Diagnosing moyamoya syndrome using ultrasound - a case report

Background

Moyamoya syndrome is a vasculopathy characterised by progressive occlusion of the cerebral arteries resulting in the development of abnormal collateral circulation. To diagnose this syndrome, imaging of the cerebral arteries is required including CT- or MR-angiography and conventional angiography. We present a case of moyamoya disease with typical findings detected in the sonography. The diagnosis was suspected after reviewing the initial ultrasound images of the cerebral arteries with evidence for obliterated intracranial arteries and the detection of an existing collateral circulation network.

Case presentation

A 62 years old male patient presented in the hospital’s emergency department with symptoms indicating a subacute cerebrovascular event. Immediate sonographic studies showed a right-sided pulsatile Doppler-signal in the common and internal carotid arteries, suggestive of distal stenoses. In addition, the transcranial examination indicated obliteration of both middle cerebral arteries. Numerous arterial vessels suggestive of leptomeningeal collateral arteries revealed a strong arterial leptomeningeal flow. At this stage of the diagnostic work-up, the collateral circulation network, characteristic of moyamoya disease, was indicated by sonography. Moyamoya syndrome was verified by conventional angiography. The aetiological work remained empty, so the diagnosis of moyamoya disease was established.

Conclusion

Our case report indicates that sonography can be a useful tool for detecting the vaculopathy in moyamoya syndrome. In case routine procedures, such as the CT- or MR-angiography, with evidence for obliterated intracerebral arteries, ultrasound studies might provide important information regarding an existing collateral network in the scope of a moyamoya syndrome.

     

Septal collateralization: demonstration of canine intramyocardial collaterals

The anatomical distribution of intramyocardial collateral arteries that develop from the septal to the other major coronary arteries was studied in dogs following gradual Ameroid occlusion of the circumflex artery. The septal artery was cannulated and injected with Batson's plastic compound resulting in a cast of the coronary circulation. Collateral vessels radiated from the septal vascular bed to both the circumflex and anterior descending arteries. The collaterals developed from the entire base-to-apex extent of the septal artery and were found on both the right and left sides of the septum. Collateral growth appeared to be more concentrated at the apex of the heart. The anatomical details of septal collateral circulation illustrate the importance of intramyocardial collateralization in the dog, which was thought to exhibit primarily epicardial collaterals.     

The role fo collateral circulation in the course of coronary heart disease: 10-year clinical and angiographic follow-up

The paper presents the results of a 10-year prospective follow-up of 59 patients with coronary heart disease (CHD) concurrent with functional classes II-IV angina pectoris. Coronarography was made in all the patients whose coronary arteries and collateral blood flow were assessed. The experimental group comprised 37 patients with CHD and collateral circulatory insufficiency. The control group included 22 patients with effective collateral circulation. The experimental group showed a worse prognosis than did the control one. Myocardial infarction developed in 54 and 27% of cases, coronary heart disease mortality was 29.7 and 9% in the experimental and control groups, respectively. Effective collateral circulation is a prerequisite of successful surgical myocardial infarction.     

Abnormalities of the Vertebrobasilar Circulation due to Subclavian Artery Disease

The studies of Hutchinson and Yates on caroticovertebral stenosis have stimulated further interest in cerebrovascular disease. As a consequence, investigation of the four vessels, supplying the brain is now routine in the assessment of patients being considered for surgery for occlusive disease of the vessels. This has led to some interesting and unexpected findings.The effect of subclavian artery disease on the vertebrobasilar circulation was studied in two patients. Angiography showed the vertebral arteries to function as a collateral pathway when an occlusion of the subclavian artery was present proximal to the origin of the vertebral arteries. In one case both the carotid and the vertebral arteries were implicated in the collateral supply. A normal circulation was restored by subclavian endarterectomy.Studies of other workers have shown that such a circulation may reduce the cerebral blood flow by about 40%, but neither patient described in this report had signs or symptoms of cerebrovascular disease. It is evident that our understanding of the effects of cerebrovascular disease is far from complete.     

Experimental study of hemodynamics in the circle of willis

Background

The Circle of Willis (CoW) is an important collateral pathway of the cerebral blood flow. An experimental study of the cerebral blood flow (CBF) distribution in different anatomical variations may help to a better understanding of the collateral mechanism of the CoW.

Methods

An in-vitro test rig was developed to simulate the physiological cerebral blood flow in the CoW. Ten anatomical variations were considered in this study, include a set of different degrees of stenosis in L-ICA and L-ICA occlusion coexist with common anatomical variations. Volume flow rates of efferent arteries and pressure signals at the end of communicating arteries of each case were recorded. Physiological pressure waveforms were applied as inlet boundary condition.

Results

In the development of L-ICA stenosis, the total CBF decreases with the increase of stenosis degree. The blood supply of ipsilateral middle cerebral artery (MCA) was affected most by the stenosis of L-ICA. Anterior communicating artery (ACoA) and ipsilateral posterior communicating artery (PCoA) function as important collateral pathways of cerebral collateral circulation when unilateral stenosis occurred. The blood supply of anterior cerebral circulation was compensated by the posterior cerebral circulation through ipsilateral PCoA when L-ICA stenosis degree is greater than 40% and the affected side was compensated immediately by the unaffected side through ACoA. Blood flow of the anterior circulation and the total CBF reached the minimum among all cases studied when L-ICA occlusion coexist with the absence of PCoA.

Conclusion

The results demonstrated the flow distribution patterns of the CoW under anatomical variations and clarified the collateral mechanism of the CoW. The flow ACoA is the most sensitive indexes to the morphology change of ipsilateral ICA. The relative independence of the circulation in anterior and posterior sections of the CoW is not broken and the function of ipsilateral PCoA is not activated until a severe stenosis of unilateral ICA occurs. PCoA is the most important collateral pathway of the collateral circulation and the missing of PCoA has the highest risk of stroke when the ipsilateral ICA has severe stenosis. These findings may provide the basis for future therapeutic and diagnosis applications.

     

Kidney of the sumatran rhinoceros,Dicerorhinus sumatrensis

The kidney of Diceros sumatrensis has the rhinocerotic form and lobation. The ureter divides intrarenally into two fibromuscular conduits which receive, at separate loci, the terminal collecting ducts of the lobes. The kidney is 67% cortex. Total renal mass is 0.46% of body mass. There are about 34 lobes and 23 primary orifices at the conduits. Glomeruli are relatively small and of the same size across the cortex. They number about 14.6 million in one kidney but the glomerular mass is relatively low. Unlike other rhinocerotic species, the kidney of D. sumatrensis lacks interlobar septa. The interlobar arteries nevertheless enter the renal parenchymas as in the other rhinoceroses, i.e., between the cortices of adjacent lobes rather than in the common mammalian manner between cortex and medulla. Thus, internal “perforator” arteries pass from cortical periphery to interior giving off cortical twigs on their way to the corticomedullary border, along which they branch while releasing centrifugal arteries back toward the cortical periphery. The arcuate veins are wide centrally where they enter the paraconduital veins. The latter form prominent central anastomoses between the large interlobar veins. © 1993 Wiley-Liss, Inc.     

Effects of exercise training on coronary collateralization and control of collateral resistance

Coronary collateral vessels serve as a natural protective mechanism to provide coronary flow to ischemic myocardium secondary to critical coronary artery stenosis. The innate collateral circulation of the normal human heart is typically minimal and considerable variability occurs in extent of collateralization in coronary artery disease patients. A well-developed collateral circulation has been documented to exert protective effects upon myocardial perfusion, contractile function, infarct size, and electrocardiographic abnormalities. Thus therapeutic augmentation of collateral vessel development and/or functional adaptations in collateral and collateral-dependent arteries to reduce resistance into the ischemic myocardium represent a desirable goal in the management of coronary artery disease. Tremendous evidence has provided documentation for the therapeutic benefits of exercise training programs in patients with coronary artery disease (and collateralization); mechanisms that underlie these benefits are numerous and multifaceted, and currently under investigation in multiple laboratories worldwide. The role of enhanced collateralization as a major beneficial contributor has not been fully resolved. This topical review highlights literature that examines the effects of exercise training on collateralization in the diseased heart, as well as effects of exercise training on vascular endothelial and smooth muscle control of regional coronary tone in the collateralized heart. Future directions for research in this area involve further delineation of cellular/molecular mechanisms involved in effects of exercise training on collateralized myocardium, as well as development of novel therapies based on emerging concepts regarding exercise training and coronary artery disease.     

High-salt diets during pregnancy increases renal vascular reactivity due to altered soluble guanylyl cyclase-related pathways in rat offspring

Adverse prenatal factors such as overtake of salt or fat food are potential risks for cardiovascular diseases in offspring. This study tested the hypothesis that prenatal high-salt (HS) diets may influence renal vascular tone and attenuates signaling pathways related to soluble guanylyl cyclase (sGC) or/and large-conductance Ca²⁺-activated K⁺ (BK_Ca) channels in the offspring.Pregnant rats were fed either normal salt (NS) (1% NaCl) or HS (8% NaCl) diet for the whole gestation. Offspring were maintained on NS diets. Renal interlobar arteries in offspring were tested for vascular responses to phenylephrine (Phe), K⁺ channels and signal pathways related to sGC.Phe induced higher vessel tension in interlobar arteries of the HS offspring. Following pretreatment with BK_Ca channel inhibitor iberiotoxin, Phe-mediated vasoconstrictions were decreased in HS offspring compared to NS. Phe-mediated constrictions following pretreatment with NO synthase inhibitor N(G)-nitro-l-arginine methyl ester or sGC inhibitor 1H-1,2,4-oxadiazolo-4,3-quinoxalin-1-one in the HS offspring were less sensitive than NS. The whole-cell K⁺ currents and the component of BK_Ca channels were not changed in smooth muscle cells from interlobar arteries, whereas the K⁺ currents stimulated by sGC activator BAY41-2272 were reduced in the HS offspring. The protein expressions of sGC β1 and β2 in the interlobar arteries of HS offspring were reduced.The results showed that chronic overintake of salt during pregnancy could increase renal vascular tone in the offspring. The affected signal pathways included down-regulation of sGC function and expression.     

Alpha 1 adrenergic receptor control of renal blood vessels during aging

Aging humans and rats have a reduced renal vascular constriction response to stress, change in posture, or exercise. In this study, renal interlobar arteries from 9- (intermediate age) to 15-month-old (aging) male Wistar rats constricted less to alpha-adrenergic agonists than those of 4-month-old (young adult) rats. The reduced contraction to A61603 (alpha 1 A agonist) was similar to that to norepinephrine and phenylephrine. Therefore, it appears that the reduction in constriction is primarily related to alpha 1 A receptor stimulation. GeneChip microarray hybridization analysis of the interlobar arteries with the RAE 230A GeneChip indicated that there were no significant differences in gene expression for alpha 1 A/C, 1B, or 1D receptors between 4-month-old (young adult) and 1-year-old (aging) male Wistar rats. Competitive binding experiments (prazosin) revealed that maximal binding (Bmax, fmol/mg protein) of the alpha 1 receptors of interlobar arteries was reduced 25% by 10 months of age and 50% by 18+ months of age. Alpha 1 receptor-induced arterial constriction and prazosin binding were both down-regulated. The loss of receptor-initiated constriction likely includes down-regulation of maximum agonist binding by alpha 1 adrenergic receptors.     

Uncertainty quantification in cerebral circulation simulations focusing on the collateral flow: Surrogate model approach with machine learning

Collateral circulation in the circle of Willis (CoW), closely associated with disease mechanisms and treatment outcomes, can be effectively investigated using one-dimensional–zero-dimensional hemodynamic simulations. As the entire cardiovascular system is considered in the simulation, it captures the systemic effects of local arterial changes, thus reproducing collateral circulation that reflects biological phenomena. The simulation facilitates rapid assessment of clinically relevant hemodynamic quantities under patient-specific conditions by incorporating clinical data. During patient-specific simulations, the impact of clinical data uncertainty on the simulated quantities should be quantified to obtain reliable results. However, as uncertainty quantification (UQ) is time-consuming and computationally expensive, its implementation in time-sensitive clinical applications is considered impractical. Therefore, we constructed a surrogate model based on machine learning using simulation data. The model accurately predicts the flow rate and pressure in the CoW in a few milliseconds. This reduced computation time enables the UQ execution with 100 000 predictions in a few minutes on a single CPU core and in less than a minute on a GPU. We performed UQ to predict the risk of cerebral hyperperfusion (CH), a life-threatening condition that can occur after carotid artery stenosis surgery if collateral circulation fails to function appropriately. We predicted the statistics of the postoperative flow rate increase in the CoW, which is a measure of CH, considering the uncertainties of arterial diameters, stenosis parameters, and flow rates measured using the patients’ clinical data. A sensitivity analysis was performed to clarify the impact of each uncertain parameter on the flow rate increase. Results indicated that CH occurred when two conditions were satisfied simultaneously: severe stenosis and when arteries of small diameter serve as the collateral pathway to the cerebral artery on the stenosis side. These findings elucidate the biological aspects of cerebral circulation in terms of the relationship between collateral flow and CH.     

The atrioventricular branches of the human coronary arteries

Branches of the coronary arteries of normal human hearts, supplying both atria and ventricles, were found by fine dissection and have been named rami atrioventriculares. They comprise atrial branches from ventricular arteries and ventricular branches from atrial arteries. Their incidence was 74% in the 50 adult individuals studied. The subjects had committed suicide with a poison which did not damage the coronary arteries. The atrioventricular branches constitute communications across the coronary sulcus, thus establishing a continuity between the atrial and the ventricular arterial supply. Therefore, there is not invariably a sharp demarcation of blood supply between atria and ventricles, as has been commonly taught. Neither atrial nor ventricular branches consist exclusively of ascending and descending branches of the coronary arteries, as has been assumed. Atrioventricular branches can play a role in collateral circulation and may, in individuals who are born with them, provide an explanation for some of the variability in signs and symptoms incidental to heart attacks.     

Surgical anatomy of the anastomotic branch between the occipital and vertebral arteries

Using anatomical material of mature persons and, for the first time, that of newborns, the discovered well developed anastomotic branch between the occipital and vertebral arteries can be considered as an anatomical variant of the vascular system, important for the collateral blood circulation. Similar branch in mature persons can also serve as an intermediate link for transmitting irritation at the neck osteochondrosis along the periarterial plexuses from the vertebral artery to the occipital one, that results in reflectory pains in the posterior area of the neck.     

Coronary three-vessel disease with occlusion of the right coronary artery: What are the most important factors that determine the right territory perfusion?

With progressive occlusion of a coronary main artery, some anastomotic vessels are recruited in order to supply blood to the ischemic region. This collateral circulation is an important factor in the preservation of the myocardium until reperfusion of the area at risk. An accurate estimation of collateral flow is crucial in surgical bypass planning as it alters the blood flow distribution in the coronary network and can influence the outcome of a given treatment for a given patient. The evaluation of collateral flow is frequently achieved using an index based on pressure measurements. It is named collateral flow index (CFI) and defined as: (P_w − P_v)/(P_ao − P_v), where P_w is the pressure distal to the thrombosis, P_ao the aortic pressure and P_v the central venous pressure. In the present work, we study patients with severe coronary disease (stenoses on the left branches and total occlusion of the right coronary artery). Using a mathematical model that describes the coronary hemodynamics in that situation, we demonstrate that the dependence of the collateral circulation to the pressure values is not as simple as it is commonly believed: using pressures alone as an index of collateral flow is likely to result in misinterpretation of the collateral flow contribution, because collateral flow depends on many other factors related to the status of the native stenosed arteries and to the microvascular resistances (capillary and collateral resistances, and the proportion between them).     

Changes in the fast uterine muscle biopotentials in rabbits with chronic hypoxia

Changes of the high-frequency electrical activity in the myometrium after a separate and simultaneous ligation the uterine and ovarian vessels were studied in chronic experiments (137 experiments on 18 non-pregnant rabbits which had already had pregnancies in the past). The highest and most prolonged (up to 45 days) inhibition of the amplitude and frequency of rapid (peak) potentials of the myometrium follows a simultaneous ligation of the uterine and ovarian arteries and veins. The amplitude and frequency of biopotentials is gradually restored with the compensation of blood circulation by the collateral blood channels.     

The ankyrin repeat containing SOCS box protein 5: a novel protein associated with arteriogenesis

Arteriogenesis, the growth of pre-existing collateral arteries, can be induced in rabbit by occlusion of the femoral artery. In order to identify and characterize genes differentially expressed during the early phase of arteriogenesis, cDNA of collateral arteries 24h after femoral ligation or sham operation was subjected to suppression subtractive hybridization. We identified the ankyrin repeat containing SOCS box protein 5 (asb5) and cloned the rabbit full-length cDNA. Asb5 was demonstrated to be a single-copy gene. We localized the asb5 protein in vivo in endothelial and smooth muscle cells of collateral arteries as well as in satellite cells. Asb5 was significantly upregulated in growing collateral arteries on mRNA and protein level. The infusion of doxorubicin in rabbit led to a significant decrease of the asb5 mRNA. In summary, our data show that asb5 is a novel protein implicated in the initiation of arteriogenesis.     

Identification of differentially expressed genes like cofilin2 in growing collateral arteries

Arteriogenesis, the growth of pre-existing collateral arteries, can be induced in rabbits by occlusion of the femoral artery. In order to analyze the differential gene expression in arteriogenesis, cDNA of collateral arteries 24h after femoral occlusion or sham operation was subjected to suppression subtractive hybridization (SSH). We demonstrated an upregulation of the U6 snRNA binding protein Lsm5, cytochrome b, an expressed sequence tag, and the actin-depolymerizing factor cofilin2 mRNA in collateral arteries 24h after femoral ligation. For cofilin2, we also detected an increase in the protein level and a localization predominantly in smooth muscle cells of collaterals. Simultaneously with the upregulation of cofilin2 we found a downregulation of the alpha-smooth muscle actin mRNA in growing collateral arteries. In summary, our data showed an augmented expression level of genes contributing to different fundamental processes of arteriogenesis.     

Evaluation of renal blood flow by Doppler study in children and adolescents with type 1 diabetes

Renal blood flow was ultrasonographically studied in 220 children, adolescents, and young, postpubertal patients who had fell ill with type 1 diabetes mellitus (DM) in childhood (of them there were 111 (49.8%) males and 112 (50.2%) females with a disease history of 1 to 27 years (73 patients with diabetic nephropathy (DN) and 150 without DN). Thirty apparently healthy children and adolescents made up a comparison group. Doppler duplex scanning and Doppler pulse-wave study (DPWS) were made at the level of the great renal artery, segmental, interlobar, and arcuate arteries. With DPWS, the values of peripheral resistance were lower than the normal values at the level of interlobar and arcuate arteries in all patients with microangiopathies. There were significant differences between the groups of patients with different types of microangiopathies, the group of patients with DM without DMA, and the control group. Echography was used to determine the volumes of the kidneys in 220 patients. In patients with DM, renal volume was significantly greater than that in the control patients (p<0.05).     

lp-PLA2、RBP与冠心病病变程度相关性及其疾病诱发的危险因素分析

摘要 目的：研究脂蛋白磷脂酶A2（lp-PLA2）、视黄醇结合蛋白（RBP）与冠心病（CAD）病变程度的相关性及冠心病病变发生的危险因素。方法：以2019年12月至2021年12月在本院诊治的冠心病患者140例作为研究对象,所有患者均进行冠状动脉造影检查,并根据冠状动脉侧支循环形成情况进行分级。所有患者都给予血清lp-PLA2、RBP检测并进行相关性、危险因素分析。结果：在140例患者中,冠状动脉侧支循环未形成40例（对照组）,冠状动脉侧支循环形成100例（研究组）,研究组中Ⅰ级40例,Ⅱ级38例,Ⅲ级22例。研究组的血清lp-PLA2、RBP含量都高于对照组（P<0.05）;不同分级患者的血清lp-PLA2、RBP含量对比也有明显差异（P<0.05）。在冠心病患者中,Spearsman相关分析显示血清lp-PLA2、RBP含量与侧支循环形成分级存在正相关性（P<0.05）。logistic回归分析显示血清lp-PLA2、RBP含量均为影响冠心病患者侧支循环形成分级的危险因素（P<0.05）。ROC曲线分析显示血清lp-PLA2、RBP含量预测冠心病患者侧支循环分级的曲线下面积为0.891、0.805。结论：随着冠状动脉侧支循环形成,冠心病患者的血清lp-PLA2、RBP含量明显增加,lp-PLA2、RBP与侧支循环形成分级存在相关性,也是影响侧支循环分级的危险因素,也可预测侧支循环分级状况。