Abstract
Aim: To assess the clinical features, diagnosis, treatment and prognosis of newborn infants with a diagnosis of Vein of Galen Malformation (VGAM)during a 10-year period. Method: Eight patients with a diagnosis of VGAM in the neonatal period were assessed retrospectively in terms of clinical signs, diagnosis, treatment strategies and follow-up. Three of four patients who survived had neurological assessment whereas one was lost to follow-up because of moving to another city. Results: Seven of 8 patients had an antenatal diagnosis. In all cases, severe heart failure and pulmonary hypertension were present from the first day of life and hypotension, multiorgan failure, hydrocephaly and seizures developed in the following days. VGAM and its feeder arteries were mapped by cranial magnetic resonance imaging and magnetic resonance angiography. Transarterial embolization therapy was performed on 7 patients, of whom four babies survived and three babies died, while one patient died before any intervention. Conclusion: The mortality and morbidity rates of VGAM is high because of its mixed anatomy, pathophysiology and characteristic features leading to severe neurological sequelae in the survivors. Prognosis in high risk neonates can be improved with aggressive medical support and early endovascular embolization therapy.
Highlights
Introduction common formType IV is known as the secondary type and hasVein of Galen Aneurysmal Malformation (VGAM) composes 1% of all vascular malformations and 30% of vascular malformations in childhood
In this report we reviewed 8 cases of newborn babies with an antenatal diagnosis of VGAM with an emphasis on clinical features, transarterial endovascular treatment and prognosis
The newborn patients who were hospitalized in our Neonatal Intensive Care Unit (NICU) between 2005 and 2015, with a diagnosis of VGAM were included
Summary
Vein of Galen Aneurysmal Malformation (VGAM) composes 1% of all vascular malformations and 30% of vascular malformations in childhood. VGAM is a small, deep venous internal cerebral vessel with a thin wall. The conjunction of choroidal and thalamostriate veins in the interventricular foramen creates internal cerebral veins. VGAM is generated between the 6th and 11th weeks of gestation by the connections between primitive choroidal vessels and median prosencephalic vein (Markowski) [1]. Three subtypes: In Type IVA an aneurysmal dilatation develops in the vein of Galen by the neighbouring thalamic arteriovenous malformation. Type IVB is similar to type IVA, but arteriovenous malformation is mesencephalic rather than thalamic. In Type IVC thalamomesencephalic or mesodiencephalic plexiform malformation drains into the vein of Galen by a nearby and different cisternal AVF. Yasargil classification is helpful when an open surgery is indicated, whereas Lasjaunias classification is helpful if endovascular intervention is the treatment option [4]
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