Abstract / Summary
Abernethy malformation is a rare congenital extrahepatic portosystemic shunt in which portal venous blood bypasses the liver and drains into the systemic circulation. Anesthetic management for surgical shunt closure requires attention to altered venous return, oxygen delivery, coagulation, hepatic metabolic function, and tolerance of portal-flow redirection. We report the perioperative management of a 19-year-old man with type II Abernethy malformation, lifelong hematochezia, severe chronic anemia, coagulation abnormalities, and a giant inferior mesenteric-iliac-caval shunt undergoing laparoscopic inferior mesenteric vein ligation. Multidisciplinary planning focused on hemoglobin optimization, blood-product and coagulation-factor preparation, blood conservation, invasive hemodynamic monitoring, and postoperative intensive care observation. General anesthesia was managed with invasive arterial pressure, CVP, FloTrac-derived CO and SVV trends, arterial blood gas analysis, BIS, temperature, and urine output monitoring. Preoperative transthoracic echocardiography showed normal right ventricular size and systolic function and a low echocardiographic probability of pulmonary hypertension. As no specific indication for continuous intraoperative echocardiography was identified, TEE was considered but was not required. Temporary shunt occlusion increased portal pressure only from 17 to 19 mmHg and improved intrahepatic portal and hepatic parenchymal opacification, supporting definitive ligation. During the procedure, 1,000 mL of crystalloid and 260 mL of washed salvaged red cells were administered; no rescue fluid boluses or vasoactive agents were required. The patient was extubated in ICU on the day of surgery, returned to the ward on postoperative day 1, and was discharged on postoperative day 8 without early recurrent hematochezia. This case illustrates a physiology-based anesthetic approach to the interacting challenges of severely limited oxygen-delivery reserve and abrupt portal-systemic flow redistribution during closure of a giant high-flow portosystemic shunt.