Anesthetic management of a patient with Alagille's syndrome undergoing living donor liver transplantation without blood transfusion

Alagille's syndrome (AGS), which has five main characteristics including chronic cholestasis; typical peculiar facies; posterior embryotoxon; butterfly-like vertebral-arch defects; and cardiovascular malformations, is rarely seen in Taiwan, especially in a liver transplantation setting. We present the successful anesthetic management of a 3-year-11-month-old boy with AGS. The patient was anemic with a preoperative hemoglobin of 9.1 g/dl and had mild aortic stenosis and mild pulmonary artery stenosis. He underwent living donor liver transplantation without blood transfusion. The key points of successful anesthetic management included complete pre-operative evaluation of the cardiovascular system, and intra-operative maintenance of normothermia, normal ionized calcium, normal pH and stable hemodynamics. Surgical blood loss, ascites and intraoperative transudate loss were primarily replaced with 5% albumin and crystalloids to maintain the central venous pressure around 10 cm H2O. No blood transfusion was given for a hemoglobin level higher than 6-7 g/dl, but the intravascular volume was sufficient to maintain stable hemodynamics. Our patient tolerated the anemia well, it did not seem to affect the recovery of the new liver allograft postoperatively.