Survival among children with portal vein thrombosis and end-stage liver disease Occlusive PVT is a relatively common complica- tion of chronic liver disease in both adults and children (1–4). The pathogenesis of PVT is linked to local factors in the portal vein related to increased resistance to portal venous flow in cirrhosis, resulting in venous stasis that contrib- utes to thrombophilia. Systemic factors, includ- ing acquired or inherited coagulation abnormalities, dovetail with the local vascular changes, resulting in varying degrees of vessel thrombosis (1, 5, 6). Patients with PVT may present with acute hepatic decompensation in the setting of chronic liver disease but, more com- monly, PVT is noted as an incidental finding upon radiographic evaluation (1, 7, 8). The natural history of patients with chronic liver disease complicated by PVT is largely unknown (7). PVT causes significant morbidity in children, including intractable ascites, variceal bleeding, hypersplenism, and encephalopathy. These com- plications can be particularly difficult to manage in children, and associated mortality is reportedly high (3, 6, 8–11). In the context of pediatric patients, this fact is particularly troubling. Beyond its associated morbidity, PVT can also complicate the liver transplant operation, but advanced sur- gical techniques have made this manageable (4, 9, 12–15). Expedited access to transplantation through allocation policy maneuvers may be warranted if children with chronic liver disease complicated by PVT have significantly inferior survival on the liver transplant waiting list. Our hypothesis is that in a pediatric popula- tion with chronic liver disease, those with a co- morbid diagnosis of PVT have a significantly increased risk of death vs. their non-PVT coun- terparts prior to transplant. In this study, we aimed to address this hypothesis by reviewing pediatric patients with chronic liver disease who were evaluated for transplant at our center. Al-Holou S, Mathur AK, Ranney D, Kubus J, Englesbe MJ. Survival among children with portal vein thrombosis and end-stage liver disease. Pediatr Transplantation 2010: 14: 132–137. Ó 2009 John Wiley & Sons A/S. Abstract: Occlusive PVT concurrent with chronic liver disease is a common clinical entity among pediatric patients referred for trans- plantation. The natural history of PVT is unknown. Our aim was to determine, using a retrospective cohort design, if children under 13 yr with chronic liver disease and concomitant PVT have an increased mortality risk prior to and after transplantation. A total of 203 patients were included in the study. Nearly 10% of the population had PVT (n = 19); 63.2% of PVT patients (5.9% of total cohort) under- went liver transplantation (n = 12). PVT patients tended to be younger than non-PVT patients at evaluation (1.94 ± 3.51 vs. 3.79 ± 4.11, p = 0.059). Clinical and demographic factors were similar between the two groups. Regarding survival, four PVT patients died, of which two had undergone transplantation. Kaplan–Meier analyses indicated that PVT and non-PVT patients had similar survival from the time of evaluation, on the waiting list, and after transplant. Although limited by sample size, our study suggests that a diagnosis of PVT does not increase the mortality risk for children waiting for a liver transplant. Further study is needed to discern variations in mortality risk that may occur in the pediatric chronic liver disease population with PVT. Shaza Al-Holou, Amit K. Mathur, David Ranney, James Kubus and Michael J. Englesbe Division of Transplantation, Department of Surgery, University of Michigan, Ann Arbor, MI, USA Key words: portal vein thrombosis – end-stage liver disease – pediatric liver transplantation – pediatric end-stage liver disease – mortality Michael J. Englesbe, MD, University of Michigan Health System, 2926 Taubman Center, 1500 E. Medical Center Drive, Ann Arbor, MI 48109-0331, USA Tel.: +734-936-9623 Fax: +734-763-3187 E-mail: englesbe@med.umich.edu Accepted for publication 12 February 2009 Abbreviations: MR, magnetic resonance; PELD, pediatric end-stage liver disease; PV, portal vein; PVT, portal vein thrombosis; SMV, superior mesenteric vein. Pediatr Transplantation 2010: 14: 132–137 Ó 2009 John Wiley & Sons A/S. Pediatric Transplantation DOI: 10.1111/j.1399-3046.2009.01175.x 132