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: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: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.

ABSTRACTThere are no standard management protocols for the treatment of bile leak (BL) after liver transplantation. The objective of this study is to describe treatment options for BL after pediatric LT.MethodsRetrospective analysis (January 2010–March 2023). Variables studied: preoperative data, status at diagnosis, and postoperative outcome. Four groups: observation (n = 9), percutaneous transhepatic cholangiography (PTC, n = 38), ERCP (2), and surgery (n = 27).ResultsNine hundred and thirty‐one pediatric liver transplantation (859 LDLT and 72 DDT); 78 (8.3%) patients had BL, all in LDLT. The median (IQR) peritoneal bilirubin (PB) level and fluid‐to‐serum bilirubin ratio (FSBR) at diagnosis was 14.40 mg/dL (8.5–29), and 10.7 (4.1–23.7). Patients who required surgery for treatment underwent the procedure earlier, at a median of 14 days (IQR: 7–19) versus 22 days for PTC (IQR: 15–27, p = 0.002). PB and FSBR were significantly lower in the observation group. In 11 cases, conservative management had resolution of the BL in an average time of 35 days, and 38 patients underwent PTC in a median time of 22 days (15–27). Twenty‐seven (34.6%) patients were reoperated as initial treatment for BL in a median time of 17 days (1–108 days); 25 (33%) patients evolved with biliary stricture, 5 (18.5%) after surgery, and 20 (52.6%) after PTC (p = 0.01).ConclusionPatients with BL who were observed presented significantly lower levels of PB and FSBR versus those who underwent PTC or surgery. Patients treated with PTC presented higher rates of biliary stricture during the follow‐up.

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.

BackgroundThe management of complex, intra- and extrahepatic portal vein thrombosis (PVT) after liver transplantation (LT) is challenging. Although most of the patients remain asymptomatic or oligosymptomatic in the chronic setting, some of them may develop severe portal hypertension and related complications, notably gastrointestinal (GI) bleeding. In the emergency scenario, clinical and endoscopic treatments as well as intensive support constitute the bases of conservative management, while more definitive treatment options such as surgical shunting and retransplantation are related to high morbidity rates. Transjugular intrahepatic portosystemic shunt (TIPS) was largely considered of limited role due to technical difficulties arising from extensive PVT. Recently, however, new minimally invasive image-guided techniques emerged, allowing portal vein recanalization and TIPS creation simultaneously (TIPS-PVR), even in complex PVT pretransplant patients. MethodsHerein, we describe a novel indication for TIPS-PVR in a post-LT adolescent presenting with life-threatening, refractory GI bleeding. ResultsThe patient presented with complete resolution of the hemorrhagic condition after the procedure, with no deterioration of hepatic function or hepatic encephalopathy. Follow-up Doppler ultrasound after TIPS-PVR showed normal hepatopetal venous flow within the stents, and no evidence of complications, including intraperitoneal or peri splenic bleeding. ConclusionsThis report describes the feasibility of TIPS-PVR in the post-LT scenario complicated by extensive PVT. In this case, a complete resolution of the life-threatening GI bleeding was achieved, with no major complications. Other patients with complex chronic PVT might benefit from the use of the described technique, but further studies are required to determine the correct timing and indications of the procedure, eventually before the occurrence of life-threatening complications.

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 COVID-19 infection has received the attention of the scientific community due to its respiratory manifestations and association with evolution to severe acute respiratory syndrome (SARS-CoV-2). There are few studies characterizing SARS-CoV-2 in pediatric immunocompromised patients, such as liver transplanted patients. The aim of this study was to analyze the outcomes of the largest cohort of pediatric liver transplant recipients (PLTR) from a single center in Brazil who were infected with COVID-19 during the pandemic. Methods: Cross-sectional study. Primary outcomes: COVID-19 severity. The Cox regression method was used to determine independent predictors associated with the outcomes. Patients were divided into two groups according to the severity of COVID-19 disease: moderate–severe COVID and asymptomatic–mild COVID. Results: Patients categorized as having moderate–severe COVID-19 were younger (12.6 months vs. 82.1 months, p = 0.03), had a higher prevalence of transplantation from a deceased donor (50% vs. 4.3%, p = 0.02), and had a higher prevalence of COVID infection within 6 months after liver transplantation (LT) (75% vs. 5.7%, p = 0.002). The independent predictor of COVID-19 severity identified in the multivariate analysis was COVID-19 infection <6 months after LT (HR = 0.001, 95% CI = 0.001–0.67, p = 0.03). Conclusion: The time interval of less than 6 months between COVID-19 infection and LT was the only predictor of disease severity in pediatric patients who underwent liver transplantation.

Background & Aims: Published studies suggest that children (<18 years) infected with COVID-19 are less likely to progress to the severe form of disease when compared to adults, however, scarce data are available on morbidity and mortality in immunocompromised children and adolescents. The aim of this study was to evaluate the outcomes of Pediatric Liver Transplant Recipients (PLTR) who were infected with COVID-19. Methods: Prospective longitudinal cohort study. Between 1st March 2020 and 31st October 2021 clinical, laboratory and evolution data from PLTR (<18 years) were collected in person or by remote telemedicine, who tested positive for COVID-19, for presenting symptoms suggestive of COVID-19 infection or positive epidemiological screening or for presenting positive in laboratory screening before procedure – incidental positive. Results: A total of 74 PLTR were diagnosed with COVID-19. The mean age was 8.2 years (SD 4.49) and 51.4% were girls. The prevalent indication for liver transplantation (LT) was biliary atresia 48 (64.8%) and in 69 (93.2%) patients LT was performed with a living donor. The mean time between LT and COVID-19 infection was 60 months (SD 57.7) and 5 (6.7%) patients became infected up to 3 months after LT, 2 in the early postoperative period. Most patients developed mild symptoms (66%) such as fever (25%), cough (20.2%) and runny nose (20.2%). Hospitalization of outpatients was necessary in 6 children (8.1%), all in hospital ward for suspected sepsis (1), respiratory distress (2), periorbital cellulitis requiring parenteral antibiotics (1), vomiting (1) and gum-stomatitis (1) both requiring hydroeletrolitic replacement. Only 1 child, previously admitted to hospital ward, required transfer to the ICU for respiratory support. The main comorbidities were atopic dermatitis in 5 patients (6.7%), asthma in 4 (5.4%), heart disease, neurological diseases and food allergy in 3(4%), none of which was related to the severity of presentation of COVID-19 infection. Baseline immunosuppression was based on tacrolimus, being associated with prednisone and mycophenolate in 28% and 13.7% respectively. Change in immunosuppression was required with mycophenolate discontinuation in 3 (4%) and a decrease tacrolimus level in 1 patient. Most patients had a good recovery, and only 1 child developed Multisystem Inflammatory Syndrome associated with COVID-19 infection. No child died in this study cohort. Conclusion: In pediatric population LT and immunosuppression cannot be considered independently as factors associated with the risk of severity and death due to COVID-19.

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: 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.