Incidence, management and outcomes in hepatic artery complications after paediatric liver transplantation: protocol of the retrospective, international, multicentre HEPATIC Registry.

INTRODUCTION: Hepatic artery complications (HACs), such as a thrombosis or stenosis, are serious causes of morbidity and mortality after paediatric liver transplantation (LT). This study will investigate the incidence, current management practices and outcomes in paediatric patients with HAC after LT, including early and late complications. METHODS AND ANALYSIS: The HEPatic Artery stenosis and Thrombosis after liver transplantation In Children (HEPATIC) Registry is an international, retrospective, multicentre, observational study. Any paediatric patient diagnosed with HAC and treated for HAC (at age <18 years) after paediatric LT within a 20-year time period will be included. The primary outcomes are graft and patient survivals. The secondary outcomes are technical success of the intervention, primary and secondary patency after HAC intervention, intraprocedural and postprocedural complications, description of current management practices, and incidence of HAC. ETHICS AND DISSEMINATION: All participating sites will obtain local ethical approval and (waiver of) informed consent following the regulations on the conduct of observational clinical studies. The results will be disseminated through scientific presentations at conferences and through publication in peer-reviewed journals. TRIAL REGISTRATION NUMBER: The HEPATIC registry is registered at the ClinicalTrials.gov website; Registry Identifier: NCT05818644.

Surgical outcomes and quality of life following exercise-based prehabilitation for hepato-pancreatico-biliary surgery: A systematic review and meta-analysis.

BACKGROUND: Hepato-pancreatico-biliary (HPB) patients experience significant risk of preoperative frailty. Studies assessing preventative prehabilitation in HPB populations are limited. This systematic review and meta-analysis evaluates outcomes for HPB patients treated with exercise prehabilitation. DATA SOURCES: A comprehensive search of MEDLINE (via Ovid), Embase (Ovid), Scopus, Web of Science Core Collection, Cochrane Library (Wiley), ProQuest Dissertations, Theses Global, and Google Scholar was conducted with review and extraction following PRISMA guidelines. Included studies evaluated more than 5 adult HPB patients undergoing ≥ 7-day exercise prehabilitation. The primary outcome was postoperative length of stay (LOS); secondary outcomes included complications, mortality, physical performance, and quality of life. RESULTS: We evaluated 1778 titles and abstracts and selected 6 (randomized controlled trial, n = 3; prospective cohort, n = 1; retrospective cohort, n = 2) that included 957 patients. Of those, 536 patients (56.0%) underwent exercise prehabilitation and 421 (44.0%) received standard care. Patients in both groups were similar with regards to important demographic factors. Prehabilitation was associated with a 5.20-day LOS reduction (P = 0.03); when outliers were removed, LOS reduction decreased to 1.85 days and was non-statistically significant (P = 0.34). Postoperative complications (OR = 0.70; 95% CI: 0.39 to 1.26; P = 0.23), major complications (OR = 0.83; 95% CI: 0.60 to 1.14; P = 0.24), and mortality (OR = 0.67; 95% CI: 0.17 to 2.70; P = 0.57) were similar. Prehabilitation was associated with improved strength, cardiopulmonary function, quality of life, and alleviated sarcopenia. CONCLUSIONS: Exercise prehabilitation may reduce LOS and morbidity following HPB surgery. Studies with well-defined exercise regimens are needed to optimize exercise prehabilitation outcomes.

Liver Transplantation in Locally Unresectable, Undifferentiated Embryonal Cell Sarcoma.

BACKGROUND: Undifferentiated embryonal cell sarcoma (UESL) of the liver is the third most common malignant liver disease of childhood presenting as a rapidly enlarging intraabdominal mass. This systematic review explores the practicality of liver transplantation as a viable option in the treatment armamentarium for locally advanced undifferentiated embryonal cell sarcoma. METHODS: A systematic review of the literature was performed using Medline and Embase, from inception of databases to December 31, 2018. Keywords and MeSH headings used were embryonal sarcoma, mesenchymal sarcoma, and liver transplant. Reviews and manuscripts with incomplete data were excluded. RESULTS: Twenty-eight patients had orthotopic liver transplantation (OLT) as a curative treatment option. The median age at presentation was 8 and 27 years in the pediatric and adult population, respectively, with a similar male to female ratio. A majority of the patients presented with abdominal pain, palpable mass, and a normal alpha-feto-protein. The median tumor size was 15 cm mainly affecting the right lobe (62%) of the liver. Eighty-two percent of the patients underwent primary OLT and 5 patients had salvage OLT. One death (3.6%) was due to initial misdiagnosis and management for hepatoblastoma. Recurrence was noted in 7.1% of the population. The median follow-up was noted to be 28.5 months. The documented survival rate post-liver transplant for UESL was 96%. CONCLUSIONS: Based on available data and the very positive results therein, liver transplantation is a practical and justifiable use of a scarce resource as a treatment option for locally unresectable, undifferentiated embryonal cell sarcoma. The authors propose (accepting existence of different proposals) neoadjuvant therapy before curative resection, and if not achievable, then liver transplantation followed by adjuvant chemotherapy is an option for suitable candidates. For recurrent tumors after surgical resection, adjuvant therapy with salvage liver transplantation is an option.