title: Vein of Galen aneurysmal malformation (VGAM) in the fetus: retrospective analysis of perinatal prognostic indicators in a two-center series of 49 cases.
abstract: OBJECTIVE
  Vein of Galen aneurysmal malformation (VGAM) is a rare fetal anomaly, the neurological outcome of which can be good with appropriate perinatal management. However, most fetal series are too small to allow reliable statistical assessment of potential prognostic indicators. Our aim was to assess, in a two-center series of 49 cases, the prognostic value of several prenatal variables, in order to identify possible prenatal indicators of poor outcome, in terms of mortality and cerebral disability.
  
  
  METHODS
  This was a retrospective study involving 49 cases of VGAM diagnosed prenatally and managed at two centers over a 17-year period (1999-2015). All cases had undergone detailed prenatal cerebral and cardiac assessment by grayscale ultrasound, color and pulsed-wave Doppler and magnetic resonance imaging (MRI). Ultrasound and MRI examination reports and images were reviewed and outcome information was obtained from medical reports. Volume of the VGAM (on ultrasound and MRI) was calculated and development of straight-sinus dilatation, ventriculomegaly and other major brain abnormalities was noted. Cardiothoracic ratio, tricuspid regurgitation and reversed blood flow across the aortic isthmus were evaluated on fetal echocardiography. Major brain lesions were considered by definition to be associated with poor outcome in all cases. Pregnancy and fetoneonatal outcome were known in all cases. Fetoneonatal outcome and brain damage were considered as dependent variables in the statistical evaluation. Poor outcome was defined as death, late termination of pregnancy due to association with related severe brain anomalies or severe neurological impairment.
  
  
  RESULTS
  At a mean follow-up time of 20 (range, 0-72) months, 36.7% of the whole series and 52.9% of the cases which did not undergo late termination were alive and free of adverse sequelae. Five (10.2%) cases showed progression of the lesion between diagnosis and delivery. On univariate analysis, dilatation of the straight sinus, VGAM volume ≥ 20 000 mm3and tricuspid regurgitation were all significantly related to poor outcome. However, on logistic regression analysis, the only variables associated significantly with poor outcome were tricuspid regurgitation and, to a lesser extent, VGAM volume ≥ 20 000 mm3. The former was also the only variable associated with brain damage.
  
  
  CONCLUSIONS
  Major brain lesions, tricuspid regurgitation and, to a lesser extent, VGAM volume ≥ 20 000 mm3are the only prenatal variables associated with poor outcome in fetal VGAM. Prenatal multidisciplinary counseling should be based on these variables. Copyright © 2016 ISUOG. Published by John Wiley & Sons Ltd.
authors: Fetal Medicine and Surgery Unit, Istituto Giannina Gaslini, Genoa, Italy.
authors: D
authors: D
authors: Paladini
authors: Maternité, Hôpital Universitaire Necker-Enfants Malades, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors: B
authors: B
authors: Deloison
authors: Neuroradiology Unit, Istituto Giannina Gaslini, Genoa, Italy.
authors: A
authors: A
authors: Rossi
authors: Maternité, Hôpital Universitaire Necker-Enfants Malades, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors: G E
authors: GE
authors: Chalouhi
authors: Neuroradiology Unit, Istituto Giannina Gaslini, Genoa, Italy.
authors: C
authors: C
authors: Gandolfo
authors: Radio-Pédiatrie, Hôpital Universitaire Necker-Enfants Malades, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors: P
authors: P
authors: Sonigo
authors: Neonatal and Pediatric Intensive Care Unit, Department of Critical Care and Perinatal Medicine, Istituto Giannina Gaslini, Genoa, Italy.
authors: S
authors: S
authors: Buratti
authors: Radio-Pédiatrie, Hôpital Universitaire Necker-Enfants Malades, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors: A E
authors: AE
authors: Millischer
authors: Department of Pediatric Cardiology and Cardiac Surgery, Istituto Giannina Gaslini, Genoa, Italy.
authors: G
authors: G
authors: Tuo
authors: Maternité, Hôpital Universitaire Necker-Enfants Malades, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors: Y
authors: Y
authors: Ville
authors: Epidemiology and Biostatistics Unit, Istituto Giannina Gaslini, Genoa, Italy.
authors: A
authors: A
authors: Pistorio
authors: Neurosurgery Unit, Istituto Giannina Gaslini, Genoa, Italy.
authors: A
authors: A
authors: Cama
authors: Maternité, Hôpital Universitaire Necker-Enfants Malades, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
authors: L J
authors: LJ
authors: Salomon
mesh_terms: D000328:Adult; D002550:Cerebral Veins / Q000002:abnormalities*; D005260:Female; D006801:Humans; D007558:Italy; D008279:Magnetic Resonance Imaging; D011237:Predictive Value of Tests; D011247:Pregnancy; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal; D054080:Vein of Galen Malformations / Q000000981:diagnostic imaging*
keywords: brain anomaly; fetal echocardiography; prenatal diagnosis