Superina Riccardo
Department of Surgery, Feinberg School of Medicine, Northwestern University, Evanston, USA.
Head of Transplant Surgery, The Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, 60611, USA.
Pediatr Surg Int. 2017 Dec;33(12):1297-1304. doi: 10.1007/s00383-017-4174-4. Epub 2017 Oct 13.
Biliary atresia (BA) is one of the most common indications for liver transplantation in children. Despite advances in biliary atresia surgical techniques, most children will ultimately require liver transplantation. Possible pre-operative predictors of outcome after the Kasai operation are: 1. Age at operation 2. Presence of the biliary atresia splenic malformation syndrome (BASM) 3. Center specific factors 4. Liver histology and 5. Anatomic pattern of bile ducts found at surgery.Age at surgery is considered a strong predictor of success after portoenterostomy. In a recent study, age of 75 days or more at surgery was associated with less frequent resolution of jaundice and decreased transplant free survival. Similarly, the Ohi type II or III anatomy was associated with a higher risk of transplantation or death than type I. Inflammatory findings on pre-operative biopsy predicted a pooreroutcome after a Kasai procedure than obstructive changes. Nodularity of the liver at surgery as well as ascites was associated with a poorer prognosis.Primary transplantation is rarely done despite excellent outcome. Deaths on the waiting list also have improved with routine use of split and live donor transplantation. The Kasai operation has the highest failure rate in its stated objective than any other operation in pediatric surgery. Failure to achieve any improvement in jaundice occurs in over 30% of all cases, even in the best of hands, and transplantation or listing for transplantation occurs in over half the children with type II and III BA by one year of age in countries where liver transplantation is readily available.There are almost no studies in children with BA that compare the outcome after liver transplantation for BA with or without a prior Kasai procedure. It is postulated that a prospective trial in children predicted to have a poor prognosis after the Kasai procedure based on anatomic pattern, liver histology and presence of BASM, would yield improved care, spare some infants needless surgery, and quite possibly result in diminished morbidity and mortality following liver transplant.
胆道闭锁（BA）是儿童肝移植最常见的适应证之一。尽管胆道闭锁手术技术有所进步，但大多数儿童最终仍需要肝移植。肝门空肠吻合术（Kasai手术）后可能的术前预后预测因素包括：1. 手术年龄；2. 胆道闭锁脾畸形综合征（BASM）的存在；3. 特定中心因素；4. 肝脏组织学；5. 手术中发现的胆管解剖模式。手术年龄被认为是肝门空肠吻合术后成功的有力预测因素。在最近一项研究中，手术时年龄在75天及以上与黄疸消退频率较低和无移植生存时间缩短相关。同样，Ohi II型或III型解剖结构比I型与更高的移植或死亡风险相关。术前活检的炎症表现预示着Kasai手术后的预后比梗阻性改变更差。手术时肝脏的结节性以及腹水与预后较差相关。尽管预后良好，但很少进行原位肝移植。通过常规使用劈离式肝移植和活体供肝移植，等待名单上的死亡情况也有所改善。在小儿外科手术中，Kasai手术在既定目标方面的失败率高于其他任何手术。即使在技术最娴熟的情况下，超过30%的病例黄疸没有任何改善，在肝移植容易获得的国家，超过一半的II型和III型BA患儿在1岁时需要进行移植或列入移植名单。几乎没有针对BA患儿的研究比较了有或没有先行Kasai手术的BA患儿肝移植后的预后。据推测，基于解剖模式、肝脏组织学和BASM的存在，对预计Kasai手术后预后不良的患儿进行前瞻性试验，将改善治疗效果，使一些婴儿避免不必要的手术，并很可能降低肝移植后的发病率和死亡率。
