PMC9630619_01
Photograph of the patient's face shows multiple nodular lesions involving the right eye lids and periorbital skin (arrow). The lesions have a bluish hue. Bluish discoloration is also noted over the forehead and skin over the right temporal region. These findings are suggestive of a vascular malformation.
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A 20-year-old man presented to us with multiple nodular swellings over the eye lids and over the lateral orbital margins on the right side since birth, which were blue in color, noncompressible, nontender, and non-pulsatile (Fig. 1).
(a) Axial CT image shows nodular soft tissue thickening over the right periorbital and right temporal region (arrow). (b) Soft tissue nodular lesion also noted in the intraconal compartment of the orbit on right side. (c) Postcontrast axial CT in venous phase shows progressive enhancement of the soft tissue lesion in right frontoparieto-temporal region which is suggestive of a venous malformation (arrow). (d) Coronal CT venogram shows multiple prominent medullary veins (arrow) converging towards a common collector vein in right periatrial region, suggestive of a developmental venous anomaly. (e) Another right periatrial developmental venous anomaly is noted (arrow) on axial CT venogram. (f) Axial CT venogram shows developmental venous anomaly involving the left cerebellar hemisphere (arrow) which was seen draining into the Vein of Galen.
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Arterial phase of CT angiography did not reveal any enlarged arterial feeders or early draining veins within a large ill-defined swelling involving the subcutaneous tissues over the right frontal, temporal, and parietal region, extending over the eye lids on the right side (Fig. 2a). Multiple nodular soft-tissue attenuation lesions were also noted within the intraconal compartment in the right orbit (Fig. 2b). There was progressive enhancement of these lesions on subsequent venous phase, suggesting venous vascular malformations (Fig. 2c). DVA (Fig. 2d). DVA were also noted in right periatrial white matter (Fig. 2e). Bilateral cerebellar DVA were also noted, draining into the vein of Galen (Fig. 2f).
(a) Axial CT venogram shows an abnormal venous structure along the right frontoparietal convexity (arrow) which could represent a meningeal venous angioma. (b) Coronal CT venogram shows the abnormal venous structure along the right frontoparietal region communicating with the extracranial venous malformation through transdiploic venous channels (arrow). (c) Axial CT venogram shows venous ectasia (arrow) along the course of the right superficial middle cerebral vein within the right Sylvian fissure. (d) Coronal CT venogram shows prominence of right choroid plexus (orange arrow) and abnormal venous channels along the right tentorial leaflet (blue arrow).
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Abnormal venous structures were noted along the right frontotemporo-parietal convexity (Fig. 3a) which were seen communicating with the soft tissue thickening over the scalp through transdiploic venous channels (Fig. 3b). The right basal vein of Rosenthal and right superficial middle cerebral vein (SMCV) were prominent on the right side with venous ectasia along the right SMCV (Fig. 3c). The choroid plexus on the right side appeared prominent and abnormal venous channels were noted along the right tentorial leaflet (Fig. 3d).
(a) T2W axial images show evidence of fluid-fluid levels within the venous malformation along the scalp in right frontoparieto-temporal region (arrow). (b) Axial susceptibility weighted image (SWI) shows prominence of medullary veins in right periventricular white matter (arrow) converging towards a common collector vein and forming a developmental venous anomaly. (c) Axial SWI image shows a developmental venous anomaly in right periatrial region (arrow). (d) Axial SWI image shows developmental venous anomalies in bilateral cerebellar hemispheres (arrows) draining into the Vein of Galen. (e) MR venogram shows transdiploic communication (arrow) between the venous angioma along the right parietal convexity and extracranial scalp venous malformation. (f) Maximum intensity projection (MIP) of the MR venogram shows abnormal dilatation of the right transverse and sigmoid sinuses (arrow) suggestive of dural venous sinus malformation.
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MRI demonstrated multiple fluid-fluid levels within the orbital and scalp venous malformation on T2W images (Fig. 4a). Multiple DVAs were appreciated on SWI sequence, correlating with the CT findings (Figs. 4b-d). Phase contrast MR venogram showed the abnormal venous structures along the right frontotemporo-parietal convexity, communicating with the scalp venous malformation through transdiploic venous connections (Fig. 4e). The posterior part of superior sagittal sinus and right transverse sinus were abnormally enlarged (Fig. 4f).