Morphological Pattern and Classification of the Superficial Middle Cerebral Vein by Cadaver Dissections: An Embryological Viewpoint.

In this study, we used 45 adult cadaveric cerebral hemispheres to investigate the anatomical classification of the superficial middle cerebral vein (SMCV) based on the number of stems, course, and anastomosis at the distal portion. We classified the SMCVs into five types based on embryological concept. Type A (18 cases, 40.0%) is that the frontosylvian veins (FSVs) merge with the vein of Trolard (VT) and the vein of Labbé (VL) at the distal portion of the sylvian fissure. Type B (5 cases, 11.1%) is that the temporosylvian veins (TSVs) merge with the VT and the VL at the distal portion. Type C (13 cases, 28.9%) is that no vein merge with the VT and the VL at the distal portion. The VT merges with the SMCV from the FSV and the VL merges with the SMCV from the TSV. They course along the sylvian fissure and merge at the proximal portion. In Type D (eight cases: 17.8%), the VT and the VL merge at the distal portion, and the SMCV from the FSV and the SMCV from the TSV join their confluence without merging. Type E (one case, 2.2%) show an undeveloped SMCV. Formation rate of intravenous anastomoses or bridging veins(BVs) at the distal portion between the frontosylvian trunk (FST) and the temporosylvian trunk (TST), between the FST and the temporal lobe, and between the TST and the frontal lobe was very low, because these formation may be difficult to occur during the embryological process in which the SMCV is formed from the telencephalic vein.

Characterization of Endothelial Cilia Distribution During Cerebral-Vascular Development in Zebrafish ( Danio rerio).

Objective- Endothelial cells (ECs) sense and respond to flow-induced mechanical stress, in part, via microtubule-based projections called primary cilia. However, many critical steps during vascular morphogenesis occur independent of flow. The involvement of cilia in regulating these stages of cranial vascular morphogenesis is poorly understood because cilia have not been visualized in primary head vessels. The objective of this study was to investigate involvement of cilia in regulating the early stages of cranial vascular morphogenesis. Approach and Results- Using high-resolution imaging of the Tg(kdrl:mCherry-CAAX) y171 ;(bactin::Arl13b:GFP) zebrafish line, we showed that cilia are enriched in the earliest formed cranial vessels that assemble via vasculogenesis and in angiogenic hindbrain capillaries. Cilia were more prevalent around the boundaries of putative intravascular spaces in primary and angiogenic vessels. Loss of cardiac contractility and blood flow, because of knockdown of cardiac troponin T type 2a ( tnnt2a) expression, did not affect the distribution of cilia in primary head vasculature. In later stages of development, cilia were detected in retinal vasculature, areas of high curvature, vessel bifurcation points, and during vessel anastomosis. Loss of genes crucial for cilia biogenesis ( ift172 and ift81) induced intracerebral hemorrhages in an EC-autonomous manner. Exposure to high shear stress induced premature cilia disassembly in brain ECs and was associated with intracerebral hemorrhages. Conclusions- Our study suggests a functional role for cilia in brain ECs, which is associated with the emergence and remodeling of the primary cranial vasculature. This cilia function is flow-independent, and cilia in ECs are required for cerebral-vascular stability.

A review of extraaxial developmental venous anomalies of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, venous varices or aneurysmal malformations, and enlarged emissary veins.

This paper is a narrative review of extraaxial developmental venous anomalies (eDVAs) of the brain involving dural venous flow or sinuses: persistent embryonic sinuses, sinus pericranii, enlarged emissary veins, and venous varices or aneurysmal malformations. The article highlights the natural history, anatomy, embryology, imaging, clinical implications, and neurosurgical significance of these lesions, which the authors believe represent a continuum, with different entities characterized by distinct embryopathologic features. The indications and surgical management options are discussed for these individual intracranial pathologies with relevant illustrations, and a novel classification is proposed for persistent falcine sinus (PFS). The role of neurointervention and/or microsurgery in specific cases such as sinus pericranii and enlarged emissary veins of the skull is highlighted. A better understanding of the pathophysiology and developmental anatomy of these lesions can reduce treatment morbidity and mortality. Some patients, including those with vein of Galen malformations (VOGMs), can present with the added systemic morbidity of a high-output cardiac failure. Although VOGM is the most studied and classified of the above-mentioned eDVAs, the authors believe that grouping the former with the other venous anomalies/abnormalities listed above would enable the clinician to convey the exact morphophysiological configuration of these lesions, predict their natural history with respect to evolving venous hypertension or stroke, and extrapolate invaluable insights from VOGM treatment to the treatment of other eDVAs. In recent years, many of these symptomatic venous malformations have been treated with endovascular interventions, although these techniques are still being refined. The authors highlight the broad concept of eDVAs and hope that this work will serve as a basis for future studies investigating the role of evolving focal venous hypertension/global intracranial hypertension and possibilities of fetal surgical intervention in these cases.

Dilated cerebral venous system observed in growth-restricted fetuses.

PURPOSE: The dilation of the fetal cerebral veins is a rare phenomenon that may be associated to a bad obstetric outcome, and is usually connected to antenatal thrombosis of the posterior dural venous sinuses. There are several descriptions of cerebral vein distension on magnetic resonance imaging (MRI), but all of them are detected postnatally. We present herein two cases of fetal antenatal cerebral dilation of the venous system, without any association to any sign of vein thrombosis, and a systematic review of literature regarding pathogenesis, diagnosis and outcomes associated to the antenatal detection of this condition with the use of MRI. MATERIALS AND METHODS: To identify potentially eligible studies, we searched PubMed, Scopus, Cochrane Library (all from inception to October 20th, 2016) and applied no language restrictions. RESULTS: The electronic database search provided a total of 22,843 results. After the exclusion of duplicates, manuscripts that resulted not relevant to the review based on title and abstract screening, and analysis of manuscripts eligible for full-text assessment, no papers were found related to the subject reported in the present manuscript. CONCLUSIONS: Our report adds importance to MRI as a tool in cases of complex ultrasound finding with the presence of fetal heart failure and deterioration of fetal growth, in order to improve the prognostic evaluation and patient?s counseling.

Nongalenic pial arteriovenous fistula: Prenatal diagnosis.

Pial arteriovenous (AV) fistulae have rarely been diagnosed in utero. They are characterized by one or more pial arteries flowing directly into a cortical vein without any shunt or interposed capillary bed. In the fetus and the newborn up to 2 years of age, the most common clinical manifestation is heart failure resulting from fistula overload. Later on, hydrocephalus, focal neurologic deficits, headaches, seizures, and cerebral hemorrhage are the most common manifestations. We present a case of nongalenic pial AV fistula diagnosed in the 25th week of pregnancy, which resulted in intrauterine fetal death due to congestive heart failure. © 2017 Wiley Periodicals, Inc. J Clin Ultrasound 45:621-625, 2017.

Bilateral thalamic developmental venous variations (DVVs) draining into same internal cerebral vein: a case report and review with emphasis on DVVs with outflow restriction.

Developmental venous variations (DVVs) are anatomic variations of normal transmedullary veins which are often discovered incidentally. Although they are accepted as benign compensatory venous drainage systems, they may become symptomatic or clinically significant due to flow-related causes. The fragile venous drainage systems increase vulnerability to in-out flow alterations. Increased inflow or decreased outflow causes rise in venous pressure, which may subsequently produce ischemic symptoms. Obstruction or stenosis of the collector vein is the most common cause of decreased outflow of a DVV. However, in the absence of collecting vein stenosis, venous hypertension may still exist due to volume overload. In case of multiple DVVs with single combined drainage pathway, functional outflow restriction may occur due to diminished capability of the vessel to adapt to pressure changes. In this report, we present a case with bilateral thalamic DVVs, which cause parenchymal amorphous calcification and drain into the left internal cerebral vein. A review of the literature on DVVs with outflow restriction including pathophysiological mechanisms is also discussed.

Developmental venous anomalies of the brain in children -- imaging spectrum and update.

Developmental venous anomalies (DVAs) are the most common vascular malformation of the brain and are commonly identified on routine imaging of the brain. They are typically considered incidental findings, usually with no clinical significance. However the increasing identification of DVAs as a result of improved imaging technology has led to recognition of their association with a variety of abnormal imaging findings and clinically important conditions. This pictorial essay explores the suspected embryological origin, associated imaging features, and proposed pathophysiological mechanisms of DVAs in the pediatric population. This paper emphasizes newer physiological imaging data, which suggest that DVA drainage has less physiological flexibility than otherwise normal venous drainage development.

Ultrasound of the Fetal Veins Part 3: The Fetal Intracerebral Venous System.

The study of the intracerebral venous system in the fetus can only be achieved by means of high-resolution ultrasound equipment with sensitive color Doppler. In the past two decades, there has been a growing interest in the ultrasound examination of the fetal brain with few studies reporting on the brain vasculature during various stages of gestation. In comparison to other fetal venous systems, reports on the assessment of the fetal cerebral venous system are still scarce. This article presents a review on the fetal intracranial venous system with detailed discussions on the anatomy of the superficial and deep cerebral veins. Color Doppler of the main fetal cerebral veins to include the superior sagittal sinus, the straight sinus, the vein of Galen, the internal cerebral veins, the transverse sinuses and others is also discussed. Furthermore, this article highlights abnormal clinical conditions such as aneurysm of the vein of Galen, thrombosis of the dural sinus and variation in the course of some veins such as the straight sinus and falcine sinus. The role of pulsed Doppler examination in normal and growth-restricted fetuses is also discussed.

Fetal MRI demonstrating vein of Galen malformations in two successive pregnancies--a previously unreported occurrence.

PURPOSE: Vein of Galen malformations are rare and are usually detected in utero using ultrasonography. No definite genetic predisposition has been described in the literature. We present a case with two successive pregnancies complicated by vein of Galen malformations, which were assessed using fetal MRI. The putative role of genetic mutations is also discussed. METHODS: A 30-year-old primigravida presented in the third trimester with a fetus diagnosed with vein of Galen malformation on sonography. MRI and MR angiography were performed for further assessment. The subsequent pregnancy was again complicated by vein of Galen malformation. In addition to MRI, genetic analysis was carried out on both fetuses and on the parents. RESULTS: MR angiography revealed that both fetuses suffered from the choroidal sub-type of vein of Galen malformation, with multiple arterial feeders fistulating onto a midline venous pouch. The visualised anatomy obtained was far superior than on sonography and allowed categorisation of vein of Galen malformation sub-type. Genetic analysis on the mother and both fetuses showed variant RASA1 gene mutation. CONCLUSIONS: This case demonstrates that fetal MRI is a powerful tool in the investigation of in utero neurovascular malformations. A genetic mutation was identified, but this was of uncertain significance.

Visualizing cerebral veins in fetal brain using susceptibility-weighted MRI.

AIM: To explore the feasibility of two-dimensional (2D) susceptibility-weighted imaging (SWI) in the visualization of cerebral veins in the foetal brain. MATERIALS AND METHODS: Forty-two pregnant healthy women (gestational age: 19-37 weeks, mean: 28.5 ± 7.1 weeks) underwent SWI examination using a 1.5 T MRI system. Two neurologists independently analysed all magnetic resonance imaging (MRI) studies. The relationship between the veins detected and the gestational age was investigated. The prominence of veins was assessed using a categorical score. RESULTS: In total, 167 veins were detected by SWI in 29 subjects with a symmetric hemisphere distribution (p > 0.05). An additional vein was detected by SWI biweekly from 24 weeks of gestation. Most veins of Galen and internal cerebral veins on SWI images were prominent, whereas others were faint or moderate. CONCLUSION: SWI appears to be a feasible method of detecting cerebral veins in the foetal brain.

MR venography of the fetal brain using susceptibility weighted imaging.

PURPOSE: To evaluate the feasibility of performing fetal brain magnetic resonance venography using susceptibility weighted imaging (SWI). MATERIALS AND METHODS: After obtaining informed consent, pregnant women in the second and third trimester were imaged using a modified SWI sequence. Fetal SWI acquisition was repeated when fetal or maternal motion was encountered. The median and maximum number of times an SWI sequence was repeated was four and six respectively. All SWI image data were systematically evaluated by a pediatric neuroradiologist for image quality using an ordinal scoring scheme: 1. diagnostic; 2. diagnostic with artifacts; and 3. nondiagnostic. The best score in an individual fetus was used for further statistical analysis. Visibility of venous vasculature was also scored using a dichotomous variable. A subset of SWI data was re-evaluated by the first and independently by a second pediatric neuroradiologist. Kappa coefficients were computed to assess intra-rater and inter-rater reliability. RESULTS: SWI image data from a total of 22 fetuses were analyzed. Median gestational age and interquartile range of the fetuses imaged were 32 (29.9-34.9) weeks. In 68.2% of the cases (n = 15), there was no artifact; 22.7% (n = 5) had minor artifacts and 9.1% (n = 2) of the data was of nondiagnostic quality. Cerebral venous vasculature was visible in 86.4% (n = 19) of the cases. Substantial agreement (Kappa = 0.73; 95% confidence interval 0.44-1.00)) was observed for intra-rater reliability and moderate agreement (Kappa = 0.48; 95% confidence interval 0.19-0.77) was observed for inter-rater reliability. CONCLUSION: It is feasible to perform fetal brain venography in humans using SWI.

Measuring venous blood oxygenation in fetal brain using susceptibility-weighted imaging.

PURPOSE: To evaluate fetal cerebral venous blood oxygenation, Yv, using principles of MR susceptometry. MATERIALS AND METHODS: A cohort of 19 pregnant subjects, with a mean gestational age of 31.6 ± 4.7 weeks were imaged using a modified susceptibility-weighted imaging (SWI) sequence. Data quality was first assessed for feasibility of oxygen saturation measurement, and data from five subjects (mean ± std gestational age of 33.7 ± 3.6 weeks) were then chosen for further quantitative analysis. SWI phase in the superior sagittal sinus was used to evaluate oxygen saturation using the principles of MR susceptometry. Systematic error in the measured Y(v) values was studied through simulations. RESULTS: Simulations showed that the systematic error in Yv depended upon the assumed angle of the vessel, θ, relative to the main magnetic field and the error in that vessel angle δθ. For the typical vessel angle of θ = 30° encountered in the fetal data analyzed, a δθ as large as ±20° led to an absolute error, δYv, of less than 11%. The measured mean oxygen saturation across the five fetuses was 66% ± 9.4%. This average cerebral venous blood oxygenation value is in close agreement with values in the published literature. CONCLUSION: We have reported the first in vivo measurement of human fetal cerebral venous oxygen saturation using MRI.

Petrosquamosal sinus: clinical characteristics based on radiologic and operative findings.

OBJECTIVE: Petrosquamosal sinus (PSS) is an embryonic emissary vein of the temporal bone connecting the intracranial and extracranial venous networks, which is present in some variants of venous cerebral drainage. The aim of the present study was to analyze 20 cases of PSS and to present its clinical characteristics and implications. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. INTERVENTION: Diagnostic. MAIN OUTCOME MEASURE: By reviewing retrospective medical records and TBCT findings, a total of 20 PSS cases were found. Based on the shapes of PSS demonstrated on TBCT, PSS was classified into tortuous and straight types. The course and thickness of PSS were also investigated. The average thicknesses of PSS between tortuous and straight types were compared. RESULTS: The mean age of the patients was 54.1 ± 16.2 years. The study group consisted of 7 male (35.0%) and 13 female (65.0%) patients. Eleven cases were found on the right side and 8 cases on the left side. The mean diameter of the bony canal that PSS courses on TBCT was 2.57 ± 0.88 mm. Its maximal and minimal diameters were 4.2 and 0.7 mm. The average diameter of tortuous type PSSs (3.04 ± 0.75 mm) was significantly larger compared with that of straight-type PSSs (2.09 ± 0.76 mm) (p < 0.05). CONCLUSION: Preoperative identification of PSS using TBCT may be important for safe mastoid surgery. The presence of PSS should be identified with thorough examination of radiographic findings before mastoid surgery.

The intracranial bridging veins: a comprehensive review of their history, anatomy, histology, pathology, and neurosurgical implications.

INTRODUCTION: The intracranial bridging veins are pathways crucial for venous drainage of the brain. They are not only involved in pathological conditions but also serve as important landmarks within neurological surgery. METHODS: The medical literature on bridging veins was reviewed in regard to their historical aspects, embryology, histology, anatomy, and surgery. CONCLUSION: Knowledge on the intracranial bridging veins and their dynamics has evolved over time and is of great significance to the neurosurgeon.

[The development of the cerebral venous system--from the embryo to the adult definitive structure]. / O desenvolvimento do sistema venoso cerebral--do embrião à estrutura definitiva no adulto.

INTRODUCTION: The human development consists of a continuous process where an uninterrupted pattern of extremely complex repetitive cycles of growth, modulation and modification take place. Despite this extreme complexity, the normal development is ordered by an impressive regularity, namely, in chronological, anatomical, topographic and physiological trends. In some organic systems, this development will not be totally ended by the time of birth. In this situation, further changes will have to take place until the adult definitive pattern is achieved. The cerebral venous system (CVS) is such a paradigm. PURPOSE: The authors pretend to present a synopsis of the specific organogenesis of the cerebral venous system in order to allow a correct interpretation of the vascular structures, recognise the anatomical variations and better comprehend the topographic correlations between neighbouring structures. METHOD AND RESULTS: Following a structured and synthetic order, the main guiding lines of the principal evolutional steps will be presented from the first embryological stages until the final and definitive adult pattern. CONCLUSION: The detailed study of the continuous development stages of the CVS constitutes a fundamental tool for the whole recognition of the anatomical structures, their correct interpretation and detection of possible variants as well as a better comprehension of topographic relationship between neighbouring structures. In other words and following KL Moore, Embryology illuminates anatomy.

Vein of Galen malformations: epidemiology, clinical presentations, management.

The vein of Galen aneurysmal malformation is a congenital vascular malformation that comprises 30% of the pediatric vascular and 1% of all pediatric congenital anomalies. Treatment is dependent on the timing of presentation and clinical manifestations. With the development of endovascular techniques, treatment paradigms have changed and clinical outcomes have significantly improved. In this article, the developmental embryology, clinical features and pathophysiology, diagnostic workup, and management strategies are reviewed.

Cerebral venous development in relation to developmental venous anomalies and Vein of Galen aneurysmal malformations.

Cerebrovascular venous development and intracranial vascular malformations are extensive topics for which volumes of text may be devoted. However, a basic knowledge of the embryology of cerebral venous system and venous architecture is essential for understanding of cerebral vascular malformations. The aim of this work is to provide the reader with a brief overview of the development of the cranial venous anatomy. We will highlight the superficial and deep venous systems with special attention to developmental venous anomalies and vein of Galen aneurysmal malformations.

Normal and abnormal embryology and development of the intracranial vascular system.

The brain vascular system develops in such a way that it continuously adapts the supply of oxygen and other nutrients to the needs of the parenchyma. To accompany the developing brain vesicles, it evolves in several steps: superficial meningeal network first; intraventricular choroid plexuses which determine the arterial pattern; penetrating capillaries from the surface to the ventricular germinal matrix forming simple transcerebral arteriovenous loops; cortical capillaries last, mainly in the last trimester. The venous return becomes connected to both the surface and to the choroidal veins, so forming distinct meningeal and subependymal venous drainage systems, while the arteries are on the surface only. While the arterial system was determined early (week 8), the venous system is continuously remodeled by the morphological changes of the base of the skull and the expansion of the brain vesicles. Until late in gestation, the vascular system is made of simple endothelial channels in which the arterial or venous fate is determined primarily by the direction of flow.

The dural entrance of cerebral bridging veins into the superior sagittal sinus: an anatomical comparison between cadavers and digital subtraction angiography.

INTRODUCTION: Intracranial venous structures have received increasing attention due to improved neuroimaging techniques and increased awareness of cerebral venous disease. To date, few studies have attempted to investigate the dural entrance of the cerebral bridging vein (BV). The aim of this study was to use the superior sagittal sinus (SSS) as an example to identify anatomical features of the dural entrance of the BVs into the SSS in both human cadavers and digital subtraction angiography (DSA) images. METHODS: A total of 30 adult and 7 fetal human cadavers and 36 patients were examined with anatomical dissections, vascular casting and DSA. The number, diameter and angle of the BVs entering the SSS were measured and compared between the cadavers and DSA images. RESULTS: The results demonstrated that (1) the way a BV entered the SSS varied in three dimensions, and thus the BV dural entrance was difficult to precisely localize by DSA, (2) the distribution pattern of the dural entrance of the BVs into the SSS was relatively constant and a nontributary segment of the SSS was centered at the coronal suture and was identifiable by DSA, and (3) nearly all the BVs (97%, 561/581) entered the SSS at an angle opposite to the direction of blood flow. CONCLUSION: Unique anatomical features of the dural entrance of a BV into the SSS should be considered in neuroimaging interpretation of the sinus and its associated veins.