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.
ObjectivePrevious outcome reports of congenital diaphragmatic hernia (CDH) have described neuroimaging anomalies and neurodevelopmental impairment. However, the link between imaging and outcome has not been described. We aimed to determine whether routine postoperative neonatal neuroimaging in infants with CDH detects later neurodevelopmental impairment.MethodsIn a prospective cohort study within a clinical service in The Royal Children’s Hospital Newborn Intensive Care. Cerebral ultrasound was performed in 81 children and MRI in 57 children who subsequently underwent neurodevelopmental follow-up after surgery for CDH. MRI scans were analyzed using a scoring system designed to identify injury, maturation and volume loss. Neurodevelopmental assessment occurred at 2 years (48) and neurocognitive assessment at 5 years (26) and/or 8 years (27). Brain imaging scores corrected for gestational age at scan time were correlated with outcome measures, adjusting for known clinical confounders.ResultsClinically significant findings were identified on MRI of 16 (28%) infants. Mean scores were in the normal range for all domains assessed at each age. Language impairment was seen in 23% at 2 years and verbal intellectual impairment in 25% at 8 years. Mean cognitive scores were lower in 2-year-old children with white matter injury on MRI (p=0.03). Mean motor scores were lower in 2-year-old children with brain immaturity (p=0.01). Associations between MRI and 5-year and 8-year assessments were no longer significant when adjusting for known clinical confounders.ConclusionsNeuroimaging abnormalities were associated with worse neurodevelopment at 2 years, but not with later neurocognitive outcomes, after accounting for clinical risk factors.
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