Chonan Masashi, Suzuki Yasuhiro, Haryu Shinya, Mashiyama Shoji, Tominaga Teiji
Department of Neurosurgery, Iwaki Kyoritsu Hospital, 16 Kusehara, Uchigo Mimaya-machi, Iwaki, Fukushima 973-8555 Japan.
Department of Neurosurgery, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8575 Japan.
Springerplus. 2016 Oct 7;5(1):1746. doi: 10.1186/s40064-016-3439-z. eCollection 2016.
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.
A 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.
His postoperative course was uneventful. Sudden congestion of the left ICV may have caused left thalamic hemorrhage.
SWS with major sinus occlusion needs to be diagnosed with utmost caution in order to allow for preoperative neurological and radiological assessments.
斯特奇-韦伯综合征（SWS）是一种罕见的先天性疾病，累及脑、皮肤和眼睛，是一种偶发性的神经皮肤综合征，影响脑内静脉，与静脉血栓形成有关。然而，SWS患者发生颅内出血的情况较为罕见。我们在此报告一例罕见的SWS合并源于静脉窦血栓形成的脑出血病例。
一名62岁男性突然昏迷，被送入我院。其神经状态评估为格拉斯哥昏迷量表（GCS）评分为6分（睁眼1分、语言1分、运动4分），伴有右侧偏瘫。出生时，他有典型的SWS右侧面部葡萄酒色斑，累及三叉神经眼支。实验室检查结果显示他脱水，血清D-二聚体浓度升高。计算机断层扫描显示左侧丘脑出血伴急性脑积水以及右侧枕叶皮质钙化。磁共振成像显示右侧大脑半球血管畸形，符合SWS表现。磁共振静脉血管造影显示上矢状窦、直窦和左侧大脑内静脉（ICV）狭窄闭塞。进行了紧急脑室引流。术后7天，他的意识恢复到GCS 14分（睁眼4分、语言4分、运动6分）。进行了补液治疗以预防静脉窦血栓形成。
他术后恢复过程顺利。左侧ICV突然充血可能导致了左侧丘脑出血。
对于伴有主要静脉窦闭塞的SWS，需要极其谨慎地进行诊断，以便进行术前神经学和放射学评估。
