124 Vein of Galen aneurysmal malformation: case series of 14 neonates in Croatian national referral centre for neonate and pediatric intensive care

• Published in: Archives of disease in childhood Vol. 106; no. Suppl 2; pp. A52 - A53
• Main Authors: Nada, Sindičić Dessardo, Marija, Čičak Ana, Ruža, Grizelj, Iva, Vukšić, Vesna, Benjak, Andrea, Dasović Buljević, Dorotea, Ninković, Tomislav, Ćaleta, Boris, Filipović-Grčić, Dražen, Perkov, Marko, Radoš
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health 11.10.2021; BMJ Publishing Group LTD
• Subjects: Abstracts; Aneurysm; Bleeding; Cognition; Congestive heart failure; Developmental Delays; EEG; Embolization; Heart failure; Hydrocephalus; Infants; Intensive care; Ischemia; Magnetic resonance imaging; Medical prognosis; Medical records; Medical referrals; Neonates; Neurodevelopment; Neurodevelopmental disorders; Newborn babies; Patients; Pediatrics; Peritoneum; Prenatal diagnosis; Seizures; Visual Impairments; Young Children
• ISSN: 0003-9888; 1468-2044
• DOI: 10.1136/archdischild-2021-europaediatrics.124

Vein of Galen aneurysmal malformation (VGAM) is a complex congenital cerebro-vascular malformation. Despite some controversy, the overall prognosis of VGAM has improved over time.We present a series of VGAM patients with hyper-dynamic heart failure during the neonatal period treated in our Department in the past 20 years.All cases diagnosed with chorioidal VGAM in a single tertiary centre between 2001 and 2020 were ascertained from medical records. All infants underwent postnatal cardiac ECHO, electroencephalogram and brain MRI, along with calculation of Bicetre severity score at admission. Embolization procedures were analysed based on timing and presence of peri- and post-procedural complications. Long-term survivors had standard neurocognitive assessment.For the purpose of outcome analysis we divided patients into two period cohorts: 2001-2010 and 2011-2020.Overall fourteen out-born neonates were diagnosed with chorioidal VGAM. Bicetre neonatal score were similar in both chronological sub-groups. First cohort comprised six neonates. Prenatal diagnosis of VGAM was obtained in one patient. Four newborns died owing to refractory heart failure without neuroembolization procedure. Two survivors underwent the intervention at fourth and eleventh day, respectively. Post-procedural analysis revealed minor intraventricular bleeding and neonatal seizures in one patient that eventually has been lost to follow up. Other survivor developed ischaemic lesions and hydrocephalus. Ventriculo-peritoneal drainage was performed.Severe neurodevelopmental delay and visual impairment were observed on follow-up. Second cohort comprised eight neonates, all prenatally diagnosed. Three patients died: at the age of five (intra-procedural rupture of aneurism), fifteen (multi-organ failure), and 205 days (pulmonary hypertension and congestive heart failure). All infants undergone the first embolization within the first ten days of life. Satisfactory immediate outcome including control of cardiac failure was achieved in six patients. Post-procedural analysis revealed minor intraventricular bleeding (n=2), ventriculomegaly (n=2), presence of ischaemic lesions (n=2), neonatal seizures (n=2) and abnormal encephalogram (n=2). Normal neurodevelopment was observed in four, and mild neuro-developmental delay was observed in one patient on follow-up.Given a small sample size it is difficult to quantify the difference between the two groups in a statistically relevant manner. However, the observed difference in survival and long-term outcome between the cohorts are most probably related to an overall improvement of prenatal diagnosis, intensive care management and embolization techniques. Early neuroembolization may decrease the risk of refractory cardiac failure and improve the long-term neurocognitive outcome.