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.     

Sturge-Weber syndrome. The current neuroradiologic data

Sturge-Weber syndrome (SWS) is a rare congenital sporadic disease with neuro-ocular and cutaneous vascular findings. Clinically, the full-blown condition consists of a facial port-wine stain (PWS) involving the V1 facial trigeminal skin area, alone or in combination with V2 and V3PWS, seizures and ocular abnormalities (glaucoma and choroidal angioma). Radiologically, a leptomeningeal (pial) capillary and venous malformation, mostly located in the parieto-occipital area, cerebral atrophy and calcifications are demonstrated. An ipsilateral enlarged choroid plexus may be an early anatomic symptom. Developmental venous anomalies (DVA) of the brain are sometimes associated. MR with gadolinium enhancement is the optimal neuro-diagnostic imaging technique for the screening of infants with an at-risk V1PWS, as well as for the follow-up of patients with evidence SWS. Accelerated myelination in the involved hemisphere may be an early diagnostic feature before 6 months of age. Later, hyperintensity of white matter on T2 is considered a symptom of gliosis. Clinically, progression of the diseases is associated with anatomic changes and correlates with the extent of the pial vascular anomaly, extent and severity of cerebral atrophy, and white matter abnormalities. A neonatal neuro-imaging work-up, using CT or MRI, may not demonstrate the pial anomaly and should be repeated after 6 to 12 months in an at-risk infant with V1PWS.     

Sturge-Weber综合征在小于1岁婴儿的颅脑MRI表现

目的：回顾性分析Sturge-weber综合征在小于1岁婴儿中的颅脑MRI表现及其临床诊断价值。方法：回顾性分析7例小于1岁的Sturge—Weber综合征患儿的MRI图像,7例行MRI平扫,6例行MRI增强扫描。结果：本组患儿均有面部三叉神经分布区的血管瘤。5例病变累及单侧大脑半球,均可见病变区脑白质于T2wI上信号明显减低,伴有局部脑萎缩改变,其中4例可见患侧脉络丛扩大;2例累及双侧大脑半球脑叶。6例增强后均可见脑回表面软脑膜强化,其中5例可见异常强化的软脑膜血管瘤,1例同时见幕下结构受累。结论：对临床怀疑为Sturge—Weber综合征的婴儿,MRI增强扫描可以帮助明确诊断及确定病变范围,为临床评估患儿的预后提供有效的参考信息。     

MR susceptibility weighted imaging (SWI) complements conventional contrast enhanced T1 weighted MRI in characterizing brain abnormalities of Sturge-Weber Syndrome

PURPOSE: To evaluate the efficacy of susceptibility weighted imaging (SWI) in comparison to standard T1 weighted postgadolinium contrast (T1-Gd) MRI in patients with Sturge-Weber Syndrome (SWS). MATERIALS AND METHODS: Twelve children (mean age, 5.6 years) with the diagnosis of SWS and unilateral hemispheric involvement were recruited prospectively and examined with high resolution three dimensional SWI and conventional T1-Gd. Both SWI and T1-Gd images were evaluated using a four-grade scoring system according to six types of imaging findings (enlargement of transmedullary veins, periventricular veins, and choroid plexus, as well as leptomeningeal abnormality, cortical gyriform abnormality, and gray matter/white matter junctional abnormality). The scores of SWI versus T1-Gd images were then compared for each type of abnormality. RESULTS: SWI was superior to T1-Gd in identifying the enlarged transmedullary veins (P = 0.0020), abnormal periventricular veins (P = 0.0078), cortical gyriform abnormalities (P = 0.0020), and gray matter/white matter junction abnormalities (P = 0.0078). Conversely, T1-Gd was better than SWI in identifying enlarged choroid plexus (P = 0.0050) and leptomeningeal abnormalities (P = 0.0050). CONCLUSION: SWI can provide useful and unique information complementary to conventional contrast enhanced T1 weighted MRI for characterizing SWS. Therefore, SWI should be integrated into routine clinical MRI protocols for suspected SWS.     

鼻腔鼻窦原发恶性黑色素瘤的影像学表现

目的：分析鼻腔鼻窦原发恶性黑色素瘤CT及 MR 表现,探讨其诊断要点。方法回顾性分析经活检或手术病理及免疫组化证实的16例鼻腔鼻窦恶性黑色素瘤的CT、MR特征及临床资料,均行 CT 平扫及增强,其中9例行 MR 平扫及增强。结果16例CT表现均为单侧发病;肿瘤位于鼻腔8例,鼻窦4例,同时累及鼻腔、鼻窦4例;形态欠规整,呈软组织密度,其内未见钙化和囊变;周围组织均受侵;增强后不均匀中-重度强化。9例 MR表现均为单侧发病;肿瘤位于鼻腔4例,鼻窦3例,同时受累鼻腔、鼻窦2例;形态欠规则,1例呈T1 WI高信号,T2 WI低信号,3例呈T1 WI等信号,T2 WI等/稍高信号,5例呈混杂信号。增强后不均匀轻-中度强化;周围组织均有侵犯。结论鼻腔鼻窦原发恶性黑色素瘤 CT表现缺乏特异性;典型恶性黑色素瘤有 MR 特征性表现,但罕见,以非色素及混合型常见。CT联合 MRI能准确显示肿瘤部位及与周围组织、邻近骨质的关系。     

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.     

Sturge-Weber综合征的CT分析(附13例报告)

目的 探讨Sturge-Weber综合征的病理变化和CT表现特征.方法 收集13例Sturge-Weber综合征的临床和CT资料,所有病例均进行常规螺旋CT扫描.结果 在CT像上,13例均见病灶内钙化,其中,轻度钙化4例,中度钙化8例,重度钙化1例;脑萎缩10例,其中,6例受累脑叶灰白质分界不清;脉络丛扩大3例;颅骨增厚6例.结论 脑叶皮层“脑回样”钙化是Sturge-Weber综合征的特征性表现,CT是确诊该病的重要检查手段.     

儿童头颈部横纹肌肉瘤的CT及MR影像学特点

 目的 探讨儿童头颈部横纹肌肉瘤(rhabdomyosarcoma,RMS)的CT及MR影像学特点。方法 回顾性分析29例病理证实的儿童头颈部RMS的影像学资料,其中23例行MRI检查,18例行CT检查。结果 10例发病部位位于鼻咽部,4例位于鼻腔及鼻窦,5例位于鼻翼部,3例位于颌面部,6例位于耳部,1例位于左侧颞下窝。肿瘤形态不规则,呈浸润性生长,多侵蚀破坏邻近骨质。CT图像病灶密度不均匀,瘤体内均无钙化。MR检查T₁WI呈等或稍低信号,T₂WI呈稍高信号,信号不均匀,增强后瘤灶明显不均匀强化。8例侵犯眼眶,13例进入并侵犯鼻腔,14例侵犯副鼻窦,15例侵犯颞骨乳突,12例侵犯颞骨岩部,14例突破颅底,累及硬脑膜,同时4例侵犯邻近脑组织,2例发生脑脊液转移。结论 儿童头颈部RMS好发于脑膜旁区。CT能够很好地显示肿瘤对周围骨质结构的破坏,而MR能更清楚显示肿瘤软组织受累范围,两者结合可提供更全面的影像信息。     

Diagnostic imaging in 13 cases of Rasmussen's encephalitis: can early MRI suggest the diagnosis?

Rasmussen's encephalitis (RE) is a rare, progressive, chronic encephalitis characterised by drug-resistant epilepsy, progressive hemiparesis and mental impairment. It typically involves only one cerebral hemisphere, which becomes atrophic. We present neuroradiological findings in 13 children with RE. MRI was performed in all patients, fluorodeoxyglucose positron-emission tomography (PET) in three, Tc-99m hexamethylpropylenamine oxime single-photon emission computed tomography (SPECT) in two and proton MR spectroscopy ((1)HMRS) in two. MRI showed progression of the hemisphere atrophy, always prevalent in the region primarily involved (13 patients), spread of the abnormal signal in white matter (11) and cortex (10) and progression of atrophy of the head of the caudate nucleus (nine). Associated secondary changes were: atrophy of the contralateral cerebellar hemisphere (in four patients), the ipsilateral hippocampus (in five) and the brain stem (in five). The earliest CT and MRI abnormalities, seen between 1 day and 4 months after the first seizure (in 12 patients examined, nine of whom had MRI) in one cerebral hemisphere included: high signal on T2-weighted images in the cortex (seven patients) and white matter (nine), cortical atrophy usually involving the frontoinsular region, with mild or severe enlargement of the lateral ventricle (eight) and moderate atrophy of the head of the caudate nucleus (seven). Cortical swelling in the early stage of the disease was recognisable only in two patients. PET revealed hypometabolism, SPECT decreased perfusion, and (1)HMRS reduction of N-acetylaspartate in the affected hemisphere. PET and SPECT were usually performed in the late stages and did not provide specific findings. MRI thus demonstrates the progression of RE and may suggest the diagnosis in the early stages, often before the appearance of neurological deficits. Early diagnosis of RE may be crucial for selecting patients for aggressive medical therapy or major surgical interventions such as hemispherectomy.     

MR imaging and CT in three cases of Sturge-Weber syndrome: prospective comparison

Three patients with Sturge-Weber syndrome (5 months, 6 years, and 17 years old) were studied prospectively with MR imaging and CT of the brain. Both techniques demonstrated parenchymal volume loss, choroid plexus enlargement, calvarial hemiatrophy, and proptosis. In regions of parenchymal volume loss, MR alone demonstrated thickened cortex with decreased convolutions and abnormal white matter. A focal thalamic lesion and prominent medullary and subependymal veins were also shown better by MR. However, CT definitively demonstrated the characteristic cortical calcification, while T2-weighted MR images detected only smaller, nonspecific foci of hypointense signal. MR and CT are complementary in the evaluation of Sturge-Weber syndrome.     

鼻窦真菌球的 CT 及磁共振成像表现

目的：探讨鼻窦真菌球的CT、磁共振成像（MRI）特征性表现及诊断价值。方法回顾性分析35例经手术、组织病理学证实的鼻窦真菌球的影像学资料,35例患者均做CT平扫,其中4例做MRI平扫加增强扫描。结果鼻窦真菌球的主要CT表现为：①病变为单侧性（100%）;②病变部位以上颌窦为主（80%）,部分突入同侧鼻腔;其次为蝶窦（14%）;③病变窦腔内不均匀软组织密度影充填,其内可见呈斑点状、云絮状或条带状高密度钙化影（89%）;④局限性骨质增生（74%）及破坏（9%）。 MRI表现为：病变中心T1WI呈等信号,T2WI呈低信号,周围增厚黏膜T1WI呈低信号,T2WI呈高信号,增强扫描病变中心不强化,周围增厚黏膜明显强化。结论病变内钙化影是本病特征性的CT征象,是诊断该病较为可靠的依据,CT检查是诊断本病的重要手段;MRI能显示病变内真菌球的范围,有助于诊断。     

CT and MR of angiomatous malformations of the choroid plexus in patients with Sturge-Weber disease

Eight patients with Sturge-Weber disease were evaluated by CT (six patients), MR (one patient), or both (one patient). CT scans of five of seven patients showed enlargement and increased enhancement of the choroid plexus on the same side as the facial and intracranial lesions. MRI showed similar findings in both patients examined. This enlargement, seen in six of eight cases of Sturge-Weber disease, is compatible with the presence of angiomatous malformations of the choroid plexus. It appears to be a common finding in this disease.     

低颅压综合征的MRI诊断

目的 分析低颅压综合征的MRI表现,以提高对本病的认识. 资料与方法 搜集经临床证实的低颅压综合征10例,治疗前均行MRI检查,其中3例仅行平扫,7例同时平扫和增强,3例加MRA,1例加MRV. 结果 10例均有硬脑膜增厚、静脉窦和脑静脉扩张;6例可见脑下垂和脑室缩小表现;4例可见侧枝静脉窦显影(基底丛和岩下窦);5例可见垂体增大;4例出现硬膜下积液;增强7例均见硬脑膜均匀性增厚强化,软脑膜无强化,2例见脊膜强化. 结论 低颅压综合征具有特征性的MRI表现,结合脑脊液压力检测,可以明确诊断.     

Cranial imaging in autosomal recessive osteopetrosis. Part I. Facial bones and calvarium.

Cranial imaging studies (radiographs, computed tomographic [CT] scans, magnetic resonance [MR] images, and bone marrow scintigrams) in 13 infants and children with autosomally recessive osteopetrosis were reviewed to characterize patterns of facial and calvarial involvement at presentation and with progression of disease. In the mandible, a characteristic triangular opacity representing calcification within the secondary condylar cartilage ossification center was seen in 10 of the 13 patients. Defective dentition with incomplete enamel formation and/or caries was encountered in all patients. The paranasal sinuses were poorly pneumatized in all patients, but the ethmoid sinuses tended to be the least severely affected. Hypertelorism was present in five of the 13 patients, with a characteristic "space-alien" appearance on frontal radiographs. In younger patients, the calvarium demonstrated a high-attenuation inner table, a broad, low-attenuation diploic space, and a less high-attenuation outer table at CT. In three older children, a "hair-on-end" appearance was seen, which, at bone marrow scintigraphy, corresponded to areas of marked hematopoietic activity. Regions of sclerotic bone demonstrated low signal intensity on both T1- and T2-weighted MR images; areas containing marrow had intermediate signal intensity. These many new radiologic features of osteopetrosis are related to its pathophysiologic characteristics.     

Dyke-Davidoff-Masson syndrome

We reported five cases of Dyke-Davidoff-Masson syndrome (DDMS) with different clinical and radiological findings. The evaluated parameters were the location of the lesions, midline structural shift effect, pathological and morphological changes on the ipsilateral calvarium, paranasal sinuses and mesencephalon, presence of compensatory contralateral hypertrophy. With the help of both magnetic resonance (MR) and computerized tomography (CT) images, changing degrees of all the evaluated parameters were observed in all five of our patients. In conclusion, no relationship was found between parenchymal and calvarial changes and between the time after onset of the disease and amount of the morphologic and pathological changes.     

Sinonasal computed tomography in patients with Wegener's granulomatosis

The goal of this study was to describe pathologies of sinonasal CT in patients with Wegener's granulomatosis. Between 1993 and 2004, sinonasal CT was performed in 28 patients (15 male, 13 female) with Wegener's granulomatosis. The following imaging findings were assessed on the CT scans: mucosal thickening, subtotal opacification, air-fluid level, bony destruction, sclerosing osteitis, bony thickening, orbital mass, and saddle nose deformity. Of the 28 patients, 61% showed mucosal thickening in the nasal cavity and 75% in the paranasal sinuses. A subtotal opacification of the paranasal sinuses was detected in 25%, and of the mastoid cells in 7% of patients. Fifty-seven percent of patients had bony destruction of the nasal cavity, and 54% of the paranasal sinuses. CT revealed sclerosing osteitis of the paranasal sinuses in 21%, and of the mastoid cells in 18% of patients. Bony thickening of the paranasal sinuses was detected in 18%, and of the mastoid cells in 14% of patients. With respect to all imaging findings, the maxillary sinuses were the most frequently affected paranasal sinuses. The authors conclude that the following sinonasal CT findings are observed in patients with Wegener's granulomatosis: (1) bony destruction, mainly of the nasal cavity, maxillary sinuses, and mastoid cells; (2) sclerosing osteitis, mainly of the maxillary sinuses and mastoid cells; (3) bony thickening, mainly of the maxillary sinuses and mastoid cells; and (4) mucosal thickening, mainly of the maxillary sinuses. These findings are, however, nonspecific and should be combined with other clinicopathologic and laboratory studies to confirm the diagnosis of Wegener's granulomatosis.     

The skull in gonadal dysgenesis. A roentgenometric study.

Skull radiographs of 54 patients with Turner's syndrome have been studied. The following characteristic findings were frequently noted: mastoid processes of definitely decreased dimensions, small facial bones which were proportional to the stunted growth of the patients, enlargement and increased thickness of the mandible, extensive calcification of the petro-clinoid ligaments, excessive pneumatisation of the paranasal sinuses and increased angle of the base of the skull.     

鼻窦和颞骨成骨细胞瘤CT和MRI诊断

目的探讨鼻窦和颞骨成骨细胞瘤CT和MRI表现，提高其诊断准确性。方法回顾性分析8例经病理证实的鼻窦和颞骨成骨细胞瘤的影像学资料，分析其CT和MRI表现。结果6例为良性成骨细胞瘤，2例为侵袭性成骨细胞瘤；发生于鼻窦者6例，颞骨者2例。CT表现：良性成骨细胞瘤边界清楚，圆形2例、椭圆形4例，周边伴有完整的骨壳，病灶内见点状、结节状、条状钙化或骨化，压迫邻近的结构；侵袭性成骨细胞瘤部分边界模糊，形态不规整，骨壳不完整，外周可见软组织肿块，内伴有不规则形的钙化或骨化，侵犯邻近结构。MRI表现：与脑实质比较，T1WI为等信号者4例，低信号者2例，T2WI信号不均匀，但病灶内部均见数量不一的极低信号影，对应CT上钙化或骨化区；4例骨壳在T1WI和T2WI均表现为低信号环，1例骨壳仅在T2WI显示低信号，1例骨壳在T1WI和T2WI均难以显示；增强后扫描呈中至显著强化，良性为较均匀强化，而侵袭性为不均匀强化；邻近脑膜可见不同程度强化，良性较均匀，侵袭性不规则且范围更大。结论CT是该病诊断和鉴别诊断的主要影像检查方法，MI可更清楚、准确显示病变侵犯的范围，为治疗提供可靠依据，可作为1种补充的影像检查方法。     

Non-invasive aspergillosis of the paranasal sinuses: CT and MRI findings

Aspergillosis is the most common fungal infection of the paranasal sinuses, and needs to be recognized because it requires surgical removal. Twenty proven cases of aspergillosis of the paranasal sinuses are reported here. CT was performed in all the cases and MRI in 2 cases. The maxillary sinus was affected in 19 patients and the sphenoid sinus in 1. Mycosis was unilateral in all but 1 of the cases. Foci of increased attenuation at CT were observed in 18 cases, with calcification in 10 cases and/or dental material in 13 cases. An increased bony wall thickness was observed in 16 cases. All the patients but 1 had at least one of the signs. At MRI the fungal mass displayed a hypointense signal on T1- and T2-weighted images. No enhancement was noted on post-contrast T1-weighted images. The diagnosis of paranasal sinus aspergillosis is suggested by the CT findings, when a hyperdense mass with calcifications and/or dental material is noted with thickening of the sinus wall. MRI may have a complementary diagnostic role in doubtful cases. Correspondence to: Y. Robert     

肿瘤软脑膜-蛛网膜转移的CT、MRI诊断

目的研究肿瘤软脑膜蛛网膜转移的ＣＴ、ＭＲＩ表现,并探讨两种检查方法对该病的诊断价值。方法回顾分析２１例肿瘤软脑膜蛛网膜转移的临床及影像学资料。病变经ＣＴ检查１６例,ＭＲＩ检查７例,其中经两种方法检查者２例。结果全部病例软脑膜蛛网膜下腔均出现病理性强化,其中１０例呈弥漫性,８例呈结节性,３例呈弥漫与结节混合性。弥漫性强化沿脑和脑干表面分布,并延伸入脑沟、脑池;结节性强化病灶数目１个或多个不等,直径０．２～３．０ｃｍ。病变见于基底脑池及相邻蛛网膜下腔者共１８例。伴室管膜结节性强化４例,天幕增厚强化１０例,脑积水１３例,合并脑内转移９例。结论增强ＣＴ和ＭＲＩ对病变的诊断具有重要临床意义,且ＭＲＩ优于ＣＴ。但两者在定性诊断上均有局限性。正确诊断有赖于结合临床资料和影像征象的综合分析