Background: Paediatric liver transplantation (pLT) is the treatment of choice for many liver conditions. However, it still poses relevant challenges, mainly related to the size of the recipients. Unlike in adults, exc...Background: Paediatric liver transplantation (pLT) is the treatment of choice for many liver conditions. However, it still poses relevant challenges, mainly related to the size of the recipients. Unlike in adults, excessive graft volume might represent an issue when the estimated graft-recipient-weight-ratio (GRWR) is significantly disproportionate. In this situation, the traditional left lateral sector (LLS) grafts are too big and other alternatives are required, such as monosegmental or reduced (including hyper-reduced) grafts (RLLS/HRLLS). Results with conventional LLS-pLT are excellent and replicating them with monosegmental or RLLS is challenging given (I) the technical complexity and (II) the need to overcome the large-for-size scenario. This article is to review the existing experience with monosegmental, RLLS/HRLLS grafts and appraise its results. Methods: Systematic search of the electronic databases, conducted from their inception until May 2020. Results: After scrutiny of the available literature, 16 studies were included reporting 330 patients transplanted with monosegmental and RLLS/HRLLS grafts. There were 10 re-grafts (6 of them <90 days);90% of grafts were LDLT. Overall, median recipient’s age and weight were 7 months (range, 5 days-22 months) and 5.8 kg (range, 2.6-8 kg) respectively. Median graft weight was 209 grams (range, 124-264 grams) and median GRWR was 3.5% (range, 2.7-5.6%). Hepatic artery and portal vein thrombosis overall incidence were 1.5% and 4.2%;120 out of the 330 pLT were monosegmental (37%) producing a smaller graft (median of 164 grams) and accordingly a lower GRWR (median 3.2%) compared to reduced LLS. With a median follow-up of 39 months (range, 6-87 months), the overall graft and patient survival were 84% (285/340) and 89% (295/330). Discussion: Monosegmental and RLLS/HRLLS grafts provide access to liver transplantation for very small recipients with excellent results comparable to the standard LLS.展开更多
We report a case involving a rescued low birth weight infant(LBWI) with acute liver failure. Case: The patient was 1594 g and 32^(3/7) gestational wk at birth. At the age of 11 d, she developed acute liver failure due...We report a case involving a rescued low birth weight infant(LBWI) with acute liver failure. Case: The patient was 1594 g and 32^(3/7) gestational wk at birth. At the age of 11 d, she developed acute liver failure due to gestational alloimmune liver disease. Exchange transfusion and high-dose gamma globulin therapy were initiated, and body weight increased with enteral nutrition. Exchange transfusion was performed a total of 33 times prior to living donor liver transplantation(LDLT). Her liver dysfunction could not be treated by medications alone. At 55 d old and a body weight of 2946 g, she underwent LDLT using an S2 monosegment graft from her mother. Three years have passed with no reports of intellectual disability or liver dysfunction. LBWIs with acute liver failure may be rescued by LDLT after body weight has increased to over 2500 g.展开更多
文摘Background: Paediatric liver transplantation (pLT) is the treatment of choice for many liver conditions. However, it still poses relevant challenges, mainly related to the size of the recipients. Unlike in adults, excessive graft volume might represent an issue when the estimated graft-recipient-weight-ratio (GRWR) is significantly disproportionate. In this situation, the traditional left lateral sector (LLS) grafts are too big and other alternatives are required, such as monosegmental or reduced (including hyper-reduced) grafts (RLLS/HRLLS). Results with conventional LLS-pLT are excellent and replicating them with monosegmental or RLLS is challenging given (I) the technical complexity and (II) the need to overcome the large-for-size scenario. This article is to review the existing experience with monosegmental, RLLS/HRLLS grafts and appraise its results. Methods: Systematic search of the electronic databases, conducted from their inception until May 2020. Results: After scrutiny of the available literature, 16 studies were included reporting 330 patients transplanted with monosegmental and RLLS/HRLLS grafts. There were 10 re-grafts (6 of them <90 days);90% of grafts were LDLT. Overall, median recipient’s age and weight were 7 months (range, 5 days-22 months) and 5.8 kg (range, 2.6-8 kg) respectively. Median graft weight was 209 grams (range, 124-264 grams) and median GRWR was 3.5% (range, 2.7-5.6%). Hepatic artery and portal vein thrombosis overall incidence were 1.5% and 4.2%;120 out of the 330 pLT were monosegmental (37%) producing a smaller graft (median of 164 grams) and accordingly a lower GRWR (median 3.2%) compared to reduced LLS. With a median follow-up of 39 months (range, 6-87 months), the overall graft and patient survival were 84% (285/340) and 89% (295/330). Discussion: Monosegmental and RLLS/HRLLS grafts provide access to liver transplantation for very small recipients with excellent results comparable to the standard LLS.
文摘We report a case involving a rescued low birth weight infant(LBWI) with acute liver failure. Case: The patient was 1594 g and 32^(3/7) gestational wk at birth. At the age of 11 d, she developed acute liver failure due to gestational alloimmune liver disease. Exchange transfusion and high-dose gamma globulin therapy were initiated, and body weight increased with enteral nutrition. Exchange transfusion was performed a total of 33 times prior to living donor liver transplantation(LDLT). Her liver dysfunction could not be treated by medications alone. At 55 d old and a body weight of 2946 g, she underwent LDLT using an S2 monosegment graft from her mother. Three years have passed with no reports of intellectual disability or liver dysfunction. LBWIs with acute liver failure may be rescued by LDLT after body weight has increased to over 2500 g.
