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

Chang Gung Med J. 2004 Jun;27(6):449-53.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Alagille Syndrome / surgery*
  • Anesthesia / methods*
  • Blood Transfusion*
  • Child, Preschool
  • Humans
  • Liver Transplantation*
  • Living Donors*
  • Male