Successful Anesthetic Management of Deceased Donor Liver Transplant in a Patient With Neonatal Sclerosing Cholangitis As a Result of DCDC2 (doublecortin domain-containing protein 2) Mutation: A Case Report.

Neonatal sclerosing cholangitis, which develops due to mutations in doublecortin domain -containing protein 2, is a rare and serious autosomal recessive inherited end -stage liver disease and frequently requires liver transplant due to decompensated biliary cirrhosis in childhood. Here, we present the anesthetic management of a deceased donor liver transplant in an 8y -ear -old female patient with hepatopulmonary syndrome and pulmonary arteriovenous fistula due to neonatal sclerosing cholangitis. Liver transplant is the only treatment option for hepatopulmonary syndrome and pulmonary arteriovenous fistula due to neonatal sclerosing cholangitis, but liver transplant has high mortality and morbidity. In patients with neonatal sclerosing cholangitis, detailed evaluations of preoperative conditions and underlying pathophysiological factors are crucial. Anesthesia management requires a multidisciplinary approach, comprehensive perioperative assessment, and close intraoperative monitoring to achieve successful outcomes and improve prognosis, thereby reducing mortality rates.