F. Lopez-Verdugo2, A. S. Muñoz-Abraham4, J. Merola7, Z. Kastenberg2, A. Bertacco3, D. Alonso2, W. R. Hewitt5, M. Facciuto6, S. H. Emre7, M. I. Rodriguez-Davalos2 6Mount Sinai School Of Medicine,Department Of Transplantation,New York, NY, USA 7Yale University School Of Medicine,Department Of Surgery,New Haven, CT, USA 2Intermountain Primary Children’s Hospital,Department Of Surgery, Transplant And Hepatobiliary Surgery,Salt Lake City, UT, USA 3Università di Padova,Department Of Surgery, Hepatobiliary Surgery And Liver Transplantation,Padova, PD, Italy 4Saint Louis University School Of Medicine,Pediatric Surgery,St. Louis, MO, USA 5Mayo Clinic In Arizona,Department Of Surgery,Phoenix, AZ, USA
Introduction: Extrahepatic portal venous obstruction (EHPVO) as well as intrinsic liver disease (ILD) are major causes of portal hypertension in children often accompanied by development of cavernoma. Decompressive surgeries, including Meso-Rex bypass (MRB) and portosystemic shunts (PSS) are offered for definitive therapy in such cases. The study aims to review surgical outcomes in a cohort of patients who underwent MRB or PSS over the last decade.
Methods: Medical records from all children who received a MRB or PSS at across different institutions in the US between 2005 and 2018 were reviewed and described. Data collected included age, gender, diagnosis, clinical manifestations of PTH, type of procedure, and clinical outcomes. Patency and patient survival were assessed at 6-months and at the last follow-up visit.
Results: 34 surgeries were performed in 29 children. The mean age at the time of surgery was 8.1 years (range: 1.5-19),14 patients (48.3%) were male. Common causes of PTH were EHPVO (n=17) and ILD (n=9). PTH manifestations at presentation were GI bleeding, splenomegaly and hepatic encephalopathy (HE). There were 18 MRB and 8 selective PSS: 6 distal splenorenal and 2 spleno-caval. 4 proximal splenorenal, 2 mesorenal, one mesocaval, and one side-to-side splenorenal shunts. Nine patients (26.4%) had treatment failure before the 6-month follow-up, 4 of which happened on postoperative day 1. Treatment failure for MRB resulted from thrombosis (n=5), and collateral steal phenomenon (n=3) and was managed with thrombectomy (n=3), creation of an alternative PSS (n=2), or successful re-do MRB with occlusion of collaterals (n=2). 25 patients (73.5%) have been followed for more than 6 months (median: 28, range: 7-116) 23 are still alive and 2 died with a patent shunt. 18 procedures did not require further intervention, 2 required balloon angioplasty, 2 ILD needed LTx, and 1 patient required conversion of MRB to PSS.
Conclusion: In this series, MRB in patients without ILD was successful in 81.2%. Early shunt thrombosis may occur in the setting of an underdeveloped intrahepatic portal system though secondary patency rates of 60% were achieved without converting to PSS. These results support the notion that MRB should remain the standard of care for children with EHPVO or portal vein thrombosis after transplantation as it re-establishes hepatopetal flow; selective PSS should be the next line therapy for those patients with concurrent ILD or when MRB is not feasible due to technical or functional constraints. Creation of non selective shunts should be reserved for patients with massive splenomegaly or when the anatomy is not favorable, and HE is not a concern. Multidisciplinary work with interventional radiology is key to the successful treatment of these patients.