Cerebral Venous Development in Relation to Developmental Venous Anomalies and Vein of Galen Aneurysmal Malformations
Section snippets
Developmental Anatomy
The works of Padget1, 2, 3 and Streeter4 provided the foundation of the embryologic knowledge on the development of the cranial venous and arterial systems. Raybaud et al5, 6, 7 further complement their works and contribute greatly to the understanding of cerebrovascular pathologies. The following summary of cranial vascular development is based largely on their excellent research.
The development of the cranial vasculature can be divided into 4 stages.5 During stage 1 (weeks 2-4), the neural
Venous Anatomy
The cerebral veins may be compartmentalized into 2 systems of drainage: superficial and deep (Fig. 1). This concept may also be applied to the infratentorial brain, in particular when considering formations of DVAs.10, 11
Clinical Presentation
DVAs are typically benign, asymptomatic lesions and generally provide sufficient venous drainage of the involved territory. Conservative management is strongly suggested,24 reflecting this type of clinical course. When symptomatic, they may present with headaches, seizures, focal neurological deficits, and hemorrhage.25, 26 The clinical sequelae of a DVA are likely related to the regional changes that occur near the DVA. The brain parenchyma drained by the DVA is usually considered normal;
Imaging Findings
A DVA consists of a radial complex of venous radicles draining normal brain parenchyma that converge into a dilated collecting vein, resulting in its characteristic caput medusae appearance (Figure 3, Figure 4). DVAs are often incidental findings on routine imaging studies and their radiological appearance has been well described.28, 35 DVAs of the brain range from a small, single draining vein, involving a small portion of the brain to a large hemispheric venous anomaly draining an entire
Vein of Galen Aneurysmal Malformation
A VGAM is a rare vascular anomaly disproportionately represented in the pediatric population, where it is said to account for up to 30% of intracranial vascular malformations.46, 47 The hallmark feature of a VGAM is the presence of one or more arteriovenous shunts draining into a dilated median cerebral venous collector. This midline venous structure corresponds to a persistent embryonic channel, the MProsV, which normally regresses with the development of the ICVs.7
The MProsV is a single,
Anatomical Considerations
A VGAM is a type of choroidal malformation that shares the same choroidal arterial feeders but differs in its venous drainage. VGAMs drain into the MProsV, whereas other types of choroidal arteriovenous malformations drain into normally developed, although dilated, internal cerebral veins. The difference in venous outflow reflects the period during which these entities develop, with choroidal arteriovenous malformations occurring at a later period, after the MProsV has disappeared and been
VGAM Classification
The 2 dominant VGAM classifications, described by Lasjaunias51 and Yasargil,52 are widely referenced and distinguish between true VGAM and “false” VGAM (ie, vein of galen aneurysmal dilatation: VGAD53). A true VGAM (Fig. 7B) involves the MProsV and is subdivided in both classification schemes (choroidal and mural types in Lasjaunias; Yasargil types I, II, and III) based on the anatomy of the arteriovenous shunt. The “false” VGAM or VGAD (Yasargil type IV; Fig. 8) describes an AVM that drains
Clinical Presentation
Three classic clinical presentations have been established by Gold et al47 in 1964. In the neonatal group, direct arteriovenous connections result in high-volume blood shunting and an early presentation characterized by cardiac and respiratory insufficiency. The second group, a delayed presentation group, is composed of infants and young children suffering from seizures, developmental delay, macrocephaly, and hydrocephalus. The last group is made of older children and adults who present with
Prenatal Diagnosis with Ultrasound and MRI
Prenatal diagnosis of VGAMs may be accomplished with prenatal ultrasound (US) and fetal MRI. Although VGAMs are thought to develop during the choroidal embryonic stage (around 8 weeks), sufficient dilatation of the MProsV is required before it can be detected on first- or even second-trimester ultrasound.54 Therefore, most diagnoses by ultrasound occur in the third trimester. Grayscale US imaging may detect a midline, tubular anechoic structure superior to the thalamus,55 representing the
Digital Subtraction Angiography
DSA remains the gold standard technique for the assessment of the intracranial vasculature. It is the only available modality that provides a detailed evaluation of the VGAM angioarchitecture necessary for endovascular treatment. Analysis of the arterial contributors in size, number, origin, as well as assessment of high-flow arteriopathy and venous stenoses is possible with DSA.48 It also may help the morphology of the fistulae and detailed assessment of shunt hemodynamics. The angiography can
Management
Optimal management of a patient with a VGAM remains challenging and requires a comprehensive, multidisciplinary approach. Treatment now is primarily endovascular, with surgery reserved for the evacuation of intracranial hematomas and the management of hydrocephalus.59 Shunt placement, however, should only be considered after endovascular treatment has failed to improve the hydrocephalus, as shunt placement is associated with a significant increase in morbidity and mortality.60
Endovascular
Conclusions
The development of the cranial venous system is a dynamic process that evolves with the progressive stages of brain development. This explains the considerable normal variability in the venous anatomy, as it forms to adapt to the current hemodynamic environment. An appreciation of this complex process is fundamental to understanding cerebral vascular malformations.
References (62)
Normal and abnormal embryology and development of the intracranial vascular system
Neurosurg Clin N Am
(2010)The cranial venous system in man in reference to development, adult configuration, and relation to the arteries
Am J Anat
(1956)The development of cranial arteries in the human embryo
Contr Embryol Carneg Instn
(1948)The Development of the Cranial Venous System in Man, from the Viewpoint of Comparative AnatomyContributions to Embryology, 36
The Developmental Alterations in the Vascular System of the Brain of fhe Human Embryo
(1918)Development of the arterial supply to the brain tissue
- et al.
Aneurysms of the vein of Galen: Embryonic considerations and anatomical features relating to the pathogenesis of the malformation
Neuroradiology
(1989) - et al.
Persisting abnormal embryonic vessels in intracranial arteriovenous malformations
Acta Radiol Suppl
(1986) - Markowski J: Entwicklung der Sinus durae matris und der Hirnvenen des Menschen: Bulletin international de l'Academie...
- et al.
Microsurgical anatomy of the veins of the posterior fossa
J Neurosurg
(1983)
Cerebral developmental venous anomalies: Current concepts
Ann Neurol
Veins of the white matter of the cerebral hemispheres (the medullary veins)
Am J Roentgenol Radium Ther Nucl Med
Micro-angiographical studies of the medullary venous system of the cerebral hemisphere
Neuropathology
The functional significance of the arrangement of the cerebral and cerebellar Veins
J Anat
Venous architecture of cerebral hemispheric white matter and comments on pathogenesis of medullary venous and other cerebral vascular malformations
Mt Sinai J Med
Intracerebral venous angiomaCase report and review
Arch Neurol
Developmental venous anomalies (DVA): The so-called venous angioma
Neurosurg Rev
Cerebral venous angiomas: Clinical evaluation and possible etiology
Radiology
Cryptic vascular malformations
Clin Neurosurg
Ischemic complication of a cerebral developmental venous anomaly: Case report and review of the literature
J Comput Assist Tomogr
Magnetic resonance angiography of cerebral developmental venous anomalies: Its role in differential diagnosis
Neuroradiology
Developmental venous anomaly (DVA) with arterial component: A rare cause of intracranial haemorrhage
Neuroradiology
Evaluation of developmental venous anomalies: Medullary venous anatomy of venous angiomas
AJNR Am J Neuroradiol
The case for conservative management of venous angiomas
Can J Neurol Sci
Clinical significance of intracranial developmental venous anomalies
J Neurol Neurosurg, Psychiatry
The natural history of intracranial venous angiomas
J Neurosurg
Parenchymal abnormalities associated with developmental venous anomalies
Neuroradiology
Venous angioma of the brain: History, significance, and imaging findings
AJR Am J Roentgenol
Ischemic accident caused by thrombosis of a venous angiomaApropos of a case [in French]
J Radiol
Thrombosis of a drainage vein in developmental venous anomaly (DVA) leading venous infarction: A case report and review of the literature
J Neuroimaging
The pathology of vascular (“arteriovenous”) malformations
J Neurosurg
Cited by (25)
Functional engagement of white matter in resting-state brain networks
2020, NeuroImageCitation Excerpt :A potential confound is that white matter BOLD signals may arise secondary to gray matter functional activity because of venous drainage effects. However, according to the venous system configuration reported by Pearl et al. (2011), there are two separate venous systems for GM and WM: a superficial venous system that drains deoxygenated blood in the cortex and superficial WM into venous sinuses via cortical veins, and a deep venous system that drains deoxygenated blood in deep WM into the subependymal veins. As such, there are minimal vascular interactions between the two different tissue types, and the blood flowing out of activated cortical regions is unable to reach deep WM to modulate the signals therein (exceptions exist for developmental venous anomalies but these have a maximum incidence of only 2.6% (Pearl et al., 2011)).
Venous anatomy of the supratentorial compartment
2020, Handbook of Clinical NeurologyCitation Excerpt :As development progresses, the anterior portion of this vein regresses and the drainage is taken over by the developing ICV. The remaining posterior portion of the median prosencephalic vein gives rise to the vein of Galen (Pearl et al., 2011). Several tributaries then develop from the ICV, giving rise to the subependymal and deep medullary veins.
Cerebral infarction secondary to a developmental venous anomaly in a cancer patient
2017, Neurologia ArgentinaDecreased susceptibility of major veins in mild traumatic brain injury is correlated with post-concussive symptoms: A quantitative susceptibility mapping study
2017, NeuroImage: ClinicalCitation Excerpt :It has been reported that straight sinus mostly receives the deep cerebral venous system draining deep gray matter, the inferior sagittal sinus and some regions of the upper brainstem through the confluence of the inferior sagittal sinus and great vein of Galen (Jain et al., 2013; Krishnamurthy et al., 2014). The great vein of Galen receives bilateral cerebral internal, basal, thalamostriate and septal veins (Pearl et al., 2011). The straight sinus also drains the deep brain structures including thalami, cerebral nuclei, midbrain, pontine nuclei and parahippocampal gyri.
Venous angiomas and headache in children. A case report
2016, Revista Chilena de PediatriaDiagnostic Imaging: Brain
2016, Diagnostic Imaging: Brain