scholarly journals Liver Transplantation for Biliary Atresia in Adulthood: Single-Centre Surgical Experience

2021 ◽  
Vol 10 (21) ◽  
pp. 4969
Author(s):  
Miriam Cortes-Cerisuelo ◽  
Christina Boumpoureka ◽  
Noel Cassar ◽  
Deepak Joshi ◽  
Marianne Samyn ◽  
...  

Background: Biliary atresia (BA) is the most common indicator for liver transplant (LT) in children, however, approximately 22% will reach adulthood with their native liver, and of these, half will require transplantation later in life. The aim of this study was to analyse the surgical challenges and outcomes of patients with BA undergoing LT in adulthood. Methods: Patients with BA requiring LT at the age of 16 or older in our unit between 1989 and 2020 were included. Pretransplant, perioperative variables and outcomes were analysed. Pretransplant imaging was reviewed to assess liver appearance, spontaneous visceral portosystemic shunting (SPSS), splenomegaly, splenic artery (SA) size, and aneurysms. Results: Thirty-four patients who underwent LT for BA fulfilled the inclusion criteria, at a median age of 24 years. The main indicators for LT were synthetic failure and recurrent cholangitis. In total, 57.6% had significant enlargement of the SA, 21% had multiple SA aneurysm, and SPSS was present in 72.7% of the patients. Graft and patient survival at 1, 5, and 10 years was 97.1%, 91.2%, 91.2% and 100%, 94%, 94%, respectively Conclusions: Good outcomes after LT for BA in young patients can be achieved with careful donor selection and surgery to minimise the risk of complications. Identification of anatomical variants and shunting are helpful in guiding attitude at the time of transplant.

Cytokine ◽  
2018 ◽  
Vol 111 ◽  
pp. 382-388 ◽  
Author(s):  
Omid Madadi-Sanjani ◽  
Joachim F. Kuebler ◽  
Stephanie Dippel ◽  
Anna Gigina ◽  
Christine S. Falk ◽  
...  

2019 ◽  
Vol 2 (1) ◽  
pp. 38-40
Author(s):  
Panyavee Pitisuttithum ◽  
Piyawat Komolmit

Biliary atresia is a progressive fibro-obliterative disease of the bile duct, commonly diagnosed within a few months of life. After hepatoportoenterostomy was successfully performed, about one fourth of biliary atresia patients have grown up to the age of twenty with their native liver. However, progression to liver cirrhosis, development of portal hypertension, or cholangitis are the major problems of adult with biliary atresia. Monitoring of these complications and listing for liver transplantation whenever indicated are the key when caring adult with biliary atresia.   Figure 1  แสดง CT abdomen พบว่ามีตับโต และมีลักษณะของตับแข็งและ ภาวะความดันของระบบหลอดเลือด portal สูงขึ้น (collateral vessels, splenomegaly, ascites) พบการขยายตัวของท่อน้ำดีในตับ (multifocal dilated ducts and peribiliary cysts along bilateral intrahepatic ducts) และต่อมน้ำเหลืองโตในช่องท้อง (mesenteric, gastrohepatic, hepatoduodenal, aortocaval, para aortic, right anterior diaphragmatic, lower paraesophageal lymph node ขนาดสูงสุด 1.8 ซม.)


2018 ◽  
Vol 53 (2) ◽  
pp. 277-280
Author(s):  
Takanori Ochi ◽  
Hiroki Nakamura ◽  
Momoko Wada ◽  
Tsuyoshi Tamura ◽  
Hiroyuki Koga ◽  
...  

Medicina ◽  
2020 ◽  
Vol 57 (1) ◽  
pp. 16
Author(s):  
Raluca-Cristina Apostu ◽  
Vlad Fagarasan ◽  
Catalin C. Ciuce ◽  
Radu Drasovean ◽  
Dan Gheban ◽  
...  

Background and objectives: In patients with biliary atresia (BA), hepatoportoenterostomy (HPE) is still a valuable therapeutic tool for prolonged survival or a safer transition to liver transplantation. The main focus today is towards efficient screening programs, a faster diagnostic, and prompt treatment. However, the limited information on BA pathophysiology makes valuable any experience in disease management. This study aimed to analyze the evolution and survival of patients with BA referred for HPE (Kasai operation) in our department. Materials and Methods: A retrospective analysis was performed on fourteen patients with BA, diagnosed in the pediatric department and further referred for HPE in our surgical department between 2010 and 2016. After HPE, the need for transplantation was assessed according to patients cytomegalovirus (CMV) status, and histological and biochemical analysis. Follow-up results at 1–4 years and long term survival were assessed. Results: Mean age at surgery was 70 days. Surgery in patients younger than 60 days was correlated with survival. Jaundice’s clearance rate at three months was 36%. Total and direct bilirubin values had a significant variation between patients with liver transplants and native liver (p = 0.02). CMV was positive in eight patients, half with transplant need and half with native liver survival. Smooth muscle actin (SMA) positivity was proof of advanced fibrosis. The overall survival rate was 79%, with 75% for native liver patients and an 83% survival rate for those with liver transplantation. Transplantation was performed in six patients (43%), with a mean of 10 months between HPE and transplantation. Transplanted patients had better survival. Complications were diagnosed in 63% of patients. The mean follow-up period was six years. Conclusions: HPE, even performed in advanced cirrhosis, allows a significant survival, and ensures an essential time gain for patients requiring liver transplantation. A younger age at surgery is correlated with a better outcome, despite early CMV infection.


2019 ◽  
Author(s):  
M Fodor ◽  
A Woerdehoff ◽  
J Walte ◽  
S Neururer ◽  
H Esser ◽  
...  

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