New born male baby presented with congestive heart failure. On examination enlarged fontanelles, a loud cranial bruit and following radiological finding was noted-the mostly likely diagnosis:
Ans. a. Vein of Galen malformationVein of Galen malformations (VOGMs) are one type of disorder known as cerebral vascular malformations, which affect the blood vessels in the brain.It is a very rare vascular malformation of the brain that develops before birth and is sometimes diagnosed in the womb or soon after birth.The congenital malformation develops mainly during weeks 6-11 of fetal development as a persistent embryonic prosencephalic vein of Markowski; thus, VGAM is actually a misnomer.The vein of Markowski drains into the vein of Galen.It causes high-output heart failure in the newborn resulting from the decreased resistance and high blood flow in the lesion.The clinical findings include cerebral ischemic changes such as strokes or steal phenomena that result in progressive hemiparesis.The malformation may result in mass effects, which causes progressive neurological impairment.Alternatively, the malformation may cause obstruction of the cerebrospinal fluid (CSF) outflow and result in hydrocephalus.
Vein of Galen malformation
Dandy walker syndrome
Pneumocephalus
Crouzon syndrome
Nervous System