Management of Vein of Galen Malformations in Children

Initial Management at Presentation

• Stabilization in the ICU: Early admission to a neonatal intensive care unit and cardiovascular/respiratory stabilization may be required prior to intervention.
• Timing of intervention and risk stratification: Some patients may decompensate shortly after birth; these patients will likely require urgent treatment. Others may be relatively stable and can undergo elective treatment in a delayed fashion at several months of age (51).
• Endovascular embolization is the gold standard of intervention: The standard of care for VOGM involves neurointerventional techniques, namely endovascular embolization (73). Transarterial and transvenous embolization may be used, and treatment often follows a staged approach with multiple procedures (25). Compared to open microsurgery (16,20), endovascular embolization is associated with more favorable outcomes and substantially lower mortality (73).

Adjunctive Therapies

• Medical management: Medical management of heart failure, pulmonary hypertension, and organ dysfunction may be required before and after endovascular intervention.
• CSF diversion for hydrocephalus: Hydrocephalus, in rare cases (when not responding to embolization), may need to be treated via CSF diversion.

Follow-up

• Follow-up imaging: Following the completion of a staged (or single) intervention, regular imaging via cranial ultrasound and MRI is indicated. Cranial ultrasound is less invasive and, therefore, more frequently applied until the anterior fontanelle closes. MRI is more invasive but is also more informative in terms of the vasculature and changes in the ventricular configuration and brain parenchyma. Cerebral angiography should be reserved for assessment of clinical or radiographic changes and is typically combined with a planned embolization.
• Cardiac follow-up: Echocardiogram titrated to clinical need (often monthly if the patient can be discharged from the hospital), and follow-up with a pediatric cardiologist for evaluation of cardiac function is crucial.
• Neurodevelopmental follow-up: Combined programs of physical, occupational, and speech therapy may be appropriate to evaluate subtle delays in neurodevelopment and facilitate development in infants.