Vein of Galen aneurysmal malformation: diagnosis and treatment of 13 children with extended clinical follow-up

Abstract

Background and purpose: We herein present our experience in diagnosing and treating 13 children with vein of Galen aneurysmal malformations (VGAM), with an emphasis on possible prognostic indicators, endovascular strategies, factors affecting treatment during the neonatal period, and long-term follow-up. With this review, we hope to identify those factors that have the most significant prognostic value in determining long-term outcomes in children with VGAM.

Methods: We retrospectively reviewed the radiology studies, hospital charts, and outpatient clinic chart notes (when applicable) of 13 children evaluated and treated for VGAM at a single tertiary care pediatric hospital. Clinical presentation, diagnostic methods, treatment strategies, and outcome were documented for each child. The present neurologic status and level of function of each patient was determined by review of the outpatient charts and direct contact with the clinicians who were conducting the follow-up. Outcome was graded on a 5-point scale, ranging from 0 (death) to 4 (normal), taking into account only neurologic and developmental characteristics.

Results: Eight of 13 patients presented as neonates with congestive heart failure. The other five patients ranged in age from 4 months to 13 years at the time of presentation. The five patients presenting outside of the neonatal period achieved normal or near-normal outcomes. Two of the eight patients presenting during the neonatal period achieved normal or near-normal outcomes, one experienced significant impairment, and the other five died. We were unable to identify significant differences in outcome on the basis of differences in treatment strategies.

Conclusion: Our experience confirms that children with VGAM presenting during the neonatal period have a generally much worse prognosis than do those presenting later in childhood. Complicating factors in the management and treatment of these children are discussed in light of their impact on outcome.

Fig 1.

Images from the case of a 19-month-old patient with mild hydrocephalus and engorged scalp veins. A, Sagittal view T1-weighted MR image shows the markedly enlarged median prosencephalic vein of Markowski, characteristic of VGAM (arrow). Arterial feeders can be seen along the anterior wall of the vein. B, The complex arterial maze (arrows) is well seen on this conventional angiogram obtained with injection of the left vertebral artery. Coils can be seen along the right side of the varix, occluding several arterial feeders.

Fig 2.

Images from the case of a 3-month-old triplet with worsening congestive heart failure. A, Volume-rendered MR angiogram shows, from an inferior prospective, the arterial feeders supplying the lesion from the left side (arrows). B, Carotid injection during arterial embolization procedure performed when the patient was 6 months old shows residual flow to the lesion from pericallosal and posterior choroidal arteries (arrowheads). C, Coronal view fast spin-echo T2-weighted MR image obtained 6 years after embolization shows continued patency of the varix and mild enlargement of extra-axial fluid spaces.