1282 Epidemiology and Natural History of Infantile Brain Arteriovenous Malformations: A 20-Year Retrospective

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INTRODUCTION: Pediatric arteriovenous malformations (AVM) are rare lesions with significant morbidity and mortality. Presentations may vary widely between the neonatal period, the infantile period, and older age groups. Literature characterizing the incidence of these complex lesions, their symptoms, and overall prognosis in the setting of modern improved endovascular techniques and understanding of pathophysiology is limited. METHODS: Data from the 2006-2019 HCUP KID database identified 1,224 AVM patients with age less than one year using ICD-9 and ICD-10 codes. Cohorts were stratified by greater or less than 28 days of age to separately assess neonatal AVM patients. Demographics, clinical presentation, comorbidities, mortality, and procedural history were assessed. RESULTS: Brain AVMs were diagnosed in 1 of every 15,160 patients under the age of 1: 504 infants and 600 neonates were identified. Neonates more frequently required non-elective embolizations, while infants received more embolizations and did not require interhospital transfers for care (all p<0.001). Neonatal patients experienced greater mortality (17.5 vs. 2.2%), and were at higher risk of seizures, cardiac failure, pulmonary hypertension, and intraventricular hemorrhage (all p<0.001). Infants more frequently presented with hydrocephalus, intracranial hypertension, developmental delays, hemiplegia/paraplegia, and stroke with or without residual neurological deficit (all p<0.001). Neonates more frequently required ventilatory support and were more likely to receive blood transfusions in surgery, while infants were more likely to receive ventriculoperitoneal shunts and PEG placement (all p<0.001). CONCLUSIONS: Though infantile AVMs are a rare disease, neonates and infants continue to demonstrate a wide constellation of emergent symptoms that require timely endovascular embolization and personalized management. Outcomes continue to improve over time as our understanding of the pathophysiology and management of AVMs evolves.

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