The vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation caused by the maldevelopment of its embryonic precursor, the median pros encephalic vein of Markowski. Although most of the VGAM cases are diagnosed in the neonatal period, sometimes it can also present during early childhood. It is very crucial to intervene immediately following the diagnosis because if left untreated, morbidity and mortality are imminent. The most common causes of morbidity and mortality are high-output congestive heart failure (most common neonatal presentation), hydrocephalus (most common presentation in infants), headache, and seizures. We are presenting the case of a two-year-old male with global developmental delay, failure to thrive, and macrocephaly who presented with recurrent generalized tonic-clonic seizures. MRI/magnetic resonance venography (MRV)/magnetic resonance angiography (MRA) brain showed an enlarged vein of Galen with venous hypertension and aqueduct stenosis. Treatment intervention included trans-arterial embolization of the right pericallosal, right/left lateral posterior, and medial posterior choroidal feeders with coils. The patient has had significant improvement in his neurocognitive functions including significant improvement in his speech/language development with outpatient therapies in between embolization.
Keywords: congenital vascular malformation; endovascular embolisation; extra cardiac shunting; transvenous embolization; vein of galen malformation.
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