Sarah Verbeeck1, Djalila Mekhali2, David Cassiman3, Geert Maleux4, Peter Witters5. 1. Department of Pediatrics, University Hospital Leuven, Leuven, Belgium. Electronic address: sarah.verbeeck@uzleuven.be. 2. Department of Pediatric Nephrology, University Hospitals Leuven, Leuven, Belgium. Electronic address: djalila.mekahli@uzleuven.be. 3. Department of Gastroenterology-Hepatology, University Hospitals Leuven, Belgium. Electronic address: david.cassiman@uzleuven.be. 4. Department of Interventional Radiology, University Hospitals Leuven, Leuven, Belgium. Electronic address: geert.maleux@uzleuven.be. 5. Department of Pediatric Hepatology, University Hospitals Leuven, Leuven, Belgium. Electronic address: peter.witters@uzleuven.be.
Abstract
BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) with congenital hepatic fibrosis (CHF) causes portal hypertension and its complications. A transjugular intrahepatic portosystemic shunt (TIPSS) could serve as a symptomatic treatment for portal hypertension-related symptoms in these children. AIMS: To study the effect of TIPSS on portal hypertension, liver and kidney function and the long term complications. MATERIALS AND METHODS: We report on 5 children with CHF treated with a TIPSS to manage severe portal hypertension related symptoms. RESULTS: Mean follow-up time was 7 years and 2 months. At the end of follow-up there was a reduction of spleen size (p = 0.715) and hypersplenism with a rise in platelet count (p = 0.465). Esophageal varices and ascites disappeared in all patients. Liver and kidney function remained stable. In two patients endotipsitis was suspected and two patients developed an in-stent stenosis. There was no sign of encephalopathy in our patients. CONCLUSION: TIPSS using ePTFE-covered stent is a feasible and effective alternative for surgical portosystemic shunting in children with CHF, also on the long term. It can postpone the need of a liver transplantation but close monitoring remains important for early diagnosis of endotipsitis or stent dysfunction related to stenosis.
BACKGROUND:Autosomal recessive polycystic kidney disease (ARPKD) with congenital hepatic fibrosis (CHF) causes portal hypertension and its complications. A transjugular intrahepatic portosystemic shunt (TIPSS) could serve as a symptomatic treatment for portal hypertension-related symptoms in these children. AIMS: To study the effect of TIPSS on portal hypertension, liver and kidney function and the long term complications. MATERIALS AND METHODS: We report on 5 children with CHF treated with a TIPSS to manage severe portal hypertension related symptoms. RESULTS: Mean follow-up time was 7 years and 2 months. At the end of follow-up there was a reduction of spleen size (p = 0.715) and hypersplenism with a rise in platelet count (p = 0.465). Esophageal varices and ascites disappeared in all patients. Liver and kidney function remained stable. In two patients endotipsitis was suspected and two patients developed an in-stent stenosis. There was no sign of encephalopathy in our patients. CONCLUSION: TIPSS using ePTFE-covered stent is a feasible and effective alternative for surgical portosystemic shunting in children with CHF, also on the long term. It can postpone the need of a liver transplantation but close monitoring remains important for early diagnosis of endotipsitis or stent dysfunction related to stenosis.
Authors: Andrew J Woerner; David S Shin; Jeffrey Forris Beecham Chick; Kevin S H Koo; Evelyn K Hsu; Elizabeth R Tang; Eric J Monroe Journal: Pediatr Radiol Date: 2021-03-30
Authors: Erum A Hartung; Juan S Calle-Toro; Carolina Maya Lopera; Jessica Wen; Robert H Carson; Mohini Dutt; Kathryn Howarth; Susan L Furth; Kassa Darge; Suraj D Serai Journal: Abdom Radiol (NY) Date: 2020-08-05