ABSTRACT 321

Abstract

Background and aims: Vein of Galen Malformation (VGAM) is a rare condition. VGAM is hallmarked by multiple arterio-venous shunts from choroidal vessels into median prosencephalic vein of Markoswki. Arterial-venous shunt at choroidal vessels leads to overload of venous system and high cardiac output failure. When untreated, this malformation has poor outcome leading to death. Aims: We present two newborn cases of VGAM who presented with signs of cardiomegaly and a normally structured heart. Methods: Case I: One-day old newborn who presented with tonic-clonic seizures and cardiac failure (cardiomegaly and hepatomegaly). Initial cranial ultrasound scan (CUSS) revealed arterio-venous malformation (AVM) confirmed in Head CT with areas of cerebral infarction. Patient underwent endovascular embolisation which was complicated with diffuse subarachnoid hemorrhage. Patient developed fixed pupils, extensive brain injury and died. Case II: Four-day old baby who presented with poor feeding and signs of cardiac failure (cardiomegaly and refractory metabolic acidosis). Routine CUSS revealed a midline AVM suggesting VGAM. Patient underwent endovascular embolisation complicated by pulmonary embolism and extensive hypoxic brain injury leading to death. Results: We had differentialdiagnosis of sepsis, congenital heart defect, AVM and metabolic disorder. Echocardiographyshowed normally structured heart. Case I had neurological symptoms but Case IIhad normal neurological examination and AVM was found in a routine CUSS. Both cases confirmed the poor prognosis of this congenital condition. Conclusions: We recommend routine CUSS in newborns with unclear diagnosis of cardiac failure. Complete neurological examination (including auscultation of the fontanel) and CUSS as a non-invasive screening method should be performed in all cardiac patients.