Abernethy Malformation with Pulmonary Arteriovenous Malformations in a Pediatric Patient: An Anesthetic Challenge

Abernethy malformation is a rare congenital vascular anomaly of the splanchnic venous system, leading to portosystemic shunting and related symptoms. A 7-month-old child was admitted with cyanosis and respiratory distress. Echocardiography revealed dilated biatrium and left superior vena cava into the coronary sinus. A bubble contrast study suggested pulmonary arteriovenous malformation (AVM) necessitating cardiac catheterization study for embolization. The child was induced with fentanyl, ketamine, and vecuronium after preoxygenation. Hypothermia prevention and measures to address pulmonary venous resistance including air embolism were implemented. The study confirmed bilateral pulmonary AVM and Type 1b Abernethy malformation. Given the complexity of the procedure, the parents were counseled for liver transplant. The management focuses on hemodynamic stability, to mitigate air embolism, counteract intraoperative pulmonary hypertension, and ensure adequate anesthesia and analgesia.