Children (Basel, Switzerland) · 2026
Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy (ALPPS) in Children with Advanced Hepatoblastoma-Lessons from a Case Series and Literature Review.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Garnier Hanna, Murawski Maciej, Wojciechowska Ewelina, Kalinchuk Oleksandr, Sinacka Katarzyna, Izycka-Swieszewska Ewa, Czauderna Piotr
Аннотация
BACKGROUND: Liver transplantation is the standard treatment for children with advanced hepato-blastoma when complete resection is not feasible. However, transplantation may be contraindicated because of persistent metastatic disease, severe comorbidities, poor clinical condition, or donor-related limitations. Associating Liver Partition and Portal Vein Ligation for Staged Hepatectomy (ALPPS) has emerged as a potential rescue strategy in highly selected patients. This study evaluated the feasibility, safety, and oncological outcomes of ALPPS in pediatric hepatoblastoma.
METHODS: A retrospective analysis was performed of four consecutive children with advanced hepatoblastoma who underwent classical ALPPS between 2013 and 2025 in two specialized centers. Patient characteristics, indications, future liver remnant (FLR) volumetry, perioperative outcomes, complications, and oncological follow-up were reviewed.
RESULTS: The median age at surgery was 22 months. In all patients, the FLR was considered insufficient for one-stage hepatectomy, leading to ALPPS. Rapid hypertrophy of the FLR was achieved in every case, allowing completion of the second stage after a median of 8.5 days (range, 7-11 days). FLR volume increased by 50-89% following the first stage. Despite successful liver hypertrophy, outcomes remained poor in three patients. One patient died intraoperatively from venous air embolism during the second stage, two died from disease recurrence despite aggressive multimodal treatment, and one remains in complete clinical and radiological remission following ALPPS and adjuvant chemotherapy.
CONCLUSIONS: ALPPS reliably induces rapid FLR hypertrophy and may provide a potentially curative option for carefully selected children when liver transplantation is not feasible. However, its substantial perioperative risk and generally unfavorable oncological outcomes support its role only as a rescue procedure in experienced pediatric hepatobiliary centers. Further multicenter studies are needed to better define indications and patient selection.
METHODS: A retrospective analysis was performed of four consecutive children with advanced hepatoblastoma who underwent classical ALPPS between 2013 and 2025 in two specialized centers. Patient characteristics, indications, future liver remnant (FLR) volumetry, perioperative outcomes, complications, and oncological follow-up were reviewed.
RESULTS: The median age at surgery was 22 months. In all patients, the FLR was considered insufficient for one-stage hepatectomy, leading to ALPPS. Rapid hypertrophy of the FLR was achieved in every case, allowing completion of the second stage after a median of 8.5 days (range, 7-11 days). FLR volume increased by 50-89% following the first stage. Despite successful liver hypertrophy, outcomes remained poor in three patients. One patient died intraoperatively from venous air embolism during the second stage, two died from disease recurrence despite aggressive multimodal treatment, and one remains in complete clinical and radiological remission following ALPPS and adjuvant chemotherapy.
CONCLUSIONS: ALPPS reliably induces rapid FLR hypertrophy and may provide a potentially curative option for carefully selected children when liver transplantation is not feasible. However, its substantial perioperative risk and generally unfavorable oncological outcomes support its role only as a rescue procedure in experienced pediatric hepatobiliary centers. Further multicenter studies are needed to better define indications and patient selection.