Prognosis for newborns with vein of Galen malformation diagnosed prenatally based on a new scale

Introduction
Vein of Galen malformation occurs rarely and accounts for 1% of intracranial vascular abnormalities, and they usually have poor outcome. Therefore, we focused on the prenatal features that might suggest favourable prognosis.

Material and methods
We analysed fetal echocardiograms and neonatal management from 10 fetuses with diagnosis of vein Galen malformations.

Results
The size of Galen malformation was mean 35 mm ×19 mm. Five fetuses had transplacental digoxin treatment. Cardiovascular profile score (CVPS) at the initial evaluation was mean 6 ±2, the minimum value was 4, and the maximum value was 10. Cardiovascular profile score at the last evaluation was mean 7.3 ±2.4, but an increase was seen only in two survivors (the third survivor had only one echo examination and did not undergo transplacental treatment). There were two intrauterine demises and eight pregnancies with live born newborns. Mean gestational time of delivery for live born newborns was in the 37th week. There were four neonatal deaths on days 1, 2, and 3 before attempted neurosurgery. Three newborns had neurosurgery procedures on day 3 (two newborns) and on day 7 (one newborn). The last one died on day 27. Based on our prenatal data we suggested a new scale to predict a prognosis for newborns with vein of Galen malformation.

Conclusions
Prenatal detection of Galen aneurysm, CVPS of seven or more before birth, transfer in utero, delivery at term, and earliest possible neurosurgical treatment might be beneficial and change the poor natural outcome known from the past in these cases. Good cooperation between members of the perinatology team might be crucial for saving lives in prenatally diagnosed AVGM.