BACKGROUND:Hepatic hemangiomas are common benign liver tumors, typically asymptomatic with a low complication rate in liver transplantation (LT). However, post-transplant rupture, though rare, can result in significant morbidity. This report describes the rupture of a small hepatic hemangioma in a liver graft following pediatric living donor liver transplantation (LDLT). CASE REPORT:An 11-month-old boy with biliary atresia underwent LDLT using a left lateral segment from his healthy mother. Preoperative imaging revealed a 2x3 cm hemangioma in Segment III of the donor's liver. Post-transplant, the child experienced significant bleeding 4 h after surgery, leading to hemodynamic instability and a drop in hemoglobin. Re-exploration revealed a large hematoma and rupture at the hemangioma site. Additional resection of the hemangioma and hematoma was performed. The child recovered after a prolonged ICU stay and remains well after 8 years of follow-up. DISCUSSION:Hepatic hemangiomas are generally asymptomatic, but rupture post-transplant can lead to severe complications. There is no standardized approach for managing hemangiomas in liver grafts. This case highlights the need for careful evaluation of lesion size, location, and potential vascular and biliary implications in donor selection. Further research is needed to define optimal management strategies for liver graft hemangiomas. CONCLUSION:While rare, hepatic hemangiomas in liver grafts require careful evaluation, with further studies needed to establish management protocols.

Background The discrepancy in size between donor and recipient presents a complex challenge in pediatric liver transplantation (PLT), often necessitating secondary abdominal closure to prevent abdominal compartment syndrome. The aim of this study is to determine the variables associated with an increased risk of requiring secondary closure in PLT.Methods The retrospective study analyzed all primary liver transplantations performed in patients under 18 years of age from January 2014 to July 2022. The primary endpoint was the risk of secondary abdominal closure. Variables analyzed included pretransplant status, perioperative and postoperative data.Results A total of 664 PLT recipients were identified, of which 58 required secondary abdominal closure (8.7%). Most patients had biliary atresia (n=412, 62.0%), followed by metabolic diseases (n=78, 11.7%). Statistical difference were found in donor gender (p=0.020) and the recipient-to-donor body weight ratio (RDBW), which was lower in the secondary closure group (0.1±0.1 vs. 0.2±0.27; p=0.001), lower in secondary closure. The mean hospital and intensive care unit (ICU) stay after PLT was significantly longer in the intervention group compared to those with primary abdominal closure (24.4±20.4 days vs. 12.5±13.1 days, p<0.001). Multivariable Cox regression analysis identified male donor as an independent risk factor for secondary abdominal closure (hazard ratio 1.9, p=0.030).Conclusions Patients requiring secondary closure were smaller, had a lower RDBW, and received grafts with a higher graft-to-recipient weight ratio (GRWR), Graft size modulation and secondary abdominal closure are currently the techniques used to prevent compartment syndrome in PLT, particularly for children with low body weight.

BACKGROUND:Currently, graft options for pediatric liver transplantation (PLT) include whole (WL) and partial (P) grafts, in the form of either deceased donor transplantation (DD) or living donor liver transplantation (LD). WL transplants from LD are commonly referred to as domino LT. The objective of this manuscript is to compare the outcomes of PLT performed with each of the available graft options. METHODS:Retrospective cohort study from Jan. 2010 to Dec. 2022. The variables included data on the recipients' preoperative clinical status, intraoperative technical aspects, post-operative complications, and survival studies. There were 4 groups: SPLIT (17), DD-WL (55), LD-WL (824), and LD-P (22). RESULTS:The median age and BW of the recipients was smaller in SPLIT, LD-P, and LD-WL compared to DDT-WL groups. HVOO (HR 15.87, 95% CI 1.89-133.06, P = 0.01), retransplantation (HR 7.94, 95% CI 2.63-24.02, P < 0.01), and malignancies (HR 3.08, 95% CI 1.29-7.37, P = 0.01) were independently associated with decreased patient survival. HAT (HR 27.54, 95% CI 10.44-72.68, P < 0.01) and malignancies (HR 2.42, 95% CI 1.10-5.34, P = 0.03) increased the risk of graft loss. The overall survival in this series was 91.4% (mean follow-up of 74.3 months). Patient and graft survival were not different among groups. CONCLUSION:HAT and malignancies were associated with reduced graft survival. Whole liver from living donors with MSUD presented 100% patient survival at 120 months. Even without statistical differences in survival among the studied groups, LD-P and LD-WL recipients presented a trend towards better outcomes. LEVEL OF EVIDENCE:LEVEL III.

Background: The techniques involved in neonatal and infantile transplantation require approaches that can sculpt a left lateral segment (LLS) to the right shape and size and avoid large-for-size syndrome. The aim of this article is to describe the anterior hepatic resection (AHR) of the LLS in pediatric LDLT. Methods: A retrospective anatomical study of preoperative image studies, description of the technique for AHR, and short-term results. Results: The AHR was performed in eight cases. All donors were male, with average age, BW, and BMI of 28.3 +/- 5.9 years, 74.2 +/- 9.3 kg, and 24.3 +/- 2.6 kg/m2, respectively. Donors were discharged at an average of 3.6 +/- 0.8 days. The median recipient age and BW at transplantation were 6.9 (2.7 to 11) months and 5.9 (3.9 to 8) kg, respectively, and the recipient-to-donor body weight ratio (RDBW) was <0.1 in all but one case. The mean percentage reduction in graft weight and in the antero-posterior diameter were 33.2% +/- 5.5% and 38.3% +/- 12.6%, respectively. The average (SD) GRWR was 4.8% +/- 1.7% before all the resections and 3.5% +/- 1.0% after the procedures. Seven patients were primarily closed. Conclusion: After LLS resection, a nonanatomical anterior resection of the LLS was accomplished without hilar vascular dissection to segments II/III. The final liver graft allowed primary abdominal wall closure in all but one patient, with meaningful adjustments in GRWR. AHR proved to be simple, safe, reproducible, and effective in the presented case series.

Background: The mainstays of irresectable hepatoblastoma (HB) treatment are surgical resection and cisplatin based (CB) chemotherapy (CHT). However, adequate patient selection is a key to achieve acceptable disease-free survival in patients with unresectable HB undergoing liver transplantation (LT). Procedure: This single-center retrospective analysis of 28 children with HB submitted to LDLT from 1996 to 2019 aimed at determining the pre-transplant factors associated with worse post-transplant event-free survival. The clinical variables collected were gender, age, PELD score (Pediatric End-Stage Liver Disease scoring system), type of neoadjuvant CHT (CB versus other regimens), pre- and post- CHT AFP levels, %AFP reduction post CHT (AFP pre-CHT – AFP post- CHT /AFP pre- CHT), PRETEXT stage, primary versus rescue LDLT, time between diagnosis and LDLT, presence of metastases at diagnosis, follow-up time. Results: Patients were divided in groups according to the occurrence of the event (recurrence/death) after LDLT – 10 patients in the event-yes and 18 patients in the event-no. Probability of 5-y event-free survival was 63.9%. AFP reduction < 70% (HR=4.33, 95%CI 1.1 to 16.95, p=0.03), and time from diagnosis to LT > 12 months (HR=4.11, 95%CI 1.14 to 14.76, p=0.03) were associated with higher recurrence/death in the Cox regression analysis. Alpha-fetoprotein (AFP) reduction post-CHT > 70% had a good performance in determining disease-free survival, with a calculated AUC of 0.8. Conclusion: LT for HB is the preferred treatment option for unresectable HB, with no distant metastasis and adequate response to CHT. AFP reduction < 70%, and time from diagnosis to LT > 12 months were associated with higher recurrence/death However, due to the limited number of patients in this study, a larger number of patients is required to corroborate these findings.

Background & Aims: One of the challenges in Segmental Liver Transplantation is the reconstruction of Hepatic Vein (HV). The occurrence of Hepatic Vein Outflow Obstruction (HVOO) can result in graft loss. The aim of this study was to describe the outcomes in different Types of HV distribution of Left Lateral Segments (LLS) grafts. Patients & Methods: Children (< 18 years) who underwent a Living Donor Liver Transplantation (LDLT) with LLS grafts during the period from February 2017 to August 2021 with follow-up until February 2022. Retrospective cohort study through data review of medical records and from a prospectively collected data base. The LLS grafts were classified according to the number and distance between HV – Graft Hepatic Vein (GVH) classification, determining the vascular reconstruction performed. Type I: a single orifice; type II: two close orifices – wedge unification; Type IIIa: two separated orifices up to 20 mm distance – venoplasty to achieve a single orifice and Type IIIb (Anomalous Hepatic Vein – AHV): two separated orifices beyond 20 mm distance – Homolog Vein Graft (HVG) interposition. Recipient and Intraoperative variables included age, diagnosis, recipient weight, PELD scores, ascites, Graft-to-Recipient Weight Ratio (GRWR), Graft Hepatic Vein (GHV) diameter, Recipient Hepatic Vein (RHV) diameter, GHV and RHV correlation, Cold Ischemia Time (CIT), Warm Ischemia Time (WIT), need for IVC exclusion during implant, use of PV graft and mesh closure. Post-LT outcomes included the occurrence of HVOO, early (≤ 30 days) portal vein thrombosis (EPVT), late (> 30 days) portal vein thrombosis (LPVT), hepatic artery thrombosis (HAT), and retransplantation. Results: 303 LDLT were performed in which LLS grafts were used. According to the GHV classification, the distribution of the LLS grafts was Type I: 174 (57.42%), Type II: 97 (32.01%), Type IIIa: 25 (8.26%) and Type IIIb: 7 (2.31%). Comparative analysis of recipient and intraoperative variables showed Type IIIb grafts presented a higher proportion of larger LLS and consequently a higher GRWR, as well as a higher mean of GHV, consequently higher GVH/RVH correlation. It was observed a higher CIT average in the LLS that required vascular reconstruction in the bench surgery – Types IIIa and IIIb grafts. There was no statistically significant difference between the LLS graft Types, in the post-transplant outcomes. Up to last follow up no HVOO was observed in this cohort study. The comparative analysis of the cumulative graft survival rate showed no difference according to the LLS graft Type used. Conclusion: The reconstruction of venous drainage plays as essential role in the surgical management of LDLT with LLS grafts. The use of HVG interposition is a good surgical strategy in the use of LLS grafts that have AHV.

Background: infants who require liver transplantation represent a treatment challenge because chronic liver disease at this early age affects the child's growth and development during a critical phase. The aim is to compare demographics, operative data, and long-term outcomes according to recipient weight at the time of LDLT.Methods: This retrospective study included primary LDLT analyzed in 2 groups: BW < 7 kg ( n = 322) and BW > 7 kg ( n = 756). A historical comparison between periods was also investigated.Results: BW < 7 kg had significantly lower height/age and weight/age z-scores, with median PELD score of 19. Transfusion rates were higher in the BW < 7 kg group (30.9 ml/kg versus 15.5 ml/kg, P < 0.001). Higher frequencies of PV complications were seen in the BW < 7 kg cohort. HAT and retransplantation rates were similar. Those with BW < 7 kg required longer ICU and hospital stays. Patient and graft sur-vival were similar. Patient survival in BW < 7 kg was significantly better in the most recent period.Conclusion: Malnutrition and advanced liver disease were more frequent in BW < 7 kg. Despite increased rates of PVT and longer hospital stay, patient and graft long-term survival were similar between groups.(c) 2022 Elsevier Inc. All rights reserved.

Background Acquired diaphragmatic hernia (DH) following liver transplantation (LT) is usually considered a surgical emergency. Interplay of contributing elements determines its occurrence but, in children, LT with partial liver grafts seems to be the most important causative factor. Methods This retrospective study describes the clinical scenario and outcomes of 11 patients with acquired DH following LDLT. Results During the study period, 1109 primary pediatric LDLT were performed (0.8% DH). The median age and BW of the recipients with DH at transplantation were 17 months and 11.1 kg, respectively; 63.7% of the cases had a weight/age Z-score of less than -2 at transplantation. The median interval between transplantation and diagnosis of DH was 114 days (32-538 days). A total of 6 (54.5%) of the patients had bowel obstruction due to bowel migration into the hemithorax. Ten defects were right-sided. Three patients required enterectomy and enterorrhaphy. Two patients required a new bilioenteric anastomosis, and one of them had complete necrosis of the Roux-in-Y limb. The patient with left-side DH presented gastroesophageal perforation. Conclusion Most defects necessitate primary closure as the first treatment, and recurrence is rare. The associated problems encountered, especially related to intestinal complications, can determine increased morbidity following DH repair. Early diagnosis and intervention are required for achieving better outcomes.

Background The impact of the COVID pandemic on liver transplant (LT) programs varied among countries. Few data are available about that impact in pediatric liver transplant (PLT) programs. This study aimed at comparing the data of our program in Brazil (2019 vs. 2020). Methods Retrospective cohort study. Results One hundred and seventy-four PLT were performed in the period (93% living donors). Patients were divided into two groups according to the LT date: pre-COVID-19 period (march/2019-February/2020) and COVID-19 period (March/2020-February 2021). In the pre-COVID-19 period, 97 LTs were performed, and 77 LTs were performed in the COVID-19 period. Patients in the COVID-19 period were younger (10.9 months vs. 16 months, p 0.009), had higher PELD scores (15 vs. 14, p 0.04), more ascites (66.2 vs. 51.5%, p 0.03), and more frequently hospitalized before LT (27.3 vs. 17.5%). However, there was no difference in post-LT complications, retransplantation nor survival rates. Six (6.2%) patients from pre-COVID-19 period were COVID positive at a median of 15.5 months (14-17.5), and 6 (7.8%) patients from COVID-19 period were COVID positive at a median of 3 months (20 days-6 months) from LT. There was neither mortality nor complications in those patients. Four (33%) were hospitalized, and one had prolonged intubation. Four (33%) were asymptomatic, 4 (33%) had upper airways symptoms, and the remaining had gastrointestinal symptoms. Conclusion Overall, PLT was not affected during COVID-19 period. Even though patients from COVID-19 period were sicker, there was no significant impact in LT outcomes. All the recipients who tested positive for COVID had a favorable outcome.

Pediatric living donor liver transplantation (PLDLT) is a successful therapeutic option for children with chronic and acute liver disease. After early transplant results, many technical advancements were introduced in the field to reduce the rate of complications and improve survival. The aim of this study is to present the outcomes of 975 primary PLDLTs in 3 periods: initial practice (period 1, 29 patients, January 1995 to December 1999), second period (period 2, 331 patients, January 2000 to December 2009), and third period (period 3 [P3], 615 patients, January 2010 to September 2019). Among the technical refinements introduced in P3 are the use of hyperreduced left lateral segment grafts, abdominal wall prosthetic mesh closure, double hepatic artery anastomosis, and increased use of vascular grafts for portal vein reconstruction. The outcomes included significant reductions of hepatic artery thrombosis (HAT), early portal vein thrombosis (EPVT), and retransplantation, with better patient and graft survival in P3. Additional analyses showed that the factors independently associated with worse 90‐day patient survival were HAT, EPVT, and increasing Pediatric End‐Stage Liver Disease score. In conclusion, the introduction of technical refinements in P3, in addition to improvements in patient care, determined a reduction in EPVT, HAT, and retransplantation. Consequently, patient and graft survival rates increased in all time points studied.