A newborn presents with congestive heart failure, on examination has bulging anterior fontanelle with a bruit on auscultation. Transfontanellar USG shows a hypoechoic midline mass with dilated lateral ventricles. Most likely diagnosis is –
Correct Answer: Vein of Galen malformation
Description: Midline intracranial mass with bruit on ausculation and features of hydrocephalus and congestive heart failure All point towards Vein of Galen Malformation.
Vein of Galen Malformations(VGM)
The vein of Galen is a large deep vein at the base of the brain. It is located under the cerebral hemispheres and drains the anterior and central regions of the brain into the sinuses of the posterior cerebral fossa .
VGM results from an aneurysmal malformation with an arteriovenous shunting of blood.VGM usually causes high-output heart failure in the newborn resulting from the decreased resistance and high blood flow in the lesion.
Associated findings include cerebral ischemic changes such as strokes or steal phenomena that result in progressive hemiparesis.
The malformation may result in mass effects, causing progressive neurological impairment. Alternatively, the malformation may cause obstruction of the cerebrospinal fluid (CSF) outflow and result in hydrocephalus.
Loud intracranial bruit may be heard because of the blood turbulence in VGM.
The most effective treatment for the malformation has been embolization rather than surgery
The vein of Galen abnormality is the most frequent arteriovenous malformation in neonates.
Category:
Pediatrics
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