We report the case of a 16-year-old boy with primary
s
clerosing
c
holangitis 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
t
ranshepatic
c
holangiography 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
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ranshepatic
c
holangiography and drainage and balloon dilation are safe and effective, but may require several attempts before being successful.
IntroductionPortal vein obstruction (PVO) consists of anastomotic stenosis and thrombosis, which occurs due to a progression of the former. The aim of this large-scale international study is to assess the prevalence, current management practices and efficacy of treatment in patients with PVO.Methods and analysisThe Portal vein Obstruction Revascularisation Therapy After Liver transplantation registry will facilitate an international, retrospective, multicentre, observational study, with 25 centres around the world already actively involved. Paediatric patients (aged <18 years) with a diagnosed PVO between 1 January 2001 and 1 January 2021 after liver transplantation will be eligible for inclusion. The primary endpoints are the prevalence of PVO, primary and secondary patency after PVO intervention and current management practices. Secondary endpoints are patient and graft survival, severe complications of PVO and technical success of revascularisation techniques.Ethics and disseminationMedical Ethics Review Board of the University Medical Center Groningen has approved the study (METc 2021/072). The results of this study will be disseminated via peer-reviewed publications and scientific presentations at national and international conferences.Trial registration numberNetherlands Trial Register (NL9261).
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