Longitudinal Neuroimaging of a Pediatric Patient With Sturge–Weber Syndrome: From Birth to Adolescence

Figures

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  Figure 1. Contrast-enhanced MRI brain image at one month of age Post-contrast T1-weighted non-fat-saturated axial image (red arrow in A) demonstrates enhancing pial angiomas over the right cerebral hemisphere. Post-contrast T1-weighted non-fat-saturated axial image (yellow arrow in B) shows a diffuse enhancing choroidal hemangioma of the right eye and (White arrow in B) indicates asymmetric enlargement of the right choroid plexus, confirming the diagnosis of Sturge–Weber syndrome.

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  Figure 2. Results of imaging studies done at one month, one year, and 12 years of age Red arrows in A, B, and D (axial post-contrast T1-weighted non-fat-saturated images at one month, one year, and 12 years of age, respectively) demonstrate enhancing pial angiomatosis. The red arrow in C (axial non-contrast Fluid-Attenuated Inversion Recovery (FLAIR) image at six years of age) shows pial angiomatosis as sulcal hyperintensity with underlying parenchymal hyperintensity reflecting hypoxic injury. These images illustrate the persistent but increasingly prominent appearance of angiomatosis with age, largely due to progressive underlying cerebral atrophy.

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  Figure 3. Axial GRE images Axial Gradient Echo (GRE) images (A–C) show progressive gyriform calcifications as blooming artifacts (red arrows) at one month, five years, and 12 years of age. Corresponding axial CT images (D–F) demonstrate the same process (green arrows) at one year, six years, and 10 years, illustrating the age-related progression of calcifications in Sturge–Weber syndrome.

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  Figure 4. Axial non-fat-saturated T1-weighted images Axial non-fat-saturated T1-weighted images (A–F) demonstrate progressive right cerebral atrophy (red arrows) at one month, one year, five years, six years, 10 years, and 12 years of age, respectively.

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  Figure 5. Axial CT bone window images at one year and 11 years of age Axial CT bone window images at one year (A) and 11 years (B) demonstrate progressive right-sided bony hemi-calvarial hypertrophy (red arrows), confirmed by interval increase in calvarial thickness measurements, i.e., from the initial 1-2 mm to follow-up thickness of 4-14 mm.

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  Figure 6. Axial CT bone window images Axial CT bone window images (A–C) demonstrate frontal sinus pneumatization with right-sided enlargement (red arrows) at one, six, and 11 years of age, respectively. Images (D–F) show ethmoidal and sphenoidal sinus pneumatization with right-sided enlargement (green arrows)at one, six, and 11 years of age, respectively.

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  Figure 7. Lateral and frontal scout CT images at six years of age Lateral (A) and frontal (B) scout CT images at six years of age demonstrate right-sided gyriform ‘tram-track’ calcifications of the skull, corresponding to underlying parenchymal calcifications.

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  Figure 8. Axial post-contrast T1 non-fat-saturated images Axial post-contrast T1 non-fat-saturated images (A–B) and MIP reconstructions (C–F) demonstrate right cerebral pial angiomatosis (red arrows), a right-sided developmental venous anomaly (yellow arrow), and aberrant venous channels (green arrows) connecting prominent subependymal and superficial veins. Arterial abnormality is also seen, with a relative hypoplastic right middle cerebral artery (blue arrow). MIP: Maximum intensity projection.

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  Figure 9. Axial CT bone window image Axial CT bone window image (A) and 3D reconstruction (B) demonstrate right eye Baerveldt tube implantation (red arrow) for glaucoma management, performed at seven years of age.