Article Figures & Data
Figures
- 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).
Tables
Region Reader 1 Reader 2 ICC NAR Infant Treatment P Value NAR Infant Treatment P Value No. Median IQR No. Median IQR No. Median IQR No. Median IQR Varix ML (mm) 10 18 13–22 4 19 11–25 1.00 10 18 13–24 5 20 12–23 1.00 0.98 Varix AP (mm) 10 30 28–33 4 19 12–25 <.01 9 30 21–34 5 20 16–27 .15 0.75 Varix CC (mm) 10 20 18–21 4 16 8–25 .64 10 18 12–21 5 18 11–23 .77 0.89 Varix V (CM3) 10 5 2–5 4 3 1–5 .73 9 5 2–6 5 5 2–6 1.00 0.93 SS-MD (mm) 10 10 8–12 4 6 4–6 <.01 10 8 5–11 5 3 3–5 <.001 0.95 SS-P (mm) 9 43 39–49 4 28 22–34 <.001 10 39 28–41 5 20 17–31 <.01 0.87 SS-A (mm2) 10 85 56–112 5 20 18–45 <.01 BA-MD (mm) 5 3 3–4 3 3 2–0 .57 R ICA-MD (mm) 5 4 3–5 3 3 2-0 .57 L ICA-MD (mm) 5 3 3–5 3 3 2-0 .39 R Sig-MD (mm) 8 10 9–12 4 6 4–8 <.001 10 7 6–8 5 5 4–6 <.001 0.72 L Sig-MD (mm) 8 11 7–12 4 6 5–7 .05 10 9 7–9 5 6 4–6 <.01 0.94 Note:—AP indicates anterior-posterior diameter; IQR, interquartile range; L, left; ML, mediolateral diameter; R, right; V, volume; ICC, intraclass correlation coefficient. Statistical significance was set using a conservative criterion of P < .01 to account for multiple testing.
Region Reader 1 Reader 2 ICC NAR Infant Treatment P NAR Infant Treatment P No. Med IQR No. Med IQR No. Med IQR No. Med IQR Varix ML (mm) 21 24 18–27 11 21 19–25 .46 21 23 17–27 11 21 18–26 .97 0.921 Varix AP (mm) 21 30 19–35 11 20 20–27 .22 21 30 25–35 11 22 20–29 .05 0.633 Varix CC (mm) 20 24 20–27 11 19 16–24 .16 21 21 16–29 11 18 15–24 .14 0.875 Varix V (CM3) 21 6 4–10 11 4 3–8 .24 20 9 4–13 11 4 3–8 .15 0.961 SS-MD (mm) 17 10 8–14 11 6 5–8 .00 21 9 7–11 11 3 2–6 <.001 0.983 SS-A (mm2) 21 6 4–8 11 3 3–4 .00 20 85 65–123 11 28 18–55 <.001 SS-P (mm) 17 40 36–48 11 29 21–37 .00 20 35 32–43 11 27 21–35 .03 0.969 BA-MD (mm) 21 4 3–5 11 4 3–4 .03 21 4 3–4 11 3 2–3 <.01 0.877 R ICA-MD (mm) 21 4 4–5 11 3 2–4 .00 21 4 3–4 11 3 2–3 <.001 0.851 L ICA-MD (mm) 21 4 3–5 11 3 3–3 .00 21 4 3–4 11 3 2–3 <.01 0.655 R Sig-MD (mm) 21 9 7–10 11 7 4–9 .04 21 6 5–8 11 5 2–6 .02 0.642 L Sig-MD (mm) 21 8 8–11 11 7 6–9 .10 21 7 6–8 11 4 3–6 <.001 0.775 Note:—Med indicates median; AP, anterior-posterior diameter; IQR, interquartile range; L, left; ML, mediolateral diameter; R, right; V, volume; ICC, intraclass correlation coefficient. Statistical significance was set using a conservative criterion of P < .01 to account for mutliple testing.
- Table 3:
Presence of VOGM morphology in early MR imaging scan data for neonatal at-risk and infant treatment cohorts
VOGM Morphology Choroidal Mural Total Reviewer 1 Cohort NAR 19 2 21 IT 9 2 11 Total 28 4 32 χ²(1, n = 32) = 0.495, P = .48 Reviewer 2 Cohort NAR 21 0 21 IT 4 7 11 Total 25 7 32 χ²(1, n = 32) = 17.105, P < .001 κ = 0.48, P = .05 - Table 4:
Presence of occipital sinus morphology in early MR imaging scan data for neonatal at-risk and infant treatment cohorts
Presence of Occipital Sinus No Yes Total Reviewer 1 Cohort NAR 0 21 21 IT 6 5 11 Total 6 26 32 χ²(1, n = 32) = 14.1, P < .001 Reviewer 2 Cohort NAR 0 21 21 IT 4 7 11 Total 4 28 32 χ²(1, n = 32) = 8.3, P < .01 κ = 0.819, P < .001 AUC 95% CI P Value Cutoff Value Sensitivity Specificity Neonatal SS-MD 0.866 0.736–0.996 <.001 >6.2 mm 86% 82% SS-A 0.836 0.690–0.983 .002 >58 mm2 80% 82% Fetal scans SS-MD 0.980 0.919–1.000 .003 >5.2 mm 90% 100% SS-A 0.941 0.823–1.000 .007 >33 mm2 90% 80% ↵a Probabilities determined by logistic regression, where straight or falcine sinus diameter on MR imaging was identified as a multivariable predictor of clinical outcome (AUC = 0.89, P < .001).
- Table 6:
Likelihood of clinical evolution to NAR as a function of the mediolateral diameter of the straight or falcine sinus at its shortest pointa
Straight or Falcine Sinus Diameter (mm) Probability of NAR 95% CI 0 4% 1%–30% 1 8% 2%–38% 2 14% 3%–44% 3 24% 8%–52% 4 38% 18%–60% 5 53% 33%–72% 6 68% 49%–83% 7 80% 60%–92% 8 88% 69%–96% 9 93% 75%–98% 10 96% 80%–99% 11 98% 84%–99% 12 99% 87%–99% 13 100% 90%–100% ↵a Probabilities determined by logistic regression, where straight or falcine sinus diameter on MR imaging was identified as a multivariable predictor of clinical outcome (AUC = 0.89, P < .001).
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