脑颜面血管瘤综合征的CT诊断和临床分析(附8例报告)

目的:探讨脑颜面血管瘤综合征的CT表现特点及临床诊断价值。方法:回顾分析经临床和CT证实的8例脑颜面血管瘤综合征的CT和临床资料。结果:8例脑颜面血管瘤综合征均有脑皮层不同程度的脑回样或条带状钙化,4例伴病侧大脑萎缩,3例伴病侧颅板增厚,1例合并眼部脉络膜血管瘤和侧脑室脉络丛增大。8例均出现与CT表现相对应的临床症状和体征。结论:CT能准确诊断脑颜面血管瘤综合征,对临床有重要的指导意义。     

脑颜面血管瘤病的CT表现

目的：探讨脑颜面血管瘤病的CT表现及其诊断价值。材料和方法：回顾性分析7例脑颜面血管瘤病的临床和CT资料。结果：7例CT表现均有特征性，一则或两侧枕顶叶表浅部位脑回状或曲线状钙化5例，伴病测大脑半球发育不全或萎缩/病侧颅板增厚2例。结论：本病CT表现有特征性，具有重要的诊断价值。     

脑颜面血管瘤病的CT表现

目的: 探讨脑颜面血管瘤病的CT表现及其诊断价值.材料和方法: 回顾性分析7例脑颜面血管瘤病的临床和CT资料.结果: 7例CT表现均有特征性,一侧或两侧枕顶叶或额顶叶表浅部位脑回状或曲线状钙化5例,伴病侧大脑半球发育不全或萎缩/病侧颅板增厚2例.结论: 本病CT表现有特征性,具有重要的诊断价值.     

Sturge-Weber综合征1例报告

患者　女 ,7岁 ,出生后 3个月开始出现癫痫 ,反复发作至今。体检 :左侧肢体乏力 ,智力低下 ,右颜面部可见紫红色血管瘤。CT表现 :头颅不对称 ,右侧大脑半球明显较左侧小 ,右额、颞、顶、枕叶脑回广泛曲线状钙化 ,CT值约 95HU ,右侧脑沟、脑裂增宽、加深 ,右侧脑室较对侧大 (图     

Sturge—Weber氏综合症一例

郭×,女,16岁。出生时即有左侧颜面皮肤血管痣,1岁时右侧肢体发作性抽搐、弛缓性不全瘫痪,智力发育较同龄人明显低下。查体:左侧颜面皮肤大片紫红色扁平状血管瘤,部分隆起呈结节状,累及同侧软腭及口唇左半侧(图1),右侧上下肢肌力Ⅱ—Ⅲ级,肌萎缩明显。头颅平片见整个左侧大脑半球呈典型的双层波浪状钙化(图2)。 CT平扫:左侧颅腔较小,颅板增厚,幕上整个左侧大脑半球皮层及皮层下区广泛脑回状钙化,部分已累及脑髓质,左侧大脑半球及大脑脚明显萎缩,左侧侧脑室和蛛网膜下腔扩大,此外右侧枕叶局部皮层及皮层下区亦可见到片状钙化(图3)。 讨论:Sturge—Weber氏综合症临床主要特征为癫痫发作、颜面皮肤血管瘤及脑部异常钙化。颅内主要病变为软脑膜上血管瘤,常为小静脉型或毛细血管型,大都位于大脑半球后部枕叶或顶枕叶,也可以累及整个半球、异常     

Sturge-Weber综合征的影像学诊断(附11例报告及文献复习)

目的　探讨Sturge -Weber综合征的影像学表现。方法　收集 11例Sturge -Weber综合征的临床及影像学资料 ,10例有面部三叉神经分布区皮肤血管瘤。所有病例都进行了常规CT扫描 ,其中头颅X线平片 2例 ,进行MR检查 8例 ,增强CT检查 5例 ,MRI增强 3例。结果　患侧皮层钙化 11例 ;脑萎缩 9例 ,受累脑叶灰白质分界不清 ;侧脑室脉络丛增大及深静脉血管扩张 7例 ;一侧颅板板障增厚 8例 ;增强脉络丛明显强化并呈囊状扩大 5例 ,患侧脑回状强化及脑表面血管扩张 6例。结论　头颅平片诊断本病作用有限 ,CT和MRI综合分析是确诊本病和判断病情最有价值的影像学方法     

新生儿和婴儿期Sturge-Weber综合征的CT诊断

目的:探讨新生儿和婴儿期Sturge-Weber综合征的CT表现。方法:回顾性分析1例新生儿期和7例婴儿期Sturge-Weber综合征患儿的临床和CT资料,均行常规CT平扫,其中5例同时行增强CT。结果:8例均有面部三叉神经分布区皮肤血管瘤,4例患儿出现患侧皮层钙化,4例未出现皮层钙化的患儿显示受累脑实质密度略增高,伴灰白质分界不清。8例患儿均表现为局部脑外间隙增宽及脑沟增宽等脑萎缩样改变,3例随访患儿出现脑萎缩。5例行增强扫描的患儿出现脑回样强化,4例患侧的侧脑室脉络丛增大,1例双侧Sturge-Weber综合征于10个月随访时出现双侧脉络丛增大。结论:CT对新生儿和婴儿期Sturge-Weber综合征的诊断有重要价值。     

脑颜面血管瘤病CT和MRI诊断

目的 探讨脑颜面血管瘤病的CT和MRI表现，并评价其临床应用价值。方法 分析脑颜面血管瘤病8例，均有颜面部三叉神经分布区皮肤血管痣及同侧颅内相关的病理改变。结果 CT显示患侧皮层钙化8例，脑萎缩6例，颅骨板增厚3例，侧脑室脉络丛增大1例。MRI扫描显示沿皮层弧条状低信号3例，静脉血栓形成1例。结论 CT对脑颜面血管瘤病的诊断有重要价值，MRI可以帮助诊断。     

脑颜面血管瘤病的MRI和CT诊断

目的:探讨脑颜面血管瘤病的CT和MRI表现,并评价其临床价值。方法:回顾分析经临床和CT、MRI证实的8例脑颜面血管瘤病临床及CT、MRI资料。结果:CT显示患侧皮质钙化8例,脑萎缩6例,患侧脑室脉络丛增大1例,颅骨增厚2例。MRI扫描显示沿皮层弧形低信号3例,5例增强检查软脑膜呈线样强化。结论:CT对脑颜面血管瘤病的诊断有重要价值,MRI可以帮助诊断。     

脑面血管瘤综合征的CT诊断

本文报导了9例脑面血管瘤综合征（Sturge－Weber综合征）的CT表现，认为此病CT的特征性改变为：面部血管瘤或痣的同侧大脑表面的高密度的钙化影，呈均匀的宽大锯齿状或脑回样。其它表现为病侧局部或广泛的脑萎缩或发育不良，蛛网膜下胜及脑室相应扩大，中线结构移向患侧。颅板增厚，头颅不对称等。     

CT findings of atypical forms of phakomatosis

Summary Three cases with unusual manifestations of phakomatosis are reported. The first two had clinical symptoms of neurofibromatosis but CT disclosed nodular subependymal calcifications as in tuberous sclerosis. The third one presented with cerebral calcifications as found in both tuberous sclerosis and Sturge-Weber syndrome, though he had no clinical symptoms of phakomatosis.     

Cranial CT and MR in the Klippel-Trenaunay-Weber syndrome.

This report describes the intracranial CT and MR findings in two cases of Klippel-Trenaunay-Weber Syndrome. The findings are 1) markedly enhancing choroid plexuses, 2) severe cerebral atrophy, 3) cerebral calcifications, and 4) angiomatous leptomeningeal enhancement. The findings may resemble those seen in cases of bilateral Sturge-Weber syndrome. The two diseases should be distinguishable by the external stigmata. The authors raise the question of a spectrum of involvement in the angiodysplasias of Klippel-Trenaunay-Weber syndrome and Sturge-Weber syndrome with considerable overlap.     

Diagnosis of Sturge-Weber syndrome: comparison of the efficacy of CT and MR imaging in 14 cases.

Sturge-Weber syndrome is a neurocutaneous syndrome that includes facial and leptomeningeal angiomas. Imaging findings include cerebral lobar atrophy, brain calcifications, choroid plexus enlargement, cranial diplo? prominence, and venous abnormalities. We compared the efficacy of CT and MR imaging in making the diagnosis in 14 consecutive patients. CT, with and without contrast enhancement, was performed in all patients, and 11 of the 14 had MR imaging (eight before and after administration of IV gadopentetate dimeglumine). MR imaging was better than CT in showing the extent and degree of brain parenchymal atrophy, the presumed ischemic changes affecting the gray and white matter, and the cranial diploetic prominence on the affected side. MR imaging after contrast administration permitted a better evaluation of the extent and patency of the leptomeningeal angiomatous malformation and the parenchymal venous anomalies. CT was better than MR imaging in showing the presence and extent of cortical calcifications. Enhanced CT and MR imaging were equal in evaluating the prominence of the ipsilateral choroid plexus. Our experience indicates that contrast-enhanced MR imaging is the method of choice in the diagnosis of Sturge-Weber syndrome. Unenhanced CT should be used only if MR findings are normal, to exclude the presence of intracranial calcifications.     

MR imaging of Sturge-Weber syndrome: role of gadopentetate dimeglumine and gradient-echo techniques

Five patients with Sturge-Weber syndrome were evaluated by conventional noncontrast spin-echo MR imaging, a gradient-recalled echo (GRE) technique, and T1-weighted spin-echo imaging after administration of gadopentetate dimeglumine. In four of five cases the full extent of intracranial disease was appreciated only on the postcontrast images. In one patient precontrast and GRE images were entirely normal, while only the postcontrast study demonstrated extensive involvement of both brain and retina. Nevertheless, some abnormal vessels with higher flows were seen better on precontrast T2-weighted images than on postcontrast T1-weighted images. GRE techniques demonstrated calcifications to best advantage, in one case even better than on CT. Contrast enhancement with gadopentetate dimeglumine is necessary for the complete MR evaluation of patients with suspected Sturge-Weber syndrome. Traditional noncontrast T2-weighted and GRE images may provide additional complementary information.     

The Sturge-Weber syndrome: comparison of MR and CT characteristics

Four patients with Sturge-Weber syndrome were evaluated with CT and MR. MR demonstrated the characteristic features of the disease: cerebral atrophy (four patients), ipsilateral bone and sinus hypertrophy (three), ocular findings (one), intracranial calcification (four), prominent deep venous system (three), and enlarged choroid plexus (two). CT demonstrated the following: cerebral atrophy (four), ipsilateral bone and sinus hypertrophy (three), calcification (four), gyral enhancement (two), prominent deep venous system (two), and enlarged choroid plexuses (three). The features of Sturge-Weber syndrome were visualized equally well with MR and CT with the exception of intracranial calcification. Conventional spin-echo MR revealed fewer calcifications, and those visualized appeared smaller than with CT. Gradient-echo acquisition sequences were more effective in the detection of intracranial calcification.     

特发性甲状旁腺机能减退症颅脑CT及MRI表现

目的探讨颅脑CT及MRI检查对特发性甲状旁腺机能减退症(IHP)的诊断价值。方法回顾性分析16例经临床确诊的IHP患者头颅CT(16/16)和MRI(3/16)表现,观察病变的范围和密度/信号等特点。结果IHP颅内主要表现为多发钙化性病变,在CT上表现为高密度,CT值114～373HU,在MRT1WI呈稍高信号或高信号,T2WI呈等信号。双侧尾状核、苍白球、壳核及丘脑的对称性、条形或团块状钙化16例;双侧额、颞、顶、枕叶皮层下钙化10例;小脑齿状核钙化6例(双侧4例,单侧2例);侧脑室旁钙化2例。结论CT和MR对IHP的诊断非常重要,其中CT对病变的显示优于MR,与临床结果结合,对IHP具有定性诊断意义。     

Abnormal ocular enhancement in Sturge-Weber syndrome: correlation of ocular MR and CT findings with clinical and intracranial imaging findings.

PURPOSETo estimate the prevalence of abnormal ocular enhancement in children with Sturge-Weber syndrome as detected with MR imaging and CT and to correlate this with the clinical, fundoscopic, and intracranial imaging findings.METHODSFifteen children, 4 years old or younger, with Sturge-Weber syndrome were examined with enhanced CT and MR imaging. Eleven children had unilateral intracranial involvement and 4 had bilateral involvement, for a total of 19 abnormal hemispheres and related orbits. The presence of ocular enhancement was compared with the fundoscopic findings independently. Ocular enhancement was correlated with the extent of leptomeningeal disease, the severity of the cutaneous lesion, and the presence of glaucoma by the calculation of likelihood ratios and 95% confidence limits.RESULTSSeven of the 15 patients had abnormal ocular enhancement, which was present in 10 (53%) of the eyes associated with the 19 abnormal hemispheres. MR imaging showed choroidal hemangioma in 7 of 8 patients in whom hemangiomas were shown at fundoscopy. The likelihood of ocular enhancement was increased with the presence of bilateral disease, extensive facial nevi, and glaucoma; there was no significant correlation with the extent of hemispheric involvement.CONCLUSIONBoth enhanced MR imaging and CT can show diffuse choroidal hemangioma in patients with Sturge-Weber syndrome. However, MR imaging is more sensitive and is recommended to aid in the detection of abnormalities with preventable late complications.