{"id":"c2186966-2b43-4238-a994-53c1f4611914","slug":"pediatric-knowledge-update-approach-to-the-management-of-vein-of-galen-aneurysmal-malformations-in-neonates","title":"Pediatric knowledge update: Approach to the management of vein of Galen aneurysmal malformations in neonates","authors":["Daniel Hansen","Peter T. Kan","Gaddum D. Reddy","Arvind Chintagumpala Mohan","Andrew Jea","Sandi Lam"],"abstract":"A newborn female born via cesarean section at 39 weeks gestation was transferred to our institution for severe respiratory distress after delivery. She was intubated and required 100% fraction of inspired oxygen to maintain adequate saturations. She was also started on vasopressor medications secondary to hypotension. An echocardiogram of the heart showed diastolic flow reversal in the transverse aortic arch and increased flow in the superior vena cava. A bruit was auscultated over the anterior fontanelle that was concerning for an intracranial vascular malformation. Bedside, head ultrasound confirmed the presence of a vein of Galen malformation. Her liver was enlarged without signs of liver failure. Her anuria resolved with improved renal function after her hypotension was treated. Electroencephalography was negative for seizure activity. Her Bicêtre score was calculated to be between 9 and 11. As per the Lasjaunias algorithm, the baby was a candidate for emergent endovascular embolization. Cerebral angiogram revealed an extensive choroidal type vein of Galen malformation with severe arteriovenous shunting [Figures 1 and 2 ]. Partial embolization was achieved using a combination of platinum coils and the liquid embolic agent Onyx, with a significant reduction in posttreatment arteriovenous shunting. Posttreatment echocardiogram showed improvement in the left ventricular function. Repeated partial embolizations were performed 3 more times over the next 3 months [ Figure 3 ]. Ventriculomegaly remained stable on serial imaging. Her cardiac function as assessed by her brain natriuretic peptide normalized. Her pulmonary hypertension steadily improved, allowing her to be discharged to home on room air. First embolization treatment. Right internal carotid injection. Pretreatment (top) and posttreatment (bottom). Blue arrows highlighting arterial feeders pre- and post-coil embolization. Note slightly decreased contrast filling of venous outflow after coil placement, highlighted by red arrows Magnetic resonance imaging of the brain without contrast. Sagittal T1 (left) sequence and axial T2 (right) sequence highlighting dilated vessels (arrows) converging to the vein of Galen varix, dilatations of the draining straight sinus Third and fourth embolization treatments. Left vertebral injection. Pretreatment (top) and posttreatment (bottom). Multiple coil constructs (blue arrows) are now visible with further reduction in contrast filling of the venous outflow (red arrows)","thumbnailUrl":"https://sni-digital-videos.s3.amazonaws.com/articles/c2186966-2b43-4238-a994-53c1f4611914/featured/hero-1782420996361.png","publishDate":"2016-05-13T00:00:00.000Z","doi":"10.4103/2152-7806.182415","categories":["Pediatric Neurosurgery","Original Article"],"fullTextUrl":"http://surgicalneurologyint.com/wp-content/uploads/2016/05/6733/SNI-7-317.pdf"}