Evidence map›Paper›PMID 42177454›Full record

ArticleBMC pediatrics2026

Liver transplantation for Type I Abernethy malformation with liver tumors in children: a report of two cases with contrasting portal vein reconstruction strategies.

Huanli Han, Mingman Zhang, Xiaoke Dai

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Article in BMC pediatrics, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 · Who and what money

Authors and funding

3 authors.

Huanli HanDepartment of Pediatric Hepatobiliary Surgery, Children's Hospital of Chongqing Medical University, Chongqing, 400014, P.R. China.
Mingman ZhangDepartment of Pediatric Hepatobiliary Surgery, Children's Hospital of Chongqing Medical University, Chongqing, 400014, P.R. China.
Xiaoke DaiDepartment of Pediatric Hepatobiliary Surgery, Children's Hospital of Chongqing Medical University, Chongqing, 400014, P.R. China. 243479093@qq.com.

Funding

National Clinical Key Specialty Construction Project National Health Commission Office Medical Administration Letter〔2024〕189
6 · The paper itself

Abstract

backgroundType I Abernethy malformation is a rare congenital portosystemic shunt with an absent intrahepatic portal vein (PV). It often presents with liver tumors or metabolic disorders. Liver transplantation (LT) is the only definitive treatment, but it poses unique technical challenges due to the absent native PV. Detailed surgical guidance for variable anatomies remains scarce. CASE PRESENTATION: We report two children with Type I Abernethy malformation who underwent LT in 2023. Both presented with benign liver tumors, hyperammonemia and abnormal liver function. Case 1 (2.5 y/m) received a deceased-donor left-lobe LT. Owing to insufficient PV length, a deceased-donor iliac vein graft was used for PV reconstruction. Case 2 (5.7 y/f) underwent living-donor left-lateral-sector LT with direct anastomosis of the donor left PV to the recipient's shunt vessel. Graft-to-recipient weight ratios (GRWR) were 2.29% and 1.45%, respectively.

resultsBoth transplant procedures were successful. Postoperative liver function and ammonia normalized. Case 2 developed hepatic venous outflow obstruction (HVOO) postoperatively, which was successfully managed with balloon angioplasty on POD 24. At 18-month follow-up, both patients were alive with good graft function, without tumor recurrence or metabolic abnormalities. To our knowledge, this is among the few reports with 18-month follow-up for pediatric patients with this disease after LT using these techniques.

conclusionThis report offers a practical and anatomy-based surgical framework for PV reconstruction in pediatric Type I Abernethy malformation: direct anastomosis when an adequate shunt vessel is present, or interposition grafting (e.g., with iliac vein) when the native portal stump is absent. These contrasting strategies achieved good early outcomes at 18 months, suggesting that LT can be a curative option for this challenging anomaly. These observations are primarily hypothesis-generating. Validation will require larger, multicenter cohorts.

Indexed as

Congenital Portosystemic ShuntLiver NeoplasmsLiver TransplantationPortal VeinChild, PreschoolFemaleHumansLiving DonorsMaleRecipient VesselsAbernethy malformationChildrenCongenital portosystemic shuntLiver transplantationPortal vein reconstruction

Identifiers

PMID42177454
PMCPMC13378170

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.