Outcome of vein of Galen malformation presenting in the neonatal period

• Published in: Archives of disease in childhood 2019-11, Vol.104 (11), p.1064-1069
• Main Authors: Malarbi, Stephanie, Gunn-Charlton, Julia K, Burnett, Alice C, Prentice, Trisha M, Williams, Amy, Mitchell, Peter, Wray, Alison, Hunt, Rod W
• Format: Article
• Language: English
• Subjects: Age; Attrition (Research Studies); Australia - epidemiology; Brain; Brain research; Cardiovascular system; Children; Childrens health; Congenital defects; Congenital diseases; Critical Illness; Developmental Delays; Female; Hospitals; Humans; Infant, Newborn; Infants; Intellectual Disability; Intelligence; Intensive care; Intensive care units; Intensive Care Units, Neonatal; Male; Medical Services; Memory; Mortality; Mortality Rate; neonatal; Neonatal care; Neonates; Neurodevelopmental Disorders - diagnostic imaging; Neurodevelopmental Disorders - mortality; Neurodevelopmental Disorders - physiopathology; neurodevelopmental outcome; Neuroimaging; Neurological Impairments; Neurology; Newborn babies; NICU; Occupational Therapy; Original article; Patients; Pediatrics; Prognosis; Retrospective Studies; Systematic review; Test Selection; Vein & artery diseases; vein of Galen malformation; Vein of Galen Malformations - diagnostic imaging; Vein of Galen Malformations - mortality; Vein of Galen Malformations - physiopathology; Veins & arteries; VGAM; Young Children
• ISSN: 0003-9888; 1468-2044
• DOI: 10.1136/archdischild-2018-316495

ObjectiveVein of Galenaneurysmal malformation (VGAM) is a rare but important congenital malformation presenting to neonatal intensive care units (NICUs), and with a change from surgical to endovascular management, survival for this condition has improved. However, there is little reported about the medical management decisions of infants with this condition and the associated long-term neurodevelopmental outcomes. We aim to report a single centre experience of both acute treatment and long-term outcomes of VGAM for those infants admitted to our NICU soon after birth.DesignRetrospective cohort study over a 15-year period from 2001 to 2015 inclusive.SettingA quaternary NICU at The Royal Children’s Hospital, Melbourne, Australia.Participants24 newborn infants referred for management of VGAM. There were no eligibility criteria set for this study; all presenting infants were included.InterventionsNone.Main outcomes measuresClinical neuroimaging data were gathered. Surviving children were formally assessed with a battery of tests administered by a neuropsychologist and occupational therapist/physiotherapist at various ages across early to middle childhood.ResultsFifteen neonates with VGAM did not survive beyond their NICU admission. 10 of these were not offered endovascular intervention. Of the nine surviving infants, only one had a normal neurodevelopmental outcome.ConclusionsThe mortality of VGAM presenting in the neonatal period was high, and rates of normal neurodevelopmental outcome for survivors were low. These findings contribute to our understanding of which neonates should be treated and highlights the importance of providing clinical neurodevelopmental follow-up to survivors beyond their infant years.