Prenatal diagnosis and postnatal consequences of vein of Galen malformation (analysis of literature data and personal observations)

Abstract

Purpose — to assess ultrasound criteria and diagnostic value at vein of Galen malformation (VGAM) throughout perinatal period with possible further mortality rate and psychomotor development prognosis.

Materials and methods. This was retrospective study involving 9 cases of VGAM diagnosed prenatally and managed at two institutions over a 5-year period (2014–2019). All cases had undergone detailed prenatal and perinatal cerebral, cardiac and fetoplacental unit assessment by grayscale ultrasound, color and pulsed–wave Doppler. In order to determine further treatment tactics neurosurgical consultation was involved into all confirmed VGAM cases.

Results. Pregnancy and fetoneonatal outcome were known in all cases. Minor size supratentorial arachnoid cysts were detected in 6 VGAM cases. Vascular origin of formations was confirmed with Doppler scan. However, no signs of parenchymal abnormalities, liquor system of the brain damage and heart failure have been identified. All newborns were discharged with further outpatient follow-up. Vascular malformation with cardiomegaly correlation, tricuspid regurgitation, dilation of the right atrium and upper cava vein, severe brain abnormalities were considered by definition to be associated with poor outcome in 3 cases. Poor outcome was defined as death.

Conclusions. VGAM diagnosis in newborns is highly determined by timely prenatal diagnosis and must involve postnatal neurosurgical assessment. Clarification of the diagnosis contributes to establishing the prognosis and inpatient care tactics. Color and pulsed+wave Doppler assessment is necessary for differential diagnosis with other midline cystic abnormalities of the brain. It is recommended to consider delivery within the perinatal clinic. Care must be provided by highly qualified perinatal team of obstetricians, neurosurgeons and neonatologists with an extensive experience in managing high risk pregnancies. Fetoneonatal outcome is poor due to congestive heart failure, severe brain damage and neurological impairment with tendency to worsen if diagnosed prenatally.

The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies.