Living Donor Whole and Partial Liver Grafts, Deceased Donor Whole Liver and SPLIT: Outcome Comparison.

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.

Anterior hepatic resection: A simple and safe technique for reducing the antero-posterior diameter of the liver graft for small pediatric recipients.

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.

Liver transplantation for hilar inflammatory myofibroblastic tumor: Case report and review of the literature.

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.

The impact of low recipient weight [≤ 7kg] on long-term outcomes in 1078 pediatric living donor liver transplantations.

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 survival 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.

Living Donor Liver Transplantation as a Backup Procedure: Treatment Strategy for Hepatocellular Adenomas Requiring Complex Resections.

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.

Intestinal complications are common in patients with acquired diaphragmatic hernia after pediatric living donor liver transplantation.

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.

Pediatric liver transplantation activity in a high-volume program during the COVID-19 pandemic in Brazil.

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.

Impact of hypoxemia on pediatric liver transplantation for hepatopulmonary syndrome.

BACKGROUND: 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. METHODS: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO2  ≥ 50 mm Hg, 11 patients, and G2-PaO2  < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups. RESULTS: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22). CONCLUSION: The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O2 weaning time than those with mild, moderate, or severe HPS.

Technical Choices in Pediatric Living Donor Liver Transplantation: The Path to Reduce Vascular Complications and Improve Survival.

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.

Current Practice in Immunosuppression in Pediatric Liver Transplantation.

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.

Domino Liver Transplant in Maple Syrup Urine Disease: Technical Details of Cases in Which the First Surgery Involved a Living Donor.

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.

Pancreas-preserving duodenectomy after living donor liver transplantation for invasive cytomegalovirus disease.

CMV infection plays an important role in the postoperative course following solid organ transplantation. We present the case of an 11-year-old male patient who underwent LDLT due to severe hepatopulmonary syndrome and biliary cirrhosis. Four weeks after LDLT, he developed persistent GI bleeding and was subjected to repeated endoscopic treatment and radiological arterial embolization to stop the bleeding from duodenal ulcers. Diagnostic workup was negative for CMV disease. Because the bleeding persisted, surgical treatment was indicated, and a pancreas-preserving duodenectomy was performed. Immunohistochemical staining of the surgical specimen demonstrated diffuse endothelial infiltration by CMV. Despite ganciclovir treatment, the patient developed new erosions in the jejunal mucosa and melena; ganciclovir was discontinued, and foscarnet was started, resulting in clinical improvement and the cessation of bleeding. This case highlights the technical aspects of performing a complex upper GI resection in a patient recently subjected to LDLT, taking care to avoid injury to the previous liver graft anastomosis and restore GI continuity. Moreover, CMV tissue-invasive disease compartmentalized in the GI tract may be difficult to diagnose, as indicated by the negative results of antigenemia and PCR assays and endoscopic superficial mucosal biopsies.

Living donor liver transplantation for neonatal fulminant hepatitis due to herpes simplex virus infection.

Although rare, ALF caused by disseminated HSV infection is associated with high mortality in the neonatal population. This condition is often diagnosed relatively late due to the absence of specific signs. We present a case involving a neonate with ALF submitted to living donor liver transplantation without a prior diagnosis. The patient had no skin or mucosal lesions, and IgM serology was negative for HSV-1 and HSV-2. Immunohistochemical staining of the liver explant was positive for herpes virus infection, and the patient subsequently received antiviral drug treatment, with a good outcome. Due to organ shortages and the rarity of the aforementioned condition, LT has seldom been reported for the treatment of ALF caused by herpes virus infection; however, LT may be the only option for neonates with fulminant hepatitis. The use of living donors in an urgent scenario is well established in Eastern countries and safely applicable for pediatric patients with ALF.

Simultaneous or sequential gastrectomy in pediatric liver transplant recipients.

The association between LT and gastrectomy is not common. Only two studies reported the gastrectomy/LT association in children. Here, we report three children who underwent LT who required a concomitant or sequential gastrectomy for different reasons. Patient 1, a 16-yr-old boy, during the LT, underwent a partial gastrectomy due to extensive injury to the duodenum. He had a previous and unusual portoenterostomy performed in the duodenum. Bowel reconstruction was performed using an intestinal loop that was first used for the bilio-enteric anastomosis and then connected to the gastric stump. Patient 2, a 22-month-old female child, underwent a partial gastrectomy with a Roux-en-Y reconstruction during a retransplantation. She had a large perforated gastric ulcer blocked by the allograft liver. Patient 3, a 26-month-old male child, five yr after living donor LT, was submitted to a partial gastrectomy because of gastric outlet obstruction. The histopathology was compatible with eosinophilic gastritis. The association between LT and gastrectomy in the pediatric population is extremely rare. Appropriate knowledge of the previous transplantation technique is very important. Further studies are required to assess the outcomes of the different types of gastric reconstruction in pediatric recipients.