LAUSR

Presenting a female neonate born in DGH at term by ventouse delivery with birth weight of 4.25 kg to non consanguineous Caucasian parents. Antenatally there was polyhydromnios and no risk factors for sepsis. She was born blue without any respiratory effort. APGARS were 4/1 and 5/5.IPPV breaths and eventually started non invasive ventilation(CPAP, BiPaP) Preductal saturation was 95% pre ductal and 100% post ductal Antibiotics started for suspected sepsis. Gases showed persistent metabolic acidosis. CXR showed cardiomegaly. She was intubated, ventilated and transferred to tertiary unit with a diagnosis of congenital cardiac disease. She had an audible scalp murmur and ECHO showed right ventricular dysfunction and PDA with right to left shunting. Cranial ultrasound diagnosed Vein of galen malformation. Commenced on ionotropes, furosemide, and transferred to speciality centre. she continued to be on conventional ventilation with nitric oxide for PPHN, needing ionotropic support for hypotension, eventually developed hepatorenal failure. CT brain showed a large VGOM abnormality with significant scarring and calcification in the remaining brain resulting in only a very thin cortex of brain tissue. There was significant mortality and chances of brain damage on survival with operative management. Decided for palliative care in agreement with parents and passed away after extubation in DGH. VOGM is a rare congenital, cerebral, arteriovenous malformation with an estimated incidence of 2.5 out of 1 00 000 live births. Choroidal (complex) form usually causes high cardiac output failure in neonates, respiratory distress in new born, PPHN and multiorgan failure. If no obvious cause for heart failure is found, diagnosis can be made by signs such as cranial bruit, expanded facial veins, ultrasound/doppler 0.94% diagnosed in the neonatal period present with high output cardiac failure. Treatment is therapeutic embolisation of feeding arteries in the VOGM with a significant high mortality rate.