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Vein of Galen arteriovenous malformation mimicking coarctation of the aorta

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Abstract

Arteriovenous malformation of the vein of Galen is a rare congenital intracranial anomaly lacking a capillary bed and subsequent aneurysmal enlargement of the arterial and venous system, warranting careful management due to associated morbidity and mortality. Coarctations of aorta demonstrate similar neonatal echocardiographic signs to the vein of Galen arterial malformation (VGAM). We present a boy at 37 weeks of gestation whose initial ultrasound and echocardiographic investigations showed a dominant right ventricle and isthmal hypoplasia, suggestive of coarctation of aorta. Follow-up ultrasound and echocardiography revealed an arteriovenous malformation involving middle and posterior cerebral artery branches, eliminating coarctation of aorta. VGAM was confirmed by further ultrasound and angiographic investigation, which demonstrated a tangle of cerebral and choroidal arterial branches centrally feeding into an enlarged vein of Galen. The boy’s hemodynamic and neurological statuses were confirmed to be stable despite increased venous pressure. Elective embolization at 7 months of age was complicated by a cerebrovascular accident, resulting in right hemiparesis despite no residual cardiac issues. This case demonstrates that rarely, arteriovenous malformations such as the vein of Galen malformations may be the primary cause of patients presenting with coarctation of aorta. The rarity of this condition and its guarded prognosis make our case of special interest to cardiologists and the perinatal care team.

Riassunto

Le malformazioni artero-venose della vena di Galeno sono rare anomalie intracraniche congenite caratterizzate da assenza del letto capillare e successiva dilatazione aneurismatica dei sistemi arterioso e venoso, che richiedono un’attenta gestione, per le associate morbilità e mortalità. La coartazioni dell’aorta presenta segni ecocardiografici neonatali simili alla malformazione artero-venosa della vena di Galeno (VGAM). Presentiamo il caso di un ragazzo che, alla 37 settimana di gestazione, mostrava all’esame ecocardiografico un ventricolo destro dominante e un’ipoplasia dell’istmo, suggestivi per coartazione dell'aorta. Il follow-up ecografico ed ecocardiografico hanno rivelato una malformazione artero-venosa che coinvolgeva rami delle arterie cerebrale media e posteriore, eliminando l’ipotesi di coartazione dell'aorta. La diagnosi di VGAM è stata confermata da ulteriori indagini ecografiche e angiografiche, che hanno dimostrato un groviglio di rami arteriosi cerebrali e dei plessi corioidei alimentato da una vena di Galeno ingrandita. I parametri emodinamici e neurologici del ragazzo erano stabili nonostante l'aumento della pressione venosa. A 7 mesi di età l’embolizzazione elettiva è stata complicato da un incidente cerebrovascolare, con conseguente emiparesi destra, nonostante non vi siano state complicanza cardiache. Questo caso dimostra che rare malformazioni arterovenose, come le malformazioni della vena di Galeno, possono essere la causa primaria di pazienti che si presentano inizialmente con la diagnosi di coartazione dell'aorta. La rarità di questa condizione e la sua prognosi severa rendono il nostro caso di particolare interesse per i cardiologi e per i team di assistenza perinatale.

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References

  1. Ciricillo SF, Edwards MS, Schmidt KG, Hieshima GB, Silverman NH, Higashida RT et al (1990) Interventional neuroradiological management of vein of Galen malformations in the neonate. Neurosurgery 27:22–28

    Article  PubMed  CAS  Google Scholar 

  2. Gupta AK, Varma DR (2004) Vein of Galen malformations: a review. Neurol India 52:43–53

    PubMed  CAS  Google Scholar 

  3. Casasco A, Lylyk P, Hodes JE, Kohan G, Aymard A, Merland JJ (1991) Percutaneous transvenous catheterization and embolization of vein of Galen aneurysms. Neurosurgery 28:260–266

    Article  PubMed  CAS  Google Scholar 

  4. de Koning TJ, Gooskens R, Veenhoven R, Meijboom EJ, Jansen GH, Lasjaunias P et al (1997) Arteriovenous malformation of the vein of Galen in three neonates: emphasis on associated early ischemic brain damage. Eur J Pediatr 156:228–229

    Article  PubMed  Google Scholar 

  5. Rao PS (2005) Coarctation of the aorta. Curr Cardiol Rep 7(6):425–434

    Article  PubMed  Google Scholar 

  6. Schievink WI, Mokri B, Piepgras DG, Gittenberger-de Groot AC (1996) Intracranial aneurysms and cervicocephalic arterial dissections associated with congenital heart disease. Neurosurgery 39:685–689

    Article  PubMed  CAS  Google Scholar 

  7. McElhinney DB, Halbach VV, Silverman NH, Dowd CF, Hanley FL (1998) Congenital cardiac anomalies with vein of Galen malformations in infants. Arch Dis Child 78:548–551

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  8. Tomlinson FH, Piepgras DG, Nichols DA, Rüfenacht DA, Kaste SC (1992) Remote congenital cerebral arteriovenous fistulae associated with aortic coarctation. J Neurosurg 76:137–142

    Article  PubMed  CAS  Google Scholar 

  9. Srinivas L, Changela V, Francis E, Kumar RK (2008) Vein of Galen arteriovenous malformation: diagnostic clues from echocardiography. Indian Heart J 60:349–351

    PubMed  Google Scholar 

  10. Harahsheh AS, Kulkarni A, Becker C, Ross RD (2007) Conditions mimicking coarctation of the aorta. Pediatr Cardiol 28:385–388

    Article  PubMed  CAS  Google Scholar 

  11. Doyle NM, Mastrobattista JM, Thapar MK, Lantin-Hermoso MR (2005) Perinatal pseudocoarctation: echocardiographic findings in vein of Galen malformation. J Ultrasound Med 24:93–98

    PubMed  Google Scholar 

  12. Hamdan MA (2006) Coarctation of the aorta: a comprehensive review. J Arab Neonatal Forum 3:5–13

    Google Scholar 

  13. (2012) In: Moller JH, Julien IE, Hoffman (eds) Pediatric cardiovascular medicine, 1st edn. Wiley, West Sussex

  14. Stos B, Le BJ, Fermont L, Bonnet D (2007) Le diagnostic anténatal de la coarctation de l’aorte, estil possible? Arch Mal Coeur Vaiss 100(5):428–432

    PubMed  CAS  Google Scholar 

  15. Quarello E, Stos B, Fermont L (2011) Diagnostic prénatal dese co- arctations de l’aorte. Gynecol Obstet Fertil 39:442–453

    Article  PubMed  CAS  Google Scholar 

  16. Bagdure D, Bartakian S, Kaufman J (2011) Coarctation of aorta and vein of Galen aneurysmal malformation in a neonate. Curr Opin Pediatr 23:249–252

    Article  PubMed  Google Scholar 

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Conflict of interest

Mohammed Firdouse, Arnav Agarwal, Tapas Mondal declare no conflicts of interest.

Informed consent

All procedures followed were in accordance with the ethical standards of the responsible committee on humannexperimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000 (5). All patients provided written informed consent to enrolment in the study and to inclusion in this article of information that could potentially lead to their identification

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The authors confirm that all human and animal studies have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the Helsinki Declaration of 1975 and its late amendments. Additional informed consented was obtained from all patients for which identifying information is not included in this article.

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The study was conducted in accordance with all institutional and national guidelines for the care and use of laboratory animals

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Correspondence to Tapas Mondal.

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Firdouse, M., Agarwal, A. & Mondal, T. Vein of Galen arteriovenous malformation mimicking coarctation of the aorta. J Ultrasound 17, 297–301 (2014). https://doi.org/10.1007/s40477-014-0080-y

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  • DOI: https://doi.org/10.1007/s40477-014-0080-y

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