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There 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. METHODS: Retrospective 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). RESULTS: Nine 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). CONCLUSION: Patients 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.

Assuntos

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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.

Assuntos

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BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) of the liver are rare neoplasms. These tumors are difficult to distinguish from other neoplasms by radiological examination, have uncertain evolution, and there is no consensus on the treatment of these lesions. Hilar tumors can involve the portal vein, hepatic artery, bile duct, and spread to the branches of the portal triad, causing obstructive symptoms, occlusive phlebitis, and portal hypertension. Thus, liver transplantation (LT) is an excellent alternative for locally advanced hilar tumors, since it ensures complete tumor resection with free margins. METHODS/RESULTS: We present a literature review and a case report showing a 3-year-old boy with liver IMT invading the hepatic hilum and inferior vena cava, who underwent a successful living donor liver transplantation that required portal vein and vena cava replacement. CONCLUSION: The incidence of hilar IMTs is low and there is no well-established standard treatment. Liver transplantation for a hilar tumor with vascular invasion was acceptable in this case because the radically of the surgery was the key for the cure, and because the tumor was benign in nature.

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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.

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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.

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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.

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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.

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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.

Assuntos

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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.

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BACKGROUND: Data describing the technical aspects of living donor (LD) domino liver transplantation (DLT) in maple syrup urine disease (MSUD) are limited. The largest published series includes only 3 cases. One great challenge of this procedure is to ensure adequate vascular stumps for the LD, the MSUD patient, and the recipient of the domino graft. Here, we describe our experience in 11 cases of LD-DLT in MSUD, highlighting the technical aspects of LD-DLT. METHODS: From September 2012 to September 2017, 11 patients with MSUD underwent LD liver transplantation at our institution, and MSUD livers were used as domino grafts in 11 children. RESULTS: (1) MSUD patients: 10 patients received a left lateral segment. The donor's left hepatic vein (HV) was anastomosed to the confluence of the recipient's 3 HVs. No vascular grafts (VG) were required for portal vein (PV) anastomosis. Single arterial anastomosis was performed with microsurgery in 10 of 11 patients. (2) MSUD graft recipients: In 8 cases, HV reconstruction was performed between the graft's HV confluence and the recipient's HV confluence, and in 3 cases, a vena cava triangulation was necessary; 6 MSUD grafts required HV venoplasty. No VG were needed for HV reconstruction. VG were used for PV reconstruction in 3 cases due to sclerotic PV. In 2 cases, double arterial anastomoses were performed in the MSUD liver. All patients remain alive and well. CONCLUSIONS: Living donor liver transplantation followed by DLT for MSUD is a complex procedure and demands technical refinement. Special attention must be paid to vascular reconstruction.

Assuntos