Outcome of Therapies for Vein of Galen Malformations in Children

Outcome after surgery

• Good neurological outcomes: Favorable neurological outcomes of endovascular embolization have been observed in 62% to 68% of patients (25,73,84).
• Outcomes vary by patient age: Neonates, compared to infants, children, and adults, have significantly lower rates of good neurological outcomes. An estimated 32.7% to 48.0% of neonates, 75.3% to 77.0% of infants, and 75.8% of children and adults have good neurological outcomes (25,84).
• Complete occlusion may not be the goal: Complete occlusion is achieved in an estimated 56% to 57% of patients (25,73). This may or may not be a treatment goal, depending on a balance of risk and clinical benefit.
• Endovascular embolization mortality rate: The overall mortality rate of endovascular embolization for VOGM is estimated to be between 10% and 15% (25,73,84).
• Microsurgery mortality rate: Microsurgery is associated with a mortality rate of 84.6% and is no longer the standard of care (84).
• Mortality varies by patient age: Neonates, compared to infants, children, and adults, have significantly higher mortality rates following endovascular embolization. Estimated mortality rates of 27.0% to 35.6% in neonates, 1.0% to 6.5% in infants, and 3.2% in children and adults have been observed (84).
• Predictors of poor outcomes: Factors associated with poor outcomes include the presence of congestive heart failure, arterial steal, neurological symptoms at presentation, lower Bicêtre Neonatal Evaluation Scores, and multiple groups of arterial feeders (25,85).

Outcome after nonsurgical treatments

• Significant mortality in the absence of definitive intervention: A 76.7% mortality rate has been observed in patients who do not receive definitive intervention for VOGM (84).

Outcome after multimodal therapies

• Outcomes of radiosurgery: Gamma Knife radiosurgery may be viable in older patients (children and adults) who are more clinically stable/asymptomatic but have residual AV shunting. One report noted favorable outcomes in 8 of 9 patients with VOGM who were treated with Gamma Knife radiosurgery (86).