Madadi-Sanjani Omid, Herden Uta, Uecker Marie
Klinik für Viszerale Transplantationschirurgie, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland.
Klinik für Kinderchirurgie, Medizinische Hochschule Hannover, Hannover, Deutschland.
Chirurgie (Heidelb). 2025 Jun;96(6):474-481. doi: 10.1007/s00104-025-02259-2. Epub 2025 Feb 27.
Biliary atresia (BA) is a rare disease in neonates of unknown etiology. BA is defined by the extent of extra- and intrahepatic bile duct destruction, which results in liver deterioration and cirrhosis within the first years of life. Liver transplantation (LT) is the only curative treatment for BA, accompanied by LT-associated risks and complications; however, more than 60 years after it's first report, the Kasai hepatoportoenterostomy (KPE) is still an essential procedure in the sequential management of BA, as the primary surgical treatment option that can achieve long-term survival with a native liver. We highlight the key surgical steps of KPE and discuss relevant aspects.
胆道闭锁（BA）是一种病因不明的新生儿罕见疾病。BA是根据肝外和肝内胆管破坏的程度来定义的，这会导致在生命的最初几年内肝脏恶化和肝硬化。肝移植（LT）是BA唯一的治愈性治疗方法，但伴有与LT相关的风险和并发症；然而，在首次报道60多年后，Kasai肝门肠吻合术（KPE）仍然是BA序贯治疗中的一个重要手术，作为一种能使患儿依靠自身肝脏长期存活的主要外科治疗选择。我们重点介绍KPE的关键手术步骤并讨论相关方面。
