See “Feasibility and Efficacy of Transjugular Intrahepatic Portosystemic Shunt (TIPS) in Children” by Di Giorgio et al on page 594.
The management of gastrointestinal bleeding, the consequence of portal hypertension in children, remains a controversial issue (1). Several pharmacologic, endoscopic, radiologic, and surgical procedures are available (2). No randomized controlled trial comparing the various modalities is available to help make an informed decision in children, and such a study is unlikely to occur in the future (3). The preference of 1 particular therapy at any individual institution is often the result of physicians’ experience and the skills of the individuals at the facility. Nonselective β-blockers and endoscopic treatment of esophageal varices (banding or sclerotherapy) are the usual first-line weapons in the pediatric gastroenterologists’ armamentarium to combat gastrointestinal bleeding from varices. Surgical shunts between the portal and caval systems are effective in resolving the portal hypertension, hypersplenism, and ascites in many patients with cirrhosis; however, the result of portal blood flow bypassing the liver can result in increased encephalopathy and hepatopulmonary complications (arteriovenous shunts and pulmonary arterial hypertension). Furthermore, many of these children have significant thrombocytopenia and coagulopathy secondary to their end-stage liver disease and hypersplenism, making surgery extremely risky. Additionally, earlier abdominal surgery may lead to peritoneal adhesions, making dissection more difficult if a liver transplant is subsequently performed.
Not all children with gastrointestinal bleeding and portal hypertension have liver disease. Extrahepatic portal hypertension, frequently the result of umbilical catheters or dehydration with resultant variable degrees of thrombosis of the portal venous system, is a frequent cause of portal hypertension observed in young children. In this scenario, esophageal band ligation or sclerotherapy are the preferable therapies because transformation of the portal vein may occur over time, alleviating the increased portal pressure. In recent years, a surgical shunt (Rex shunt) that reestablishes physiologic portal venous flow to the liver has been used successfully (4). When the portal vein obstruction is limited to the main portal vein, a bypass can be established between the superior mesenteric vein and the left intrahepatic portal vein using a portion of the patient's internal jugular vein or iliac vein. It is important that normal hepatic parenchyma (often established by liver biopsy) is verified before initiation of this surgical procedure.
In selected children with gastrointestinal bleeding secondary to portal hypertension, an alternative and potentially short- and medium-term solution is the use of the transjugular intrahepatic portosystemic shunt (TIPS) (5). In this issue of JPGN, Di Giorgio et al (6) from Italy report their experience in the feasibility and efficacy of TIPS in children. Children with severe portal hypertension that is unresponsive to nonselective β-blockers and endoscopic treatment were selected for TIPS placement using a coated stent. Patients studied included children with cirrhosis and portal hypertension or noncirrhotic portal hypertension with portal vein thrombosis in both native and transplanted livers. Thirteen children between 2005 and 2010 were selected for TIPS, 8 with gastrointestinal bleeding and 5 with ascites. TIPS was successfully placed in 11 of 13 (85%) children. Following TIPS, the portosystemic gradient decreased to 10 mmHg, portal hypertension complications resolved in 10 of 11, no clinically apparent encephalopathy developed, and TIPS revision was necessary in 3 patients. All of the shunts were patent at follow-up (0.2–67 months) in 7 children without transplant and in 4 children (1.5–33 months) with transplant. The authors appropriately concluded that TIPS is feasible and effective in children with ascites or gastrointestinal hemorrhage unresponsive to medical and endoscopic therapies. TIPS should be used to manage portal hypertension in children with native or transplanted livers, and TIPS can be used as a bridge to transplantation and for longer-term management.
So then, when do we proceed to a TIPS procedure in a child? Should we insist that there be failure of pharmacologic and endoscopic therapies before TIPS is attempted? Should gastrointestinal bleeding and ascites be prerequisites for TIPS placement? Should TIPS only be used as a bridge to transplant? We have many unanswered questions desperately needing answers. As Ling et al (3) concluded, the sample size calculations, costs and ethical challenges of randomized controlled trials to obtain evidence-based recommendations for such questions are nearly impossible to accomplish. Thoughtful, well-designed alternative approaches to obtain the answers to such questions remain our responsibility to our young patients and their families. Although TIPS is feasible and efficacious, we must balance the benefits and risks of the procedure in each of our patients in guiding our decision to proceed or not when there are few evidence-based data.
1. Gana JC, Valentino PL, Morinville V, et al. Variation in care for children with esophageal varices: a study of physicians’, patients’, and families’ approaches and attitudes. J Pediatr Gastroenterol Nutr
2. Mileti E, Rosenthal P. Management of portal hypertension in children. Curr Gastroenterol Rep
3. Ling SC, Walters T, McKiernan PJ, et al. Primary prophylaxis of variceal hemorrhage in children with portal hypertension: a framework for future research. J Pediatr Gastroenterol Nutr
4. Scholz S, Sharif K. Surgery for portal hypertension in children. Curr Gastroenterol Rep
5. Heyman MB, LaBerge JM, Somberg KA, et al. Transjugular intrahepatic portosystemic shunts (TIPS) in children. J Pediatr
6. Di Giorgio A, Agazzi R, Alberti D, et al. Feasibility and efficacy of transjugular intrahepatic porto-systemic shunt (TIPS) in children. J Pediatr Gastroenterol Nutr