A case report of general anaesthesia for the surgeries of cleft lip-plate in an infant with congenital portosystemic venous shunt

Congenital portosystemic venous shunt (CPSVS) is a rare complication in which the ductus venosus does not close naturally after birth and the shunt forms between the portal vein and vena cava, resulting in pathophysiological states, such as pulmonary hypertension, high serum galactose, hepatic encephalopathy and acute liver failure. Cleft palate is a more frequent congenital abnormality that can hamper feeding and physical growth. Both disorders present potential problems for surgical anaesthesia, including changes in hepatic drug metabolism and difficult airway management. This report summarises our experience of the managements of general anaesthesia for ear tube insertion and reconstructive surgery of cleft lip and cleft palate in a female infant with complication of CPSVS. A preoperative blood test showed high serum levels of aspartate aminotransferase and alanine aminotransferase but no clinically significant elongation of prothrombin time or activated partial thromboplastin time. The patient preoperatively received a strict galactose-free diet. Although general anaesthesia for two surgeries in this case were well managed and uneventful, appropriate preoperative galactose management and lower doses of medication are recommended even if preoperative liver function is normal in a CPSVS patient.