Incidence of pulmonary arteriovenous malformations among pediatric patients presenting with acute ischemic stroke.

OBJECTIVES

Pulmonary arteriovenous malformations (pAVMs), most commonly associated with hereditary hemorrhagic telangiectasia (HHT), are linked to paradoxical embolization however the risk of pediatric stroke and brain abscesses is unclear. We aimed to estimate the national incidence of acute ischemic stroke (AIS) and brain abscess among children with HHT and pAVMs.

METHODS

Using the 2016, 2019, and 2022 Kids' Inpatient Database (KID), we performed a retrospective analysis with pediatric patients (<18 years) hospitalized for AIS or brain abscess using validated ICD-10 codes. Based off U.S. Census data, published prevalence, and occurrence rates, the annual incidence of AIS and brain abscess was estimated among children with pAVMs associated with HHT.

RESULTS

6,780 children were hospitalized for AIS were identified, of whom 23 (0.03%) were associated with a diagnosis of HHT or pAVM. During the same period, 3,048 children were hospitalized for brain abscess, with fewer than 11 cases associated with HHT or pAVM. Based on population estimates and the stroke events identified in our query of the KID database, we estimate that the annual incidence of AIS among children living with HHT is approximately 0.05% per year (23 cases per 14,620 children per 3 years), and, assuming all stroke events occurred in patients with pAVMs, the annual stroke incidence for children with pAVMs associated with HHT is estimated to be no more than 0.1% per year. The annual incidence of brain abscess appears even lower at below 0.02-0.05%.

CONCLUSION

The incidence of AIS and brain abscess in pediatric patients with pAVMs associated with HHT is lower than previously reported, warranting reconsideration of indications to embolize pAVMs based more off clinical significance than routine stroke prevention.