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

摘要: 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 a liver transplantation setting. We present the successful anesthetic management of 3-year-11-month-old boy with AGS. The patient was anemic preoperative hemoglobin 9.1 g/dl had mild aortic stenosis pulmonary artery stenosis. He underwent living donor without blood transfusion. key points included complete pre-operative evaluation system, intra-operative maintenance normothermia, normal ionized calcium, pH stable hemodynamics. Surgical loss, ascites intraoperative transudate loss were primarily replaced 5% albumin crystalloids to maintain central venous pressure around 10 cm H2O. No transfusion given for level higher than 6-7 g/dl, but intravascular volume sufficient Our tolerated anemia well, it did not seem affect recovery new allograft postoperatively.