Vein of Galen Malformation

Vein of Galen Malformation (VOGM) is a rare condition in which there is an abnormal malformation involving one of the important veins in the brain that returns blood to the heart.

What causes VOGM?

The exact cause of VOGM is unknown. It is seen most often in infants and children. It can be detected as early as during pregnancy during routine ultrasound examination. The condition is not inherited and is not transmitted in the family. Rarely, it can be seen in older individuals. Signs and Symptoms The abnormal communication between arteries and veins in the brain leads to increased blood flow into the veins and to the heart. All the symptoms are as a result of this increased blood flow. the most common signs and symptoms are

• heart failure, often in the first few days of life
• abnormally increased head size and circumference due to excessive fluid accumulation within the brain
• prominent veins on the veins and scalp
• developmental delay
• failure to thrive
• seizures
• headache
• rarely, brain bleed
• if untreated, the condition may be life threatening

Diagnosis

• the condition may be detected on antenatal ultrasound
• CT scan, MRI may be required if there is a suspicion of VOGM
• cerebral angiography is the definitive test to confirm the diagnosis

Treatment

• A Neurosurgeon and neurointerventionist should be consulted to assess the extent and location of VOGM and plan management
• A Cardiologist should be consulted to assess heart function
• The neonatologist or pediatrician takes care of general health of the child
• Embolization is performed to close the abnormal connections. The procedure

involves inserting a catheter through one of the arteries in the groin and navigating all the way into the tiny abnormal connections. Coils (metal threads) and glue are used to close the abnormal connections.

• Multiple sessions of embolization are often needed to close all the abnormal connections
• Open surgery for VOGM is never required. However, shunt surgery may be required to drain the fluid from accumulating in the brain.

Outcome

It is often possible to cure the malformation. The extent of brain and heart damage at the time of diagnosis and treatment determines the outcome.