Vein of Galen aneurysmal malformations

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Vein of Galen aneurysmal malformations (VGAM) are rare vascular malformations of the brain that occur in the region of the Vein of Galen, a large deep cerebral vein. These malformations are characterized by an abnormal connection between cerebral arteries and the vein, leading to a high-flow arteriovenous shunt.

Presentation

VGAMs are typically diagnosed in infancy or early childhood. They can present with a variety of symptoms, including heart failure, hydrocephalus, and seizures. In severe cases, they may lead to developmental delay or neurological deficits.

Pathophysiology

The malformation involves a direct connection between the arterial and venous systems, bypassing the capillary network. This results in increased blood flow and pressure in the venous system, which can lead to congestive heart failure and other complications. The exact cause of VGAM is not well understood, but it is believed to result from developmental anomalies during embryogenesis.

Diagnosis

VGAMs are often diagnosed using imaging techniques such as ultrasound, magnetic resonance imaging (MRI), and computed tomography (CT) scans. Angiography is used to confirm the diagnosis and to assess the anatomy of the malformation.

Treatment

The primary treatment for VGAM is endovascular embolization, a minimally invasive procedure that involves the insertion of materials to block the abnormal blood vessels. This procedure aims to reduce the blood flow through the malformation and alleviate symptoms. In some cases, surgical intervention may be necessary.

Prognosis

The prognosis for individuals with VGAM varies depending on the size and location of the malformation, as well as the presence of associated complications. Early diagnosis and treatment are crucial for improving outcomes. With advances in endovascular techniques, the prognosis has improved significantly in recent years.

Related pages

• Arteriovenous malformation
• Cerebral aneurysm
• Neurosurgery

References

• Lasjaunias, P., et al. (2006). "Vein of Galen aneurysmal malformations." In: Vascular Diseases in Neonates, Infants and Children. Springer.
• Jones, B. V., et al. (2002). "Vein of Galen aneurysmal malformation: diagnosis and treatment." Journal of Neurosurgery: Pediatrics.

Images

Axial view of a Vein of Galen aneurysmal malformation.

3D reconstruction of a Vein of Galen aneurysmal malformation.

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  Vein of Galen aneurysmal malformation axial view

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  Vein of Galen aneurysmal malformation 3D view