Tratamiento endovascular a malformación aneurismática de la Vena de Galeno / Endovascular treatment of aneurysmal malformation of the Vein of Galen

Gestante de 19 años sin antecedentes de embarazos previos, presenta a las 27 semanas en ecografía del segundo trimestre hallazgo sugestivo en el feto de MAVG; a las 38 semanas se realiza parto por cesárea sin complicaciones, se planificó angiografía cerebral con embolización. Se abordó por la arteria femoral derecha utilizando lavado heparinizado continuo y sistema de navegación coaxial se estudió la MAVG de tipo mural, se canalizo de forma selectiva a las arterias coroideas postero medial y postero lateral derecha y a la arteria coroidea posteromedial izquierda y se embolizó bajo control radiológico utilizando espirales de coils y líquido embolizante Onix. Control inmediato mostro cierre completo de conexiones fistulosas y preservación de ramas sanas.

A 19-year-old pregnant woman with no history of previous pregnancies, presented at 27 weeks in a second-trimester ultrasound suggestive finding in the fetus of MAVG; At 38 weeks, cesarean delivery was performed without complications, cerebral angiography with embolization was planned. It was approached through the right femoral artery using continuous heparinized lavage and a coaxial navigation system. The mural-type AVM was studied, it was selectively channeled to the right posteromedial and posterolateral choroidal arteries and to the left posteromedial choroidal artery and it was embolized under control. radiological using coil spirals and Onix embolizing liquid. Immediate control showed complete closure of fistulous connections and preservation of healthy branches.

Relevancia clínica del diagnóstico ecográfico de la malformación aneurismática en la vena de Galeno / Clinical relevance of ultrasound diagnosis of Vein of Galen aneurysmal malformation

Introducción: La malformación aneurismática en la vena de Galeno es infrecuente, pero tiene alta mortalidad en neonatos. Objetivo: Examinar la relevancia clínica del diagnóstico ecográfico de la malformación aneurismática en la vena de Galeno Métodos: Investigación observacional, prospectiva y transversal realizado en el Cardiocentro William Soler. (noviembre 1999-diciembre 2016) La muestra la conformaron 18 neonatos con diagnóstico de malformación aneurismática en la vena de Galeno variedad coroidea mediante ecografía doppler. Se configuraron dos grupos de referencia: 1) 70 niños supuestamente sanos. 2) 73 niños con malformación de la vena Galeno de diferente naturaleza que la variante coroidea estudiada. El procesamiento de la información incluyó elementos de estadística inferencial y herramientas de la medicina basada en la evidencia. Resultados: La presencia de fallo cardíaco neonatal, signos electrocardiográficos de isquemia miocárdica y detección de soplo continuo transcraneal, fueron significativamente diferentes en el grupo de estudio en relación con el grupo de referencia (p= 0,000001), con predominio en el número de pacientes del grupo estudio en todas las variables. Los resultados anteriores conjugados con diversos hallazgos ecográficos: la dilatación anómala de la vena, exceso de vasculatura aferente, reducción de los índices circulatorios encefálicos e incremento en los diámetros vasculares supraaórticos, identificaron de forma precisa la malformación aneurismática en la vena de Galeno. La evaluación del riesgo relativo reafirmó la documentación de los hallazgos expuestos. Conclusiones: La ecografía doppler, por su relevancia clínica y vínculo con otros elementos diagnósticos, es mandatoria en la detección de la malformación aneurismática en la vena de Galeno(AU)

Introduction: Vein of Galen aneurysmal malformation is not frequent but it has high mortality rates in newborns. Objective: To assess the clinical relevance of ultrasound diagnosis of Vein of Galen aneurysmal malformation. Methods: Observational, prospective and cross-sectional research conducted in William Soler Cardiocentro (November 1999- December 2016). The sample was formed by 18 newborns with diagnosis of Vein of Galen aneurysmal malformation (choroidal variety) through doppler echocardiography. Two reference groups were formed: 1) 70 supposedly healthy children; 2) 73 children with Vein of Galen malformation with a nature different to the studied choroidal variety. Processing of the information included elements of inferential statistics and tools from medicine based in evidences. Results: The presence of neonatal heart failure, electrocardiographic signs of myocardial ischemia and detection of transcranial continuous murmur were significantly different in the study group in relation with the reference group (p= 0,000001), with predominance in the number of patients of the study group in all the variables. The previous results combined with different ultrasound findings as the anomalous dilation of the vein, the excess of afferent vasculature, the reduction of encephalic circulation indexes and the increase of the supraaortic vascular diameters identified in a precise way the vein of Galen aneurysmal malformation. The assessment of the relative risk reaffirmed the information on the exposed findings. Conclusions: Doppler echography, due to its clinical importance and its links with other diagnostic elements, is mandatory in the detection of the vein of Galen aneurysmal malformation(AU)

Malformación aneurismática de la vena de Galeno / Aneurysms malformation of vein of Galen

Introducción: La malformación aneurismática de la vena de Galeno es una infrecuente variedad de fístula arteriovenosa cerebral de origen embriológico. Se localiza en la línea media de la fisura coroidal y tiene morfología de amplio espectro. Objetivo: Exponer información actualizada acerca del tema. Método: La información se obtuvo de la búsqueda automatizada realizada fundamentalmente en bases de datos MEDLINE, Current Contents y Scielo. Resultados: La malformación aneurismática de la vena Galeno se manifiesta con síntomas y signos derivados de la insuficiencia cardiaca severa refractaria al tratamiento médico y del daño neurológico que provoca. La entidad implica morbilidad grave y mortalidad en el periodo neonatal. La angiorresonancia es la técnica de referencia, pero la ecografía doppler es buen método de valoración. El tratamiento de elección es la embolización endovascular. Consideraciones finales: Debe existir índice elevado de sospecha para no fracasar en el diagnóstico precoz de la enfermedad. Es importante definir la anatomía de la lesión, por las implicaciones clínicas, terapéuticas y pronósticas que ello acarrea. El tratamiento exitoso sigue siendo un reto terapéutico complejo(AU)

Introduction: Vein of Galen aneurysm is a rare and congenital cerebral arteriovenous abnormality with reported incidence of 1:25 000 live births. It represents the 30 percent of the vascular congenital cerebral malformations that harm the pediatric population. Objective: To show updated information about the topic. Method: The information was obtained from the automated search mostly done in MEDLINE, CurrentContents and Scielo databases. Development: Vein of Galen malformation presents with symptoms and signs derived from the severe refractary heart failure to medical treatment and the neurologic damage it causes. That entity implies severe morbidity and mortality on the neonatal period. Angioresonance is the reference technique, but the Doppler ecography is a good valuation method. The election treatment is the endovascular embolization. Final considerations: There must be an elevated rate of suspect to do not fail on the early diagnostic of the disease. It is important to define the lesion's anatomy, because of the clinic, therapeutic and prognostic implications this represent. The successful treatment is still a complex therapeutic challenge(AU)

Adjuvant Coil Assisted Glue Embolization of Vein of Galen Aneurysmal Malformation in Pediatric Patients / 신경중재치료의학

PURPOSE: Adjuvant coils may offer advantages in flow control during glue embolization of high flow vein of Galen aneurysmal malformation (VGAM) patients but involves specific issues such as feasibility, durability and coil mass effect. The purpose of this study is to assess the outcome of adjuvant coils in addition to transarterial glue embolization for treatment of these patients. MATERIALS AND METHODS: Five pediatric VGAM patients (age range; 11 weeks to 2 yrs 2 mos) with high flow fistulous angioarchitecture were treated with adjuvant coils 1) in the distal feeding artery and/or 2) in the vein of Galen followed by glue embolization of the shunt. The angiographic / clinical outcomes were assessed. RESULTS: Adjuvant coils were deployed in the distal feeding artery (n=3), vein of Galen pouch plus distal feeding artery (n=2). Additional transarterial glue embolization of the fistulae was successfully performed (n=4). Complete occlusion was achieved with coils in one case. Complete occlusion was achieved for all mural type cases (n=4). Residual feeders remained in a case of choroidal type of VGAM. No complications were noted related to the treatment. All patients showed normal development on follow up (range: 7.6 to 88.8 mo, mean 49.3 mo). Initial hydrocephalus improved on follow up despite coil mass effect in dilated vein of Galen. CONCLUSION: Adjuvant coils for flow control with glue embolization may be a safe and effective treatment method for VGAM patients with high flow fistulous feeders.

Vein of Galen Aneurysmal Malformation with Transvenous Endovascular Treatment in an Adult: Case Report

Vein of Galen aneurysmal malformation (VGAM) is the result of the direct communication between the arterial network and the median prosencephalic vein. It is a rare vascular congenital malformation representing less than 1% of intracranial abnormalities. This finding is very rare in adults, and it may or may not present symptoms during childhood. Most cases of VGAM can be detected in the fetus by ultrasonography. The referral of pregnant women with fetuses with this condition to centers where better facilities and resources for childbirth and immediate postpartum care are available has resulted in considerable improvement in the prognosis of newborns. Regarding treatment, the endovascular approach to VGAM includes arterial embolization and percutaneous transvenous techniques. The transvenous endovascular treatment was chosen in the case presented in this article.

A malformação aneurismática da veia de Galeno (MAVG) é resultado da comunicação direta entre a rede arterial e a veia prosencefálica mediana. Trata-se de uma malformação vascular congênita rara, que representa menos de 1% das anormalidades intracranianas. Sua ocorrência é muito rara em adultos, e a malformação pode ou não apresentar sintomas durante a infância. A maioria dos casos pode ser detectada em fetos por ultrassonografia. O encaminhamento de grávidas com fetos com esta malformação para centros mais bem estruturados, com recursos para cuidados no parto e pós-parto, tem resultado em considerável melhora do prognóstico de recémnascidos. Quanto ao tratamento, o acesso endovascular à MAVG inclui a técnica de embolização arterial e o tratamento transvenoso percutâneo. O tratamento transvenoso endovascular foi escolhido no caso apresentado neste artigo.

Prenatal diagnosis of ultrasound and MRI in the vein of Galen aneurysmal malformation / 中华医学超声杂志（电子版）

Objective To explore the clinical value of prenatal ultrasound and magnetic resonance imaging in the vein of Galen aneurysmal malformation. Methods The ultrasonographic characteristics and magnetic resonance imaging features of 17 fetuses who were diagnosed as the vein of Galen aneurysmal malformation in Maternal and Child Heath Hospital of Hubei from January 2011 to December 2016 were compared. Results Fifteen cases of Galen aneurysmal malformation. Were diagnosed by prenatal ultrasound during the third trimester. The sonographic feature was a hypoechogenic structure located in the middle line with arteriovenous fistula waveform in doppler analysis, and the cerebral venous sinus of all cases were dilated. Real time three dimensional power doppler can display the angioarchitecture and the communications between widened vascular sinus. MRI feature was the expansion of venous aneurysm located in the middle line, greater cerebral venous pool and quadrigeminal area and tortuous flow void vessels. Among 17 cases, 2 cases of Galen venous aneurysm malformation were misdiagnosed and 7 cases were diagnosed by MRI. There were 15 terminations and 2 neonatal died after birth. Conclusions Galen aneurysmal malformations have typical sonographic findings, and MRI can show neurologic development and impairment. Combined application of ultrasound and magnetic resonance imaging technology can more accurately and comprehensively observe the pathological features of Galen venous aneurysm malformation and diagnose the related complications early.

Vein of Galen Aneurysmal Malformation : Endovascular Management of 6 Cases in a Single Institute

OBJECTIVE: The aim of this study was to analyze the treatment outcome of patients with vein of Galen aneurysmal malformations (VGM). METHODS: Clinical and angiographic data of six consecutive patients with VGM were retrospectively reviewed. VGMs were angiographically classified by Yasargil's method. Treatment outcomes were evaluated. RESULTS: Mean age at initial treatment was 4.4+/-5.7 months. Angiographic types of VGMs were type II in two patients and type III in four. Three patients had cardiac symptoms and the others were asymptomatic. Two patients were treated with transvenous embolization, three with transarterial embolization, and one was managed conservatively. Two patients died due to venous hypertension few days after transvenous approach. Of three patients who were transarterially embolized, one was completely occluded with Onyx and two were incompletely occluded. During the follow-up period (range, one to six years) two of three patients treated with transarterial approach were asymptomatic and the other showed mild symptoms. One patient who was managed conservatively showed normal performance. CONCLUSION: Transarterial embolization of VGMs may be better than transvenous approach in terms of the treatment outcome and complication. Further studies are needed because of the rarity of the disease and rapid advancement of endovascular techniques.