Pediatric Congenital Cerebrovascular Anomalies

Abstract

Congenital cerebrovascular anomalies in the pediatric age group are myriad with diverse etiologies. The purpose of this paper is to provide an imaging overview of congenital vascular malformations and vascular tumors, as these conditions are varied and the characteristic vascular abnormality may even suggest the underlying systemic condition in helping to guide further management. For example, the identification of an arterial anomaly such as agenesis/hypoplasia/duplication may warrant further evaluation for an associated syndrome. Recognition of other pertinent findings, such as persistent carotid‐vertebrobasilar anastomoses for instance, would be particularly important for the planning of potential embolization/coiling of associated vascular malformation and/or aneurysm. Awareness of congenital dural sinus anomalies, including hypoplasia, aplasia, and or duplication, prevents the incorrect or overdiagnosis of sinus thrombosis. Moreover, multiple developmental venous anomalies in a patient may require further investigation for its familial association. In a similar context, there may be familial association with multiple cavernous malformations and children with central nervous system vascular malformations may have an association with other vascular conditions, eg, hereditary hemorrhagic telangiectasia, etc. An accurate description of vascular lesions is critical for understanding the natural history of the disease process and ultimately for guiding treatment. An important example of this includes Sturge‐Weber syndrome, which is often incorrectly viewed as an arteriovenous malformation, but actually a type of venous malformation. Another example which is important to recognize includes the differentiation between an infantile hemangioma and a vascular malformation, particularly as the course of the pathology and the treatment is so different between the two.

Pediatric Brain Tumors: From Modern Classification System to Current Principles of Management

“…Infantile CNS hemangiomas are rare benign tumors composed of endothelial cells. They are seen in about 1% of children with cutaneous hemangiomas and have a female predilection [112,113]. They may be associated with PHACES (posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta/ cardiac defects, and eye abnormalities) syndrome, and are more common in the posterior fossa [112].…”

Hirayama Disease in a Patient with a History of Late-Onset Symptomatic Vein of Galen Aneurysmal Malformation

“…Vein of Galen aneurysmal malformations (VGAMs) are rare congenital cerebrovascular malformations, consisting of a dilated median prosencephalic vein fed by multiple, deep, high-flow arteriovenous shunts [14]. VGAMs are essentially arteriovenous fistulae between arterial feeders, usually the choroidal, pericallosal, transmesencephalic, and thalamoperforator arteries, and an abnormally persistent median prosencephalic vein of Markowski, an embryonic precursor of the vein of Galen that usually degenerates after the 11th week of gestation [15].…”

“…VGAMs are essentially arteriovenous fistulae between arterial feeders, usually the choroidal, pericallosal, transmesencephalic, and thalamoperforator arteries, and an abnormally persistent median prosencephalic vein of Markowski, an embryonic precursor of the vein of Galen that usually degenerates after the 11th week of gestation [15]. The Lasjaunias classification of VGAMs divides them into 2 types, the mural type and the choroidal type, depending on their exact morphology [14, 15]. Clinical features of VGAMs include high-output cardiac failure secondary to the central arteriovenous shunting, usually in the neonatal period, and ventriculomegaly and/or hydrocephalus secondary to cerebrospinal malabsorption and venous hypertension, usually in the infantile period [14, 15].…”

Neuroimaging of Pediatric Arteriopathies

“…The diagnosis of definite and possible PHACES can be made according to the expert consensus‐based diagnostic criteria . In our previous paper, we described PHACES in relation to congenital cerebral arterial anomalies including agenesis of major cerebral arteries, aberrant course, arterial dysplasia and persistent of embryonic vessels. However, it can also be associated with intracranial arterial stenoses with an MMS like appearance of the distal ICA (Figs A,B), which may lead to pediatric AIS .…”

“…Congenital arteriopathies such as persistent fetal arteries, aplasia, hypoplasia, duplication, arteriovenous malformations have been discussed in a recent review of congenital cerebrovascular anomalies and will not be discussed here. Congenital vascular anomalies may, however, predispose patients to acquired arteriopathies such as saccular aneurysms or MMS and these complications will be included in our review.…”