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 chroniccholestasis; typical peculiar facies; posterior embryotoxon; butterfly-like vertebral-archdefects; and cardiovascular malformations, is rarely seen in Taiwan, especially in a livertransplantation 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 9.1g/dl and had mild aortic stenosis and mild pulmonary artery stenosis. He underwent livingdonor liver transplantation without blood transfusion. The key points of successful anestheticmanagement included complete pre-operative evaluation of the cardiovascular system,and intra-operative maintenance of normothermia, normal ionized calcium, normal pH andstable hemodynamics. Surgical blood loss, ascites and intraoperative transudate loss wereprimarily replaced with 5% albumin and crystalloids to maintain the central venous pressurearound 10 cm H2O. No blood transfusion was given for a hemoglobin level higher than 6-7g/dl, but the intravascular volume was sufficient to maintain stable hemodynamics. Ourpatient tolerated the anemia well, it did not seem to affect the recovery of the new liver allograftpostoperatively.