A VEIN OF GALEN MALFORMATION - A RARE CAUSE OF CONGESTIVE CARDIAC FAILURE IN NEONATE

Abstract

Vein of Galen aneurysmal malformation (VGAM) is a rare congenital vascular malformation caused by the maldevelopment of its embryonic precursor, the median prosencephalic vein of Markowski. Vein of Galen aneurysmal malformation (VGAM) is a rare cause of congestive heart failure in the neonatal period and carries a high mortality rate. It is a difcult diagnosis because the clinical picture often presents with symptoms suggesting that the patient has a congenital cardiac malformation.1,2 Patients are subjected to high-risk procedures such as cardiac catheterization to establish a diagnosis. These investigations often have negative ndings and can delay correct diagnosis. Here we report a case of Four days old neonate with c/o Increased respiratory activity since 2 days with tachycardia, tachypnea and oliguria. Continuous murmur was noted with features suggestive of Congestive cardiac failure i.e. Tachycardia, tachypnoea, hepatomegaly. Bruit + over Anterior Fontanelle. USG skull revealed the presence of Vein of Gallen Aneurysmal Malformation. 2D ECHO was done which suggestive of Congenital Heart Disease; moderate OS ASD with left to right shunt Dilated cardiac chambers; No PAH; Normal biventricular function .CECT Brain showed the presence of Aneurysmal deep venous structure measuring 20 x 25 mm noted in the supratentorial region of posterior fossa (in quadrigeminal cistern) which drained to vein of galen (VGAM) and the vessel feeding the aneurysm was arising from basilar artery.Hence diagnosis was made.Child initially treated symptomatically for cardiac failure then Endovascular treatment i.e. embolization was performed on 30th day of life.The VGAM was partially occluded.Then symptoms got relieved after Embolization