Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd‐Chiari syndrome in children. (17th February 2018)

• Record Type: Journal Article
• Authors: Singh, Sumit K.; Sen Sarma, Moinak; Yadav, Rajanikant; Kumar, Sheo; Prasad, Raghunandan; Yachha, Surender K.; Srivastava, Anshu; Poddar, Ujjal
• Is Part Of: Liver international. Volume 38:Number 7(2018)
• Page Start: 1308
• Page End: 1315
• Publication Date: 2018-02-17
• Subjects: Budd‐Chiari syndrome -- children -- prognostic scores -- radiological intervention; Liver -- Periodicals; Liver -- Diseases -- Periodicals; 616.362
• DOI: 10.1111/liv.13683
• Languages: English
• ISSNs: 1478-3223

Abstract

Abstract: Background and Aim: 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. Methods: 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. Results: 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). Conclusions: 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.