Cerebral Venous Development in Relation to Developmental Venous Anomalies and Vein of Galen Aneurysmal Malformations

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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.

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.

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