Abstract
Introduction Sturge–Weber syndrome (SWS) is a rare congenital disease that affects the brain, skin, and eyes, and is a sporadically occurring neurocutaneous syndrome that affects intracerebral veins, which is associated with venous thrombosis. However, intracranial hemorrhage in patients with SWS is rare. We herein report a rare case of SWS with intracerebral hemorrhage derived from sinus thrombosis.Case descriptionA 62-year-old man suddenly fell into a coma and was admitted to our hospital. His neurological status was assessed as GCS 6 (E1V1M4) with right-sided hemiparesis. At birth, he had a right-sided facial port-wine stain typical of SWS that involved the ophthalmic division of the trigeminal nerve. Laboratory findings showed that he was dehydrated, and his serum D-dimer concentration was increased. Computed tomography revealed left thalamic hemorrhage with acute hydrocephalus and cortical calcification in the right occipital lobe. Magnetic resonance imaging displayed a vascular malformation of the right cerebral hemisphere consistent with SWS. Magnetic resonance venography showed steno-occlusion of the superior sagittal sinus, straight sinus, and left internal cerebral vein (ICV). Emergency ventricular drainage was performed. Seven days after surgery, his consciousness improved to GCS 14 (E4V4M6). Rehydration therapy was performed to prevent sinus thrombosis.Discussion and EvaluationHis postoperative course was uneventful. Sudden congestion of the left ICV may have caused left thalamic hemorrhage.ConclusionsSWS with major sinus occlusion needs to be diagnosed with utmost caution in order to allow for preoperative neurological and radiological assessments.
Highlights
Sturge–Weber syndrome (SWS) is a rare congenital disease that affects the brain, skin, and eyes, and is a sporadically occurring neurocutaneous syndrome that affects intracerebral veins, which is associated with venous thrombosis
SWS with major sinus occlusion needs to be diagnosed with utmost caution in order to allow for preoperative neurological and radiological assessments
SWS is a congenital neurocutaneous syndrome that is characterized by facial angioma in the upper facial region and intracranial leptomeningeal angiomatosis is common
Summary
Sturge–Weber syndrome (SWS) is a rare congenital disease that affects the brain, skin, and eyes, and is a sporadically occurring neurocutaneous syndrome that affects intracerebral veins, which is associated with venous thrombosis. A few cases of SWS with intracranial hemorrhage have been reported to date (Table 1). We present a rare case of SWS with intracerebral hemorrhage derived from sinus thrombosis. We speculated that intracranial hemorrhage may have been derived from sinus thrombosis; magnetic resonance imaging (MRI) was performed. Gadolinium-enhanced MRI showed left thalamic hemorrhage, leptomeningeal enhancement in the right temporo-occipital lobe, and choroid plexus thickening with enhancement on both sides (Fig. 2a).
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