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Prognostic scoring systems and outcome of endovascular radiological intervention of chronic Budd‐Chiari syndrome in children
Author(s) -
Singh Sumit K.,
Sen Sarma Moinak,
Yadav Rajanikant,
Kumar Sheo,
Prasad Raghunandan,
Yachha Surender K.,
Srivastava Anshu,
Poddar Ujjal
Publication year - 2018
Publication title -
liver international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.873
H-Index - 110
eISSN - 1478-3231
pISSN - 1478-3223
DOI - 10.1111/liv.13683
Subject(s) - medicine , budd–chiari syndrome , liver transplantation , univariate analysis , liver disease , chronic liver disease , model for end stage liver disease , surgery , gastroenterology , multivariate analysis , transplantation , cirrhosis , inferior vena cava
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.

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