Vein of Galen aneurysmal dilatation

Vein of Galen Aneurysmal Malformation
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Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Heart failure, Hydrocephalus, Seizures
Complications	Developmental delay, Neurological deficits
Onset	Congenital
Duration	N/A
Types
Causes	Abnormal embryonic development of cerebral vasculature
Risks
Diagnosis	MRI, CT scan, Angiography
Differential diagnosis	N/A
Prevention	N/A
Treatment	Endovascular embolization, Surgical intervention
Medication	N/A
Prognosis	Variable
Frequency	Rare
Deaths	N/A

The Vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation characterized by an abnormal connection between cerebral arteries and the vein of Galen, a large deep cerebral vein. This condition is a type of arteriovenous malformation (AVM) that occurs during embryonic development.

Anatomy and Pathophysiology

The vein of Galen is a short midline vein that drains blood from the deep structures of the brain into the straight sinus. In VGAM, there is an abnormal arteriovenous shunt where high-flow arterial blood bypasses the capillary system and drains directly into the vein of Galen. This results in its aneurysmal dilatation and can lead to significant hemodynamic changes.

Clinical Presentation

VGAM can present in neonates, infants, or children with a variety of symptoms depending on the severity of the malformation and the age of presentation. Common symptoms include:

• Heart failure due to high cardiac output from the shunt.
• Hydrocephalus resulting from impaired cerebrospinal fluid absorption.
• Seizures and other neurological deficits.
• Developmental delay and failure to thrive.

Diagnosis

Diagnosis of VGAM is typically made using imaging studies.

• Magnetic Resonance Imaging (MRI) provides detailed images of the brain and the vascular malformation.
• Computed Tomography (CT) scan can be used to assess hydrocephalus and other structural abnormalities.
• Cerebral angiography is the gold standard for visualizing the vascular anatomy and planning treatment.

Treatment

The primary treatment for VGAM is endovascular embolization, a minimally invasive procedure where the abnormal vessels are occluded using coils or glue to reduce blood flow through the malformation. In some cases, surgical intervention may be necessary.

Prognosis

The prognosis for patients with VGAM varies widely and depends on the size of the malformation, the presence of associated complications, and the success of treatment. Early diagnosis and intervention are crucial for improving outcomes.

Also see

• Arteriovenous malformation
• Hydrocephalus
• Pediatric neurology
• Neurosurgery