Over the past 34 years, a single transplant team in São Paulo, Brazil, has performed 1560 pediatric liver transplants (PLT)-including 1352 LDLT, 179 DDT, and 29 domino procedures using donors with maple syrup urine disease-achieving outstanding long-term outcomes. In our most recent cohort of 500 PLT (2018-2024), 1- and 5-year patient survival rates were 96.7% and 94.8%, respectively. From 2015 to 2024, our team performed 35.1% of all PLT in Brazil and 45.5% of those in children under 5 years of age, with 98.1% of the latter using LDLT. This milestone highlights not only clinical achievement but also the persistent structural challenges facing PLT in low- and middle-income countries. We outline some key barriers to sustainable and equitable PLT development in Brazil, including underfunding, geographic disparities, lack of outcome transparency, bureaucratic delays, and gaps in transition to adult care. A set of guiding principles is proposed to support national progress and inform similar efforts elsewhere. Our center's experience demonstrates that excellence in PLT is achievable in resource-constrained settings, but long-term success depends on institutional commitment, strategic investment, and national coordination to ensure equitable access for all children.

BACKGROUND:Endovascular management of portal vein thrombosis (PVT) is challenging. Transsplenic access (TSA) is growing as an access option to the portal system but with higher rates of bleeding complications. The aim of this article is to evaluate the efficacy and safety of transsplenic portal vein recanalization (PVR) using a metallic stent after pediatric liver transplantation. MATERIALS AND METHODS:This is a retrospective review of 15 patients with chronic PVT who underwent PVR via TSA between February 2016 and December 2020. Two children who had undergone catheterization of a mesenteric vein tributary by minilaparotomy were excluded from the patency analysis but included in the splenic access analysis. The technical and clinical success of PVR and complications related to the procedure via TSA were evaluated. RESULTS:Thirteen children with PVT were treated primarily using the TSA. The mean age was 4.1 years (range, 1.5-13.7 years), and the most common clinical presentation was hypersplenism (60%). Technically successful PVR was performed in 11/13 (84.6%) children, and clinical success was achieved in 9/11 (81.8%) children. No major complications were observed, and one child presented moderate pain in the TSA (from a total of 17 TSA). The median follow-up was 48.2 months. The median primary patency was 9.9 months. Primary patency in the first 4 years was 75%, and primary assisted patency was 100% in the follow-up period. CONCLUSIONS:Transsplenic PVR is a safe and effective method for the treatment of PVT after pediatric liver transplantation.

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.

Left lateral segment grafts have become a suitable option in pediatric liver transplantation (PLT). The correlation between hepatic vein (HV) reconstruction and outcome is relevant when assessing the safe use of these grafts. We retrospectively reviewed the medical records prospectively collected from a pediatric living donor liver transplantation database and conducted a comparative analysis of the different left lateral segment graft types according to HV reconstruction. Donor, recipient, and intraoperative variables were analyzed. Post-transplant outcomes included vascular complications such as hepatic vein outflow obstruction, early (≤30 d) and late (>30 d) PVT, hepatic artery thrombosis, and graft survival. From February 2017 to August 2021, 303 PLTs were performed. According to venous anatomy, the distribution of the left lateral segment was as follows: single HV (type I) in 174 (57.4%), close HVs, simple venoplasty for reconstruction (type II) in 97 (32.01%), anomalous hepatic vein (AHV) with a distance between the HVs orifices that allowed simple venoplasty (type IIIA) in 25 (8.26%) and AHV with a distance between the HVs orifices requiring homologous venous graft interposition (type IIIB) in 07 (2.31%) grafts. Type IIIB grafts came from male donors ( p =0.04) and had a higher mean donor height ( p =0.008), a higher mean graft weight, and a higher graft-to-recipient weight ratio, both p =0.002. The median follow-up time was 41.4 months. The overall cumulative graft survival was 96.3%, and comparative graft survival showed no difference (log-rank p =0.61). No hepatic vein outflow obstructions were observed in this cohort study. There was no statistically significant difference in the post-transplant outcomes between the graft types. The venous reconstruction of the AHV with homologous venous graft interposition had similar outcomes in the short and long term.

In biliary atresia (BA), efforts to prevent premature liver transplantation (LT) are aimed at early diagnosis, timing of Kasai-portoenterostomy (KPE), and centralization of care. This report presents the clinical picture, treatment strategies, and outcomes of BA patients with no previous treatment. A retrospective cohort study (Jan/2001 to Jan/2021) was conducted to evaluate the outcome of patients with BA referred to a single team. Study groups were: 1) Kasai-only group (K-only) n=9), 2) LT-only group (n=7), and 3) Kasai+LT group (K+LT) (n=23). Survival with native liver and overall survival were 22.9 and 94.8%, respectively, at 120 months of follow-up. There was no difference in age at KPE in the K-only group (46.8±21.8 days) vs K+LT (52.1±22 days), P=0.4. Ten (25.6%) patients were babies conceived through in vitro fertilization (IVF). Four IVF patients (40%) presented associated congenital heart disease vs 5 patients (17%) in the remaining group (P=0.14). Two of the IVF patients were premature (<37 weeks). Median maternal age at birth was 35 years (33 to 41 years). Excellent patient survival is expected for patients with BA with the available treatment strategies. IVF+BA was an unexpected prevalent association in this cohort, and further studies are required to better understand these findings.

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: In patients with Biliary Atresia (BA), the efforts at avoiding premature liver transplantation (LT) target early identification and diagnosis, timing of Kasai-portoenterostomy (KPE) before 45 days of age, and centralization of surgery at experienced centers. However, a large percentage of infants still require LT in the first 2 years of life, and many referals to liver transplant centers are still late. Aim: The aim of this study was to analyze the outcomes of all patients with BA, with no previous treatment, referred to a single team, on an intent-to-treat basis. Methods: Retrospective study in patients with no previous treatment refferred to our center from Jan/2001 to Jan/2021. All the patient’s care, which included diagnosis of BA, portoenterostomy (PE), clinical follow-up, transplantation, and post-transplant follow-up was provided by the same pediatric hepatology and transplant team throughout the years. All patients included in this study had BA. They were evaluated according to their age (days) at first assessment, clinical presentation (jaundice, coluria, acolic stools), physical examination (size of the liver and spleen, presence of ascites), laboratory values (liver function tests, infectious workup, serum protein electrophoresis), image studies (total abdominal ultrasound and hepatobiliary scintigraphy - DISIDA Scan), liver biopsy, and intraoperative cholangiography when necessary. The patients were divided in three groups according to the initial assessment: LT only, Kasai portoenterostomy (KPE) only, and KPE + LT group. Results: Thirty-nine patients with biliary atresia were followed from an early age. Seven patients did not undergo Kasai-PE, and received liver grafts at various time points (LT-only Group). Thirty-two children underwent KPE. Nine of them composed the Kasai only Group: 8 patients survived with their native liver at a median time of 5 years (range 2y to 11y), but one patient in this group died during the follow-up. Twenty-three patients were transplanted after the PE (Kasai + LT Group). Median follow-up in this group was 11 years (range 2 to 20). The overall patient survival of the entire cohort was 94.8%, and the survival with native liver was 28.1%. Interstingly, 10 patients in this series (25.6%) were babies conceived through in vitro fertilization (IVF), distributed proportionaly in the observed groups. Conclusion: This abstract shows that when all the treatment options are at hand and timely offered to patients with BA, the expected long-term results are excellent (95% patient survival). A interesting finding in this study, which has not been reported elsewhere, was the 25% association of BA and IVF.

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 & Aims. The most dangerous complications of hepatocellular adenomas are hemorrhage and malignant transformation, both of which require surgical treatment. The surgical treatment strategy for patients with benign large or central tumors is challenging because complex liver resections are required. The strategy of using a live donor as a backup procedure is described in this series. Methods. We present a series of three patients with large hepatocellular adenoma lesions showing a central location, for which the living donor liver transplantation strategy was used as a backup procedure. Results. Hepatocellular adenoma was confirmed by biopsy in all patients. Surgical resection was indicated because of the patients’ symptoms and lesion size and growth. All patients had a lesion that was central or in close contact with major vessels. The final decision to proceed with the resection was made intraoperatively. A live donor was prepared for all three patients. Two patients underwent portal vein embolization associated with extended hepatectomy, and a total hepatectomy plus liver transplantation with a living donor was performed in one patient. All patients had good postoperative outcomes. Conclusions. In the treatment of hepatocellular adenomas for which complex resections are necessary and resectability can only be confirmed intraoperatively, surgical safety can be improved through the use of a living donor backup. Center expertise with living donor liver transplantation is paramount for the success of this approach.

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.

Introduction: Intestinal transplantation remains the treatment of choice for patients with irreversible intestinal failure (IIF) and severe complications of parenteral nutrition. We describe the first case of a living donor intestinal transplantation (LDIT) performed in Brazil. This strategy was used after the clinical condition of the patient deteriorated while on the waiting list for a deceased donor. Methods: Case report, highlighting the planning and execution of the technique of LDIT. Result: A 3-year-old patient with IIF due to intestinal atresia was referred for intestinal transplantation due to progressive loss of venous access sites, with worsening of the venous thrombosis during the waiting time on the list for a deceased donor. After 8 months on the list, a LDIT was performed: the donor was her mother, and a 160 cm length small bowel graft was harvested. Total ischemia time was 62 minutes, and the graft was transplanted using the recipient’s aorta and inferior vena cava for vascular anastomosis. On postoperative day (POD) 15, the patient presented a severe acute cellular rejection (ACR) resistant to corticosteroids and thymoglobulin, successfully treated with alemtuzumab. She was discharged home on POD 80, totally free of parenteral nutrition or any intravenous fluid/electrolyte supplementation. The patient remained stable with a totally functioning graft during all the follow-up period; 11 months after LDIT, she was diagnosed with a central nervous system lymphoma, and died after treatment failure, 17 months after LDIT. Conclusion: LDIT is a viable and safe strategy for the treatment of children with IIF, especially in the scenario of the shortage of pediatric organ donors. Possible immunological advantages using a graft obtained from a living donor are uncertain.

The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health‐related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO2 < 50 mm Hg and those with PaO2 ≥ 50 mm Hg.

EBV-associated post-transplant smooth muscle tumors (EBV-PTSMTs) is a rare complication following solid organ transplantation and has been observed more frequently in pediatric and young adult populations. This manuscript reports a 10-month-old female child who developed multicentric EBV-PTSMTs in a liver graft and in the distal ileum 2 years after living donor liver transplantation. The patient was successfully treated with resection and radiofrequency ablation of a liver nodule in a central position in the graft parenchyma.

The last decades have witnessed a significant improvement in the field of pediatric liver transplantation (LT), resulting in longer patient and graft survival; adequate graft selection, surgical refinement, the use of live donors and optimal postoperative care are among the reasons why pediatric recipients are living longer. With this new condition, pediatric recipients are now more exposed to the deleterious effects of immunosuppression, including metabolic, infectious and neoplastic complications, nephrotoxicity and neurotoxicity. Due to all those particularities, the approach to avoid overimmunosuppression or underimmunosuppression may be more difficult in children than in adult recipients. Moreover, pediatric recipients are exposed to growth issues and specific problems during adolescence, like nonadherence to immunosuppressive therapy. This article highlights the current immunosuppressive strategies for pediatric liver transplant recipients.