Chun-Chen Sun, Chen-Hsiu Chen
Taoyuan Armed Forces General Hospital. National Defense Medical University. Kaohsiung Veterans General Hospital. National Yang Ming Chiao Tung University. National Sun Yat-Sen University.
Taiwan
Respirology Case Reports
Respirol Case Rep 2026; 14:
DOI: 10.1002/rcr2.70707
Abstract
A 4-year-old girl with no prior respiratory history developed refractory intraoperative hypoxemia during elective dental surgery under general anaesthesia. Despite 100% inspired oxygen and systematic exclusion of all conventional causes of desaturation, pulse oximetry (SpO2) failed to exceed 94%. Postoperative contrast-enhanced chest computed tomography identified a 2.01-cm pulmonary arteriovenous malformation (PAVM) in the right lung, and subsequent molecular genetic testing confirmed hereditary hemorrhagic telangiectasia (HHT). The patient was discharged with baseline SpO2 of 85%-95% on room air and established on long-term paediatric pulmonary follow-up. This case highlights that PAVMs associated with HHT can manifest as unexplained perioperative hypoxemia in asymptomatic children, and that general anaesthesia with positive pressure ventilation may unmask previously compensated right-to-left shunting. Early recognition enables appropriate perioperative management and timely HHT surveillance.
Category
Pulmonary Arteriovenous Malformations
Genetic Factors Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes