The Many Faces of Cerebral Developmental Venous Anomaly and Its Mimicks: Spectrum of Imaging Findings

Developmental venous anomalies (DVAs) are the most common cerebral vascular malformations and are usually found incidentally on neuroimaging studies. Despite the benign nature of DVAs, occasionally, they can be symptomatic. The objective of this article is to review the spectrum of imaging findings of DVAs on conventional and advanced imaging studies. In addition, neuroimaging findings of symptomatic DVAs as well as imaging mimicks will also be described to assist in the approach to differential diagnosis.     

脑动静脉畸形患者癫痫间发作的相关因素分析

目的 探讨脑动静脉畸形并发癫痫间发作的相关危险因素以及癫痫间的合理预防措施。方法 回顾性分析286例脑动静脉畸形患者的临床及脑动静脉畸形基线资料。根据是否合并癫痫间发作分为癫痫间组和对照组。分析脑动静脉畸形患者并发癫痫间发作的危险因素,观察癫痫间组92例患者的发作类型及发作频率以及药物治疗反应情况。结果 癫痫间组与对照组畸形血管团的位置、畸形血管团直径、静脉引流方向等因素比较差异明显(P<0.05); 非条件多因素Logistic回归分析显示对脑动静脉畸形患者癫痫间发作有显著影响的因素为畸形团直径大于3 cm和由浅静脉或者浅深静脉共同引流(P<0.05)。经过抗癫痫间药物治疗,癫痫间组有69例患者癫痫间发作得到了完全控制,余患者发作频率均有不同程度减低。结论 畸形团直径大于3cm和由浅静脉或者浅深静脉共同引流是脑动静脉畸形患者并发癫痫间的危险因素; 脑动静脉畸形合并的癫痫间发作是一个相对良性过程,抗癫痫间药物对多数癫痫间患者治疗有效。     

以出血为表现的脑静脉畸形的诊断和治疗

目的 探讨脑静脉畸形出血的影像学特点及其手术治疗方法.方法 回顾性分析8例脑静脉畸形出血的临床资料.7例采取显微手术治疗,1例出血破入脑室系统采取双侧脑室穿刺体外引流术.结果 8例经影像学及手术确诊为静脉畸形,其中2例合并海绵状血管瘤.随访2-60个月,平均21个月.所有患者均未发生再次出血,4例恢复日常生活工作,3例有不同程度恢复,但遗留神经功能缺损,1例加重(卧床).结论 静脉畸形MRI、DSA表现呈"水母头"改变.血肿量大、反复出血及神经功能障碍进行性加重是脑静脉畸形的手术指征,手术应尽量保留有正常引流功能的静脉畸形.     

自发性低颅压并发静脉血栓形成和出血性梗死一例临床分析

目的自发性低颅压(SIH)多以与体位相关的剧烈头痛为主要症状,误诊率高。SIH并发静脉和(或)静脉窦血栓形成非常罕见。方法我们报道1例SIH并发静脉血栓形成和出血性梗死的患者,并复习相关文献。结果本例患者自发性低颅压在前,静脉血栓形成和出血性梗死在后,同时经过检查排除了其他可导致静脉血栓形成的危险因素。所以我们考虑SIH是静脉血栓形成的危险因素。假如SIH患者与体位相关的间断的头痛变成持续性头痛时,应考虑到有静脉血栓形成的可能。结论因为SIH的患者有形成硬膜下血肿的风险,所以对于静脉血栓形成的抗凝治疗应谨慎。     

Cerebral developmental venous anomalies: Current concepts

Cerebral developmental venous anomalies are the most frequently encountered cerebral vascular malformation, and as such, are frequently reported as fortuitous findings in computed tomography (CT) and magnetic resonance imaging (MRI) studies. Developmental venous anomalies (DVAs) are generally considered extreme anatomical variations of the cerebral vasculature, and follow a benign clinical course in the vast majority of cases. Here we review current concepts on DVAs with the aim of helping clinicians understand this complex entity. Morphological characteristics that are necessary to conceptualize DVAs are discussed in depth. Images modalities used in diagnosing DVAs are reviewed, including new MRI or CT techniques. Clinical presentation, association with other vascular malformations and cerebral parenchymal abnormalities, and possible physiopathological processes leading to associated imaging or clinical findings are discussed. Atypical forms of DVAs are also reviewed and their clinical significance discussed. Finally, recommendations as to how to manage asymptomatic or symptomatic patients with a DVA are advanced. Ann Neurol 2009;66:271–283     

Morphological and blood flow MR findings in cerebral vascular malformations

The authors report MR findings in 139 patients with 159 cerebral vascular malformations (36 arteriovenous malformations, 25 venous angiomas and 98 cavernous angiomas). Morphological aspects and hemodynamic findings due to rapid and slow flow are analysed. MR is able to differentiate the various type of lesions and permits the visualization of angiographically occult vascular malformations. In the case of cavernous angiomas MR information may not be sufficient for an absolute diagnosis because of differential diagnostic problems; correlation with the clinical history, CT and angiographic findings and follow-up MR study are certain to be necessary to increase specificity. Usually it is possible to distinguish histopathological components of the lesion and perilesional modifications in the cerebral parenchyma.     

Extensive Cerebellar Venous Malformation Associated with a Varix and Cavernous Malformations: a Case Report

Venous malformations are the most common cerebral vascular malformation, frequently associated with cavernous malformation, and rarely accompanied by a varix. We report the case of a 67-year-old woman with tinnitus, whose cranial magnetic resonance images and selective catheter angiograms showed extensive cerebellar venous malformation with cavernous malformations and varix. Injury of vessel walls by increased blood flow in the draining vein of the malformation may have led to the development of cavernous malformations and a varix. To our knowledge, such association with an extensive cerebellar venous malformation has not been reported.     

Pericapillary arteriovenous malformations angiographically manifested as cerebral venous malformations

Cerebral venous malformations have been diagnosed by angiographic features and are considered to be a benign anomaly. However, ample evidence indicates that stroke or similar symptomatology occurs in patients harboring a cerebral vascular malformation that was diagnosed angiographically as a venous malformation. The purpose of the study is to confirm the presence of a pericapillary arteriovenous malformation in these patients by analyzing the clinical history and surgical findings and correlating them with histological features. Thirteen patients were included in this study. Each patient fulfilled four criteria: 1. the patient was neurologically symptomatic; 2. the angiographic diagnosis was a venous malformation; 3. at operation, shunting arterioles (50-100 microns) were found to contribute to the malformation; and 4. histologically, a mixture of venous channels and arterioles with arterioles directly connected to venules was found. Based on the above findings, the malformation present in the 13 patients can be termed a 'pericapillary arteriovenous malformation'. Its angiographic distinction from the cerebral venous malformation requires technological advancement in the capability of magnifying images of arterioles and venules, along with improvement in image resolution.     

Familial cavernous angioma without clinical haemorrhage

The incidence and natural history of cerebral cavernous malformations remain poorly defined. MRI now provides anaccurate means of diagnosis and follow-up of these lesions. Hereditary cavernous angiomata have been reported in several racial groups, revealing asymptomatic family members with significant numbers of lesions. We report a family of Anglo-Saxon origin with nine members among three generations affected via an autosomal dominant mode of inheritance. MRI of seven affected family members revealed 74 lesions. Autopsy of two further family' members who died of unrelated causes also demonstrated the presence of cavernous angiomata. No lesion was associated with anomalous venous drainage. Two affected family members had seizures. Although six lesions exhibited the characteristic mixed intensity T2 spin echo signal suggesting haemorrhage, no lesion has caused a clinically apparent haemorrhage. This family offers a unique perspective of the natural history of this disease. No clinically apparent haemorrhage has occurred in 2418 lesion years. The benign nature of these lesions suggests that, in clinically unruptured familial cavernous angiomas, without accompanying anomalous venous drainage, a conservative approach may be warranted irrespective of their location.     

Electroconvulsive therapy and venous angiomas: two case reports and review of the literature

According to the American Psychiatric Association, the risk for complications related to the electroconvulsive therapy (ECT) treatment of patients with cerebrovascular malformations is small. The literature contains a number of case studies presenting the uneventful treatment of patients with cerebral aneurysms with ECT. However, there is a paucity of cases presenting ECT in the context of a cerebral venous angioma. In this article, we present 2 cases of patients treated with ECT who were found to have documented venous angiomas. This is followed with a brief review of the literature.     

Two cases of cystic enlargement of vestibular schwannoma as a late complication following gamma knife surgery

Cyst formation is the most common long-term complication after gamma knife surgery (GKS) for cerebral arteriovenous malformations; however, this rarely occurs after GKS for vestibular schwannoma (VS). We describe two patients that developed aggressive, symptomatic large cysts at more than 4 and 12 years after GKS for VS, although the tumor control for these patients had been acceptable at 3 and 11 years, respectively. During the surgery, a reddish lesion, which was distinct from the surrounding tumor, was observed. The microscopic examination of the reddish lesion revealed dilated capillary vessels and hemorrhage, which was compatible with late radiation changes; these were usually seen in arteriovenous malformations following radiosurgery. The present patients indicated that cyst formation and enlargement may occur as a late complication following GKS even in extra-axial benign tumors such as VS.     

Dural arteriovenous malformation causing brainstem haemorrhage

A 40 year old man developed a brainstem haemorrhage from an arteriovenous malformation of the tentorial incisura. Although the location of the haemorrhage represents a unique report, this case illustrates 3 important points that can be generally applied to dural arteriovenous malformations. Firstly, the dangers of dural arteriovenous malformations with cortical venous drainage. Secondly, with regards their risk of haemorrhage, dural arteriovenous malformations (tentorial arteriovenous malformations in particular) cannot be dismissed as benign until angiography has been performed. Finally, angiography performed for the purpose of investigating the possibility of an arteriovenous malformation being responsible for an intraparenchymal haemorrhage cannot be considered truly negative unless the external carotid circulation has failed to demonstrate an arteriovenous malformation in addition to the internal carotid and vertebral circulations.     

Paradoxical clinicoradiologic features of a cavernous dural arteriovenous malformation

A case of bilateral Barrow type D dural arteriovenous malformations (DAVMs) of the cavernous sinuses is described in a 66-year-old woman who, despite having moderate symptoms with partial resolution and benign findings on non-invasive imaging, was ultimately found to have associated leptomeningeal venous drainage on conventional angiography. This case illustrates the problem of discordance between clinical symptoms, non-invasive imaging and actual radiologic features predisposing to aggressive clinical behaviour in DAVMs detected by conventional angiography.     

Embolie gazeuse cérébrale iatrogène d’origine veineuse sans signe respiratoire : un mécanisme rétrograde ?

IntroductionAir embolism is a rare complication of various invasive medical procedures. Venous cerebral air embolism is usually the consequence of paradoxical embolism. We report a case of isolated cerebral air embolism resulting from a non-paradoxical mechanism.Case reportA few minutes after his central venous catheter had been accidentally disconnected, a 63-year-old man developed left-sided rhythmic jerking movements followed by left hemiplegia. There were no associated cardiologic or pulmonary signs. Brain CT showed air bubbles in the right frontal cortical sulci. The brain MRI DWI and FLAIR sequences showed a high intensity right frontal cortical lesion without reduction in ADC. Transesophageal echocardiogram did not find a patent foramen ovale.ConclusionsIn this case of venous cerebral air embolism, the lack of any cardiopulmonary manifestation, the lack of a patent foramen ovale and the neuroradiological findings are not in favor of the hypothesis of paradoxical embolism. The hypothesis of retrograde venous cerebral air embolism is discussed.     

Cerebellar venous anomalies with symptomatic vascular malformations

Cerebral and cerebellar venous anomalies (previously known as venous angiomas) form the alternative venous drainage of the surrounding nervous tissue because of the un-development of the normal venous system. They are made up of veins with abnormal structure: thick walls, lumens dilated and of irregular calibre that converge radially towards a wide draining vein (caput medusae). They are thought to be a benign condition although they are sometimes associated with cerebellar hemorrhages. Authors report three patients with cerebellar venous anomalies associated either with pontine cavernoma, cerebellar arteriovenous malformation or cerebellar infarct. They illustrate that cerebellar venous anomalies are benign conditions, but their presence might be a marker for additional, pathogenic malformation. It might be difficult to detect the associated malformations even by sophisticated imaging methods, but their presence can modify the treatment options.     

A sixty-eight-year-old man with giant intracranial arteriovenous malformation and right-sided hemicrania -- a case report

Arteriovenous malformations (AVMs) are the most dangerous congenital vascular malformations. Intracranial AVMs occur in about 0.1 percent of the population and account for 1 to 2 percent of all strokes. The angioarchitecture of AVMs consists of direct arterial to venous connections without an intervening capillary network. The main symptom reported by half of all patients with arteriovenous malformations is an episodic headache. The headache is associated with nausea and vomiting and due to this resembles the hemicrania or cluster headache attacks. Although cerebral angiography is considered as the gold standard for the diagnosis, planned treatment and after treatment observation, AVMs are usually identified with advanced visual imaging techniques such as magnetic resonance imaging, computed tomography, and computed angiotomography. The treatment options consist of surgery, radiosurgery and endovascular embolization. We report a case of a 68-year-old man with giant intracranial arteriovenous malformation and right-sided hemicrania.     

Cerebral venous angiomas. 12 personal cases and review of the literature]

Cerebral venous angiomas (CVAs) are made up of veins with abnormal structure: thick walls, lumens dilated of irregular calibre that converge radially towards a wide draining vein. The arteries are normal. The veins are separated by a normal nervous tissue. The malformation is thought to develop as a compensatory venous drainage consecutive to the occlusion, or lack of development, of one or several transcerebral veins during the formation of the mature venous system. Together with arteriovenous angiomas, capillary telangiectasias and cavernous angiomas, CVAs belong to the vascular angiomatous malformations, also called hamartomas. The present study is based on 12 cases collected between 1984 and 1989, all explored by CT and angiography, and by MRI in 2 cases. The diagnosis therefore was neuroradiological, except in 1 case where it was obtained by neuropathological examination. The malformation was supratentorial in 10 cases and cerebellar in 2 cases. Most CVAs were discovered in patients whose symptoms could hardly be attributed to these malformations and consequently were termed asymptomatic (6 cases). Two cases were found in subjects with generalized epileptic seizures without clear-cut relationship with the angioma; 4 cases were revealed by haemorrhages: subarachnoidal haemorrhage in 2 cases and supratentorial intraparenchymatous haematoma in 1 case. These 3 cases had a spontaneously favourable outcome after a follow-up of several years. One patient with a cerebellar hematoma died postoperatively of edematous infarction of the cerebellum. One of these patients had two symmetrical CVAs, one in each cerebral hemisphere (multiple venous angiomas), and in another patient the CVA was probably associated with a cavernous angioma.(ABSTRACT TRUNCATED AT 250 WORDS)     

Cutaneous Venous Malformations Related to KRIT1 Mutation: Case Report and Literature Review

Cavernous malformations (CMs) are vascular anomalies of the nervous system mostly located in the brain. Cerebral cavernous malformations can present sporadically or familial, as a consequence of an autosomal dominant condition, with incomplete penetrance and variable clinical expression. Occasionally, extraneural manifestations of CMs involving the skin have been described. We report the case of two siblings presenting in adulthood diffuse cutaneous vascular lesions associated with cerebral CMs that, after surgical excision and histopathologic analysis, resulted to cavernous haemangiomas. Genomic DNA was extracted from peripheral blood, and molecular evaluation of KRIT1 gene was performed. Although no signs of neurological impairment were reported, cerebral MRI revealed multiple images in both patients, suggestive of cavernous haemangiomas. The genetic study demonstrated a nonsense mutation (c.535C>T) in the KRIT1 (Krev-1/rap1 interaction trapped 1) gene. Few reports describe extraneural manifestations of Cavernous malformation syndrome (CMs) related to a KRIT1 mutation; these involve the skin and are associated with hyperkeratotic cutaneous capillary–venous malformation. CMs should be suspected in patients developing multiple nodular cutaneous venous lesions in adulthood.     

Cerebrovascular diseases in childhood--etiology, clinical aspects and prognosis

Over a period of nine years we observed 52 children with acute neurological symptoms which were caused by a cerebrovascular disease. Fourteen patients had congenital vascular malformations, most frequently AV-angiomas (9 patients). A Sturge-Weber-Syndrome and a venous angioma were found in two cases and one patient had an aneurysm of the middle cerebral artery. Thirty-eight patients had acquired cerebrovascular diseases such as ischaemic infarctions (22), intracranial haemorrhages without vascular malformations (14) and thromboses of the dural sinus (2). The cerebral infraction was a complication of a congenital heart disease in 8 children, two others suffered from chronic renal insufficiency and were on haemodialysis. Two children had a trauma of the internal carotid artery and in one patient a large haemorrhagic infarct was caused by hypernatremic dehydration. In 9 patients (6 females, 3 males) no obvious aetiology of the infarct could be found. However, in most of these cases a nonspecific febrile illness preceded the neurological manifestations. The thrombosis of the dural sinus occurred in a 6-week old previously healthy infant and in a 3-year old boy as a complication of a nephrotic syndrome. Intracranial haemorrhages (without cerebrovascular malformations) occurred in 14 patients, mainly as a complication of haematological diseases (acute lymphatic leukaemia, severe aplastic anaemia, haemophilia A, lupus erythematodes). Four children had spontaneous intracerebral haemorrhages without obvious causes. The prognosis for survival was good in children with infarcts, but persisting neurological deficits were more severe than in children with haemorrhages. At the acute stage the lethality was higher in children with intracranial haemorrhages.     

Venous infarction mimicking top of basilar syndrome; An uncommon complication of adult influenza infection

Influenza infection increases the risk of ischemic stroke. Here, we represent a case of a 32 year-old female who presented with acutely developed altered mental status after influenza A infection. The clinical manifestation and initial lesion on brain DWI mimicked top of basilar syndrome, but without an arterial occlusion or stenosis. Follow-up neuro imaging analysis suggested cerebral venous infarction. It is valuable to report this case of venous infarction in a healthy young adult, as an uncommon complication of adult influenza infection.