Pediatric organ transplantation has evolved significantly since the 1950s, with breakthroughs in immunosuppressive therapy in the 1980s. Worldwide, around 2,700 pediatric liver transplants are performed annually, with living donor transplants dominating in Japan due to cultural and legal limitations surrounding brain-dead organ donation. Regional disparities in transplant practices are evident, with brain-dead transplants being more common in North America and Europe, while living donor transplants prevail in Asia and the Middle East. Technological innovations such as machine perfusion and minimally invasive surgery have enhanced transplant outcomes and addressed the ongoing donor shortage. Despite excellent long-term survival rates, challenges persist, particularly with graft fibrosis, which affects over 60% of pediatric recipients within a decade post-transplant. A critical issue is the transition of pediatric transplant patients to adult care facilities, where nonadherence with immunosuppressive regimens is associated with a high mortality rate. As the number of pediatric transplant cases rises, the need for increased human and financial support becomes more pressing to ensure these patients receive comprehensive care throughout their lives.

An uncommon and aggressive malignancy, perihilar cholangiocarcinoma, may arise in the bile ducts at the intersection of the right and left hepatic ducts. The implementation of surgical resection and transplantation has greatly enhanced the management of the condition, resulting in improved survival rates and enhanced quality of life for patients. Nevertheless, there are still obstacles to overcome, such as restricted therapeutic alternatives for later phases, possible onset of complications, and the scarcity of donor organs. For early-stage illness, surgical resection, typically a right hepatectomy, is the recommended treatment, whereas transplantation is specified for instances that cannot be resected. Liver transplantation provides extended longevity for some patients, but it necessitates lifelong immunosuppression and entails the possibility of recurrence. The choice between resection and transplantation is contingent upon several circumstances, including the stage of the disease, the health of the patient, and the availability of a living liver donor. For patients diagnosed with perihilar cholangiocarcinoma, adopting an interdisciplinary strategy is essential to maximizing treatment success.

Background: This investigation delves into the intricacies of graft-versus-host disease (GVHD) in the context of liver transplantation (LT), focusing on the experiences from a Korean single center. Despite GVHD’s relatively low incidence, its severe implications on patient mortality underscore the urgent need for advanced management and comprehension strategies. Methods: In a retrospective analysis at Severance Hospital, Korea, we reviewed 1,107 adult LT recipients from January 2009 to March 2023, excluding those who succumbed within 14 days post-transplantation, to scrutinize the manifestation, treatment, and outcomes of GVHD. Diagnostic approaches ranged from skin to colonoscopic biopsies, with interventions including high-dose corticosteroids and tailored immunosuppressive adjustments. Results: GVHD was diagnosed in 1.3% of the study cohort, predominantly identified through skin biopsies. Critical findings include the significant role of donor liver characteristics and recipient pre-transplant conditions in GVHD development. Notably, GVHD affected patients exhibited markedly lower survival rates at one year compared to their non-GVHD controls (21.4% vs. 90.2%, p<0.001), with deceased donor liver transplants and human leukocyte antigen one-way mismatches between donor and recipient identified as significant GVHD risk factors. Conclusion: This study reaffirms the severe impact of GVHD on post-LT patient survival and highlights specific risk factors associated with its development. Enhanced understanding and targeted management of these risk factors are crucial for improving outcomes for LT recipients facing this complex complication.

Background: Emergency pediatric living donor liver transplantation (LDLT) is vital for acute liver failure patients in life-threatening situations. However, we do not know the outcomes of emergency pediatric ABO-incompatible (ABOi)-LDLT, which is an alternative treatment for patients without ABO-compatible (ABOc) living liver donors. The purpose of our study is to compare the outcomes between emergency pediatric ABOi-LDLT and emergency pediatric ABOc-LDLT using data from the Korean Network for Organ Sharing (KONOS). Methods: We analyzed retrospective KONOS data for consecutive pediatric emergency LDLT patients between 2017 and 2021 in Korea. Results: The incidence of ABOc-LDLT and ABOi-LDLT was 83% (n=44) and 17% (n=9), respectively. The baseline, pre-transplant care, postoperative complications, and infectious complications of ABOi-LDLT did not differ from those of ABOc-LDLT. Graft survival and overall survival at 5 years in ABOi-LDLT were 100% and 100%, respectively, which was better than in ABOc-LDLT, but the survival difference between the two groups was not significant. No developed acute rejection in ABOi-LDLT patients. No factors in the multivariate analysis were related to patient mortality or graft failure. Neither graft failure nor death was associated with ABOi-LDLT. Conclusion: This study concludes that emergency pediatric ABOi-LDLT is safe and feasible for use in ALF patients as well as other urgent instances.

Background: Challenging clinical circumstances and high demand for liver transplantation have led to a refinement in the recipient selection criteria. This study aims to investigate the hypothesis that surgical outcomes in living donor liver transplantation (LDLT) for hepatocellular carcinoma (HCC) have improved over time with the shift from morphological to biological criteria. Methods: A retrospective analysis was conducted on 942 adult HCC patients underwent LDLT at Seoul National University Hospital between 2000 and 2022. Study populations were divided into Group A (2000.01.01-2011.06.30, n=314) and Group B (2011.07.01-2022.06.30, n=628). Baseline characteristics, perioperative factors, and survival outcomes were compared. Results: Group B demonstrated higher recurrence-free survival (RFS) compared to Group A (p=0.03). Additionally, Group B exhibited superior overall survival rates at the 1-, 3-, and 5-year intervals (95.9%, 87.9%, 84.6%, p<0.01). Moreover, Group B had a significantly lower recurrence rate (p=0.02) and mortality rate (p<0.01). The median time to recurrence was 9.1 months (interquartile range [IQR] 3.9-21.8) for Group A and 11.4 months (IQR 6.6-18.5) for Group B (p=0.92). Furthermore, Group A's median tumor-bearing survival was 12.3 months (IQR 5.2-26.1), which was significantly shorter than Group B's 20.0 months (IQR 5.4-25.7) (p<0.01). Conclusion: The use of biological tumor markers in patient selection criteria has significantly improved the effectiveness of HCC treatment in LDLT and should be encouraged for pervasive use.

Background: The use of small grafts, defined by a graft-to-recipient weight ratio (GRWR) less than 0.8, is possibly associated with an increased risk of graft loss in living donor liver transplantation (LDLT). This study aims to validate risk factors for graft loss in LDLT with GRWR<0.8 using single-center data. Methods: LDLT recipients, who received GRWR<0.8 graft at Severance Hospital, between July 2007 and December 2022, were categorized based on the number of risk factors identified in previous Korean multicentric study: recipient age ≥60 years, model for end-stage liver disease (MELD) score ≥15, and male donor. Baseline characteristics and graft survival were compared among these groups. Results: The median GRWR was 0.74 (interquartile range 0.69-0.78) and minimum was 0.49. Recipients with more risk factors exhibited lower graft survival rates: 100% at 5 years in the Risk 0 group (n=18), 72.7% in the Risk 1 group (n=20), and 54.5% in the Risk≥2 group (n=18, p=0.015). This trend was similar in subgroups of right lobe graft and the others (left lobe plus right posterior lobe), although not statistically significant. Donor age did not significantly affect graft survival in GRWR<0.8 transplants (78.9% for donor age≥45 vs. 69.2% for donor age<45, p=0.25). Conclusion: This study confirms that the number of risk factors, including recipient age, MELD score, and donor sex, significantly impacts graft survival in LDLT with GRWR<0.8. These findings highlight the need for careful recipient and donor selection to improve outcomes in LDLT.

Background: As the prevalence of obesity and nonalcoholic fatty liver disease increases in recent years, the number of living donor liver transplantation from donor with high body mass index (BMI) is increasing. However, data on the effect of high BMI on outcomes in liver donors are lacking. This study aimed to investigate the influence of donor obesity (BMI ≥30 kg/m²) on and clinical outcomes after donor right liver resection (DRLR). Methods: Records of all living donors who underwent DRLR between January 2015 and December 2020 at Asan Medical Center were retrospectively reviewed. We divided donors into obese (BMI ≥30 kg/m², n=41) and normal-weight (BMI ≤25 kg/m², n=1,125) groups. To eliminate bias, matching was performed using the greedy method, and 34 donors involved in this study in each group. Results: After matching, preoperatively, the obese group had significantly higher preoperative levels of serum aspartate aminotransferase (p=0.021), alanine aminotransferase (p=0.003), and a larger celiac artery depth ratio (p<0.001) and graft volume (p<0.001) than the normal-weight group. When comparing perioperative outcomes of donor, the operation time was longer in the obese group (p=0.019), but no difference was observed in postoperative major complication rates (p=0.314), lengths of hospital stay (p=0.607), and readmission rate (p>0.999). Furthermore, both groups had similar major surgical outcomes of recipients including biliary (p=0.163) complications and lengths of hospital stay (p=0.445). Conclusion: DRLR may be safely performed in a select group of high BMI donors with ≥30 kg/m² without uncontrolled metabolic disease and significant liver steatosis in the absence of other suitable living donors.

Backgrounds: Alcohol-related liver disease (ALD) is currently a major indication for liver transplantation (LT) in South Korea. Alcoholic recidivism is a primary concern after LT for ALD. However, it is unclear how patients who have received LT for ALD are managed regarding medical and psychological issues. Furthermore, management approaches vary across centers. This study aims to investigate how these management approaches differ among centers. Methods: We conducted a survey of 19 liver transplant centers in South Korea to gather detailed information on the management protocols and related issues of ALD in LT patients. Results: All 19 centers that responded to the survey had been performing LT for patients with ALD for more than 5 years. Nine out of the 19 centers (47%) stated that a minimum abstinence period of 1-6 months from alcohol was required before evaluating LT and enrolling on the transplant waiting list. Only 2 (10%) and 5 (26%) centers had protocols for assessing alcohol consumption while awaiting LT or for treating alcohol consumption after transplantation, respectively. Monitoring abstinence from drinking before and after LT mostly relied on direct interviews, and alcohol biomarkers were not used. Conclusion: Our findings emphasize the importance of consistent patient management guidelines for ALD-LTs in South Korea.

Background: Concurrently extrahepatic malignancy (EHM) has long been considered a relative contraindication to liver transplantation because of cancer recurrence. However, we were frequently challenged as living donor liver transplantation (LDLT) may be the only life-saving option available in the setting of end-stage liver disease, or hepatocellular carcinoma (HCC) with concurrent EHM. In this study, we aim to analyze the outcome of adult LDLT with concurrent EHM at the time of LDLT. Methods: Of 2,448 adults who underwent LDLT from May 1996 to January 2023 at our institution, we retrospectively analyzed data for 16 patients with an EHM treated within 6 months at the time of LDLT. Results: Among 16 patients, one patient died of postoperative liver failure, and another died 3 months post-surgery due to bowel perforation. The cumulative overall survival rates at 1-year, 3-year, and 5-year were 87.5%, 78.8%, and 68.9%, respectively. Five patients died during follow-up; only one patient died due to a cancer-related cause. None of the eight patients with low-risk EHM showed EHM recurrence after LDLT. EHM recurrence occurred in one patient with intermediate risk, and cancer progression was seen in one patient with high-risk EHM. Concurrent HCC was present in six patients, and HCC recurrence occurred in two (33.33%). There was no statistically significant difference in survival between patients with hematologic (n=5) and non-hematologic (n=11) EHM (p=0.891). Conclusion: Our study shows a high survival rate for LDLT in patients with concurrently EHM. Hence, we suggest that concurrent EHM should not be a contraindication to LDLT even when 'minimum remission times' have not yet elapsed.

Central pontine myelinolysis (CPM) is a severe neurological complication observed after liver transplantation (LT), with a mortality rate exceeding 50%. This study presents a case report of a 43-year-old female patient with alcoholic liver cirrhosis who underwent liver transplantation and subsequently developed CPM. The patient admitted with symptoms and signs of acute on chronic liver failure, such as hyponatremia, and hepatic encephalopathy Grade 3 Glasgow Coma Scale (GCS) 10. Sodium benzoate was started together with lactulose. Ammonia level was decreased and mentality was also improved. However, sodium level was increased up to 160 mmol/L. After liver transplantation, the patient become drowsier (GCS 6-9) even with good liver function. The brain magnetic resonance imaging at the post-LT 5th day showed CPM. The mentality was slowly improved after conservative management with low level of tacrolimus. This case gives a lesson that pre-operative sodium benzoate treatment can increase the risk of CPM by increasing sodium level in patients with severe hyponatremia. Therefore, careful monitoring of sodium level is important when sodium benzoate is prescribed.

Deceased donor liver transplantation (DDLT) raises ethical and social questions about liver transplantation for patients who are not expected to live. Patients with post-hepatectomy liver failure must have DDLT as soon as possible. Here, we detail a particular dilemma that the transplant team had while deciding whether to harvest liver from a deceased donor in the face of the recipient's non-reactive, fully dilated pupils while they awaited a liver transplant in the intensive care unit. Despite having fixed dilated pupils during the neurologic evaluation, the patient eventually had effective DDLT, indicating that DDLT should not be done unless there is obvious brain death.

Lacticaseibacillus rhamnosus (L. rhamnosus), known for its beneficial effects on gut and vaginal microflora, is considered a low-pathogenicity organism with the propensity to cause infection in immunocompromised patients. To our knowledge, no cases have been reported in liver transplant recipients (LTR). Here, we report the first case with L. rhamnosus intra-abdominal infection and pleural empyema in a LTR, along with review of the literature. The isolate found in peritoneal and pleural fluid cultures exhibited complete resistance to routinely prescribed antibiotics in LTR. The majority of infections documented in the literature occur in individuals with chronic health conditions, and are linked to high mortality rates. In conclusion, while L. rhamnosus is an opportunistic pathogen, this and previous documented examples underscore the need of heightened recognition of its potential as a pathogen, and its resistance in immunocompromised patients. In order to get a more favourable result, it is crucial to initiate tailored early antimicrobial therapy alongside efficient source control.

We present the case of a 74-year-old female who had a right hepatectomy performed laparoscopically to donate her liver to her a 40-year-old son who had alcoholic liver cirrhosis. She voluntarily cooperated, and thorough medical and psychological evaluations were carried out. The receiver underwent surgery in 296 minutes, while the donor took 158 minutes. With normal liver function, the donor and recipient were released from the hospital on days 10 and 14, respectively, after a smooth recovery. Three months following the living donor liver transplant, both the receiver and the living liver donor have not experienced any problems and are doing well.

Colorectal cancer (CRC) poses a significant global health challenge, particularly with patients often experiencing liver metastases. Surgical resection remains the standard treatment; however, many patients face ineligibility due to disease status, contributing to poor prognoses. Liver transplantation (LT) is a viable alternative for select patients with advanced disease. This case report details the clinical outcomes of two CRC patients with multiple liver metastases who successfully underwent living donor LT (LDLT). Both individuals had intricate medical histories and pre-existing liver issues. Patient 1, a 58-year-old male, received extensive pre-transplant interventions, including multiple radiofrequency ablations and pulmonary resections. Patient 2, a 50-year-old male, had cirrhosis alongside colorectal cancer with liver metastases and completed chemotherapy. The cases illustrate LDLT's viability and potential advantages for complex CRC cases with liver metastases. The patients' intricate medical backgrounds necessitate thorough pre-transplant evaluations and vigilant post-transplant supervision. Moreover, these cases highlight the critical nature of ongoing follow-up for evaluating long-term survival and recognizing possible complications.

Liver transplantation is accepted as an effective therapeutic option for unresectable hepatoblastoma. We present a pediatric case of hepatoblastoma patient with portal vein (PV) tumor thrombosis (PVTT) that occluded the main PV completely despite aggressive neoadjuvant chemotherapy. The patient was a 4-year and 2-month-old 17-kg boy suffering from hepatoblastoma with PVTT, which was partially regressed by neoadjuvant chemotherapy. Viable PVTT remained after right trisectionectomy and repetition of systemic chemotherapy. To remove the tumor completely, we performed living donor liver transplantation (LDLT) using his father's left liver graft. The native PV was completely occluded with PVTT and abundant pericholedochal collaterals developed. The native PV was meticulously dissected to fully expose the superior mesenteric vein-splenic vein confluence. This confluence portion was deeply clamped and opened to use as a viable PV inflow source. A cold-stored fresh external iliac vein homograft was anastomosed to the confluence portion as an end-to-end fashion and passed through the native retropancreatic PV tract after mechanical dilatation of the tract. Left liver graft implantation was performed using standard procedures of pediatric LDLT. The patient recovered uneventfully. He will undergo scheduled adjuvant chemotherapy. Customized design for PV reconstruction using interposition vein graft with viable PV inflow source can result in successful PV reconstruction with complete removal of PVTT.