Fetal and Neonatal MRI Predictors of Aggressive Early Clinical Course in Vein of Galen Malformation

Abstract

Background and purpose: Neonates with vein of Galen malformations are split into 2 cohorts: one needing urgent neonatal embolization, with relatively high mortality and morbidity even with expert care, and a cohort in which embolization can be deferred until infancy, with far better prognosis. We aimed to identify brain MR imaging characteristics obtained from fetal and early neonatal scans that can predict the clinical presentation.

Materials and methods: Patients with vein of Galen malformations were stratified into a neonatal at-risk cohort if the patient needed urgent neonatal intervention or if neonatal death occurred; or an infantile treatment cohort if they were stable enough not to require treatment until >1 month of age. Twelve vascular MR imaging parameters, measured by 2 independent observers, were systematically correlated with the need for early neonatal intervention and/or neonatal mortality.

Results: A total of 32 neonatal patients (21 patients in the neonatal at-risk cohort, 11 in the infantile treatment cohort) were identified. Maximal mediolateral diameter (area under the curve = 0.866, P < .001) and cross-sectional area (area under the curve = 0.836, P = .002) at the narrowest point of the straight or falcine sinus were most predictive of clinical evolution into the neonatal at-risk cohort. There were 15 patients who had fetal MRIs (10 in the neonatal at-risk cohort and 5 in the infantile treatment cohort). Here too, maximal mediolateral diameter (area under the curve = 0.980, P = .003) and cross-sectional area (area under the curve = 0.941, P = .007) at the narrowest point of the straight or falcine sinus were highly predictive of the neonatal at-risk cohort.

Conclusions: Early neonatal and fetal MR imaging can be readily used for accurate early risk stratification, assisting in directing resources, timing treatment decisions, and identifying appropriate cohorts for novel interventions.

Fig 1.

Aggressive early evolution of a vein of Galen malformation (NAR cohort) (A, left panels). After med-flighting this neonate with VOGM to our center for urgent treatment, the MR imaging obtained on day-of-life 9 showed diffuse bihemispheric brain injuries (red arrows) not seen on the initial on day-of-life 1 MR imaging (green arrows showing analogous regions). Increased soft-tissue edema in the neck and scalp on day 0 is secondary to overwhelming heart failure (B, right panels). Another patient, a one-day-old neonate with VOGM presents with complete liquefactive gliosis of both cerebral hemispheres (white brain signal resembles white CSF signal on T2-weighted imaging).

Fig 2.

Measurement of the mediolateral diameter of the falcine sinus at its shortest point. The left image shows a sagittal view of a fetal MR imaging, with the dashed red line at the section of shortest height of the falcine sinus. The right image shows a coronal view with the mediolateral diameter of the sinus demonstrated with red dashes, measured at this same shortest section. This diameter efficiently differentiated the IT from the NAR cohort, as did the cross-sectional area of the sinus, measured at this same section.

Fig 3.

ROC curves showing measurements of the straight or falcine sinus area (SS-A) and the straight or falcine sinus maximal mediolateral diameter (SS-MD) at the shortest point of the sinus. Measurements from neonatal MR imaging (A) and from fetal MR imaging (B).