Madadi-Sanjani Omid, Fischer Lutz, Uecker Marie, Tomuschat Christian, Herden Uta, Sterneck Martina, Hegen Bianca
Department of Visceral Transplantation, University Medical Center Hamburg-Eppendorf, Martinistreet 52, 20251, Hamburg, Germany.
Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany.
Pediatr Surg Int. 2025 Jun 12;41(1):165. doi: 10.1007/s00383-025-06066-y.
Biliary atresia (BA) is a rare cholangiopathy in neonates, leading to end-stage liver failure in the first years of life, when left untreated. Kasai procedure aims to restore biliary drainage to achieve native liver survival. While liver transplantation is the necessary treatment in children with failure of the Kasai procedure, the number of native liver survivors in the long-term remains around 20-30%. Reports on malignancies in native livers of children, adolescents and adults with BA are increasing, including cases of hepatocellular carcinoma, cholangiocarcinoma, hepatoblastoma and small intestinal adenocarcinoma. In this review we summarize the reports, with focus on tumor markers, imaging technologies and reported outcomes. Furthermore, we discuss recent advances in surveillance strategies in adults with chronic liver impairment.
胆道闭锁（BA）是一种新生儿罕见的胆管病，若不治疗，会在生命的头几年导致终末期肝衰竭。肝门空肠吻合术旨在恢复胆汁引流以实现自体肝存活。虽然肝移植是肝门空肠吻合术失败患儿的必要治疗方法，但长期自体肝存活者的比例仍保持在20%-30%左右。关于患有BA的儿童、青少年和成人自体肝发生恶性肿瘤的报道越来越多，包括肝细胞癌、胆管癌、肝母细胞瘤和小肠腺癌的病例。在本综述中，我们总结了这些报道，重点关注肿瘤标志物、成像技术和报道的结果。此外，我们还讨论了慢性肝功能损害成人监测策略的最新进展。
