the cerebral circulation, the proximal catheter makes a tight seal between its outer surface and the guide catheter's inner surface. During aspiration, in vitro flow rates and tip suction force under gentle retraction were compared among 10 commercially available aspiration catheters and the RQ system.

Results: The RQ 6F, 5F, 4F, and 3F catheters achieved flow rates at least 21.9%, 24.7%, 61.9%, and 244.7% greater than the other catheters tested respectively and the RQ 6F produced a 140.2% higher tip force than a catheter of similar size. Fluid flow rate in the RQ 6F increased on retraction into the guide catheter, delivering a 58.2% increase from fully extended to fully retracted.

Conclusion: The RQ design demonstrates a substantial increase in aspirated flow rate and suction force due to an increased effective diameter than standard tubular catheter designs tested. The prominent increase in the aspiration parameters measured in vitro supports the potential for improved clinical results during stroke thrombectomy procedures.
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http://dx.doi.org/10.1136/neurintsurg-2017-013702DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6582808PMC
February 2019

Expanding the clinical and molecular findings in RASA1 capillary malformation-arteriovenous malformation.

Eur J Hum Genet 2018 10 11;26(10):1521-1536. Epub 2018 Jun 11.

ARUP Institute for Clinical and Experimental Pathology, Salt Lake City, UT, USA.

RASA1-related disorders are vascular malformation syndromes characterized by hereditary capillary malformations (CM) with or without arteriovenous malformations (AVM), arteriovenous fistulas (AVF), or Parkes Weber syndrome. The number of cases reported is relatively small; and while the main clinical features are CMs and AVMs/AVFs, the broader phenotypic spectrum caused by variants in the RASA1 gene is still being defined. Here, we report the clinical and molecular findings in 69 unrelated cases with a RASA1 variant identified at ARUP Laboratories. Sanger sequencing and multiplex ligation-dependent probe amplification were primarily used to evaluate RASA1. Several atypical cases were evaluated using next-generation sequencing (NGS) and array-comparative genomic hybridization (aCGH). Sixty individuals had a deleterious RASA1 variant of which 29 were novel. Nine individuals had a variant of uncertain significance. Five large RASA1 deletions were detected, giving an overall deletion/duplication rate of 8.3% (5/60) among positive cases. Most (75.4%) individuals with a RASA1 variant had CMs, and 44.9% had an AVM/AVF. Clinical findings in several cases expand the RASA1 phenotype. Our data suggest that screening for large RASA1 deletions and duplications in this disorder is important and suggest that NGS multi-gene panel testing is beneficial for the molecular diagnosis of cases with complex vascular phenotypes.
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http://dx.doi.org/10.1038/s41431-018-0196-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6138627PMC
October 2018

Vein of Galen Aneurysmal Malformation: Advances in Management and Endovascular treatment.

Neurosurgery 2019 02;84(2):469-478

Department of Neurosurgery, Icahn School of Medicine at Mount Sinai Hospital, New York, New York.

Background: Vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation representing <1% of all arteriovenous malformations. The knowledge and strategies in the management are constantly evolving.

Objective: To review our series of postneonatal VGAM patients treated over 11-yr period.

Methods: Retrospective analysis of 113 VGAM treated between January 2004 and April 2015. After exclusions, 45 patients were included: 33 choroidal and 12 mural types.

Results: Presenting symptom in the order of frequency: enlarged head circumference, antenatal diagnosis, mild CHF, and PHT at birth. Older patients were diagnosed following trauma, headache, cognitive decline, and incidentally during workup for other diseases. Hydrocephalus due to hydrodynamic disorder was present in 70% of choroidal and 58% of mural types. Only a quarter needed cerebrospinal fluid diversion procedure. Radiological cure was achieved in 82%; the outcome graded on a 5-point scale: 0 (death) to 4 (normal). A total of 66.6% are neurologically and developmentally intact with outcome score 4, 20% had outcome score of 3, and 8.9% had outcome score of 2. There was 4.4% mortality. Dural feeders to VGAM were found either in the initial or during the treatment in 22.2% in the current series. Angiogenesis from pial vessels developed after partial embolization in 17.7% that resolved completely following complete obliteration of VGAM.

Conclusion: Technical and technological advancements in endovascular embolization along with better understanding of clinical, anatomic, and pathophysiological aspects have resulted in significantly improved outcome and prognosis in VGAM. Most patients with proper treatment can now survive and most develop normally following appropriately timed treatment.
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http://dx.doi.org/10.1093/neuros/nyy100DOI Listing
February 2019

Bleomycin sclerotherapy for eyelid venous malformations as an alternative to surgery or laser therapy.

J Neurointerv Surg 2019 Jan 19;11(1):57-61. Epub 2018 Apr 19.

Department of Neurosurgery, Cerebrovascular Center, Mount Sinai Health System, New York City, New York, USA.

Purpose: To evaluate the clinical outcome of patients with venous malformation (VM) involving the eyelid treated with bleomycin sclerotherapy.

Methods: A retrospective review was performed of 18 consecutive patients with VM involving the eyelid who underwent bleomycin sclerotherapy. Patients' clinical presentation, details of sclerotherapy, and post-sclerotherapy resolution of the lesion as well as any procedure-related complications were evaluated.

Results: Twelve women and six men of mean age 34.3±20.4 years underwent sclerotherapy with bleomycin. Chief complaints were cosmetic disfigurations with or without hemifacial deformity (n=2), pain in engorgement area (n=2), pain and swelling from venous thrombosis (n=2), swelling or engorgement obstructing their eyesight (n=2), or eyelid dysfunction (n=1). The lesions were only in the eyelid in three patients; otherwise they were extended out of the eyelid either superiorly (n=3), laterally (n=8), inferiorly (n=8), and/or posteriorly to the orbit (n=8) to various extents. Conjunctival involvement was present in 13 patients. 14 patients had received prior treatments including surgery, laser therapy, or non-bleomycin sclerotherapy. With an average three sessions of bleomycin sclerotherapy (average total dose 34.5 mg), more than 80% shrinkage was observed in seven patients (38.9%), 50-80% shrinkage in eight patients (44.4%), and 30-50% shrinkage in two patients (11.1%). One patient had recurrence, which was successfully treated again with bleomycin. No procedure-related complications were noted.

Conclusions: The use of bleomycin appears to be a simple, safe, and effective treatment for venous malformations involving the eyelid, avoiding more elaborate and challenging surgical or laser interventions, and is even effective in full thickness lesions.
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http://dx.doi.org/10.1136/neurintsurg-2018-013813DOI Listing
January 2019

Human genetics and molecular mechanisms of vein of Galen malformation.

J Neurosurg Pediatr 2018 04 19;21(4):367-374. Epub 2018 Jan 19.

1Department of Neurosurgery.

Vein of Galen malformations (VOGMs) are rare developmental cerebrovascular lesions characterized by fistulas between the choroidal circulation and the median prosencephalic vein. Although the treatment of VOGMs has greatly benefited from advances in endovascular therapy, including technical innovation in interventional neuroradiology, many patients are recalcitrant to procedural intervention or lack accessibility to specialized care centers, highlighting the need for improved screening, diagnostics, and therapeutics. A fundamental obstacle to identifying novel targets is the limited understanding of VOGM molecular pathophysiology, including its human genetics, and the lack of an adequate VOGM animal model. Herein, the known human mutations associated with VOGMs are reviewed to provide a framework for future gene discovery. Gene mutations have been identified in 2 Mendelian syndromes of which VOGM is an infrequent but associated phenotype: capillary malformation-arteriovenous malformation syndrome ( RASA1) and hereditary hemorrhagic telangiectasia ( ENG and ACVRL1). However, these mutations probably represent only a small fraction of all VOGM cases. Traditional genetic approaches have been limited in their ability to identify additional causative genes for VOGM because kindreds are rare, limited in patient number, and/or seem to have sporadic inheritance patterns, attributable in part to incomplete penetrance and phenotypic variability. The authors hypothesize that the apparent sporadic occurrence of VOGM may frequently be attributable to de novo mutation or incomplete penetrance of rare transmitted variants. Collaboration among treating physicians, patients' families, and investigators using next-generation sequencing could lead to the discovery of novel genes for VOGM. This could improve the understanding of normal vascular biology, elucidate the pathogenesis of VOGM and possibly other more common arteriovenous malformation subtypes, and pave the way for advances in the diagnosis and treatment of patients with VOGM.
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http://dx.doi.org/10.3171/2017.9.PEDS17365DOI Listing
April 2018

Surgical technique for venous patch aneurysms with no neck in a rabbit model.

J Neurointerv Surg 2018 Feb 8;10(2):118-121. Epub 2017 Feb 8.

Research Institute of Neurointervention, Paracelsus Medical University, Salzburg, Austria.

Background: Animal experimental studies are crucial for the development of endovascular devices and embolization techniques for intracranial aneurysms. The aim of the study was to describe the surgical creation technique for an aneurysm with a dilated shape and no definable aneurysm neck. The model should be reproducible in size and shape and stay patent over a long period of time. It should constitute a challenge to endovascular therapy and provide a valuable testing environment for new endovascular devices and techniques.

Methods: Surgical creation of 30 no-neck aneurysms was attempted in 15 New Zealand White rabbits using a segment of jugular vein, which was transected, longitudinally opened and sutured to the anterior aspect of the common carotid artery. The first 14 aneurysms were used to develop the technique. Once the technique was mastered and the procedure was standardized, 16 consecutive aneurysms were created. For these aneurysms, digital subtraction angiography was employed after a mean of 120 days to confirm size and patency. All aneurysms were evaluated for patency.

Results: Overall aneurysm patency was 93%. Three complications occurred, two of which were related to the vascular anastomosis. Angiographic follow-up showed a median (SD) aneurysm base and height of 6.7 (0.76) mm and 3.2 (0.85) mm), respectively.

Conclusions: This technique allows creation of aneurysms without a neck which are reproducible in size and shape, and which remain patent. The model contributes to the list of aneurysm models fit for evaluation of embolization devices and techniques. It is particularly useful in exploring treatment options for wide-neck aneurysms and aneurysms which presently do not qualify for conventional coiling.
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http://dx.doi.org/10.1136/neurintsurg-2016-012955DOI Listing
February 2018

Dilated Superior Ophthalmic Vein: Clinical and Radiographic Features of 113 Cases.

Ophthalmic Plast Reconstr Surg 2018 Jan/Feb;34(1):68-73

Department of Ophthalmology, SUNY Downstate Medical Center, Brooklyn, New York.

Purpose: Dilated superior ophthalmic vein (SOV) is an uncommon radiographic finding. The authors review the presentation, etiology, radiography, and visual implications of 113 patients with dilated SOV.

Methods: An observational case series and multicenter retrospective chart review were conducted. There were 113 patients with a dilated SOV. Outcome measures included patient demographics, clinical features, radiographic findings, diagnosis, and treatment, and treatment outcomes were assessed.

Results: Cases included 75 women (66%) and 38 men (34%) with a mean age of 49 ± 24 years (range, 0.4-90 years). Diagnoses fell under 6 categories: vascular malformation (n = 92, 81%), venous thrombosis (n = 11, 10%), inflammatory (n = 6, 5%), traumatic hemorrhage (n = 2, 2%), lymphoproliferative (n = 1, 1%), and infectious (n = 1, 1%). Imaging modalities utilized included MRI (n = 98, 87%), digital subtraction angiography (n = 77, 68%), CT (n = 29, 26%), and ultrasonography (n = 4, 4%). Disease status at last follow up included no evidence of disease (n = 57, 50%), alive with persistent disease (n = 53, 47%), and expired from disease (n = 3, 3%). Treatment and management was tailored to the underlying disease process with a mean follow up of 18 months (range, 1 day to 180 months). Visual impairment observed at presentation and last follow up across all cases was 26% and 22%, respectively.

Conclusion: Dilated SOV is a rare radiographic finding resulting from a wide spectrum of etiologies with clinical implications ranging from benign to sight- and life-threatening. Dilated SOV is most often found with dural-cavernous fistula or carotid-cavernous fistula, orbital or facial arteriovenous malformation, and venous thrombosis. Recognition of this finding and management of the underlying condition is critical.
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http://dx.doi.org/10.1097/IOP.0000000000000872DOI Listing
February 2018

Retreatment of a choroidal vein of Galen malformation with embolization 42 years after open surgical treatment in the neonatal period.

J Neurointerv Surg 2017 May 8;9(5):e19. Epub 2016 Oct 8.

Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York, USA.

In 1976 an infant boy initially presented with a choroidal vein of Galen malformation (cVOGM). Craniotomy and clipping of the anterior cerebral artery and posterior choroidal arteries was performed, which was reported in the literature 40 years ago. The patient remained asymptomatic until age 42 when he re-presented with an isolated intraventricular hemorrhage. Angiography demonstrated cVOGM with venous sinus occlusive disease leading to venous hypertension and subsequent intraventricular hemorrhage. The angiogram also demonstrated a 'pseudo-nidus' composed of multiple arterial-to-arterial anastomoses that had developed as a result of the original surgical treatment. We embolized a portion of the lesion to reduce the venous hypertension. The patient has been asymptomatic for more than 2 years. To our knowledge, this is the first reported case of a symptomatic neonatal VOGM treated with open surgery that required embolization as an adult for a delayed hemorrhagic presentation.
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http://dx.doi.org/10.1136/neurintsurg-2016-012709.repDOI Listing
May 2017

Retreatment of a choroidal vein of Galen malformation with embolization 42 years after open surgical treatment in the neonatal period.

BMJ Case Rep 2016 Oct 4;2016. Epub 2016 Oct 4.

Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, New York, USA.

In 1976 an infant boy initially presented with a choroidal vein of Galen malformation (cVOGM). Craniotomy and clipping of the anterior cerebral artery and posterior choroidal arteries was performed, which was reported in the literature 40 years ago. The patient remained asymptomatic until age 42 when he re-presented with an isolated intraventricular hemorrhage. Angiography demonstrated cVOGM with venous sinus occlusive disease leading to venous hypertension and subsequent intraventricular hemorrhage. The angiogram also demonstrated a 'pseudo-nidus' composed of multiple arterial-to-arterial anastomoses that had developed as a result of the original surgical treatment. We embolized a portion of the lesion to reduce the venous hypertension. The patient has been asymptomatic for more than 2 years. To our knowledge, this is the first reported case of a symptomatic neonatal VOGM treated with open surgery that required embolization as an adult for a delayed hemorrhagic presentation.
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http://dx.doi.org/10.1136/bcr-2016-012709DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5073684PMC
October 2016

Pediatric neurointervention: collimation on radiation exposure-associated lifetime excess tumor risk.

J Neurointerv Surg 2017 Sep 5;9(9):895-898. Epub 2016 Sep 5.

Department of Neurosurgery, Cerebrovascular Center, Mount Sinai Health System, New York, New York, USA.

Background: Intracranial vascular malformations in children are being addressed through a variety of treatment modalities including open surgery, external beam radiation, and image-guided neuroendovascular procedures. These patients often receive multiple treatments and incur serial exposures to ionizing radiation which has been linked to tumor development in population-based data.

Objective: This study quantifies the effect of collimation on exposures from single procedures and over patient lifetimes to estimate excess risk of lifetime tumor development.

Methods: 215 patients aged 0-21 years from a single center took part in the study. Radiation exposure from neuroendovascular procedures was tabulated and converted to brain doses using modeled data and extrapolated to risk ratios using results of population-based estimates found in the literature.

Results: Lifetime and per procedure risk was highest in patients with brain arteriovenous malformations, brain arteriovenous fistulas, and vein of Galen malformations, a reflection of our institutional referral patterns. Across all pathologies the per procedure excess relative risk decreased from 13.4 to 2.3 when full collimation was employed. Lifetime excess relative risk decreased from 49.0 to 7.7 for full collimation.

Conclusions: This is the first study to quantify the effect of collimation on lifetime and per procedure risk of tumor development in a pediatric population. In addition to collimation, technical and operator-based aspects of the neurointerventional suite are discussed to further reduce patient exposure without sacrificing image quality.
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http://dx.doi.org/10.1136/neurintsurg-2016-012477DOI Listing
September 2017

Vascular Disorders of the Cerebellum in Children.

Neuroimaging Clin N Am 2016 Aug;26(3):435-58

Department of Radiology, Mount Sinai Medical Center, Box 1234, One Gustave Levy Place, New York, NY 10029, USA; Department of Neurosurgery, Mount Sinai Medical Center, One Gustave Levy Place, New York, NY 10029, USA; Department of Pediatrics, Mount Sinai Medical Center, One Gustave Levy Place, New York, NY 10029, USA.

Key differences exist in the epidemiology, pathophysiology, and clinical presentation of vascular lesions of the cerebellum in children versus adults. An understanding of these differences and an appreciation of the distinct imaging features of these lesions aid in distinguishing normal vascular variations from pathology, in predicting lesion etiology, and in directing effective treatment strategies. This paper reviews the embryogenesis of the normal vascular system of the cerebellum and brainstem and then discusses the clinical and imaging features of the common vascular lesions affecting these structures in the pediatric population.
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http://dx.doi.org/10.1016/j.nic.2016.03.009DOI Listing
August 2016

Oral Surgical Procedures Performed Safely in Patients With Head and Neck Arteriovenous Malformations: A Retrospective Case Series of 12 Patients.

J Oral Maxillofac Surg 2016 Feb 23;74(2):255.e1-8. Epub 2015 Oct 23.

Site Chair, Institute for Neurology and Neurosurgery, Department of Interventional Neuroradiology, Mount Sinai St Luke's-Roosevelt Hospital, New York, NY.

Purpose: This case series describes patients with head and neck arteriovenous malformations who underwent oral and maxillofacial surgical procedures combined with interventional radiology techniques to minimize blood loss.

Materials And Methods: Twelve patients underwent femoral cerebral angiography to visualize the extent of vascular malformation. Before the surgical procedures, surgical sites were devascularized by direct injection of hemostatic or embolic agents. Direct puncture sclerotherapy at the base of surgical sites was performed using Surgiflo or n-butylcyanoacrylate glue. Surgical procedures were carried out in routine fashion. A hemostatic packing of FloSeal, Gelfoam, and Avitene was adapted to the surgical sites.

Results: Direct puncture sclerotherapy with Surgiflo or n-butylcyanoacrylate glue resulted in minimal blood loss intraoperatively. Local application of the FloSeal, Gelfoam, and Avitene packing sustained hemostasis and produced excellent healing postoperatively.

Conclusion: Patients with arteriovenous malformations can safely undergo routine oral and maxillofacial surgical procedures with minimal blood loss when appropriate endovascular techniques and local hemostatic measures are used by the interventional radiologist and oral and maxillofacial surgeon.
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http://dx.doi.org/10.1016/j.joms.2015.10.012DOI Listing
February 2016

Treatment of experimental aneurysms with a new liquid embolic agent and a retrievable stent: proof of concept and feasibility study.

J Neurointerv Surg 2016 Sep 26;8(9):934-9. Epub 2015 Aug 26.

Background: Occlusion of canine bifurcation and sidewall aneurysms was undertaken with a new liquid embolic agent (PHIL 35) assisted by a high-density partially retrievable stent (FRED) with preservation of the carotid artery.

Methods: Three dogs were used as acute preparations for development of the technique and two were used for chronic studies lasting 90 days. In one animal we intentionally did not completely fill the aneurysm to determine the long-term results of incomplete treatment. The degree of occlusion, carotid artery compromise, and dislodgement and/or migration of embolic material in treated aneurysms were assessed.

Results: All aneurysms planned for complete obliteration were totally occluded successfully. By design, we partially occluded one aneurysm. In this aneurysm, angiography performed at 30 days revealed less filling, but at 90 days it had persistent small residual filling. We did not detect any distal embolization during the injection and no angiographic occlusions, change in configuration, or delayed migration of the embolic material were found. In the inspected stent, no foreign material was noted. In four animals we successfully removed the stent with preservation of the integrity of the carotid artery. In the fifth we intentionally left both stents deployed.

Conclusions: We have developed a new treatment for cerebral aneurysms using a combination of a retrievable stent and a new liquid embolic agent.
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http://dx.doi.org/10.1136/neurintsurg-2015-011930DOI Listing
September 2016

Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies.

Pediatrics 2015 Jul 8;136(1):e203-14. Epub 2015 Jun 8.

Laboratory of Human Molecular Genetics, Christian de Duve Institute of Cellular Pathology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.

Vascular anomalies represent a spectrum of disorders from a simple "birthmark" to life- threatening entities. Incorrect nomenclature and misdiagnoses are commonly experienced by patients with these anomalies. Accurate diagnosis is crucial for appropriate evaluation and management, often requiring multidisciplinary specialists. Classification schemes provide a consistent terminology and serve as a guide for pathologists, clinicians, and researchers. One of the goals of the International Society for the Study of Vascular Anomalies (ISSVA) is to achieve a uniform classification. The last classification (1997) stratified vascular lesions into vascular malformations and proliferative vascular lesions (tumors). However, additional disease entities have since been identified that are complex and less easily classified by generic headings, such as capillary malformation, venous malformation, lymphatic malformation, etc. We hereby present the updated official ISSVA classification of vascular anomalies. The general biological scheme of the classification is retained. The section on tumors has been expanded and lists the main recognized vascular tumors, classified as benign, locally aggressive or borderline, and malignant. A list of well-defined diseases is included under each generic heading in the "Simple Vascular Malformations" section. A short definition is added for eponyms. Two new sections were created: one dealing with the malformations of individually named vessels (previously referred to as "truncular" malformations); the second groups lesions of uncertain or debated nature (tumor versus malformation). The known genetic defects underlying vascular anomalies are included in an appendix. This classification is meant to be a framework, acknowledging that it will require modification as new scientific information becomes available.
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http://dx.doi.org/10.1542/peds.2014-3673DOI Listing
July 2015

High-flow carotid cavernous fistula and the use of a microvascular plug system: initial experience.

Interv Neurol 2015 Mar;3(2):78-84

Hyman-Newman Institute of Neurology and Neurosurgery, Mount Sinai Hospital, New York, N.Y., USA.

Purpose: We report our initial experience using a detachable microvascular plug system to occlude the internal carotid artery during endovascular treatment of high-flow carotid cavernous fistula.

Case And Technique: An 87-year-old patient was admitted for acute-onset double vision with associated right-eye ptosis. Exam revealed a pupil-sparing, partial right third cranial nerve palsy. MRI showed a carotid cavernous fistula with high-flow drainage. Digital subtraction angiography showed a high-flow, right-sided, direct carotid cavernous fistula with flow from the proximal right internal carotid artery. The ophthalmic artery, posterior communicating artery and anterior communicating arteries supplied retrograde flow to the fistula through the internal carotid artery. Obliteration of the fistula was achieved through coil embolization in combination with proximal and distal microvascular plugs (Reverse Medical, Irvine, Calif., USA).

Conclusion: The microvascular plug is a new addition to current endovascular embolization devices for the treatment of high-flow, direct carotid cavernous fistulas. This technique offers easy navigability through tortuous arteries, precise localization and immediate occlusion, which may allow shorter procedure and fluoroscopy times and increased cost-effectiveness. Larger case series are needed to support our observation.
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http://dx.doi.org/10.1159/000369477DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4439797PMC
March 2015

Endoscopic transmucosal direct puncture sclerotherapy for management of airway vascular malformations.

Laryngoscope 2016 Jan 13;126(1):205-11. Epub 2015 May 13.

Vascular Birthmark Institute of New York, Department of Otolaryngology (m.w., t.o.), Lenox Hill and Manhattan Eye, Ear, and Throat Hospitals, New York, New York, U.S.A.

Objectives/hypothesis: To describe a multidisciplinary approach to the treatment of airway vascular malformations (venous or lymphatic) with direct suspension rigid laryngoscopy and direct puncture transmucosal bleomycin sclerotherapy injected under road-mapping fluoroscopic monitoring, supplemented by Dyna-computed tomography utilization.

Study Design: Case series.

Methods: We performed a retrospective medical record and imaging review of four patients with venous malformations or lymphatic malformations located in the airway. Patients were treated with a combination of direct suspension laryngoscopy or rigid nasopharyngoscopy and image-guided direct puncture bleomycin sclerotherapy.

Results: Two patients presented to our institution with extensive lymphatic malformation of the neck, parapharyngeal, and retropharyngeal spaces, and two presented with venous malformation of the nasopharynx and oropharynx. All patients were treated with multiple sclerotherapy and debulking procedures before undergoing combined direct transmucosal puncture bleomycin sclerotherapy guided by direct laryngoscopy or nasopharyngoscopy. All patients had complete resolution of disease while maintaining a safe airway.

Conclusions: A multidisciplinary approach to airway vascular malformations with a combination of endoscopy and direct puncture bleomycin sclerotherapy was demonstrated to be a safe and effective treatment in our patient cohort. Direct laryngoscopy and nasopharyngoscopy provide easy access to the nasopharynx, oropharynx, retro- and/or parapharyngeal spaces and larynx. Unlike traditional agents, bleomycin induces minimal edema and therefore is an ideal substance to treat airway lesions.
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http://dx.doi.org/10.1002/lary.25284DOI Listing
January 2016

Ruptured distal AICA pseudoaneurysm presenting years after vestibular schwannoma resection and radiation.

J Neurointerv Surg 2016 May 11;8(5):e19. Epub 2015 May 11.

Department of Neurosurgery, Mount Sinai Health System, New York, New York, USA.

Distal anterior inferior cerebellar artery (AICA) pseudoaneurysms are very rare lesions. Although cases have been previously reported, only a few have been reported as a result of vestibular schwannoma (VS) radiation, none have been reported as a result of VS resection, and only one has been reported as treated with parent vessel occlusion (PVO) with n-butyl cyanoacrylate (nBCA). We report a case of a 65-year-old man with a history of right-sided VS surgery and radiation who presented years later with a ruptured pseudoaneurysm of the distal right AICA and was treated with endovascular PVO using nBCA. The aneurysm was completely obliterated and the patient had no worsening of symptoms or neurological exam. The case illustrates a very rare complication of VS surgery and radiation as well as an effective treatment for distal AICA aneurysms.
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http://dx.doi.org/10.1136/neurintsurg-2015-011736.repDOI Listing
May 2016

Ruptured distal AICA pseudoaneurysm presenting years after vestibular schwannoma resection and radiation.

BMJ Case Rep 2015 May 6;2015. Epub 2015 May 6.

Department of Neurosurgery, Mount Sinai Health System, New York, New York, USA.

Distal anterior inferior cerebellar artery (AICA) pseudoaneurysms are very rare lesions. Although cases have been previously reported, only a few have been reported as a result of vestibular schwannoma (VS) radiation, none have been reported as a result of VS resection, and only one has been reported as treated with parent vessel occlusion (PVO) with n-butyl cyanoacrylate (nBCA). We report a case of a 65-year-old man with a history of right-sided VS surgery and radiation who presented years later with a ruptured pseudoaneurysm of the distal right AICA and was treated with endovascular PVO using nBCA. The aneurysm was completely obliterated and the patient had no worsening of symptoms or neurological exam. The case illustrates a very rare complication of VS surgery and radiation as well as an effective treatment for distal AICA aneurysms.
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http://dx.doi.org/10.1136/bcr-2015-011736DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4434381PMC
May 2015

Dural arteriovenous shunt development in patients with vein of galen malformation.

Interv Neuroradiol 2014 Dec 5;20(6):781-90. Epub 2014 Dec 5.

Hyman Newman Institute for Neurology and Neurosurgery, Centre for Endovascular Surgery, St. Luke's Roosevelt Hospital; New York, NY, USA.

Dural arteriovenous fistulas (DAVF) associated with our series of patients with vein of Galen malformations (VOGM) are analyzed and discussed. We retrospectively analyzed 87 consecutive cases of VOGM treated between May 2002 and December 2011 and identified 26 patients with DAVF. We gathered information from the clinical case records, angiographic images, MRI on presentation and during follow-up. The findings were analyzed to aid discussion. Among 87 patients treated by multi-stage endovascular embolization, age range from newborn to 19 years, 26(30%) had DAVF. In seven patients (8%), DAVF were found on initial angiogram and were all into the VOGM. Nineteen (21%) DAVF found on follow-up angiograms were all into the VOGM and distant locations. Sprouting and non-sprouting angiogenesis resulted in the formation of a network of vessels around partially thrombosed VOGM, recruiting blood from the surrounding dura mater resulting in a secondary network on the dura mater supplied by the blood vessels of dura mater in the region or from its natural collaterals. Embolization targeting DAVFs was done in 13 (52%) with complete cure in eight (32%) and recurrence in five (20%). Among 12 non-embolized patients (48%), eight (32%) had spontaneous regression with continued treatment of VOGM. In others, the DAVF either remained stable or progressed. DAVF associated with VOGM represent the dural response to angiogenic stimuli. They are observed to regress spontaneously or mature while continuing to treat the primary feeders of VOGM. It is important to include the external carotid system during angiograms. Persistent DAVF with residual VOGM that do not have access though the pial vessels are used as a conduit to treat the dural shunt and to achieve obliteration of residual VOGM at later stages of treatment.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4295253PMC
http://dx.doi.org/10.15274/INR-2014-10066DOI Listing
December 2014

Neurocognitive improvements following endovascular repair of vein of Galen malformation in a child.

J Neurosurg Pediatr 2015 Feb 5;15(2):197-202. Epub 2014 Dec 5.

Departments of 1 Neuropsychology.

Cognitive regression is a well-described presentation of vein of Galen aneurysmal malformations (VGAMs) in childhood. However, it remains unclear whether successful treatment of the malformation can reverse cognitive regression. Here, the authors present the case of a 5-year-old girl with a VGAM that was treated with staged endovascular embolization procedures. Comprehensive neurocognitive assessments were completed before intervention and approximately 6 years after initial presentation. There were significant age-matched improvements in this child's neurocognitive profile over this period. The authors believe that timely and successful treatment of VGAM in children may not only stabilize the associated cognitive deterioration but, in some cases, may ameliorate these deficits. Details of this case and a discussion of neurocognitive deficits related to VGAM are presented.
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http://dx.doi.org/10.3171/2014.10.PEDS14244DOI Listing
February 2015

N-butyl cyanoacrylate embolization using a detachable tip microcatheter: initial experience.

J Neurointerv Surg 2015 Jun 8;7(6):458-61. Epub 2014 Apr 8.

Hyman Newman Institute for Neurology and Neurosurgery, Centre for Endovascular Surgery, Roosevelt Hospital, Mount Sinai Health Stystem, New York, New York, USA.

Introduction: Endovascular embolization of intracranial vascular malformations with N-butyl cyanoacrylate (nBCA) using a detachable tip microcatheter allows prolonged injection and decreases the risk of catheter retention.

Methods: Between March and December 2013, the Apollo 1.5 cm detachable tip microcatheter was used in five patients after being approved by both the Food and Drug Administration and the institutional review board as a compassionate use device. Nine pedicles were embolized and the follow-up ranged from 1 to 3 months.

Results: Five of the nine catheter tips detached. The length of reflux was not directly associated with the detachment of the distal tip. There were no cases of premature microcatheter detachment during navigation, manipulation with multiple microguidewire reintroduction and guidance. There was no leak of embolic agent at the detachment zone. Follow-up showed the detached tip to be stable without migration.

Conclusions: A detachable tip microcatheter offers an advance in the safety and effectiveness of nBCA embolization. Catheter retrieval becomes more controlled and less traumatic. Our initial experience is encouraging, and more experience is needed to categorically ascertain its safety and efficacy.
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http://dx.doi.org/10.1136/neurintsurg-2014-011165DOI Listing
June 2015

Safety and efficacy using a detachable tip microcatheter in the embolization of pediatric arteriovenous malformations.

Childs Nerv Syst 2014 Jun 27;30(6):1099-107. Epub 2014 Mar 27.

Center for Endovascular Surgery, Hyman-Newman Institute for Neurology and Neurosurgery, Suite 10G, Mount Sinai-Roosevelt Hospital, 1000 Tenth Ave., New York, NY, 10019, USA,

Purpose: The treatment of deep-seeded pediatric brain arteriovenous malformations (AVMs) remains a challenging task. We describe our experience using a new detachable tip microcatheter in the embolization of brain arteriovenous malformations, pial arteriovenous fistulas, and vein of Galen malformations. We describe the safety and efficacy using a new detachable tip microcatheter in the treatment of pediatric deep brain arteriovenous malformations, pial malformations, and vein of Galen malformations.

Methods: During a period of 9 months from March 2013 through January 2014, 11 pediatric patients in 14 procedures with 27 total injections were selected for treatment with a detachable tip under Food and Drug Administration (FDA) compassionate use exemption and were admitted to our department for treatment of their brain AVM using a liquid embolic agent and a detachable tip microcatheter. The ages of the patients ranged from 3 months to 18 years old.

Results: Of the 27 total injections done, the tip detached in seven cases. For the 16 n-BCA injections, the tip detached six times (37.5 %), and for the 11 Onyx injections, the tip detached one time (9 %). There were no cases of premature microcatheter detachment during normal vessel navigation.

Conclusions: The introduction of these detachable tip microcatheters allows for a safe and relaxed injection that permits a true circumferential occlusion, and may further permit filling a larger amount of angioarchitecture without the risk of distal migration, or vessel damage during the usual rapid removal of non detachable micocatheters.
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http://dx.doi.org/10.1007/s00381-014-2404-9DOI Listing
June 2014

Advances and innovations in vascular neurosurgery: Foreword: Part II.

Neurosurgery 2014 Feb;74 Suppl 1:S3-4

Mount Sinai Hospital, New York, New York.

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http://dx.doi.org/10.1227/NEU.0000000000000246DOI Listing
February 2014