Transuterine Ultrasound-Guided Fetal Embolization of Vein of Galen Malformation, Eliminating Postnatal Pathophysiology.

HomeStrokeVol. 54, No. 6Transuterine Ultrasound-Guided Fetal Embolization of Vein of Galen Malformation, Eliminating Postnatal Pathophysiology Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessResearch ArticlePDF/EPUBTransuterine Ultrasound-Guided Fetal Embolization of Vein of Galen Malformation, Eliminating Postnatal Pathophysiology Darren B. Orbach, Louise E. Wilkins-Haug, Carol B. Benson, Wayne Tworetzky, Shivani D. Rangwala, Stephanie H. Guseh, Nicole K. Gately, Jeffrey N. Stout, Arielle Mizrahi-Arnaud and Alfred P. See Darren B. OrbachDarren B. Orbach Correspondence to: Darren B. Orbach, MD, PhD, Neurointerventional Radiology, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. Email E-mail Address: [email protected] https://orcid.org/0000-0002-1793-3398 Departments of Neurointerventional Radiology (D.B.O.), Boston Children’s Hospital, MA. Cerebrovascular Surgery and Interventions Center (D.B.O., A.P.S., J.N.S), Boston Children’s Hospital, MA. Search for more papers by this author , Louise E. Wilkins-HaugLouise E. Wilkins-Haug Departments of Maternal Fetal Medicine (L.E.W.-H., S.H.G.), Brigham and Women’s Hospital, Boston, MA. Search for more papers by this author , Carol B. BensonCarol B. Benson https://orcid.org/0000-0003-2639-2703 Radiology (C.B.B.), Brigham and Women’s Hospital, Boston, MA. Search for more papers by this author , Wayne TworetzkyWayne Tworetzky https://orcid.org/0000-0002-8422-2158 Cardiology (W.T.), Boston Children’s Hospital, MA. Search for more papers by this author , Shivani D. RangwalaShivani D. Rangwala Neurosurgery (A.P.S., S.D.R.), Boston Children’s Hospital, MA. Search for more papers by this author , Stephanie H. GusehStephanie H. Guseh Departments of Maternal Fetal Medicine (L.E.W.-H., S.H.G.), Brigham and Women’s Hospital, Boston, MA. Search for more papers by this author , Nicole K. GatelyNicole K. Gately https://orcid.org/0009-0000-1917-7867 Maternal Fetal Care Center (N.K.G.), Boston Children’s Hospital, MA. Search for more papers by this author , Jeffrey N. StoutJeffrey N. Stout https://orcid.org/0000-0002-7342-3018 Cerebrovascular Surgery and Interventions Center (D.B.O., A.P.S., J.N.S), Boston Children’s Hospital, MA. Search for more papers by this author , Arielle Mizrahi-ArnaudArielle Mizrahi-Arnaud Anesthesiology (A.M.A.), Boston Children’s Hospital, MA. Search for more papers by this author and Alfred P. SeeAlfred P. See https://orcid.org/0000-0003-3634-4692 Neurosurgery (A.P.S., S.D.R.), Boston Children’s Hospital, MA. Cerebrovascular Surgery and Interventions Center (D.B.O., A.P.S., J.N.S), Boston Children’s Hospital, MA. Search for more papers by this author Originally published4 May 2023https://doi.org/10.1161/STROKEAHA.123.043421Stroke. 2023;54:e231–e232Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: May 4, 2023: Ahead of Print Despite decades of technique refinement of transarterial embolization and the establishment of specialty referral centers, fetuses diagnosed with vein of Galen malformation continue to have high mortality, with survivors facing high rates of severe neurological and cognitive morbidity.1 Low resistance in the vein of Galen malformation arteriovenous shunt induces high-flow physiology that compromises cerebral perfusion and induces cardiopulmonary stress. In utero, the placental circulation is also low resistance, providing compensation and fetal protection. Thus, most infants suffer decompensation postnatally rather than in fetal life, with exacerbation upon ductus arteriosus closure. Moreover, neonatal embolization itself carries significant risk of iatrogenic cerebral injury, even at the hands of the most experienced practitioners.We have hypothesized that fetal intervention, before acute postnatal cardiovascular and cerebrovascular stress ensues, may decrease mortality and morbidity. Fetuses at great risk of acute decompensation after birth can now be identified with high reliability based on fetal magnetic resonance imaging morphological criteria,2 thereby defining an appropriate cohort for a novel in utero intervention. We present here the first embolized case in a ClinicalTrials.gov-registered study (URL: https://www.clinicaltrials.gov; Unique identifier: NCT04434729), performed with US Food and Drug Administration oversight, applying an institutional review board-approved study protocol3 of ultrasound-guided percutaneous transuterine, transcranial embolization.MethodsThe fetus was 34 2/7 weeks gestational age at intervention and was enrolled per study criteria.3 The mediolateral width of the fetal falcine sinus was associated with 99% likelihood of neonatal decompensation.2 Additional maternal and fetal data and procedural details are described in the Supplemental Methods.ResultsImmediate marked flow reduction in the varix and falcine sinus were seen on ultrasound intraprocedurally (Figure [A] through [C]). After embolization, 43% reduction in total cardiac output was seen on fetal echocardiography. Diminution in caliber of the prosencephalic varix and width of the falcine sinus (12.9 → 8 mm) was seen on fetal magnetic resonance imaging, with no evidence of subdural, subarachnoid, or intraventricular hemorrhage or infarction (Figure [D] through [F]). Detailed results are presented in the Supplemental Results.Download figureDownload PowerPointFigure. Intraprocedural ultrasound and pre- and post-procedural magnetic resonance imaging (MRI). A, Color Doppler ultrasound preceding fetal embolization shows avid flow within the prosencephalic varix (red arrow) and the falcine sinus (green arrow). B, Microwire advancing within the varix (blue arrow) before coil deployment. C, After embolization, Doppler signal is absent from within the falcine sinus (yellow arrow) and the varix, replaced by echogenic signal with posterior acoustic reverberations from the coil mass (purple arrow). D, Axial T2-weighted fetal preembolization MRI image, (E) fetal postembolization image, and (F) postnatal day 1 image show progressive diminution in varix size.Due to premature rupture of membranes, the infant was delivered by induction of vaginal birth at 34 4/7 weeks. Birth weight was 1.9 kg. The 3-week-old has required no cardiovascular support and no postnatal embolization. Neurological exam is normal. Magnetic resonance imaging on postnatal days 1 and 7 demonstrated further decrement in varix and falcine sinus caliber (Figure [F]).DiscussionWe report a novel ultrasound-guided transuterine, fetal cerebral embolization of vein of Galen malformation, resulting in complete elimination of the expected aggressive postnatal natural history. This approach represents a paradigm shift in management of this challenging condition, from a strategy focused on reversing severe multiorgan pathophysiology after onset, to one focused instead on prevention via embolization in utero.Article InformationSources of FundingThis article was funded by Sage Schermerhorn Chair for Image-Guided Therapy (D.B.O.).Supplemental MaterialSupplemental MethodsSupplemental ResultsDisclosures Dr See is a consultant for Microbot Medical, Inc.FootnotesThe podcast and transcript are available at https://www.ahajournals.org/str/podcast.Supplemental Material is available at https://www.ahajournals.org/doi/suppl/10.1161/STROKEAHA.123.043421.For Sources of Funding and Disclosures, see page e232.Correspondence to: Darren B. Orbach, MD, PhD, Neurointerventional Radiology, Boston Children’s Hospital, 300 Longwood Ave, Boston, MA 02115. Email darren.orbach@childrens.harvard.eduReferences1. D’Amico A, Tinari S, D’Antonio F, Rizzo G, Liberati M, Vasciaveo L, Buca D. Outcome of fetal Vein Galen aneurysmal malformations: a systematic review and meta-analysis.J Matern Fetal Neonatal Med. 2022; 35:5312–5317. doi: 10.1080/14767058.2021.1878494CrossrefGoogle Scholar2. Arko L, Lambrych M, Montaser A, Zurakowski D, Orbach DB. Fetal and neonatal MRI predictors of aggressive early clinical course in vein of Galen malformation.AJNR Am J Neuroradiol. 2020; 41:1105–1111. doi: 10.3174/ajnr.A6585CrossrefGoogle Scholar3. See AP, Wilkins-Haug LE, Benson CB, Tworetzky W, Orbach DB. Percutaneous transuterine fetal cerebral embolisation to treat vein of Galen malformations at risk of urgent neonatal decompensation: study protocol for a clinical trial of safety and feasibility.BMJ Open. 2022; 12:e058147. doi: 10.1136/bmjopen-2021-058147CrossrefGoogle Scholar eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. 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Authors of the article cited in the comment will be invited to reply, as appropriate.Comments and feedback on AHA/ASA Scientific Statements and Guidelines should be directed to the AHA/ASA Manuscript Oversight Committee via its Correspondence page.Sign In to Submit a Response to This Article Previous Back to top Next FiguresReferencesRelatedDetails June 2023Vol 54, Issue 6 Advertisement Article InformationMetrics © 2023 American Heart Association, Inc.https://doi.org/10.1161/STROKEAHA.123.043421PMID: 37139817 Originally publishedMay 4, 2023 Keywordsstroke in childrenembolizationheart failurevein of Galen malformationfetal surgeryPDF download Advertisement SubjectsCerebrovascular MalformationsHemodynamicsPathophysiologyTreatment