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KEY POINTS
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Key Points

  • The vein of Galen is a single, midline structure formed by the convergence of the two internal cerebral veins and the basal veins of Rosenthal posterior to the splenium of the corpus callosum; the vein courses posteriorly to empty into the straight sinus.

  • Vein of Galen malformation, an extremely rare anomaly, is a complex arteriovenous malformation affecting the vein of Galen and the cerebral arteries.

  • The size of the aneurysm determines its clinical presentation. A large aneurysm can result in as much as 50% to 60% of the cardiac output shunting through the lesion that may cause high-output congestive heart failure. This can present in utero as hydrops or as cardiac failure in early neonatal life.

  • Cerebral parenchymal injury including cerebral infarction, periventricular leukomalacia, and hemorrhagic infarction can also be associated with this anomaly.

  • Vein of Galen aneurysm may be suspected on antenatal ultrasound examination when a cystic or tubular mass is noted in the midline of the brain just above and behind the thalamus. Turbulent venous or arterial flow may be demonstrated using color Doppler within the draining vessel.

  • Generalized cardiomegaly or right ventricular enlargement is commonly noted but should not be attributed to high-output cardiac failure until targeted fetal echocardiography has been performed.

  • The differential diagnosis includes other midline cystic structures within the brain such as an arachnoid cyst or an interhemispheric cyst associated with agenesis of the corpus callosum.

  • Because of the differences in the fetal and neonatal circulations, cardiac failure does not often occur until after birth.

  • Serial antenatal ultrasound examinations should be performed to determine a change in size of the aneurysm and to monitor for hydrocephalus or congestive heart failure.

  • Joint management with a pediatric cardiologist and pediatric neurosurgeon is suggested.

  • The approach to therapy for a patient with a vein of Galen aneurysm will depend on the age of the patient, the clinical symptoms, and the angiographic architecture of the malformation.

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LONG-TERM OUTCOME OF PRENATALLY DIAGNOSED VEIN OF GALEN ANEURYSM IS STILL NOT A WELL-KNOWN CONDITION
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Vein of Galen aneurysm is also referred to as a varix of the vein of Galen or vein of Galen malformation. This anomaly is a complex arteriovenous malformation affecting the vein of Galen and the cerebral arteries. The vein of Galen is a single, midline structure formed by the convergence of the two internal cerebral veins and the basal veins of Rosenthal posterior to the splenium of the corpus callosum; the vein courses posteriorly to empty into the straight sinus. During embryologic development, cerebral arteries and veins cross in close proximity to each other; fistulous connections may exist because only a few cell layers separate these vessels (Padget, 1956). These fistulas persist because of an arteriovenous pressure gradient. The size and number of arteriovenous fistulous connections determine the eventual size of a vein of Galen aneurysm.

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Aneurysm of the vein of Galen was first ...

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