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Diagnosis of Foetal Vein of Galen Aneurysmal Malformation By Li et al. BMC Medical Imaging (2020) 20:63 https://doi.org/10.1186/s12880-020-00463-6 CASE REPORT Open Access Diagnosis of foetal vein of galen aneurysmal malformation by ultrasound combined with magnetic resonance imaging: a case series Tian-gang Li1,2†, Yao-yue Zhang1†, Fang Nie2*, Mei-juan Peng1, Yun-Zhi Li1 and Pei-long Li1 Abstract Background: Foetal vein of Galen aneurysmal malformation (VGAM) is a very rare congenital malformation of the cerebral blood vessels. We sought to evaluate the diagnostic value of ultrasound in combination with magnetic resonance imaging (MRI) in foetal VGAM. Case presentation: Prenatal ultrasound combined with MRI diagnosed five cases of VGAM. Two dimensional ultrasound images were used to find the echo-free cystic structure below the thalamus and above the cerebellum with five cases. Colour blood flow showed dilated VGAM in five cases, while the arteriovenous spectrum was explored in two cases and foetal heart failure was found in other three cases. MRI was manifested as a dilated VGAM found at the midline of the brain, demonstrating widening or dilation of the straight sinus in four cases, ventricular dilatation in one case, brain parenchyma bleeding in two cases, and grey matter softening in one case. One infant died on the day of its birth, while the other four infants died within one month to six months after birth. Conclusions: Ultrasound combined with MRI can more accurately and comprehensively observe the pathological characteristics of VGAM, diagnose related complications early and determine its prognosis. Keywords: Ultrasound, magnetic resonance imaging, Foetus, vein of Galen aneurysmal malformation, Prenatal diagnosis, Congenital malformation Background precursor to the vein of Galen, becomes significantly en- Foetal vein of Galen aneurysmal malformation (VGAM) larged and aneurysmal [2–4]. The condition is mostly is a very rare congenital malformation of the cerebral detected during late pregnancy in the third trimester, blood vessels, accounting for about 1% of foetal intracra- often combined with foetal brain injury and cardiac dys- nial malformations [1]. These malformations form be- function, and the prognosis is poor [5]. Earlier diagnoses tween the sixth and 11th week of gestation. Due to the have also been reported [6, 7]. fistula between the cerebral arteries and deep draining VGAM leads to a typical ultrasound sonogram [1, 3]. veins of the brain, the median prosencephalic vein, a Magnetic resonance imaging (MRI) can show the devel- opment of the intracranial nervous system and brain * Correspondence: [email protected] † parenchymal damage [8]. With a study of previous liter- Tian-gang Li and Yao-yue Zhang contributed equally to this work. atures, ultrasound or MRI examination was used to diag- 2Department of Ultrasound diagnosis, Lanzhou University Second Hospital, Lanzhou 730030, Gansu Province, P. R. China nose foetal VGAM. There were rarely reports of analysis Full list of author information is available at the end of the article © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Li et al. BMC Medical Imaging (2020) 20:63 Page 2 of 6 and diagnosis of VGAM by ultrasound combined MRI. premature, and all of them were SGA foetuses, with This study analysed the imaging characteristics and other basic information such as parity and delivery value in diagnosing VGAM by prenatal ultrasound and methods being described in Table 1. MRI among five foetuses at Gansu Maternal and Child The volume of VGAM could be measured by ultra- Health Hospital. sound and MRI and the results were similar. The max- imum volume as measured by MRI was 150.12 cm3 and Case presentation the minimum volume was 1.64 cm3. Three cases showed Characteristics and basic clinical information straight sinus dilatations with an inner diameter of more Between January 2013 and December 2019, five cases of than 0.6 cm. All five cases had high-risk factors or co- foetal VGAM were diagnosed by prenatal ultrasound morbidities, including three that were accompanied by and MRI examinations at Gansu Maternal and Child structural abnormalities of the foetal heart. After labour, Health Hospital. Four cases were single infants and one one infant died on the day of its birth, while the other case was one of the twins, and the age, parity, delivery four cases died within one month to six months after method, ultrasound diagnosis week, gestational age, birth (Table 2). presence of heart failure, and clinical outcomes were Ultrasound results showed arteriovenous leaks in two evaluated (Table 1). We calculated the gestational age cases, foetal heart failure in three cases, and MRI find- based on the first day of the last menstrual period and ings in four cases suggesting widening or dilation of the confirmed it by crown–rump length measurement on straight sinus. One infant had ventricular dilatation and the first trimester-ultrasound. All cases had undergone two had brain parenchyma bleeding. One case showed at least one detailed ultrasound scan (two-dimensional grey matter softening (Table 3). and, more recently, three-dimensional) and as well as Ultrasound was used to evaluate heart failure in five one MRI prenatally. cases, with three subsequently diagnosed. The cardio- thoracic ratio increased in three cases, one case showed Clinical findings pleural effusion, one case demonstrated peritoneal effu- In all five cases of foetal ultrasound, an echo-free cystic sion, three cases showed tricuspid regurgitation, and structure was found below the thalamus and above the three cases presented an abnormal umbilical artery cerebellum of approximately the same size across all spectrum. The frequency spectrum of the umbilical vein cases and with clear boundaries (Figs. 1a and 2a), reveal- was abnormal in one case and that of the uterine artery ing that the blood flow in the nonechoic area was full was abnormal in one case (Table 4). (Figs. 1b and 2b). 3D blood flow imaging revealed the three-dimensional distribution of whole blood flow and Ultrasonography blood flow in VGAM (Figs. 1c and 2c). Meanwhile, the We used E10 or E8 (GE Healthcare, USA) ultrasound spectrum approach explored the arteriovenous fistula systems with obstetric preset and completed foetal bio- and high-speed low resistance (Fig. 1d) or arteriovenous metric measurements on each scan. Subsequently, we mixed spectrum (Fig. 2d), and MRI examination of the obtained a two-dimensional image of the VGAM from foetal head cross-section (Figs. 1e and 2e) revealed that each patient to observe its shape and size. In particular, the VGAM and the sagittal section (Figs. 1fand2f) the following parameters were recorded: orthogonal di- showed the VGAM straight sinus. ameters of the VGAM (i.e., craniocaudal, laterolateral, One case had a gestational age of less than 32 weeks at anteroposterior); volume of the VGAM, calculated from the time of ultrasound, while the other four cases had a the three diameters with the ellipsoid formula; and pres- gestational age of more than 32 weeks. Five foetuses ence/absence of straight sinus dilatation. Colour Doppler were born, of which one was one of the twin pregnancy blood flow was used to detect blood-flow filling in and four were single pregnancies. Three infants were VGAM and to observe the distribution of cerebral Table 1 Characteristics and basic clinical information of the five cases of VGAM Case Maternal age GA at diagnosis GA at delivery (weeks) Birth weight (grams) Number of Parity Delivery method number (weeks) infants 1 23y 34+5 35+2 2350 Twins Primipara Caesarean section 2 30y 30+1 39+6 2950 Single Multiple Natural delivery 3 36y 35+6 36+1 2300 Single Multiple Natural delivery 4 28y 40+1 40+6 3740 Single Multiple Natural delivery 5 23y 33+3 33+5 2180 Single Primipara Natural delivery GA gestational age, VGAM vein of Galen aneurysmal malformation Li et al. BMC Medical Imaging (2020) 20:63 Page 3 of 6 Fig. 1 Case 1, 35-week prenatal ultrasound and MRI images of a VGAM foetus. a: Two-dimensional ultrasound shows a huge thin-walled echoless area next to the midline of the brain. b: Colour Doppler shows a full blood-flow signal in the brain’s echoless area. c: The skull’s echoless area shows a high-speed low-impedance blood-flow spectrum. d: The reconstructed three-dimensional energy Doppler image clearly shows the dilated Galen vein, but no obvious straight sinus is displayed. e: MRI cross-section of the foetal skull showing the dilated Galen vein through flow phenomenon (white arrow). f: Sagittal section of the foetal brain showing dilated Galen’s venous aneurysm by flow phenomenon (white arrow) arteries and VGAM connection vessels, while the there were softening lesions in the white matter of the spectrum was used to monitor the blood flow spectrum. foetus; further, real-time three-dimensional power Dop- Finally, we observed whether the echo of the brain tissue pler sonography was used to observe the relations be- surrounding the VGAM was enhanced and/or whether tween the VGAM and cerebral artery branch and that small cystic lesions were visible and determined whether between the VGAM and sagittal sinus connection, Fig.
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