When Should the Malformation be Treated?

The treatment depends on a few factors two of which include the size of the vein of Galen malformation and the extent of the congestive heart failure. The patient will often die if the high output heart failure is the presenting feature without treatment. If the malformation is large the patient will rapidly deteriorate with progressively worsening cardiac failure however with a smaller malformation the heart failure will present later on in life and the patient usually presents with “failure to thrive” and developmental delay.

Embolisation of a neonate is a high-risk procedure. There are some limitations of the procedure, one of which is the amount of contrast medium, flush solution that can be given to the patient. The amount of contrast that can safely be used depends on the patient’s weight. The contrast is radioactive and needs to be used in safe amounts so that the patient’s body can remove this waste product from it. Contrast makes the vessels visible on the angiogram. The angiogram provides the detailed pictures of where the coils need to be placed and where the catheter is inside the vessels – the road map if you like. Sometimes if the congestive heart failure is mild it is safer to manage the heart failure with medication and allow time for the baby to grow. The bigger the size of the baby the safer it is to treat the malformation.

Taking all of this information into account leaves the tricky question of: when should the patient be treated?

The “Bicetre Neonatal Evaluation Score” is used to help make this decision. The interventional neuroradiologist calculates the score with input from all the other medical professionals involved. It is the interventional neuroradiologist’s final decision of when and if treatment will take place.

The “Bicetre Neonatal Evaluation Score” is pictured below:

Bicetre neonatal evaluation score.

The following outlines how the scores relate to treatment timeframes:

 A score 8 or less  Not for treatment
 A score between 8 and 12  Immediate treatment
 A score 12 or more  Medical management until over 5 months old