Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Department of Radiodiagnosis, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Liver Int. 2018 Jul;38(7):1308-1315. doi: 10.1111/liv.13683. Epub 2018 Feb 17.
Prognostic scoring systems (PSS) have not been validated in children with chronic Budd-Chiari syndrome (BCS). We aimed to analyse the long-term outcome of radiological intervention (RI) and validate the PSS in children.
Chronic BCS children were analysed in four subgroups: (i) SI: successful intervention (primary or secondary stent patency) (ii) PO: poor outcome (refractory stent block or requirement of liver transplantation), (iii) NU: naïve unintervened (awaiting RI) and (iv) DBI: died before intervention. PSS analysed included Paediatric end-stage liver disease (PELD), Rotterdam, BCS-Transjuglar intrahepatic Portosystemic shunt (BCS-TIPS) index, Zeitoun, Child-Pugh and Model for end-stage liver disease.
Of 113 BCS children, 48 children underwent 53 successful primary RI. Actuarial probability of vascular patency was 87% at 1 year and 82% at 5 years follow-up. Four groups (SI: n = 40, PO: n = 7, NU: n = 13, DBI: n = 6) were analysed. Univariate analysis showed pre-intervention PELD score [PO: 11 (-1-23) vs SI: 2 (-8-25), P = .009] with a cut-off of 4 (AUC: 0.809, 86% sensitivity, 75% specificity) determined PO following intervention. In unintervened group (NU vs DBI), multivariate analysis demonstrated that Zeitoun score predicted death independently (OR 15.4, 95% CI: 1.17-203.56, P = .04) with a cut-off of 4.3 (AUC: 0.923, 83% sensitivity and 77% specificity).
Children with BCS have a favourable long-term outcome. Among those undergoing RI, pre-intervention PELD score determines the outcome. Survival is determined by Zeitoun score in those unintervened.
预后评分系统（PSS）尚未在慢性布加综合征（BCS）儿童中得到验证。我们旨在分析放射介入治疗（RI）的长期结果，并验证儿童的 PSS。
将慢性 BCS 儿童分为以下四个亚组进行分析：（i）SI：成功干预（主要或次要支架通畅）（ii）PO：不良结局（难治性支架阻塞或需要肝移植）（iii）NU：未干预（等待 RI）（iv）DBI：介入前死亡。分析的 PSS 包括小儿终末期肝病评分（PELD）、鹿特丹、BCS-经颈静脉肝内门体分流术（BCS-TIPS）指数、Zeitoun、Child-Pugh 和终末期肝病模型。
在 113 例 BCS 儿童中，48 例儿童接受了 53 次成功的原发性 RI。血管通畅的累积概率在 1 年时为 87%，在 5 年随访时为 82%。分析了四个组（SI：n=40，PO：n=7，NU：n=13，DBI：n=6）。单因素分析显示，干预前 PELD 评分[PO：11（-1-23）比 SI：2（-8-25），P=0.009]，以 4 分为截定点（AUC：0.809，86%敏感性，75%特异性）确定了干预后的 PO。在未干预组（NU 与 DBI）中，多因素分析表明，Zeitoun 评分独立预测死亡（OR 15.4，95%CI：1.17-203.56，P=0.04），以 4.3 分为截定点（AUC：0.923，83%敏感性和 77%特异性）。
BCS 儿童具有良好的长期预后。在接受 RI 的儿童中，干预前的 PELD 评分决定了结局。在未干预的儿童中，生存由 Zeitoun 评分决定。
