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Chapter 14: Fetal Cerebral Vascular Malformations

Zvi Leibovitz; Lerman-Sagie Tally; Dario Paladini

INTRODUCTION

Congenital vascular brain anomalies include various disorders characterized by different morphological, hemodynamic, and prognostic features. Some of them (eg, vein of Galen aneurysmal malformation [VGAM] and arteriovenous malformation) can impose a high risk for abnormal outcome, while others (eg, developmental venous anomaly and capillary telangiectasia) are usually asymptomatic.

Cerebral vascular malformations (CVM) can be classified on the basis of histopathological1 (Table 14–1) or morphological2 (Table 14–2) features, existence of arteriovenous shunting, or age-group specific anomalies.3

Table 14–1.HISTOPATHOLOGICAL CLASSIFICATION OF CEREBROVASCULAR MALFORMATIONS
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Table 14–1. HISTOPATHOLOGICAL CLASSIFICATION OF CEREBROVASCULAR MALFORMATIONS
Malformation Histopathological Features
Arteriovenous malformations Clusters of abnormal arteries and veins; intervening gliotic brain tissue; vessel walls are typically thickened, contain elastin and smooth muscle
Cavernous malformations (cavernomas) Clusters of sinusoidal channels with thin walls devoid of elastin and smooth muscle; the channels are filled with blood and thrombi at varying stages of organization; there is no intervening brain tissue except at the periphery of the lesion
Venous malformations (developmental venous anomalies) Collections of small veins usually clustering around one or more larger veins; there is typical venous wall histology and intervening normal brain tissue
Capillary telangiectasias Collections of dilated capillaries intervening normal brain tissue

Note: Developmental venous anomaly and capillary telangiectasia are not associated with brain tissue damage.

Data from McCormick WF. The pathology of vascular (“arteriovenous”) malformations. J Neurosurg. 1966;24(4):807–816.

Table 14–2.MORPHOLOGICAL AND PATHOPHYSIOLOGICAL CLASSIFICATION OF CEREBRAL VASCULAR MALFORMATIONS
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Table 14–2. MORPHOLOGICAL AND PATHOPHYSIOLOGICAL CLASSIFICATION OF CEREBRAL VASCULAR MALFORMATIONS
  Arterial Lesions Venous Lesions Capillary Lesions
Focal Arterial aneurysms as part of genetic syndromes (neurofibromatosis-I, Ehlers-Danlos syndrome type IV, familial immune deficiency syndrome) Sinus pericranii, developmental venous anomaly, cavernous malformation Associated with hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) or lesions at arteriovenous junction
Segmental Agenesis, hypoplasia, dysplasia (PHACES syndrome) Dural sinus malformation, sinus pericranii, venous varix Associated with cerebral proliferative angiopathy
Metameric Craniofacial arteriovenous metameric syndrome (Wyburn-Mason syndrome) Craniofacial venous metameric syndrome, non-familial Sturge-Weber syndrome, familial blue rubber bleb nevus syndrome Associated with craniofacial arteriovenous metameric syndrome
Other Aberrant ICA arteries, azygous ACA, arterial fenestrations, persistent embryonic arteries, variants of the circle Willis Persistent falcine sinus, duplicated sagittal sinus  
Low-flow CVM Developmental venous anomalies, cavernous malformations, dural sinus malformations
High-flow CVM Vein of Galen aneurysmal malformations, pial high-flow AV shunts, dural sinus AV fistulas

ACA, anterior cerebral artery; AV, arteriovenous; CVM, congenital vascular malformations; ICA, internal carotid artery.

Data from Gaillard F. Cerebrovascular malformations https://radiopaedia.org/articles/cerebrovascular-malformations?lang=us; Krings T, Geibprasert S, terBrugge K. Classification and endovascular management of pediatric cerebral vascular malformations. Neurosurg Clin N Am. 2010;21(3):463–482.

Common arterial anomalies include persistent primitive carotid−vertebrobasilar anastomoses, arterial dysplasias (isolated or as a part of PHACES syndrome), and abnormal branching.

Structural dural sinus anomalies consists of persistent falcine sinus, sinus ...

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