Prenatal Diagnosis of Galen Vein Aneurysm Using Ultrasonography and Magnetic Resonance Imaging and Perinatal and LongTerm Neurological Outcomes: A Case Series

Pires, Pedro; Martins, Larisse de Brito Aurélio; Pires, Norma Maria Tenório Brito; Werner, Heron; Ferreira, Adilson Cunha; Araujo, Edward

doi:10.1055/s-0037-1601401

Acessibilidade / Reportar erro

Brasil

Revista Brasileira de Ginecologia e Obstetrícia

Español English

Brasil

Español English

sumário « anterior atual seguinte »

Sumário

Case Report • Rev. Bras. Ginecol. Obstet. 39 (06) • June 2017 • https://doi.org/10.1055/s-0037-1601401 copy

Prenatal Diagnosis of Galen Vein Aneurysm Using Ultrasonography and Magnetic Resonance Imaging and Perinatal and LongTerm Neurological Outcomes: A Case Series

Diagnóstico do aneurisma de veia de Galeno por meio de ultrassonografia e ressonância magnética e resultados perinatais e neurológicos: série de casos

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

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.

Keywords:
Galen vein aneurysm; prenatal diagnosis; ultrasonography; magnetic resonance; perinatal outcomes

Case#	1	2	3	4	5	6	7
GA at diagnosis (weeks)	33	36	32wk, 1d	35	30	24	26
Associated findings	cardiomegaly, slight pericardial effusion	polyhydramnion, ventriculomegaly, cardiomegaly, cervical vascular congestion	Polyhydramnion	ventriculomegaly, cardiomegaly, cervical vascular congestion	ventriculomegaly, pericardial effusion, cardiomegaly, cervical vascular congestion	None	None
Fetal echo.	cardiomegaly,	pulmonary hypertension, heart failure	Normal	cardiomegaly, pericardial effusion, heart failure	cardiomegaly, tricuspid failure, pericardial effusion	Normal	Normal
GVA(cm)	2.9 × 1.1	3.2 × 1.8	2.2 × 1.8	7.0 × 2.5	4.2 × 2.5	3.0 × 2.3	3.5 × 3.4
Type of delivery	Vaginal	Cesarean	Cesarean	Cesarean	Cesarean	Cesarean	Cesarean
GA at birth(weeks)	37	39	38	36wk, 1d	34wk, 4d	38	39
Apgar score,1st & 5th min	8 and 9	3 and 6	7 and 9	5 and 4	6 and 4	8 and 9	9 and 10
Birth weight(g)	2900	2940	3180	2980	2550	3060	3240
Neonatal outcome	GVA embolization at 6mth	ICU, digoxin, furosemide	discharge 4d after birth	ICU for 28d	heart failure	3wk in the ICU	seizures, hydrocephalus, heart failure 2wk after birth
Survival up to 4yr of age	Normal	death at 48h	spontaneous thrombosis at 4yr, normal development	serious neurologic sequelae	death on 4th day	death during surgery	Normal

Case #	Magnetic resonance imaging	Ultrasonography
1	Ellipsoid expansive formation, ∼ 2.0 cm in its largest diameter at the level of the middle line in the tentorial region, posterior to the pituitary, hypointense T1 and T2 signals, suggesting a flow void, communicating with the sinus rectus, consistent with aneurysmal dilation of the Galen vein, with no signs of ventriculomegaly.	An elongated cystic image, measuring ∼ 2.9 × 1.1 cm, was observed in a location posterior to the thalamus, continuing on the middle line and spreading superiorly between the hemispheres. On the color Doppler, a low-resistance flow was observed, with an arterial pattern prevailing. The echographic aspect and the flow pattern are consistent with Galen vein aneurysm. Enlarged cardiac area and slight pericardial effusion.
2	Slight ventriculomegaly and expansive ellipsoid formation in a middle line location, spreading to the posterior fossa, consistent with arteriovenous malformation (Galen vein aneurysm).	Slight ventriculomegaly and an elongated anechoic image in a middle line location, spreading to the posterior fossa. On the color Doppler, an abundant flow of very low resistance, consistent with an arteriovenous malformation (Galen vein aneurysm). Enlarged cardiac area with slight pericardial effusion.
3	Intracranial ellipsoid expansive formation, 3.0 × 2.1 cm in size, located on the middle cerebral line (supratentorial), consistent with Galen vein aneurysm.	A homogeneous intracranial cystic area, measuring 2.2 × 1.8 cm, localized on the middle line (supratentorial). On color Doppler, intense arteriovenous flow. Normal heart size and shape.
4	Ellipsoid expansive formation measuring 7.0 × 2.5 cm, supratentorial, slight dilation of the posterior horn of the brain ventricle and the 3rd ventricle, consistent with Galen vein aneurysm associated with ventriculomegaly.	A cystic area with a tubular aspect, measuring 4.1 × 3.7 cm, on the middle line (supratentorial), being confirmed based on color Doppler as an arteriovenous flow within, consistent with Galen vein aneurysm. Slight ventricular dilation. Slightly enlarged cardiac area. Vascular congestion of the cervical region.
5	Ventricular dilation and ellipsoid expansive formation, measuring ∼ 6.0 × 4.0 cm, on the middle line (supratentorial), consistent with Galen vein aneurysm.	A cystic area with a tubular aspect, measuring ∼ 5.5 × 4.0 cm, on the middle line (supratentorial), confirmed based on color Doppler as an arteriovenous flow within, consistent with Galen vein aneurysm. Slight ventricular dilation.
6	Expansive lesion with lobulated contours and well-defined limits, with a hypointense T2 signal and an iso/hypointense T1 signal, measuring 3.0 × 1.8 × 2.3 cm, interhemispheric, posterior to the 3rd ventricle, with no compressive effect.	Anechoic image measuring 34 × 28 mm, located posteriorly above the thalamus. Slightly dilated lateral ventricles. Color Doppler with turbulent flow. Normal 3rd and 4th ventricles.
7	Expansive lesion with lobulated contours and well-defined limits, with a hypointense T2 signal and an iso/hypointense T1 signal, measuring 3.5 × 3.4 × 2.6 cm, interhemispheric, posterior to the 3rd ventricle, no compressive effect.	Anechoic Image measuring 26 × 24 mm, posterior to the 3rd ventricle. Normal lateral, 3rd, and 4th ventricles. Color Doppler with turbulent flow.

Federação Brasileira das Sociedades de Ginecologia e Obstetrícia Av. Brigadeiro Luís Antônio, 3421, sala 903 - Jardim Paulista, 01401-001 São Paulo SP - Brasil, Tel. (55 11) 5573-4919 - Rio de Janeiro - RJ - Brazil
E-mail: editorial.office@febrasgo.org.br

Acompanhe os números deste periódico no seu leitor de RSS

[1] Address for correspondence Edward Araujo Júnior, PhD, Department of Obstetrics, Escola Paulista de Medicina, Universidade Federal de São Paulo, Rua Belchior de Azevedo, 156, apto. 111, Torre Vitória, São Paulo, SP, Brazil (e-mail: araujojred@terra.com.br).