High-frequency color Doppler ultrasound as the first diagnostic and monitoring choice for early superficial high-flow vascular malformations

Diagnosis and monitoring of early high-flow vascular malformations could be a meaningful pursuit. However, there has been no ideal method for their long-term monitoring and prognosis. We examined 21 early high-flow vascular malformations in this study and deemed that high-frequency color Doppler ultrasound could be regarded as the first diagnostic and monitoring choice for early high-flow vascular malformations at present.     

血管生长相关因子在脑血管畸形中的差异性表达

背景：脑血管畸形是在青壮年人群中造成出血性脑卒中的常见病因,畸形血管破裂出血可引发严重的神经功能障碍。脑血管畸形的形成及发病原理尚不明确。现代分子生物学的研究表明血管生长相关因子在脑血管畸形中可能存在异常表达。目的：评价血管生长相关因子在脑畸形血管中的表达差异,探讨血管生长相关因子与脑畸形血管形成的关系。方法：选择脑血管畸形患者与颅内出血开颅治疗患者各50例,通过免疫组织化学染色方法检测脑血管畸形标本和开颅治疗患者颞浅动脉标本中血管因子的表达差异。结果与结论：正常颞浅动脉中血管生长相关因子（血管内皮生长因子和转化生长因子α）几乎不表达,畸形血管中两者高表达（P〈0.05）。结果证实,与正常血管相比,脑血管畸形患者血管内皮生长因子和转化生长因子α的表达存在明显差异,脑畸形血管患者血管组织可表达更多的血管内皮生长因子和转化生长因子α。     

US and MRI features in venous vascular malformation of the abdominal wall. A case report

Vascular anomalies are classified as vascular tumors and vascular malformations. Venous vascular malformations are the most common type of vascular malformation. They may be isolated or multiple and they rarely affect the trunk. The authors report a rare case of isolated venous vascular malformation of the abdominal wall with an emphasis on the related MRI and ultrasound (US) features.     

Aneurysms and vascular malformations

Intracranial aneurysms and vascular malformations are frequently detected following intracranial hemorrhages. A CT scan is the most sensitive method of detecting acute subarachnoid, parenchymal, and intraventricular hemorrhages. Small aneurysms are inconsistently visualized on MR scans. Angiography remains the standard for complete and accurate depiction of patent aneurysms, as well as of arteriovenous and venous malformations that have not thrombosed. Giant and thrombosed aneurysms present as mass lesions and are frequently detected when MR is used as a screening examination. Often MRI characterizes these lesions better than CT or angiography. Patients with vascular malformations who have focal neurologic symptoms without hemorrhage are best evaluated with MRI. Patent vascular malformations demonstrating flow void and other flow-related phenomena are readily demonstrated. Occult vascular malformations, including thrombosed arteriovenous, venous, and cavernous malformations and telangiectasia, are also best detected by MRI and are not visible on angiography.     

Nontumorous vascular malformations in the liver: color Doppler ultrasonographic findings.

OBJECTIVE: To investigate color Doppler and spectral wave characteristics of nontumorous vascular malformations in the liver. METHODS: From September 1995 to January 2001, 32 cases of vascular malformations were identified by means of color Doppler ultrasonography and spectral wave analysis. Computed tomography, angiography, or both were performed in all cases. RESULTS: Five arterioportal and 14 portovenous malformations, 1 arteriovenous malformation, and 4 portoportal and 8 venovenous shunts were detected. Associations with Rendu-Osler-Weber syndrome in 6 cases and with cirrhotic liver in 12 cases were found Fourteen patients were liver disease free. In 3 cases, interventional procedures were necessary to reduce portal hypertension or cardiac dysfunction. The incidence of finding vascular malformations in 12,000 patients was 0.1%. CONCLUSIONS: Nontumorous vascular malformations are rare disorders in the liver. They may appear in patients with healthy livers and in patients with portal hypertension. Color Doppler ultrasonography and spectral wave analysis are capable of showing and differentiating different types of hepatic vascular malformations.     

血管瘤和血管畸形MRI、MRA与VEGF表达的联系

目的：探讨周围软组织血管瘤和血管畸形MRI，MRA与VEGF表达的关系。方法：对71例血管瘤和血管畸形MRI，MRA表现与VEGF表达进行对照。结果：38例血管瘤，31例T2WI呈较高信号，7例呈高信号；7例VEGF表达（-），31例（＋～＋＋＋）。33例血管畸形，3例T2WI呈较高信号，30例呈高信号；31例VEGF表达（-），2例（＋）。血管瘤T2WI信号表现与VEGF表达密切相关。结论：血管瘤和血管畸形VEGF表达、MRI、MRA表现均不相同，MRI、MRA表现一定程度反应血管内皮增生程度与VEGF表达。     

基质结构蛋白在脑血管畸形中的表达差异

背景：有研究表明脑血管畸形中存在管壁结构基质蛋白的表达差异,但血管畸形间结构基质蛋白的差异及结构蛋白差异与畸形血管管壁异形关系少有报道.目的：观察基质结构蛋白在脑血管畸形中的差异性表达.方法：对50例脑血管畸形手术标本和34例正常颞浅动脉标本进行苏木精-伊红染色,并应用美国Santa Cruz公司生产单/多克隆抗体对4种基质结构蛋白-Ⅳ型胶原蛋白、a-血管平滑肌蛋白、层粘连蛋白和纤维连接蛋白免疫组织化学染色.结果与结论：苏木精-伊红染色显示,脑血管畸形壁结构与正常血管相比呈现明显紊乱和构成异形性;且脑血管畸形中,脑动静脉畸形与海绵状血管瘤结构存在明显差异.免疫组织化学染色显示：正常颞浅动脉和畸形血管中Ⅳ型胶原和a-血管平滑肌蛋白全部呈阳性表达且无显著性差异,但结构排列差异明显;正常颞浅动脉与畸形血管层粘连蛋白和纤维连接蛋白表达阳性率差异有显著性意义（P＜0.05）,正常血管表达更多层粘连蛋白,畸形血管表达更多纤维连接蛋白;畸形血管中脑动静脉畸形和海绵状血管瘤间层粘连蛋白和纤维连接蛋白的阳性率表达差异有显著性意义（P＜0.05）,脑动静脉畸形血管表达更多层粘连蛋白,海绵状血管瘤血管表达更多纤维连接蛋白.结果证实：与正常血管相比,脑血管畸形者管壁结构存在明显异形性,结构蛋白Ⅳ型胶原和a-血管平滑肌蛋白结构明显紊乱,且低表达层粘连蛋白而高表达纤维连接蛋白,这种差异可能是导致脑血管壁结构异常的原因.     

Clinical-sonographic diagnostics and laser therapy treatment options in lymphatic vascular malformations – An experience-based report

Lymphatic vascular malformations occur with a large variety of different characteristics. Color-coded duplex sonography is the most effective preoperative and intra-operative diagnosis method. According to different manifestations, suitable laser procedures can be performed with minor side effects and a noticeable stabilization of the clinical situation after only a few interventions. We report on our experience in diagnostics and the laser therapy of lymphatic vascular malformations which are based on the treatment of 168 patients over the last 8 years.     

Progress and prospects of mechanotransducers in shear stress-sensitive signaling pathways in association with arteriovenous malformation

Arteriovenous malformations are congenital vascular lesions characterized by a direct and tangled connection between arteries and veins, which disrupts oxygen circulation and normal blood flow. Arteriovenous malformations often occur in the patient with hereditary hemorrhagic telangiectasia. The attempts to elucidate the causative factors and pathogenic mechanisms of arteriovenous malformations are now still in progress. Some studies reported that shear stress in blood flow is one of the factors involved in arteriovenous malformations manifestation. Through several mechanotransducers harboring the endothelial cells membrane, the signal from shear stress is transduced towards the responsible signaling pathways in endothelial cells to maintain cell homeostasis. Any disruption in this well-established communication will give rise to abnormal endothelial cells differentiation and specification, which will later promote arteriovenous malformations. In this review, we discuss the update of several mechanotransducers that have essential roles in shear stress-induced signaling pathways, such as activin receptor-like kinase 1, Endoglin, Notch, vascular endothelial growth factor receptor 2, Caveolin-1, Connexin37, and Connexin40. Any disruption of these signaling potentially causes arteriovenous malformations. We also present some recent insights into the fundamental analysis, which attempts to determine potential and alternative solutions to battle arteriovenous malformations, especially in a less invasive and risky way, such as gene treatments.     

探讨介入治疗软组织血管畸形的临床疗效及安全性

探讨介入治疗软组织血管畸形的临床疗效及安全性。方法搜集1998年1月至2008年12月经介入治疗各种血管畸形31例,男19例,女12例,年龄12—51岁,发生于四肢的血管畸形最为常见,其他部位的有躯干、生殖器官等。治疗方法为在DSA引导下：①经皮穿刺血管畸形注射硬化剂治疗;②经导管供血动脉对血管畸形进行栓塞治疗。结果所有病例的畸形血管团得到全部或大部分闭塞、血管畸形局部肿胀减轻、溃破出血停止、软组织肿块变硬变小及固定;所有病例术中术后均无严重并发症发生。结论介入治疗软组织血管畸形是一种有效的、创伤小的治疗方法,在目前情况下应作为首选的治疗方法。     

Color Doppler sonography of vascular malformations of the liver

Nine cases of intrahepatic vascular malformations diagnosed using color Doppler sonography are described. These consisted of six cases of intrahepatic portal-hepatic venous shunts and three cases of arteriovenous fistulas. Among these is a case of multiple intrahepatic portal-systemic shunts. The sonographic findings and theories explaining the formation of vascular malformations in the liver are discussed.     

Perinatal pseudocoarctation: echocardiographic findings in vein of Galen malformation.

OBJECTIVE: Vein of Galen aneurysmal malformations (VGAMs) are rare congenital malformations thought to develop during weeks 6 to 11 of fetal life. Although they represent less than 1% of all cerebral vascular malformations, they constitute up to 30% of all pediatric vascular malformations. Vein of Galen aneurysmal malformations cause high-output heart failure in the fetus and neonate secondary to the decreased resistance and high blood flow in the lesion. We describe 2 cases, 1 prenatal and 1 postnatal, in which unusual aortic Doppler flow patterns and substantial brachiocephalic vessel dilation contributed to the discovery of a VGAM. METHODS: Echocardiographic findings associated with VGAM malformations in 2 cases are described. RESULTS: Unusual Doppler flow patterns and substantial brachiocephalic vessel dilation were seen in both cases. Pseudocoarctation of the aorta was also noted in both cases. CONCLUSIONS: The echocardiographic findings in fetal and neonatal VGAM may include pseudocoarctation of the aorta. Abnormal fetal cardiac findings should raise the practitioner's suspicion for cerebral malformations and vice versa.     

Natural history, evaluation, and management of intracranial vascular malformations

Intracranial vascular malformations are seen increasingly in clinical practice, primarily because of advances in cross-sectional brain and spinal cord imaging. Commonly encountered lesion types include arteriovenous malformations, cavernous malformations, venous malformations, dural arteriovenous fistulas, and capillary telangiectasias. Patients can experience various symptoms and signs at presentation. The natural history of vascular malformations depends on lesion type, location, size, and overall hemodynamics. The natural history for each lesion subtype is reviewed, with special consideration of the risk of hemorrhage or other adverse outcomes after the lesion is detected and any known predictors of hemorrhage or other outcomes. In practice, these data are compared with the risk of available treatment options as the optimal management is clarified. A multidisciplinary approach including neurosurgery, radiosurgery, interventional neuroradiology, and vascular neurology is most useful in determining the best management strategy.     

Color Doppler imaging is a valuable tool for the diagnosis and management of uterine vascular malformations.

OBJECTIVE: The aim of this study was to assess the spontaneous outcome of uterine vascular malformations detected with ultrasonography and color Doppler, and to investigate the predictive value of color Doppler imaging as to which patients require invasive treatment. METHODS: This was a prospective observational study conducted between January 1999 and February 2001 comprising all consecutive patients diagnosed with a uterine vascular malformation by ultrasonography and color Doppler imaging. Spectral analysis included measurement of flow velocities, pulsatility index (PI) and resistance index (RI). Close follow-up was arranged in all cases and the outcomes were recorded. RESULTS: A total of 30 consecutive patients with uterine vascular malformations were included in the study. Spectral analysis of the vessels in the vascular malformations within the myometrium and endometrium revealed the presence of a low-impedance and high-velocity flow. The average values for PI, RI, peak systolic velocity (PSV) and time-averaged maximum velocity (TAMXV) were 0.50, 0.38, 0.63 m/s and 0.46 m/s, respectively. Eight patients (27%) eventually required embolization of the uterine arteries and three of them had true arteriovenous malformations confirmed at angiography. PSV values of >/= 0.83 m/s were associated with higher probabilities of further treatment, such as an embolization, whereas no vascular malformation with a PSV value < 0.39 m/s required embolization. CONCLUSION: Conservative management is possible in more than two-thirds of patients presenting with uterine vascular malformations diagnosed by color Doppler sonography. Despite considerable overlap, PSV values appear to be useful in distinguishing between low- and high-risk patients.     

Emerging mechanisms of vascular stabilization

Summary.  Neural guidance cues are essential for a growing axon to correctly course through the body and innervate target tissues. Interestingly, the vascular network follows a parallel trajectory along nerves, suggesting that guidance cues important for neural patterning may also be required for proper vascular patterning. However, while an axon arises from one cell, a blood vessel is composed of many endothelial cells. Recent evidence suggests that neural repulsive cues are usurped by multi-cellular blood vessels to ensure vascular stabilization cues. Additional clues into the signaling mechanisms that promote vascular stabilization are emerging from cerebral cavernous malformations, a disease characterized by headache, epilepsy, and stroke. Thus, neurobiology and neurology are providing insights into the concepts of vascular stability.     

Ultrasound-Navigated Interstitial Nd:YAG LaserCoagulation of Congenital Vascular Disorders

The treatment of voluminous hemangiomas or vascular malformations, particularly of the infiltrative type, is difficult and requires a combination of methods such as surgical excision, embolisation, laser therapy, sclerotherapy and magnesium spiking. The goal of this study was to investigate the efficacy of ultrasound-navigated interstitial Nd:YAG (Neodymium:Yttrium-Aluminium-Garnet) laser coagulation of hemangiomas and vascular malformations.Ultrasound-navigated interstitial Nd:YAG laser coagulation was performed on 21 patients with cavernous hemangiomas or vascular malformations. 12 patients had vascular malformations primarily of the venous kind, whereas arteriovenous components were predominant in 5 patients. In 4 children, an extensive hemangioma was the indication for interstitial laser therapy. Laser energy with a power of 7–10 W was applied via puncture canula through a 600 μm glass fiber and with support of ultrasound guided to the vessel wall. Immediately after laser application the blood flow was controlled with color-coded duplexsonography.Three months after lasertreatment, a volume reduction between 60% and 80% in hemangiomas (n = 4), between 20% and 70% in venous malformations (n = 12) and 0% to 70% in arteriovenous malformations (n = 5) was achieved. With exception of necrosis in one patient which healed spontaneously, no further complications occurred.Ultrasound-navigated interstitial Neodym-YAG-laser coagulation is an effective and minimally invasive method for treating voluminous hemangiomas and vascular malformations and is an alternative or complimentary to other therapies.     

Lethal progression of a fetal intracranial arteriovenous malformation.

Fetal intracranial vascular tumors present unique clinical challenges. Accurate diagnosis of the lesion, as well as an understanding of the local and systemic impacts, will guide the antenatal surveillance and the treatment plan and will determine the prognosis. Management will be altered by and dependent on intrauterine progression, gestational age, and fetal condition at birth. In addition, large vascular tumors can lead to the development of the Kasabach-Merritt sequence in the fetus and to either fetal or maternal hemodynamic impairment. Vascular tumors are either malformations or neoplasms. Color and pulsed wave Doppler sonography are useful for the identification of vascular lesions and help narrow the differential diagnosis. Once a vascular malformation is identified, a comprehensive anatomic survey is mandatory to determine whether there are coexistent malformations that impact either the diagnosis or prognosis. These lesions can have local mass effects, systemic hemodynamic effects, or both. Therefore, longitudinal assessment of the fetus is focused on the detection of lesion progression and on any fetal or maternal status changes. We report the prenatal diagnosis of an intracranial arteriovenous malformation (AVM) with a dramatic progression affecting both mother and fetus.     

Peripheral vascular tumors and vascular malformations: imaging (magnetic resonance imaging and conventional angiography), pathologic correlation and treatment options

Vascular anomalies are classified into vascular tumors (infantile hemangioma) and vascular malformations. Vascular malformations are divided into slow flow and high flow subtypes. Magnetic resonance imaging helps in classification and assessing extent and distribution. Conventional angiography also known as digital subtraction angiography is pivotal in assessment of fine vascular details and treatment planning. Imaging correlates well with histopathology. We review recent development in imaging techniques of various vascular anomalies most of which are affecting the peripheral system which potentially may broaden understanding of their diagnosis, classification and treatment.     

Cerebral arterial fenestration

Fenestration or duplication of a cerebral vessel is a rare congenital variation which most commonly involves the vertebral artery and is usually of no clinical significance. Fenestrations may have associated aneurysms remote to the anomaly or involving the proximal bifurcation of the fenestration. They may also be associated with other vascular malformations e.g. arteriovenous malformations and venous angiomas. Four fenestrated cerebral vessels and their angiographic appearances are reported in this study.     

Magnetic resonance imaging of facial vascular anomalies.

In 36 patients, facial vascular anomalies were studied with 46 magnetic resonance (MR) examinations, 9 angiograms, and 5 computed tomographic scans. All lesions were categorized into classic pathologic groups on the basis of radiologic and pathologic studies, clinical examination, and behavior. Overall, 2 juvenile hemangiomas, 3 capillary malformations (port-wine stains), 18 venous malformations, 9 lymphatic malformations, and 4 arteriovenous malformations were found. MR imaging was superior to computed tomography and angiography for demonstrating the precise anatomic extent of the facial vascular anomalies and their relationship to the adjacent soft tissues but was inferior to computed tomography for demonstrating radiopaque structures such as trophic bone changes and phleboliths. MR imaging was also inferior to angiography in determining the nidus and the exact nature of collateral vascular structures in arteriovenous malformations. MR studies confirmed the clinically suspected diagnosis of facial vascular anomalies and demonstrated typical characteristics for each type of lesion. MR imaging is an ideal initial technique to triage patients with facial vascular anomalies for appropriate management, including observation, endovascular therapy, or surgical excision.