Regional association of developmental venous anomalies with angiographically occult vascular malformations

This study reviews the neuroradiological findings of 43 patients with a developmental venous anomaly In in order to the clinical significance of this entity. All patients underwent unenhanced and contrast-enhanced computer tomography and magnetic resonance tomography, as well as selective angiography, and were followed for at least 2 years In 40% (17 of 43) of patients a cryptic vascular malformation found In the proximity to the developmentmental venous anomaly. Neurolo gical symptoms were present in 8 of 17 patients (47%) in this group. Patients with an isolated developmental venous anomaly had symptoms in 19% (5 of 26), but none of them had experienced a hemorrhage. Magnetic resonance was the most sensitive method for the diagnose of both types of lesions and alterations of the adjacent parenchyma. These results further support that developmental venous anomalies represent a clinically benign entity. However, patient, with an sociation of a developmental venous anomaly and a cryptic vascular malformation are at risk for hemorage from their angiographically occult vascular malformation. Magnetic resonance proved to be the imaging modality of choice for both entities and is appropriate for diagnosis and follow-up.     

Magnetic resonance angiography of cerebral developmental venous anomalies: its role in differential diagnosis

Summary CT, MRI and contrast angiography of 20 patients with 21 developmental venous anomalies (DVAs), so-called venous angiomas, were compared with magnetic resonance angiography employing a two-dimensional time-of-flight technique (2D-MRA). MRA was diagnostic in 17 DVAs, when both the primary 2D slices and the maximum-intensity-projection images were read. Contrast angiography still provides the best visualization of both DVA components: dilated medullary veins and transcerebral draining vein; however, it is an invasive procedure and delivers no information about brain parenchyma. We regard MRI as necessary in cases with a suspected DVA because of the high rate of association with cavernomas: 33% in this study. Acute neurological symptoms were caused by haemorrhage from an associated cavernoma and not from the DVA in 4 such cases. Thus MRA combined with MRI obviates angiography in most cases and offers a noninvasive diagnostic strategy adequate for DVAs.     

脑发育性静脉异常的影像学诊断

目的:分析脑发育性静脉异常的CT、MRI和DSA表现,提高对本病的认识。方法:17例脑发育性静脉异常(DVA)患者中15例行MRI检查、CT检查6例、DSA检查4例。对DVA在不同影像检查的表现进行分析,对DVA的部位、分型、引流方式以及合并的其它异常进行探讨。结果:17例患者有21个DVA,其中3例多发,4例患者合并有海绵状血管瘤。21个DVA中幕上7个,幕下14个,根据其位置分为浅型(皮层和皮层下区)12个和深型9个,其引流静脉中单支引流14例,多支引流3例。增强CT、增强MRI和DSA静脉期均能清晰显示其特征性表现既"水母头"状扩张的髓静脉汇入粗大的引流静脉干。结论:增强CT、MRI以及DSA检查均能正确诊断和评价DVA,DSA是诊断DVA并与其它血管畸形鉴别的金标准;多种序列组合使MRI能更全面的评价DVA及其合并的其它脑实质异常,是DVA检查的首选方法。     

The spectrum of symptomatic arterialized developmental venous anomalies: case reports

Developmental venous anomaly (DVA) is an anatomical variation of the intracranial venous system, usually without clinical repercussion. In most cases, DVAs are incidentally diagnosed and should be considered as benign conditions. In rare circumstances, DVAs may become symptomatic due to mechanical or flow-related etiologies. The authors present three cases of symptomatic arterialized DVAs: a 28-year-old male with hematoma at the splenium of the corpus callosum and intraventricular hemorrhage, a 53-year-old male patient with a history of epileptic seizures starting recently, and a 25-year-old male patient, previously healthy who started with persistent headaches and hemosiderin deposition in brain parenchyma. These rare cases of arterialized DVAs are conditions that can cause symptoms or show more aggressive behavior with bleeding.     

A rare (arteriolo-)capillomedullary venous anomaly of the cerebellum

A rare (arteriolo-)capillomedullary venous anomaly of the cerebellum was examined with CT, MRI, and angiography. Unlike the usual venous angioma, this case had such extraordinary features as: infratentorial location, involvement of the whole left cerebellum, an arteriolocapillary component and a history of progressive vomiting and chronic constipation in a 7-year-old boy. CT and MRI were consistent with an extensive vascular malformation, but the actual diagnosis was reached by angiography.     

脑发育性静脉异常的低场磁共振诊断

目的:探讨脑发育性静脉异常(DVA)的低场强磁共振表现及诊断价值。方法:回顾性分析我院5例DVA的磁共振平扫及增强表现,研究其引流静脉及髓静脉信号特点。结果:5例DVA中平扫有2例异常粗大的引流静脉得以显示,其在T1WI上均为低信号,在T2WI上1例为低信号,1例为高信号,髓静脉未能显示。增强扫描5例均显示了引流静脉及髓静脉,其中两例呈典型的水母头征,另外3例分别显示了引流静脉和邻近的髓静脉。结论:在低场强磁共振上DVA有着不同于高场磁共振的特点。     

Brain parenchymal signal abnormalities associated with developmental venous anomalies: detailed MR imaging assessment

BACKGROUND AND PURPOSE: The occurrence of brain parenchymal signal-intensity changes within the drainage territory of developmental venous anomalies (DVAs) in the absence of cavernous malformations (CMs) has been incompletely assessed. This study was performed to evaluate the prevalence of brain parenchymal signal-intensity abnormalities subjacent to DVA, correlating with DVA morphology and location.MATERIALS AND METHODS: One hundred sixty-four patients with brain MR imaging with contrast studies performed from July 2005 through June 2006 formed the study group. The examinations were reviewed and data were collected regarding the following: location, depth, size of draining vein, associated increased signal intensity on fluid-attenuated inversion recovery and T2-weighted images, associated CMs, and associated signal intensity on gradient recalled-echo sequences.RESULTS: Of the 175 DVAs identified, 28 had associated signal-intensity abnormalities in the drainage territory. Seven of 28 DVAs with signal-intensity abnormalities were excluded because of significant adjacent white matter signal-intensity changes related to other pathology overlapping the drainage territory. Of the remaining DVAs imaged in this study, 21/168 (12.5%) had subjacent signal-intensity abnormalities. An adjusted prevalence rate of 9/115 (7.8%) was obtained by excluding patients with white matter disease more than minimal in degree. Periventricular location and older age were associated with DVA signal-intensity abnormality.CONCLUSION: Signal-intensity abnormalities detectable by standard clinical MR images were identified in association with 12.5% of consecutively identified DVAs. Excluding patients with significant underlying white matter disease, we adjusted the prevalence to 7.8%. The etiology of the signal-intensity changes is unclear but may be related to edema, gliosis, or leukoaraiosis secondary to altered hemodynamics in the drainage area.

Developmental venous anomalies (DVAs) are encountered frequently on postcontrast MR imaging of the brain and are usually regarded as normal variants of venous development. The association between DVAs and cavernous malformations (CMs) has been well described.^1–3 Intracranial hemorrhage in the absence of CM has been rarely reported as a complication of DVA.⁴ There have also been a few case reports of nonhemorrhagic presumed venous infarction in the drainage territory of the DVA.^5–8 Signal-intensity abnormality on T2-weighted or fluid-attenuated inversion recovery (FLAIR) sequences has been infrequently reported in the drainage territory of DVAs and has not been thoroughly investigated. Although some of the early literature described signal-intensity abnormalities in the adjacent parenchyma, these were in small case series and appeared, in some instances, to be related to prior hemorrhage.^9,10 A more recent investigation reported parenchymal alterations in up to 65% of DVAs by MR imaging and CT evaluation in a retrospectively identified patient population.¹¹ We chose to specifically evaluate the frequency of signal-intensity abnormalities in association with DVAs in a more detailed fashion by evaluating a series of consecutive DVAs identified on MR imaging examinations during a defined time interval and correlating the presence of associated signal intensity with DVA morphology, location, size, and drainage pattern. We also attempted to assess the relationship of other white matter signal-intensity alterations not in the DVA territory to presumed DVA-associated signal-intensity changes.     

Cerebral developmental venous anomalies associated with head and neck venous malformations.

PURPOSETo study cerebral developmental venous anomalies in patients with extensive venous malformations of the head and neck.METHODSAll patients had undergone carotid angiography 10 to 15 years previously. Four-vessel cerebral angiography was carried out in 40 patients with venous malformations. All patients had a physical examination, 16 had CT, and 22 were examined with MR imaging. One patient had MR angiography.RESULTSEighteen developmental venous anomalies were noted in 8 (20%) of 40 patients. Four patients had multiple anomalies, and these were bilateral in 1 patient. Developmental venous anomalies seen in association with cervicofacial, cutaneous, and mucosal venous malformations were remarkable in their absence of neurologic events and associated cavernoma; significance of ectatic venous convergence, extension, and preponderance of deep drainage routes; and frequency with which they multiple in occurrence.CONCLUSIONDevelopmental venous anomalies have a remarkable prevalence of 20% in patients with extensive superficial venous malformations. Therefore, it is important to search for a cerebral developmental venous anomaly when confronted with a cervicofacial venous malformation.     

Parenchymal abnormalities associated with cerebral venous sinus thrombosis: assessment with diffusion-weighted MR imaging

BACKGROUND AND PURPOSE: The common entity cerebral venous sinus thrombosis is associated with the poorly characterized imaging finding of parenchymal abnormalities; diffusion-weighted imaging has offered some insight into these manifestations. We assessed the relationship between the diffusion constant from apparent diffusion coefficient (ADC) maps in patients with cerebral venous thrombosis (CVT) with follow-up imaging findings and clinical outcome. METHODS: We evaluated the medical records and T2-weighted MR images of 13 patients with CVT complicated by intraparenchymal abnormality. Diffusion-weighted (DW) images and ADC maps were evaluated for increased, decreased, or unchanged signal intensity and were compared with signal intensity of contralateral, normal-appearing brain. In addition, ADCs were obtained in nine pixel regions of interest in abnormal regions in eight of the 13 patients. RESULTS: Eight patients had superficial CVT, and five had superficial and deep CVT. CVT of deep veins was associated with deep gray nucleus and deep white matter abnormalities, whereas superficial CVT was associated with cortical and subcortical abnormalities. Twenty-four nonhemorrhagic lesions were identified in 10 of 13 patients on the basis of follow-up imaging findings. Four patients without seizures had lesions with decreased diffusion that appeared hyperintense on follow-up T2-weighted images, three patients with seizures had lesions with decreased diffusion that resolved, and seven patients had lesions with increased diffusion that resolved. Three of 10 patients had more than one lesion type. No difference was noted in mean ADCs for lesions with decreased diffusion that resolved compared with lesions with decreased diffusion that persisted. CONCLUSION: DW imaging in these patients disclosed three lesion types: lesions with elevated diffusion that resolved, consistent with vasogenic edema; lesions with low diffusion that persisted, consistent with cytotoxic edema in patients without seizure activity; and lesions with low diffusion that resolved in patients with seizure activity. This information may be important in prospectively determining severity of irreversible injury and in patient treatment.     

Venous congestion associated with developmental venous anomaly: Findings on susceptibility weighted imaging

A case of venous congestion associated with developmental venous anomaly (DVA) in a 5‐year‐old girl who presented with acute deterioration of consciousness is reported. Susceptibility‐weighted imaging revealed abnormal structures connected to the central collector of a DVA, which were not shown on other sequences. These structures were considered to be presumably thrombosed medullary veins. J. Magn. Reson. Imaging 2008;28:1506–1509. © 2008 Wiley‐Liss, Inc.     

Sinus pericranii: diagnostic and therapeutic considerations in 15 patients

INTRODUCTION: Sinus pericranii (SP) is a rare, usually asymptomatic condition characterized by a large communication between the intra- and the extracranial venous drainage pathways in which blood may circulate bidirectionally through dilated veins of the skull. We describe our diagnostic and therapeutic experience with SP, with a special focus on the vascular analysis of digital subtraction angiography (DSA). METHODS: DSA images of 15 patients were evaluated with regard to the delay in opacification of the scalp vessels, the absence or distortion of the superficial cortical veins in the vicinity of the SP, the drainage patterns of the superior sagittal sinus, and the degree of maturation of the venous outlets of the brain. SP were classified either as "dominant", if the main stream of contrast flow used the SP to drain the brain bypassing usual venous outlets, or as "accessory", if only a small part of the venous outflow occurred through the extradiploic vessels. RESULTS: All patients presented with a nonpulsatile, soft-tissue mass. The lesion was on the midline in 14 of 15 patients, frontal in 12 patients, and parietal in 2 patients. In 13 patients, associated intracranial venous anomalies were present, eight of which were developmental venous anomalies. Seven patients had a dominant SP, and eight an accessory SP. CONCLUSION: SP can be considered the cutaneous sign of an underlying venous anomaly. If treatment is contemplated, analysis of the drainage pattern of the SP has to be performed. Treatment should be avoided in dominant SP or if its accessory role constitutes the only collateral pathway of an underlying venous anomaly.     

体静脉连接异常的心血管造影诊断

目的评价心血管造影对体静脉连接异常的诊断价值。方法回顾分析371例经手术证实的体静脉连接异常患儿的心血管造影。结果左上腔静脉残存共190例,其中左上腔静脉回流入冠状窦171例,左上腔静脉回流入左房9例,左上腔静脉经无顶冠状窦回流入左房10例;右上腔静脉缺如3例;下腔静脉异常连接42例,其中40例为下腔静脉中断伴奇静脉回流,1例为下腔静脉回流入左房,1例为双下腔静脉。无名静脉异常共136例。结论体静脉连接异常在先天性心脏病的患者中并不少见,先心病术前通过心血管造影了解体静脉的连接关系对手术有重要的指导意义。     

Developmental venous anomaly (DVA) with arterial component: a rare cause of intracranial haemorrhage

Introduction  To examine the clinical and radiologic findings of patients with developmental venous anomaly (DVA) associated with intracranial haemorrhage but unrelated to cavernoma. Methods  Computed tomography (CT) was used to obtain intracranial images from seven patients ranging in age from 6 to 51 years. Magnetic resonance imaging (MRI) was then performed on six patients, and two patients were further examined via CT angiography. Finally, digital subtraction angiography (DSA) was performed to confirm the initial diagnosis. Results  CT showed intraparenchymal supratentorial haemorrhage in all patients. The combined imaging modalities eventually confirmed a diagnosis of arterialised DVA in four patients and arterialised DVA associated with arteriovenus malformation (AVM) in three. Two patients were managed symptomatically, two underwent radiosurgery, one underwent surgery, one underwent combined embolisation plus radiosurgery and the remaining patient underwent combined embolisation plus surgery. Two patients died, one as a result of re-bleeding, and the other due to radiation necrosis. The mean follow-up period was 33 months (6 months to 6 years) for the remaining five patients with favourable outcome. Conclusion  DVA associated with intraparenchymal haemorrhage, but not related to cavernoma, was confirmed. Though very rare, DVA may present with non-cavernoma-related haemorrhage in the form of arterialised DVA or DVA with AVM.     

Accelerated myelination associated with venous congestion

Magnetic resonance imaging is currently the gold standard in the assessment of brain myelination. The normal pattern of brain myelination conforms to a fixed chronological sequence. Focal accelerated myelination is a usual pathological state and previously has only been associated with Sturge‐Weber syndrome. The purpose of our study is to describe alternate causes for accelerated myelination. We retrospectively reviewed serial MR scans, MR angiography, conventional angiography and the clinical progress of three children with accelerated myelination. Two patients with accelerated myelination had an underlying cerebral sinovenous thrombosis. The third patient had Sturge‐Weber syndrome. Our study strongly suggests that cerebral venous thrombosis with the consequent restriction of venous outflow could be a key factor in the induction of accelerated myelination. We recommend that in patients with accelerated myelination, the search for an underlying etiology should include careful evaluation of the intracranial vascular pathology, especially cerebral venous thrombosis.     

Brain lesions due to cerebral venous thrombosis do not correlate with sinus involvement

Cerebral venous thrombosis may be well tolerated or lead to a brain lesion; availability of collateral venous pathways may explain the great variability of the lesions. This collateral circulation involves mainly medullary and cortical veins. These are difficult to assess neuroradiologically, particularly if thrombosed. Cerebral venous thrombosis is diagnosed usually based on thrombosis of dural sinuses and of the deep venous system. We tried to correlate the site and extent of dural sinus thrombosis with the location and the size of brain lesions in 26 consecutive patients with cerebral venous thrombosis, to investigate whether a simple causal relationship exists. No significant correlation between the extent and site of thrombosis in dural sinuses and the extent and location of brain lesions was found. In some cases a clear relationship between thrombosis of cortical and medullary veins and the lesions was evident. These data suggest that sinus thrombosis alone may be well tolerated in many cases, while involvement of cortical and medullary veins leads to a worse clinical situation. Accepted: 26 November 1998     

脑发育性静脉异常并出血的影像学诊断

目的:探讨脑发育性静脉异常(DVAs)并出血的影像学表现。方法:回顾性分析7例随访证实为DVAs并出血病例的CT及MRI表现。结果:发生在左、右额叶白质内各1例,右侧基底节2例,右侧小脑中脚1例,左、右侧小脑半球各1例。CT平扫2例呈结节状高密度灶,1例呈卵圆形低密度灶。MRI平扫时DVAs呈长T1,长、短T2信号,3例呈"水母头征",出血灶呈片絮状短、长T1,长、短T2信号,条状短、长T1,短T2信号。MRI增强扫描1例未见强化,其他均明显强化,呈"水母头征",显示清晰。磁敏感加权成像(SWI)示5例呈低信号,呈典型"水母头征",显示清晰,出血灶呈结节状低信号,边界清晰。结论:MRI对DVAs并出血的诊断有明显优势,以SWI更为显著。     

颈部动静脉瘘合并深静脉高压的影像特征分析

目的分析动静脉瘘(AVF)合并深静脉高压的影像特征旨在提高认识。方法回顾性分析1例颈部AVF致颅内深静脉高压病人的临床资料及影像表现,并复习相关文献。结果CT及MRI表现为深静脉高压征象:①双侧基底节丘脑区及脑室旁、左侧小脑肿胀伴血管源性水肿;②深静脉引流区多发细小迂曲血管影及含铁血黄素沉积。DSA显示左侧颈部AVF,动脉期见左侧颈内静脉-乙状窦-横窦-直窦反流。结论AVF临床及影像表现具有多样性,当颅内静脉引流区出现肿胀伴异常信号、多发迂曲血管影时,应及早行血管检查以明确诊断,DSA是确诊该病的金标准。     

顺序节段分析法在先天性右位心合并心内畸形中的诊断价值

目的提高超声心动图对心脏位置异常合并复杂心内畸形的诊断准确率。方法选择近年来在我院经手术或心血管造影证实的先天性右位心合并心内畸形患者42例,对其超声心动图检查结果进行回顾分析。结果手术证实本组先天性右位心分型：镜像右位心13例,右旋心29例;心内畸形：单心室10例,右室双出口8例,法洛四联症6例,完全性大动脉转位5例,矫正性大动脉转位4例,完全性房室间隔缺损4例,永存动脉干3例,肺动脉闭锁2例。超声误诊、漏诊的病例：3例完全性大动脉转位伴巨大室间隔缺损误诊为单心室;2例右室双出口误诊为法洛四联症;1例矫正性大动脉转位误诊为完全性大动脉转位;1例肺动脉闭锁误诊为永存动脉干;1例肺动脉闭锁超声漏诊左肺动脉缺如及多支体肺侧枝。结论超声心动图顺序节段法对绝大多数先天性右位心并心内畸形能作出准确诊断,但对某些极为复杂的病例心导管检查仍是明确诊断和手术条件的重要手段。     

Microstructural callosal abnormalities in normal-appearing brain of children with developmental delay detected with diffusion tensor imaging

Callosal fibres play an important role in psychomotor and cognitive functions. The purpose of this study was to investigate possible microstructural abnormalities of the corpus callosum in children with developmental delay, who have normal conventional brain MR imaging results. Seventeen pediatric patients (aged 1–9 years) with developmental delay were studied. Quantitative T2 and fractional anisotropy (FA) values were measured at the genu and splenium of the corpus callosum (CC). Fibre tracking, volumetric determination, as well as fibre density calculations of the CC were also carried out. The results were compared with those of the age-matched healthy subjects. A general elevation of T2 relaxation times (105 ms in patients vs. 95 ms in controls) and reduction of the FA values (0.66 in patients vs. 0.74 in controls) at the genu of the CC were found in patients. Reductions of the fibre numbers (5,464 in patients vs. 8,886 in controls) and volumes (3,415 ml in patients vs. 5,235 ml in controls) of the CC were found only in patients older than 5 years. The study indicates that despite their inconspicuous findings in conventional MRI microstructural brain abnormalities are evident in these pediatric patients suffering from developmental delay.     

Cerebral venous sinus thrombosis presenting with excessive subarachnoid hemorrhage in a 14-year-old boy

Cerebral venous thrombosis presenting with subarachnoid hemorrhage (SAH) is very rare. We report a case of cerebral venous sinus thrombosis as an initial manifestation of SAH. A 14-year-old boy was admitted with progressive headache, nausea, vomiting, diplopia, and gait disturbance. Cerebral computed tomography scan showed a widely SAH in the basal cisterns, bilateral sylvian fissures, and anterior interhemispheric fissure. Cerebral angiography was performed to detect any aneurysm in intracranial vasculature as a cause of SAH; however, the totally thrombosed superior sagittal sinus, galenic vein, and straight sinus were the sole abnormal findings.