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

Fellows’ Journal Club

The authors aimed to identify brain MR imaging characteristics obtained from fetal and early neonatal scans that can predict the clinical presentation. 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 and cross-sectional area at the narrowest point of the straight/falcine sinus were most predictive of clinical evolution into the neonatal at-risk cohort. This measurement clearly and unambiguously differentiated between high- and low-risk cohorts. The ability to accurately predict clinical evolution after birth based on fetal MR imaging can be of help for both caregivers and families, enabling better preparedness for urgent treatment and better planning for allocation of resources.

Abstract

Figure 1 from Arko et al
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).

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.

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