Transjugular Intrahepatic Portosystemic Shunt Insertion for the Management of Portal Hypertension in Children

Objectives: To describe our 20-year experience with transjugular intrahepatic portosystemic shunt (TIPSS) procedures for children with resistant portal hypertension (PHTN). Methods: Retrospective review of all children that had a TIPSS performed at Birmingham Children's Hospital from 1st January 1995 - 1st January 2015. Results: 40 children underwent 42 attempted TIPSS for resistant PHTN with recurrent variceal bleeding (n = 35), refractory ascites (n = 4), and hypersplenism (n = 1). Median age at operation was 12 years (range, 7 months - 17 years). Thirty-four procedures were elective and 8 were emergency cases. TIPSS was established in 33 cases (79%). Median portal venous pressure reduction was 10mmHg. Variceal bleeding ceased in 27 (96%) and ascites improved in all. Clinical improvement following TIPSS enabled 7 children to be bridged to transplantation and 7 others to become suitable for transplantation. The 1-year and 5-year survival with TIPSS was 57% and 35%, respectively. Child-Pugh score C was an independent risk factor for adverse outcome (LR = 8.0; 95% CI 2.7 - 23.5; P = 0.001). There were 6 major complications: hepatic artery thrombosis and infarction (n = 1), hepatic artery pseudoaneurysm (n = 1), bile leak (n = 1), and hepatic encephalopathy (n = 3). Encephalopathy was resistant to medical treatment in 2 cases, necessitating staged closure in one. Ten patients (30%) required intervention to maintain TIPSS patency. The 1-year and 5-year freedom from reintervention was 71% and 55%, respectively. Conclusion: A TIPSS is highly successful in controlling symptoms in children with resistant PHTN and facilitating liver transplantation. However, it is technically demanding and not without risk. Patients must be appropriately selected and counselled. Address correspondence and reprint requests to Lauren Johansen, Liver Unit, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH (. e-mail: ljohansen@nhs.net). Received 15 December, 2017 Accepted 3 April, 2018 Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org). Conflicts of Interest and Source of Funding: None declared © 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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