Galen vein malformation-intrauterine treatment

Vein of Galen malformation (VOGM) is a rare and severe congenital arteriovenous malformation. Its therapeutic results are complex and often catastrophic, making its treatment a true challenge.

VOGM is characterized by the persistence of direct arteriovenous communications between the choroidal feeding arteries and the embryonic vein, known as the median prosencephalic vein of Markowski.

The malformation develops between the 8th and 11th weeks of gestation. Diagnostic suspicion is usually made through fetal ultrasound after the 18th week of gestation or later. Fetal MRI complements the diagnosis.

There are two groups of patients with VOGM: severe cases, with persistent high-flow arteriovenous shunts leading to multiorgan failure, and mild cases with a lower mortality rate and a more favorable outcome. The use of vascular parameters, based on the diameter of the falcine sinus measured by fetal MRI, can serve as a predictor of neonatal outcome and therapeutic choice.

A 33-year-old primigravida presented with a fetus diagnosed with VOGM by fetal ultrasound at 32 weeks of gestation. An aneurysmal dilation of the Galen vein was observed, with a transverse falcine sinus measuring 10.5 mm. Aneurysm dilatation dimensions: 25 × 21 × 20 mm. Fetal MRI confirmed a falcine sinus width of 10.5 mm, indicating a 96% chance of poor outcome. Fetal embolization of the VOGM was performed, followed by postnatal treatment.

In this article, we review the main principles of VOGM management and present a case treated during pregnancy, illustrating a potential therapeutic strategy. We suggest that fetal embolization of VOGM, when appropriately indicated, may represent an emerging approach in the management of this severe condition.