Perioperative Strategies and Thrombophilia in Children with Extrahepatic Portal Vein Obstruction Undergoing the Meso-Rex
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ORIGINAL ARTICLE
Perioperative Strategies and Thrombophilia in Children with Extrahepatic Portal Vein Obstruction Undergoing the Meso-Rex Bypass Rukhmi Bhat & Timothy B. Lautz & Riccardo A. Superina & Robert Liem
Received: 14 November 2012 / Accepted: 24 January 2013 / Published online: 6 February 2013 # 2013 The Society for Surgery of the Alimentary Tract
Abstract Background/Purpose Extrahepatic portal vein obstruction (EHPVO) is an important cause of chronic portal hypertension in children. Although usually idiopathic in etiology, genetic and acquired thrombophilia have been implicated in EHPVO. Meso-Rex bypass is increasingly used to treat EHPVO in children. Objective The objective of this study is to assess the relationship of postoperative anticoagulation strategies and thrombophilic risk factors to the development of bypass thrombosis following the meso-Rex bypass. Methods Records of children who underwent meso-Rex bypass for EHPVO at a single institution from 1999 to 2009 were reviewed, and preoperative thrombophilia testing, perioperative anticoagulation strategies, and postoperative bypass patency based on imaging at last follow-up were examined. Results Sixty-five children with EHPVO underwent a first time meso-Rex bypass during the study period, and 9 of 65 (14 %) developed bypass thrombosis. The use of warfarin in the postoperative period was more common among children with thrombosed shunts than among those with open shunts [63 % vs. 20 %; OR, 6.5 (95 % CI, 1.3–31.5), p=0.022]. The contribution of genetic or acquired thrombophilia to shunt thrombosis was inconclusive given variability in testing. Conclusions Choice of anticoagulation following meso-Rex bypass may affect postoperative incidence of bypass thrombosis. Role of thrombophilic risk factors in the development of shunt thrombosis remains unclear. Keywords Extrahepatic portal vein obstruction . Meso-Rex bypass . Thrombosis . Thrombophilia . Anticoagulation
Introduction Extrahepatic portal vein obstruction (EHPVO), the etiology of which is often idiopathic, is an important cause of chronic R. Bhat (*) : R. Liem Division of Hematology, Oncology & Stem Cell Transplantation, Ann & Robert H. Lurie Children’s Hospital of Chicago, Department of Pediatrics, Northwestern University Feinberg School of Medicine, 225 E Chicago Av, Box # 30, Chicago, IL 60611, USA e-mail: [email protected] T. B. Lautz : R. A. Superina Department of Surgery, Ann & Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL 60611, USA
portal hypertension in children.1,2 Thrombophilia has been reported as a risk factor for EHPVO in both adults and children.3–6 The most commonly studied thrombophilic risk factors include inherited traits such as factor V Leiden (FVL) mutation, mutations in the prothrombin gene (PG), and methylenetetrahydrofolate reductase (MTHFR) gene, as well as inherited deficiencies in the naturally occurring anticoagulants. However, existing studies have been small, and the thrombophilic risk fac
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