Hassan Anbari, MD – Attending Physician, University of Michigan; Mamadou Sanogo, MD – Attending Physician, University of Michigan; baljendra Kapoor, MD – Attending Physician, Division Director, Interventional Radiology, University of Michigan; Joseph Gemmete, MD – Attending Physician, University of Michigan
Purpose: Understand the common etiologies of portal hypertension in children and how they contrast with those affecting adults Discuss different methods of anatomic liver transplantation in children, and how this affects treatment of liver disease in children Recognize the primary role of endoscopic surveillance and therapy for treatment of variceal bleeding in children
Material and Methods: Portal hypertension affects children in different ways than adults. Different underlying etiologies, primarily extrahepatic portal vein thrombosis and biliary atresia, lead to different approaches in management. There is greater availability of donor liver grafts for children, in part due to anatomic considerations that allow for partial liver transplantation, and in very small children left lateral segment transplantation. This means more pediatric patients with severe underlying liver disease will undergo liver transplantation, which may resolve associated portal hypertension.
Results: Endoscopy remains the primary treatment modality for bleeding esophageal varices in children due to portal hypertension, though data on which patients with suspected portal hypertension should undergo endoscopic surveillance is a topic of ongoing research. Endoscopic banding is generally superior to endoscopic sclerotherapy, and sclerotherapy is generally reserved for patients in whom band ligation is not possible due to their small size. Surgical shunting in the form of mesenteric vein bypass remains popular in pediatric patients, partly due to anatomic considerations in children with isolated extrahepatic portal vein thrombosis. The Meso-Rex bypass allows for restoration of near-anatomic flow via an autologous shunt from the distal SMV to the intrahepatic portal venous system, negating risks of encephalopathy and CHF associated with more shunts to the hepatic veins such as TIPS. TIPS in children for palliation of severe symptoms of portal hypertension and as second line therapy for bleeding esophageal varices among other indications is performed. However, heightened concerns over the negative effects of hepatic encephalopathy and long-term shunt durability in children makes TIPS less popular in this population.
Conclusions: An IR working with children with portal hypertension must understand the key differences in etiology and treatment amongst this patient population. TIPS may still be pursued, but has different implications in children.