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Introduction Portal vein obstruction (PVO) is a significant vascular complication after liver transplantation (LT) in pediatric patients. Current treatment strategies include percutaneous transluminal angioplasty (PTA), with or without stent placement, mesorex bypass (MRB), splenorenal shunt, mesocaval shunt, endovascular recanalization (EVR), splenic artery embolization and splenectomy. However, specific characteristics of patients undergoing intervention and selection of individual treatment and its efficacy have remained unclear. This review systematically analyzed biochemical and clinical characteristics, selection of treatment, efficacy, and post-procedural complications. Methods We systematically searched PubMed and Embase between January 1995 and March 2021 for studies on the management of PVO after LT. We analyzed the reports for biochemical and clinical characteristics at the timing of the intervention in different patients, selection of treatment, and reported efficacies. Results We found 22 cohort studies with 362 patients who had the following characteristics: biliary atresia (83%), living-donor LT (85%), thrombocytopenia (73%), splenomegaly (40%), ascites (16%), or gastrointestinal bleeding (26%). The 3-year primary patency of PTA without stent placement was similar to that with stent placement (70%–80% and 43%–94%, respectively). MRB was used as an initial treatment with a 3-year patency of 75% to 100%. One study showed that 5-year primary patency of EVR was 80%. Secondary patency was 90% to 100% after 3 years in all studies with PTA alone, PTA/stent placement, and stent placement alone. Conclusion This is the first review of all treatment protocols in PVO after pediatric LT. We showed that an important group of patients has severe symptoms of portal hypertension. Efficacy of all treatment modalities was high in the included studies which make them important modalities for these patients.

We report the case of a 16-year-old boy with primary sclerosing cholangitis associated with inflammatory bowel disease who was initially treated and controlled pharmacologically. He underwent living donor liver transplantation (LDLT) after he developed progressive biliary tract abnormalities and portal hypertension accompanied by recurrent bile duct infections. Two months following LDLT, the hepaticojejunostomy anastomosis became occluded and it was treated surgically. Few weeks later, an increase in drain production persisted without focus; therefore, further diagnostic tests were conducted which proved the recurrence of biliary cast. Under sonographic guidance, external drainage of bile was carried out by percutaneous transhepatic cholangiography and drainage. In total, our patient underwent 5 interventions under general anesthesia and clinically, our patient's general condition improved, and he gained weight. Minimally invasive procedures such as percutaneous transhepatic cholangiography and drainage and balloon dilation are safe and effective, but may require several attempts before being successful. Keywords: Biliary strictures, Pediatric liver transplantation, Percutaneous transhepatic cholangiography and drainage