Abstract
Here, we report a case of successful resolution of severe hepatopulmonary syndrome (HPS) in biliary atresia by living donor liver transplantation (LDLT). A 14-year-old girl diagnosed as having biliary atresia (BA) with HPS was referred to our hospital for LDLT. She underwent Kasai portoenterostomy 65 days after birth. Her jaundice and hypoxia worsened from 11 days after birth. On admission, her data on oxygenation were as follows: SpO2 in room air was approximately 80%, arterial blood gas analyses showed PaO2 of 39 mmHg, and perfusion scintigraphy showed a shunt ratio of 41%. From these findings, this patient was determined to have severe HPS. LDLT was performed using an extended left lobe graft from her father. She underwent relaparotomy and ileostomy twice owing to bowel perforation 6 and 9 days after LDLT. One month after LDLT, the shunt ratio increased to 59.9% transiently, but her respiratory condition improved gradually. Six months after LDLT, her data on oxygenation significantly improved as follows: SpO2 in room air, 100%; PaO2, 90.0 mmHg; and shunt ratio, 11.9%. We recommend that although the complication rate after LDLT for BA with severe HPS is relatively high, LDLT should be performed with utmost care. However, there is no significant evidence for the long-term prognosis after LDLT; therefore, it is necessary to closely monitor such a patient for a long period of time.
