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Vein of GALEN aneurysmal malformation (VGAM) is a rare congenital disease caused by arteriovenous shunts between the choroidal arteries and the porencephalic ectatic vein. The diagnosis is often made in utero or during infancy, endovascular treatment remains the most suitable therapeutic means in a well-equipped environment. Here we report here the case of a patient complaining of headache for 1 year, and whose brain CT imaging showed the presence of Galen vein aneurysm with associated non-communicating hydrocephalus. In the absence of the appropriate technical platform, the placement of a ventriculoperitoneal shunt relieved our patient’s symptoms.

The Vein of Galen aneurysmal malformation (VGAM) is a dilatation of the vein of Galen with multiply communications between of the vein of Galen and venous sinus and the cerebral arteries, occurring between 6 and 11 weeks of gestation

Objective To describe the prenatal diagnosis of Galen vein aneurysm (GVA) based on ultrasonography and magnetic resonance imaging (MRI) in a series of cases, as well as its postnatal outcomes and follow-up until 4 years of age. Methods A retrospective longitudinal study was performed, analyzing a database comprising seven cases of prenatal diagnosis of GVA at two Brazilian institutions from February of 2000 to May of 2012. The following data were evaluated: gestational age at diagnosis, GVA dimensions on ultrasonography, associated fetal changes, findings on fetal echocardiography, gestational age at delivery, type of delivery, birth weight, Apgar score at the 1st and 5th minutes, neonatal outcomes, and survival with follow-up until 4 years of age. Results The mean gestational age ± standard deviation on the prenatal diagnosis of GVA based on ultrasonography was 25 ± 4.9 weeks. The mean length of GVA was 3.2 ± 0.4 cm. The mean gestational age at birth was 37.5 ± 0.7 weeks, and a cesarean section was performed in 85.7% of the cases (6/7). The mean birth weight was 3,070 ± 240.4 g. The total survival rate was 42.8% (4/7), with three neonatal deaths. Of the four survivors, three presented with normal neuropsychomotor development until 4 years of age and only one showed serious neurological sequelae. Ultrasonography and MRI showed similar findings for all seven cases. Conclusions Galen Vein Aneurysm is associated with a high neonatal death rate. Therefore, its prenatal diagnosis is essential for parent counseling and follow-up at tertiary care institutions.

Objective To describe the prenatal diagnosis of Galen vein aneurysm (GVA) based on ultrasonography and magnetic resonance imaging (MRI) in a series of cases, as well as its postnatal outcomes and follow-up until 4 years of age. Methods A retrospective longitudinal study was performed, analyzing a database comprising seven cases of prenatal diagnosis of GVA at two Brazilian institutions from February of 2000 to May of 2012. The following data were evaluated: gestational age at diagnosis, GVA dimensions on ultrasonography, associated fetal changes, findings on fetal echocardiography, gestational age at delivery, type of delivery, birth weight, Apgar score at the 1st and 5th minutes, neonatal outcomes, and survival with follow-up until 4 years of age. Results The mean gestational age ± standard deviation on the prenatal diagnosis of GVA based on ultrasonography was 25±4.9 weeks. The mean length of GVA was 3.2±0.4 cm. The mean gestational age at birth was 37.5±0.7 weeks, and a cesarean section was performed in 85.7% of the cases (6/7). The mean birth weight was 3,070±240.4 g. The total survival rate was 42.8% (4/7), with three neonatal deaths. Of the four survivors, three presented with normal neuropsychomotor development until 4 years of age and only one showed serious neurological sequelae. Ultrasonography and MRI showed similar findings for all seven cases. Conclusions Galen Vein Aneurysm is associated with a high neonatal death rate. Therefore, its prenatal diagnosis is essential for parent counseling and follow-up at tertiary care institutions. Resumo Objetivo Descrever o diagnóstico pré-natal de uma série de casos de aneurisma de veia de Galeno (AVG) por meio de ultrassonografia e ressonância magnética (RM), bem como os resultados pós-natais e acompanhamento até 4 anos de vida. Métodos Realizou-se um estudo retrospectivo longitudinal com análise de banco de dados de sete casos de diagnóstico pré-natal de AVG em dois serviços brasileiros entre fevereiro de 2000 e maio de 2012. Foram avaliados a idade gestacional ao diagnóstico, dimensões do AVG na ultrassonografia, alterações fetais associadas, achados da ecocardiografia fetal, idade gestacional ao parto, tipo de parto, peso ao nascimento, índice de Apgar no 1° e 5° minutos, resultados neonatais, e sobrevida com acompanhamento até 4 anos de idade. Perinatais Resultados A idade gestacional média±desvio-padrão ao diagnóstico pré-natal do AVG pela ultrassonografia foi de 25±4,9 semanas. O comprimento médio do AVG foi 3,2±0,4 cm. A idade gestacional média ao nascimento foi 37,5±0,7 semanas, sendo que, em 85,7% dos casos (6/7) o parto foi cesáreo. O peso médio ao nascimento foi de 3.070±240,4 gramas. A sobrevida total foi de 42,8% (4/7), com três óbitos neonatais. Dos quatro sobreviventes, três apresentaram desenvolvimento neuropsicomotor normal até a idade de 4 anos, sendo que apenas um apresentou sequelas neurológicas graves. Ultrassonografia e RM apresentaram achados semelhantes nos sete casos. Conclusões O AVG está associado à elevada taxa de óbito neonatal, sendo, portanto, fundamental o seu diagnóstico pré-natal precoce para aconselhamento dos pais e seguimento em serviço terciário.

Vein of Galen aneurysm malformation (VGAM) is a rare congenital vascular anomaly. Although the cause of VGAM remains to be elucidated, the current hypothesis is persistence of the embryonic vascular supply, which leads to progressive enlargement and formation of the aneurysmal component of a typical VGAM. Here, we present a 36-year-old woman at 23 weeks' gestation (gravida 3, para 2) who was evaluated using 3D power Doppler sonography for the prenatal diagnosis of a vein of Galen aneurysm. Investigation using 3D power Doppler sonography allowed for a non-invasive yet diffuse and detailed prenatal assessment of VGAM. Thus, we suggest that prenatal sonography with 3D power Doppler may be an option in cases of VGAM. © 2013 by the Turkish-German Gynecological Education and Research Foundation.