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• ALT levels may be abnormally low due to various factors, making accurate assessment difficult. • We report a case of abnormally low ALT levels that were ameliorated by LDLT. • Abnormally low ALT levels, suspected to be caused by gene mutations, were ameliorated by LDLT. • The standard ALT range for this case should be set lower than the typical range to avoid underestimating liver damage. Alanine aminotransferase (ALT) is an enzyme that catalyzes the transfer of amino groups from alanine to ketoglutaric acid. ALT is an established marker of liver diseases. Occasionally, ALT levels may be abnormally low due to various factors, making accurate assessment difficult. To date, no studies have documented ALT alterations following Living donor liver transplantation (LDLT) in patients with low ALT levels. Here, we present a case of abnormally low ALT levels that were ameliorated by LDLT. A 27-year-old woman underwent LDLT for refractory cholangitis with biliary atresia. The patient's preoperative ALT level was 1 IU/L. Following graft reperfusion, ALT levels increased (peak value, 456 IU/L), primarily attributed to the donor liver. After LDLT, ALT levels consistently surpassed the lower limit. The differential diagnosis of abnormally low ALT levels suggested a genetic mutation as the most probable underlying cause. Even after LDLT, ALT levels in organs other than the transplanted liver would remain abnormally low. Therefore, to prevent underestimating liver damage, the standard ALT range for such cases should be set lower than the typical range. [ABSTRACT FROM AUTHOR]

Patients who undergo pediatric living donor liver transplantation (LDLT) sometimes develop graft fibrosis. Recently, Mac-2 binding protein glycosylation-modified isomer (M2BPGi) was developed as a new marker of hepatic fibrosis progression. We performed this study to examine the relationship between serum M2BPGi levels and liver histologic findings in patients after LDLT for biliary atresia. Patients aged <19 years who underwent LDLT for biliary atresia at our institution and followed up for at least 1 year after LDLT were eligible. There were 56 patients in this study. Pathologic findings of the last available biopsy were assessed. Portal vein (PV) stenosis was confirmed with angiography. M2BPGi levels were compared with pathologic fibrosis scores and PV stenosis findings. The mean age at transplant was 4.3 years. The mean observation period was 8.6 years. In terms of the degree of liver fibrosis, F0 was observed in 7 patients, F1 in 36, and F2 in 13. The median serum M2BPGi value was 0.8 cut-off index (COI) overall and 0.60 COI for F0, 0.74 COI for F1, and 1.07 COI for F2. The mean M2BPGi value in F2 was higher than that in F0 (P =. 016) and F1 (P =. 012). Mean serum M2BPGi values were 1.57 COI (0.29 COI) in patients with PV complications (n = 5) and 0.72 COI in patients without PV complications (n = 51) (P =. 0001). M2BPGi is a novel marker for liver fibrosis in patients after pediatric LDLT. It is especially useful for follow-up of pediatric patients after LDLT to support liver biopsy interpretation. [ABSTRACT FROM AUTHOR]

The COVID-19 pandemic has had a major impact on liver transplantation (LT) and living donor programs globally. In this study, we aimed to present the principles and strategies of our LT program during the pandemic period and describe its achievements. We retrospectively reviewed the outcomes of 1417 LTs performed at Asan Medical Center, Seoul, Korea, from 2020 to 2022. Of these, 216 recipients who received transplants from deceased donors were excluded, and 1201 recipients who received transplants from 1268 live donors were included in the study, including 38 children <18 years old. Among the 1201 living donor LT (LDLT) recipients, the most common indication for LT was unresectable hepatocellular carcinoma (315/1163, 27.1%) in adults and biliary atresia (29/38, 76.3%) in pediatric recipients. Emergency LDLT was performed in 40 patients (3.3%). The median model of end-stage liver disease and pediatric end-stage liver disease scores were 13.9 ± 7.2 and 13.8 ± 7.1, respectively. In-hospital mortality of recipients was higher than usual at 2.2%, but the cause of death was not related to COVID-19 infection. Of the 1268 live donors who underwent hepatectomy for liver donation, 660 (52.1%) underwent hepatectomy using a minimally invasive approach. Although 17 (1.3%) live donors experienced major complications, there were no serious life-threatening complications and no mortality. Even in a pandemic era, a team with well-established infection control protocols, patient-tailored surgical strategies, and thorough perioperative care can maintain LDLT at a similar quantitative and qualitative level as in a non-pandemic era. [ABSTRACT FROM AUTHOR]

Liver failure and gastrointestinal bleeding occur in the end-stage of biliary atresia (BA). Living-donor liver transplantation (LDLT) is a standard treatment in Japan. Our program actively provides pre-transplant total parenteral nutrition (TPN) for such patients, and here we report its efficiency and safety. Patients with BA for whom LDLT was indicated were identified. Those with a long-term external central venous catheter and TPN, longer than 4 weeks before LDLT, were analyzed. Ascites was controlled with diuretics. TPN indications, efficacy, and complications were assessed along with patient growth, biochemical markers, and gastrointestinal bleeding. Fourteen patients were included in the study, of whom 8 were girls and 6 were boys. The median age at LDLT was 0.9 years. Body weight (BW) at TPN initiation averaged 6799 g, and the median serum total bilirubin was 9.5 mg per dL. The median catheterization duration was 54 days, and 1 patient received home TPN. Indications for TPN were gastrointestinal bleeding and/or massive esophageal varices in 4 patients and poor nutritional status in 10 patients. No complications were observed except for 1 catheter infection and 1 catheter occlusion. The median final body weight before LDLT was 7906 g. The mean rate of BW gain was significantly higher after TPN than before (149 vs 32 g/wk, respectively, P =.0002). Mean prothrombin time and levels of albumin, cholinesterase, and total bilirubin were not significantly different at the start and end of TPN. Pre-transplant TPN was safe and effective for patients with end-stage BA. [ABSTRACT FROM AUTHOR]

Pediatric living-donor liver transplantation (LDLT) candidates often receive long-term antibiotic treatment. Micafungin has been used as an antifungal agent after LDLT, but the adequate dose after pediatric LDLT was unknown. Here, we report micafungin blood concentrations after pediatric LDLT and discuss its safety and adequate dosing. Pediatric patients with data on micafungin concentrations after LDLT were identified. Those with surgical complications were excluded. All patients received standard tacrolimus-based immunosuppression. A micafungin dose of 1 mg/kg was administered once daily for 10 days starting on postoperative day (POD) 1. The trough and peak micafungin blood concentrations were evaluated on PODs 1, 4, 7, and 10. Beta D glucan levels and liver function tests were assessed to determine micafungin effectiveness and safety. Ten patients were enrolled, with a median age of 1.2 years. The median graft vs body weight ratio was 2.7%. The primary diseases were biliary atresia (n = 7), Alagille syndrome (n = 2), and progressive familial intrahepatic cholestasis type 2 (n = 1). Mean peak micafungin levels were 4.47, 6.27, 5.47, and 5.47 µg/mL on PODs 1, 4, 7, and 10, respectively. Mean trough levels were 2.03, 1.88, and 2.66 µg/mL on PODs 4, 7, and 10, respectively. The micafungin half-lives were 13.7, 14.7, and 14.0 hours on PODs 4, 7, and 10, respectively. Beta D glucan levels were 4.4 pg/mL and 3.7 pg/mL before and after transplantation, respectively, indicating no significant difference (P =.3). No clinical fungal infections were observed. Micafungin administration is safe and effective after pediatric LDLT. [ABSTRACT FROM AUTHOR]

This study examines the results of liver transplantation (LT) in patients with biliary atresia, considering whether they underwent the Kasai procedure beforehand. LT and determine postoperative and long-term graft outcomes. This single-center, retrospective study included 72 pediatric patients diagnosed with postpartum biliary atresia who underwent LT between 2010 and 2022. We included patients who underwent LT either after or without the Kasai procedure and compared the demographic data of the patients with various factors, such as the Pediatric End-Stage Liver Disease scores and laboratory values. The study included 72 patients, with 39 of them being female (54.2%) and 33 of them being male (45.8%). Of the 72 patients in the study, 47 (65.3%) had undergone the Kasai procedure, and 25 (34.7%) had not. The preoperative and postoperative month 1 bilirubin values were lower in patients who underwent the Kasai procedure and were higher in postoperative months 3 and 6. Preoperative bilirubin values, postoperative month 3 bilirubin values, and preoperative albumin values were higher in patients who developed mortality (P <.05). Cold ischemia time was longer in patients who developed mortality (P <.05). Our study showed a higher mortality rate in patients who underwent the Kasai procedure. The results also showed that LT was more effective in children, as patients with Kasai had higher mean bilirubin values and higher preoperative albumin values than patients without Kasai. [ABSTRACT FROM AUTHOR]

• Pediatric liver transplantation can be performed safely with technical variant grafts and whole liver grafts. • The pediatric end-stage liver disease score, hepatic artery thrombosis, and portal vein thrombosis are associated with overall mortality. • Reducing the vascular complication rates can provide superior results in pediatric liver transplantation. Liver transplantation (LT) is a treatment modality in the pediatric population for several diseases like biliary atresia, metabolic liver disease, hepatoblastoma, and so on. According to the Organ Procurement and Transplantation Network, 5-year survival was reported as 85.4% to 93.5% by age after pediatric liver transplantation (PLT). This study aimed to evaluate our single-center experience of PLT by analyzing long-term results, comparing the outcomes with the literature, and identifying predictors of patient survival. The data of 40 patients who underwent LT at <18 years of age between June 2015 and June 2021 were studied retrospectively. Recipient characteristics such as age, sex, etiology of liver disease follow-up time, postoperative vascular and biliary complications, and donor characteristics were evaluated. There were 20 (50%) girls and 20 (50%) boys, and the median age was 42 (IQR = 9-117) months. The most common indications of LT were biliary disorders (45%). A whole liver graft was used in 7 (17%), a right lobe graft in 9 (23%), a left lobe graft in 4 (10%), and a left lateral lobe graft in 20 (50%) of the recipients. The 1-, 3-, 5-, and 7-year survival rates were 85%, 82.1%, 82.1%, and 82.1%, respectively. The multivariate survival analysis revealed that the pediatric end-stage liver disease score, hepatic artery thrombosis, and portal vein thrombosis are associated with overall mortality. In conclusion, our long-term survival is similar to the literature, with satisfactory results. However, reducing the vascular complication rates can provide superior results on PLT. [ABSTRACT FROM AUTHOR]

• The timing of liver transplantation in biliary atresia remains controversial. • Liver-spleen volume ratio (LSR) reflects splenomegaly and liver atrophy. • LSR could predict long-term native liver prognosis. • LSR may guide decisions about liver transplantation in biliary atresia patients. The appropriate timing of liver transplantation (LT) in patients with biliary atresia (BA) who survived with their native livers until adolescence remains controversial. The liver–spleen volume ratio (LSR) has been reported to be efficacious in predicting the prognosis of chronic liver disease. We investigated whether LSR could predict long-term native liver prognosis and serve as an indication for LT in patients with BA. Patients with BA who survived with their native liver until the age of 15 years were included. These patients were classified into 2 groups. The unfavorable prognosis group included patients who underwent or were awaiting LT or developed complications such as refractory cholangitis or gastrointestinal bleeding due to esophagogastric or intestinal varices. The favorable prognosis group included patients who survived with their native liver without complications. We compared the 2 groups regarding LSR, hematological, and histologic data. Of 19 patients, 8 were in the unfavorable prognosis group, and 11 were in the favorable prognosis group. LSR was significantly lower in the unfavorable prognosis group (P =. 009). Analysis of the receiver operating characteristic curve showed that the area under the curve of the LSR was 0.891, which was higher than the area under the curve of liver fibrosis markers. The optimal LSR cut-off value for predicting poor native liver prognosis was 1.97, with a sensitivity of 75.0% and a specificity of 87.5%. The LSR reflects splenomegaly and liver atrophy. The LSR might be a reliable predictor of native liver prognosis and could guide decisions about LT in patients with BA. [ABSTRACT FROM AUTHOR]

• Biliary complications are common after deceased donor orthotopic liver transplantation. • Ischemia-reperfusion injury on postreperfusion biopsy does not predict the development of any biliary complication. • Hepatic artery thrombosis, older donor age (>75 years old), and a prolonged cold ischemia time are associated with a higher incidence of biliary complication after deceased donor orthotopic liver transplantation. Ischemia reperfusion injury (IRI) on postreperfusion biopsies is associated with worse outcomes after liver transplantation, although the influence on biliary complications (BC) remains poorly studied. Therefore, the primary aim of our study was to assess the influence of IRI on the incidence of BC. A secondary aim was to assess the influence of steatosis on biliary complications and determine factors that predictor BC. We report a retrospective cohort study including patients with liver transplantation and postreperfusion injury. Biopsies were classified as relevant and nonrelevant ischemia reperfusion injury for assessment of BC. BC included anastomotic stricture, ischemic cholangiopathy, leaks, and bilomas. Independent predictive factors of biliary complications were assessed using univariate and multivariate analyses. 302 patients were included, and 125 patients fulfilled the criteria for relevant IRI (41.4%). Worse IRI was not associated with biliary complications (42.5% vs 40.1%; P =.68), nor was liver graft steatosis associated with BC (40.5% vs 41.5%, P =.95). The median time until biliary complications did not differ between the 2 groups (2 months; interquartile range = 1-15 vs 3 months; interquartile range = 1-12.5; P =.18). Hepatic artery thrombosis (odds ratio [OR] = 3.4; 95% confidence interval [CI], 1.4-8.2; P =.004), older donor age (OR = 2.1; 95% CI, 1.1-4.1; P =.024), and prolonged cold ischemia time (OR = 1.9; 95% CI, 1.1-3.2) were independent factors of biliary complications. Severe IRI on the postreperfusion injury does not predict development of biliary complications. [ABSTRACT FROM AUTHOR]

Portopulmonary hypertension, a type of pulmonary arterial hypertension in the setting of cirrhotic or noncirrhotic portal hypertension, is associated with elevated morbidity and mortality during and after transplantation. Uncontrolled portopulmonary hypertension may prevent or delay listing for transplant candidates, and the prognosis without treatment and ultimately transplant is extremely poor. We present a 29-year-old White woman, who had a post-liver transplant at infancy due to biliary atresia. Later on, she developed extensive portal vein thrombosis and portopulmonary hypertension and underwent a multivisceral transplant (liver, stomach, pancreaticoduodenal complex, and small and large intestine). Preoperative mean pulmonary artery pressure was <30 mm Hg with a pulmonary vascular resistance of <300 dynes.s/cm5 on oral sildenafil and intravenous epoprostenol. Intraoperatively, management required comprehensive transfusion protocols, a careful balance between correcting blood loss and preventing thrombosis. Intravenous epoprostenol, sildenafil, milrinone, and inhaled nitric oxide were used to reduce elevated mean pulmonary artery pressure and right ventricular strain associated with vascular clamping, reperfusion, and massive fluid shifts. Nitric oxide and epoprostenol use unleashed antiplatelet effects on a patient already susceptible to coagulopathy. A multimodal and multidisciplinary approach continued throughout the surgery and in the postoperative period, which led to a successful outcome. [ABSTRACT FROM AUTHOR]

• Estimated graft volume in infants varies widely. • Grafts were functioning well with the exception of one graft loss due to antibody-mediated rejection. • Monosegment grafting can be useful for infants as well as newborns. Left lateral segment grafts are generally used for very young pediatric patients undergoing living donor liver transplantation (LDLT). Recently, graft reduction techniques were developed for LDLT. Monosegment grafting has been used in newborns. The aim of this study was to determine the usefulness of monosegment grafting for infants. Recipients <2 years of age who underwent LDLT with a monosegment graft between 2010 and 2020 were gathered. Parents comprised all LDLT donors. A segment 2 monosegment graft was resected as a graft from the donor. Standard liver volume (SLV) was estimated using Urata's equation. Graft type, graft weight (GW), and native liver weight were assessed. Eight patients were included in the study. Original diseases consisted of biliary atresia (n = 6) and fulminant hepatitis (n = 2). Final graft type included monosegment (n = 5) and reduced monosegment (n = 3). Median final GW/body weight after reduction was 3% (range, 2%-3.4%). Median native liver weight/SLV was 134% except in patients with fulminant hepatitis. Median pre-reduction graft volume (GV)/estimated GV was 113% (range, 60%-208%). Median pre-reduction GV/SLV of monosegment grafts that required reduction (n = 3) was 109% (range, 106%-121%). Median final reduced graft GV/SLV was 80% (range, 74%-91%). Complications due to large-for-size grafts were not observed. One case of bile leakage due to graft reduction occurred as a complication. Grafts were functioning well with the exception of one graft loss due to antibody-mediated rejection. Estimated GV in infants varies widely. Monosegment grafting can be useful for infants as well as newborns. [ABSTRACT FROM AUTHOR]

Approximately 50 years have passed since the Kasai operation announcement for biliary atresia. In adult liver failure cases, the so-called "the carryover cases after Kasai operation" have increased. These patients often underwent polysurgery. In such cases, adult living-donor liver transplantation (LDLT) is occasionally difficult. Many complications have been reported to be caused by severe cholangitis, hepatic portal regional inflammation, and adhesion. We investigated the complications of adult LDLT in post-Kasai biliary atresia cases with polysurgery. Between 1991 and 2021, we performed 205 LDLT cases. We investigated the outcome of adult LDLT for post-Kasai biliary atresia cases (transplanted over 16 years old) (n = 20) and the risk factors for complications after LDLT. On 5 years overall survival, there were no significant differences between "adult LDLT for post-Kasai" group and the others (81.8% vs 81.2%). Adult LDLT for post-Kasai was not found to be a risk factor for complications. However, polysurgery before LDLT was an independent risk factor for biliary stenosis and portal stenosis, as identified in our univariate and multivariate analysis. We analyzed the relationship between biliary stenosis and the frequency of laparotomies using a receiver operating characteristic curve. The analysis showed that the cutoff point (maximum point of sensitivity plus specificity) was more than 3 times that of laparotomies before LDLT. In our study, adult LDLT for post-Kasai cases was not a risk factor for any complications. However, polysurgery before LDLT has been identified as a risk factor for biliary stenosis and portal vein stenosis. [ABSTRACT FROM AUTHOR]

Post-transplant lymphoproliferative disorder (PTLD) is known as one of the most frequent post-transplant neoplastic diseases, which may lead to recipient and graft morbidity after liver transplant. A 14-year-old boy received pediatric living donor liver transplant (right living graft) 29 months ago, with etiology of biliary atresia. During 22-month follow-up after transplant, computed tomography and positron emission tomography with computed tomography scan showed a single progressive mass locating in the graft cutting surface with occlusive reconstructed middle hepatic vein (MHV). Serology was negative for Epstein-Barr virus serology. Mild fever and pneumonia did not improve after 1-week intravenous antibiotics. PTLD was considered. A surgical resection was scheduled and performed in compliance with the Helsinki Congress and the Istanbul Declaration. During laparotomy, a single mass located in the reconstructed MHV from segment V to the inferior vena cava was confirmed. Postoperative immunohistochemical result showed CD 3(+), CD 20(+), CD 38(+), CD 10(−), CD 56(−), Ki-67(+, 20%-30%), Epstein-Barr virus–encoded RNA(−), and Epstein-Barr virus nuclear antigen 2(−). Polymorphic PTLD was eventually diagnosed. No recurrence or new set lesions were detected after 6-month follow-up. This is the first case describing PTLD may originate from reconstructed MHV after pediatric living donor liver transplant. As a life-threatening complication of liver transplant, surgical resection should be considered as a safe and feasible treatment for the single resectable mass. • Post-transplant lymphoproliferative disorder (PTLD) is considered as one of the most frequent post-transplant neoplastic diseases, which may lead to recipient and graft morbidity after transplant. • Lower liver transplant age and Epstein-Barr virus naivety in recipients are reported to be associated with a higher risk of developing PTLD after liver transplant. • Epstein-Barr virus infection and immunosuppressive therapy may together lead to subsequent hyperplasia and even malignant transformation. • PTLD that may originate from the reconstructed middle hepatic vein is first described. • Surgical resection should be considered as a safe and feasible treatment for the single resectable mass. [ABSTRACT FROM AUTHOR]

Alopecia is a common complication in patients following kidney transplantation; however, reports regarding liver transplantation patients are still few. This study followed 111 children who underwent living related liver transplantation. Alopecia patients and its possible risk factors were analyzed. Alopecia occurred in 3 patients (2.7%). Underlying diseases were biliary atresia and Alagille syndrome. Clinically significant alopecia (universal alopecia) occurred in 1 patient with Alagille syndrome. All patients received tacrolimus as their immunosuppression drug. None of the patients who received cyclosporine experienced alopecia. The onset of alopecia ranged from 7 to 28 months after transplantation. Alopecia was treated with a topical corticosteroid and topical tacrolimus, but 1 patient with clinically severe alopecia required conversion from tacrolimus to cyclosporine A. Alopecia is 1 complication seen in children receiving tacrolimus therapy following living donor liver transplant. Prompt management of this cosmetic complication should be done to ensure patients' compliance to medication regimen. • Alopecia is one complication in patient following liver transplantation. • We studied alopecia in a patient who underwent liver transplantation, which was thought to be caused by tacrolimus toxicity. • Alopecia was treated with topical corticosteroid and topical tacrolimus. • In a clinically severe alopecia case, conversion from tacrolimus to cyclosporine is needed. [ABSTRACT FROM AUTHOR]

• This study clearly showed the usefulness of living donor liver transplantation after the Kasai operation. • Active introduction of living donor liver transplantation for patients with poor clearance of jaundice after the Kasai operation improved overall survival. Living donor liver transplantation (LDLT) has been actively performed for patients with poor clearance of jaundice after the Kasai operation for biliary atresia (BA). The present study clarified the usefulness of LDLT for BA. Between 2000 and 2020, 24 patients (late group) underwent radical surgery for BA in our institute. The overall survival rate, native liver survival rate, and proportion of LDLT in the late group were retrospectively compared with those of 47 patients treated before 1999 (early group). P values <.05 was considered statistically significant. The overall survival rates at 5, 10, and 15 years were 57%, 54%, and 49%, respectively, in the early group and 100%, 100%, and 100% in the late group (P <.001). The native liver survival rates at 5, 10, and 15 years were 57%, 52%, and 39%, respectively, in the early group and 57%, 49%, and 42% (P =.993) in the late group. In the early group, LDLT was performed in 7 of 47 patients (15%), and the overall survival rate after LDLT was 71%. In the late group, LDLT was performed in 11 of 24 patients (46%), and the overall survival rate after LDLT was 100%. The long-term outcomes after the Kasai operation for BA have improved in recent years. There were no marked differences in long-term native liver survival before and after 2000. LDLT was actively introduced for patients with poor clearance of jaundice after the Kasai operation, and the survival rate significantly improved. [ABSTRACT FROM AUTHOR]

Treatment options for patients presenting with life-threatening splanchnic ischemia, including that of the intestine and liver, could previously only receive salvage surgery and attempted medical revascularization. We propose that urgent liver transplantation (LT) for acute liver failure (ALF) due to massive liver necrosis should be considered as a life-saving treatment. We successfully performed urgent living donor LT for nonocclusive hepatic ischemia accompanied by nonocclusive mesenteric ischemia (NOMI). An 11-month-old boy with biliary atresia whose jaundice was re-elevated after Kasai portoenterostomy underwent cysto-jejunostomy. Three hours after the uneventful operation, tachycardia, hypotension, and unconsciousness suddenly occurred. Computed tomography revealed whole-liver and massive splenic and focal intestinal ischemia without any vessel occlusion. Urgent LT was performed on postoperative day 3 because intensive therapies, including prostaglandin E1 administration, blood transfusion, and continuous hemodiafiltration, were not effective and the patient had developed life-threatening bradycardia and hypotension. Intraoperative findings included whole-liver necrosis and splenic ischemia and segmental ileal necrosis without any vessel thrombus. LT and necrotic intestinal resections by end-to-end anastomosis were performed. Massive liver necrosis with Gram-positive cocci was histopathologically identified, indicating bacterial translocation due to NOMI. The post-LT course was uneventful, and the neurologic outcomes were uncomplicated. Urgent LT was successfully completed for ALF with NOMI. LT is the sole treatment for the refractory ALF, and undelayed determination is important to rescue. • Liver Transplant was performed for Massive liver necrosis accompanied by Nonocclusive mesenteric ischemia in Biliary atresia patient. [ABSTRACT FROM AUTHOR]

Some liver transplant recipients may present with persistent hypersplenism post transplant. Persistent thrombocytopenia and leukopenia may put immunosuppressed transplant recipients at a higher risk of serious, even life-threatening bleeding and infection and limit the use of some drugs (such as mycophenolate mofetil) that could reduce white blood cell and platelet counts during the postoperative period. Herein, we report the first case, to the best of our knowledge, of successful simultaneous anatomic subtotal splenectomy during living donor liver transplantation in a 6-year-old girl with biliary atresia, cirrhotic portal hypertension, severe hypersplenism, and massive splenomegaly. The normalization of platelet and leukocyte counts observed in this patient on the third and first day of the transplant were maintained within the reference ranges during the whole follow-up period, with no significant remnant splenic regrowth and occurrence of serious procedure-related complications. Currently at 48 months of follow-up, the patient has remained in good general condition with normal liver allograft function and peripheral blood cell counts. Our case demonstrates that simultaneous anatomic subtotal splenectomy during liver transplantation may be an effective and feasible treatment for the prevention of persistent hypersplenism post transplant, achieving a long-term desired hematological response, in a selective category of pediatric cirrhotic patients with severe hypersplenism and massive splenomegaly. • This is the first report of simultaneous, anatomic, subtotal splenectomy during orthotopic liver transplantation in pediatric cirrhotic patients with severe hypersplenism and massive splenomegaly to prevent the persistence of post-transplant hypersplenism. • The platelet and leukocyte counts remained in the reference ranges with no occurrence of serious procedure-related complications during the whole follow-up. • No significant remnant splenic regrowth was observed at the most recent follow-up at 48 months. [ABSTRACT FROM AUTHOR]

Evaluation of the efficiency of percutaneous transhepatic cholangial drainage (PTCD) for biliary stricture after living donor liver transplantation (LDLT) in pediatric patients. We retrospectively analyzed biliary stricture observed in pediatric biliary atresia. LDLT patients were studied between June 1994 and November 2017. A total of 291 patients were observed, 10 of whom were found to have biliary strictures. Among the 291 patients, we observed 10 patients with biliary stricture, which were divided into 2 groups: group A were patients who have biliary stricture with vascular complication (n = 4), and group B were patients who have biliary stricture without vascular complication (n = 4). Two children without vascular complications received PTCD to bridge the time for Rou-en-Y hepaticojejunostomy. A total of 12 procedures were carried out: only 1 patient (10%) underwent the procedure 3 times. The average interval between liver transplantation and percutaneous transhepatic drainage was 63.2 months in group A and 156.9 months in group B, and no significant difference between the 2 groups (P =.127). Clinical success was achieved in all patients. The PTCD was removed from 3 of 4 patients (75%) in group B with clinical success at a mean follow-up of 32.2 months. Higher PTCD removal rate (75%, P <.05) was found in the patients without vascular complication. All of the patients in group A were tube dependent during follow-up. No major complications were observed among all procedures. PTCD is an effective rescue therapy in pediatric LDLT patients, especially in nonvascular complication patients. Successful internal-external drainage and stenting can prevent a second operation for bile duct reconstruction. • Post-transplantation biliary stenosis in patients without vascular complication showed higher tube independence rate compared with patients with vascular complication. • Proper drainage with PTCD can prevent the patients with vascular complication from graft loss and reoperation. • Single-session balloon dilation plus large-bore drainage catheter is an effective and approachable method for post-transplantation biliary stricture pediatric patients. [ABSTRACT FROM AUTHOR]

Everolimus (EVR) is a derivative of sirolimus with a similar mechanism of action. The safety and efficacy of EVR after pediatric living donor liver transplantation (LDLT) are currently unknown. The purpose of this study was to examine the safety and efficacy of EVR as rescue therapy after pediatric LDLT. This study included patients younger than 19 years of age who received EVR after LDLT at our institution. EVR was administered as rescue treatment in addition to tacrolimus. In 21 patients, EVR dose, trough level, outcomes, and adverse effects were assessed. Original diseases of patients consisted of biliary atresia (n = 11), Alagille syndrome (n = 3), fulminant hepatitis (n = 3), hepatoblastoma (n = 2), and other (n = 2). Mean age at transplant was 2.0 years (range 0.6-6.2 years). Mean age at initial EVR administration was 8.0 years (range 0.9-18.9 years). Indications for EVR use were graft fibrosis (n = 8), refractory acute cellular rejection (n = 5), renal sparing (n = 4), hepatoblastoma (n = 2), and chronic rejection (CR) (n = 2). Mean duration of administration was 17.1 months (range 2.1-60.4 months). Mean dose was 0.5 mg/m2 twice daily. Mean EVR trough level was 2.5 ng/mL (range 1.5-5.0 ng/mL). Liver function improved and fibrosis did not progress in all patients with CR. However, 14 patients (67%) experienced adverse effects that required EVR dose reduction or discontinuation. EVR is tolerable for pediatric patients after LDLT with dose adjustment. EVR had a certain effect to relieve progression on CR. Further follow-up is required. • Everolimus can be used after pediatric living donor liver transplantation. • Everolimus is an effective treatment for chronic rejection. • Everolimus had adverse effects that required dose adjustment. [ABSTRACT FROM AUTHOR]

Liver transplantation (LT) is the gold-standard treatment for end-stage liver disease; however, late-onset complications such as fatty liver can occur in the absence of metabolic comorbidities. We report a unique case of post-transplant hepatic steatosis developing in only a part of the liver graft. A 1-year-old boy underwent ABO-incompatible living donor liver transplantation (LDLT) with a left lateral liver graft donated from his mother for biliary atresia. The biliary tract was reconstructed by hepaticojejunostomy using the previous Roux-en-Y limb. Liver function tests increased by up to 2-fold of the upper normal limit after the second year. He developed segmental steatosis in a part of the liver graft 2 years after LDLT. Venous blood drained into the area of the liver graft from veins in the Roux-en-Y limb of the jejunum. Pathologic findings from a liver biopsy showed fatty depositions without steatohepatitis, acute rejection, or tumors. Portal vein stricture (PVS) subsequently became apparent, which was complicated by the symptoms of portal hypertension, such as gastrointestinal varices. We treated PVS with 2 sessions of percutaneous transhepatic portal vein angioplasty (PTPA), after which the segmental steatosis disappeared. We hypothesize that PVS caused local hemodynamic anomalies, leading to fatty deposition in a part of the liver graft. We experienced a case of post-LT with segmental steatosis that was successfully treated by portal vein flow modification with PTPA. Steatosis of the graft might indicate a vascular abnormality, and further examinations should be performed after LT. • A 1-year-old boy underwent living donor liver transplantation for biliary atresia. • The procedure was done using a left lateral liver graft donated by his mother. • Two years later, he developed segmental steatosis in a part of the liver graft. • Portal vein stricture and symptoms of portal hypertension became apparent. • It was successfully treated with percutaneous transhepatic portal vein angioplasty. [ABSTRACT FROM AUTHOR]

Although endoscopic management is considered as the first-line treatment for biliary strictures, it may be challenging in living donor liver transplant recipients due to the complex nature of duct-to-duct reconstruction. In this study we present the use of a pigtail drainage catheter as a biliary stent to treat biliary strictures after a living donor liver transplant. Twenty-seven patients with biliary strictures were treated with our novel technique. In this technique, a pigtail catheter was trimmed into 3 parts (proximal, middle, and distal portions). A suture string was passed through the distal hole of the middle portion, which was then reversed and used as a stent while the proximal portion was used as a pusher. Following balloon dilation of the stenotic segment, the distal, reversed middle, and proximal portions were loaded over the guidewire. After proper placement of the stent, the retractor suture string, pusher, and guidewire were removed. The stent was removed during the third or fourth month of placement through endoscopic retrograde cholangiopancreatography (ERCP) in all patients. No significant complications developed during the procedure or follow-up period. Ten patients required re-stenting by ERCP during the same session. The mean follow-up period was 2 years. Cholestase enzymes and bilirubin levels were within normal limits in all patients during follow-up. Stents derived from drainage catheter facilitate treatment of biliary strictures in patients not eligible for the retrograde approach. This stent is cheap, easy to implement, can be easily removed by ERCP, and re-stenting can be applicable in retrograde if needed. • To prevent the long-term stay of percutaneous drainage catheters. • To reduce the cost of stents by deriving them from drainage catheters. • This type of stent is easy to implement and remove. • It facilitates treatment of biliary strictures in patients not eligible for the retrograde approach. [ABSTRACT FROM AUTHOR]

Our aim was to review the rate of biliary duct stones (BDS) after liver transplantation (LT), risk factors, and treatments, and to identify predictive factors for their onset. LTs performed in our center from 2004 to 2014 were studied. Risk factors for the onset of BDS were identified using univariable Cox's proportional hazards models. Three hundred and sixty-four grafts with 317 duct-to-duct end-to-end biliary anastomosis on a T-tube and 47 hepaticojejunal anastomosis (HJ) were analyzed. BDS were identified in 13 of 364 (3.5%) grafts, including 10 duct-to-duct end-to-end biliary anastomosis on a T-tube grafts (3.2%) and 3 HJ grafts (6.4%). Predictive factors for BDS were biliary strictures (hazard ratio [HR] 9.94; 95% confidence interval [95% CI] 3.25–30.4), bilirubin (HR 1.04; 95% CI 1.01–1.06, for 1 unit increase), Model for End-Stage Liver Disease score (HR 1.07; 95% CI 1.01–1.14, for 1 unit increase), surgery time (HR 1.04; 95% CI 1.01–1.08, for 10-minute increase), hepatocellular disease (HR 8.3; 95% CI 1.09–64.0), hepatic artery thrombosis (HR 6.71; 95% CI 1.47–30.6), and retransplantation (HR 3.69; 95% CI 1.02–13.43). Among 51 grafts (14%) with biliary strictures, female sex was identified as a risk factor for BDS (HR 5.19; 95% CI 1.29–20.98). Multimodality treatment of BDS was often successful but open surgery was still needed in 23% of them. One-, 5-, and 10-year graft survival was not influenced by the onset of BDS. Main predictive factor for BDS in liver grafts is biliary stricture. Recipient's age and body mass index failed to show any statistical importance. In grafts with biliary strictures, female sex is the main risk factor for BDS. In the absence of biliary strictures, hepatic artery thrombosis lead to an increase in the risk of BDS. Multimodality treatment of BDS is often successful. BDS do not influence outcome. [ABSTRACT FROM AUTHOR]

Abstract Increased biliary complications in hepatopulmonary syndrome (HPS) have been hypothesized due to post-transplant hypoxemia. Supporting this hypothesis, we report histopathological findings from an explant liver allograft where the recipient suffered severe and prolonged post-operative hypoxemia. A 4-year-old child underwent liver transplantation (LT) for decompensated chronic liver disease complicated by severe HPS. The post-operative period was complicated by severe prolonged hypoxemia. HPS resolved completely 6 months after LT only to recur 3 months later due to graft dysfunction. The child underwent retransplantation 8 months after the first LT. The explant liver showed bile duct loss along with ulceration and fibrosis of large hilar bile ducts biliary, suggestive of ischemic cholangiopathy. Based on the histopathology findings, we suggest that severe prolonged hypoxemia during post-transplant period could cause ischemic cholangiopathy, which can lead to biliary complications. Highlights • This article reports on the youngest patient to undergo retransplantation for recurrent HPS. • Severe hypoxemia post-LT contributes to biliary complications and an increased risk of graft loss. • This article presents objective evidence of ischemic cholangiopathy in severe hypoxemia-bile duct loss along with ulceration and fibrosis of large hilar bile ducts in the explanted liver. [ABSTRACT FROM AUTHOR]

Abstract Cadaveric split liver transplantation (SLT) is a valid option to increase the pool of cadaveric organs, obtaining 2 functioning grafts from a single donor. Typically, SLT is performed for 1 adult and 1 pediatric recipient. However, on the heels of great results achieved in living donor liver transplantation, splitting cadaveric liver into full right graft and full left graft for 2 adults has become a feasible idea. The rate of biliary complications remains the "Achilles heel" in partial graft liver transplantation, either from cadaveric or living donors. In cases of biliary complications, interventional radiology and/or endoscopic procedures are the cornerstone of management. Surgical revision is left as the last option. When surgical revision fails, retransplantation becomes the only rescue option. Herein we describe the case of a cadaveric SLT, complicated by biliary leakage in the presence of multiple bile ducts. A duct-to-duct anastomosis was not feasible. Therefore, a hepaticojejunostomy was performed and resulted in a high-output biliary leak from different sources. Given the anatomy of the biliary tree, radiologic interventional measures were not feasible to address the leak. The idea of performing a portoenterostomy to restore bilioenteric continuity proved to be successful. Portoenterostomy should not be performed in lieu of other alternatives, but rather as the last option to avoid retransplantation in cases of complicated biliary reconstruction after partial graft liver transplant. Highlights • Radiologic interventional measures and hepaticojejunostomy may not be feasible. • Portoenterostomy proved to be successful in saving the graft. • Portoenterostomy can be an effective way of restoring bilioenteric continuity. • Management of leakage in cases of multiple bile ducts after liver transplantation is challenging. [ABSTRACT FROM AUTHOR]

Abstract Background Cirrhosis for biliary atresia (BA) is associated with risk of gastrointestinal bleeding (GB) from gastroesophageal varices due to portal hypertension. Primary prophylaxis of GB is controversial in children who are candidates for liver transplantation (LT). The aim of the study was to define the management of gastroesophageal varices and to identify the benefit of primary prophylaxis for GB in BA children waiting for LT. Methods A retrospective single-center study including all BA children listed for LT in 2008–2016. Clinical, endoscopical, and biochemical data were analyzed. Results Of 82 children, 50 (61%) did not receive primary prophylaxis and did not present any episode of bleeding, 16 (19.5%) underwent primary prophylaxis, and 16 (19.5%) presented spontaneous GB and received secondary prophylaxis. Children without primary prophylaxis and GB were younger than patients with primary prophylaxis and those with GB (7.7 years [range, 4.1–37.9 years] vs 11.2 years [range, 5.1–43 years]; P =.03 vs 10.7 years [range, 6.9–39.9 years], respectively; P =.004). Seventy-five percent of GB occurred in children older than 8 months. Fifteen (93.8%) children with GB presented esophageal varices (grade III = 10 [62.5%]) and 10 (62.5%) required endoscopic treatments, consisting mainly of sclerotherapy. Median time to LT was similar for children with or without bleeding (2 months [range, 0–17.7 months] vs 2.2 months [0–17.9 months], respectively; P =.89). After 45.5 months (range, 13.7–105.5 months) of follow-up, the overall patient survival was 97.6%. At the intention-to-treat analysis, the survival rate was 100% for patients without bleeding episode and 87.5% for children with GB (P =.16). Conclusions Despite the risk of GB being not clinically predictable in children with BA waiting for LT, our experience suggests that primary prophylaxis of GB might be unnecessary in children younger than 6 months, while it should be considered in older children. Thus, the occurrence of GB does not delay the timing of transplantation. Highlights • Risk of gastrointestinal bleeding is not clinically predictable in biliary atresia. • Esophageal varices screening in children younger than 6 months who are waiting for liver transplant is unnecessary. • Primary prophylaxis should be always considered in selected children older than 6 months. • Occurrence of gastrointestinal bleeding does not delay the time of transplantation. [ABSTRACT FROM AUTHOR]

Abstract Purpose After undergoing the Kasai procedure for biliary atresia (BA), most patients develop severe splenomegaly that tends to be improved by liver transplantation. However, fluctuations in splenic volume long after transplantation remain to be elucidated. Patients and Methods Seventy-one consecutive patients who had undergone pediatric living donor liver transplantation (LDLT) for BA were followed up in our outpatient clinic for 5 years. They were classified into 3 groups according to their clinical outcomes: a good course group (GC, n = 41) who were maintained on only 1 or without an immunosuppressant, a liver dysfunction group (LD, n = 18) who were maintained on 2 or 3 types of immunosuppressants, and a vascular complication group (VC, n = 11). Splenic and hepatic volumes were calculated by computed tomography in 464 examinations and the values compared before and after the treatment, especially in the VC group. Results Splenic volume decreased exponentially in the GC group, with splenic volume to standard spleen volume ratio (SD) being 1.59 (0.33) 5 years after liver transplantation. Splenic volume to standard spleen volume ratios were greater in the VC and LD groups than in the GC group. Patients in the VC group with portal vein stenosis developed liver atrophy and splenomegaly, whereas those with hepatic vein stenosis developed hepatomegaly and splenomegaly. Interventional radiation therapy tended to improve the associated symptoms. Conclusions Fluctuations in splenic volume long after pediatric LDLT for BA may reflect various clinical conditions. Evaluation of both splenic and hepatic volumes can facilitate understanding clinical conditions following pediatric LDLT. Highlights • There is still minimal information available regarding the fluctuation of the spleen volume long after pediatric living donor liver transplantation (LDLT) for biliary atresia (BA). Data on the relationships among spleen volume, liver volume, and post liver transplantation (LTx) complications long after LTx are also scarce. In this study we measured the sequential changes in the spleen and liver volumes after pediatric LDLT for BA in patients followed up for more than 5 years in our outpatient clinic. • This article is the first report shedding light on the relationship between spleen and liver volume related to complications such as vascular or biliary complication after pediatric LDLT for BA in patients followed up for more than 5 years. • This information could be helpful for post-LDLT follow-up, especially in recipients after pediatric living donor liver transplantation for BA. [ABSTRACT FROM AUTHOR]

Serum beta-D-glucan (BDG) levels may increase with anti-adhesion barrier film (ABF) use during pediatric living donor liver transplantation (LDLT). It may affect detection of fungal infections after LDLT. We evaluate BDG levels after pediatric LDLT. Pediatric patients who received an ABF during LDLT were included. Patients who may have had fungal infections prior to LDLT were excluded. One sheet of ABF was placed in the peritoneum during abdominal closure. Serum BDG levels before transplantation and on postoperative days (PODs) 1, 4, 7, 14, 21, and 28 and peritoneal fluid BDG levels on PODs 1 and 7 were measured. Sixteen patients received an ABF during LDLT. Median age at transplant was 1.9 years (range, 6-11 years). Median body weight was 12.6 kg (range, 6.8-39 kg). Indications for LDLT were biliary atresia (n = 10) and other (n = 5). Prior to transplantation, the mean serum BDG level was 3.8 pg/mL. Mean Serum BDG levels were 18.1, 38.3, 5.3, 3.8, 3.3, and 3.3 pg/mL on PODs 1, 4, 7, 14, 21, and 28, respectively. Mean peritoneal fluid BDG levels were 485.9 and 240.4 pg/mL on PODs 1 and 7, respectively. No clinical fungal infections were observed. BDG levels were high in serum and peritoneal fluid after pediatric LDLT. Serum BDG levels normalized after POD 7. Careful interpretation of BDG levels until POD 7 is needed when an ABF has been used. • B-D-Glucan levels were high in serum and peritoneal fluid after pediatric LDLT. • Serum B-D-Glucan levels normalized after POD 7. • Careful interpretation of BDG levels until POD 7 is needed when an ABF has been used. [ABSTRACT FROM AUTHOR]

Objective The aim of this study was to evaluate the impact of preoperative growth status on clinical outcomes of infantile living-donor liver transplantation (LDLT). Methods From January 2009 to December 2014, 131 LDLT recipients ≤1 year of age met the eligibility criteria of this study, of whom 31 patients with weight z-scores less than −2 constituted the abnormal growth group. Patients in the abnormal growth group were randomly matched (1:1) to patients with normal growth status by means of a multivariate case-matched method, and thus 31 patients in the normal growth group and 31 patients in the abnormal growth group were finally enrolled into the study. We compared the 2 groups regarding demographic characteristics, liver functions, postoperative complications, survival outcomes, and effects of catch-up growth. Results Baseline characteristics were well matched between the 2 groups except that patients from the abnormal growth group were significantly inferior in terms of body weight (7.5 kg vs 6.0 kg; P < .01), height (65 cm vs 63 cm; P < .01), and body mass index (17.0 kg/m 2 vs 14.8 kg/m 2 ; P < .01) before LT. After LT, alanine transaminase, aspartate transaminase, alkaline phosphatase, γ-glutamyltransferase, albumin, and total bilirubin were all similar between the 2 groups. We also did not identify significant difference in any specific early complications or patient and graft survival rates (5-year: 90.2% vs 96.8%; P = .313). However, LT brought forth notable catch-up growth in both groups, especially for children with more serious growth retardation before LT. Differences of height z-scores between the 2 groups gradually diminished and lost significance 18 months after LT, and body weight z-scores between the 2 groups became similar 6 months after LT. Conclusions Preoperative growth status had no impact on liver function recovery, postoperative complications, or survival outcomes after infantile LDLT. Although biliary atresia caused severe growth impairment in infants, LT brought forth notable catch-up growth, especially for children with more serious growth retardation before LT. [ABSTRACT FROM AUTHOR]

We report a transplant of the left lateral liver segments with 3 arteries for a pediatric recipient from a living donor. A 6-month-old female infant was diagnosed with liver cirrhosis secondary to biliary atresia and scheduled for living donor liver transplantation (LDLT; mother as donor). Left lateral hepatectomy was performed at the donor site. The dissection of the left hepatic artery (HA), which was divided immediately after its origin, showed 3 branches for segments II, III, and IV. The arteries for segment II, segment III, and segment IV were anastomosed to the recipient HA. No postoperative complications were observed. The outcome of this case demonstrates that left lateral segments with 3 arteries can be successfully used if proper surgical techniques are applied. From this experience we can recommend “3-in-1 segmental resection” in the donor can be safely done by skilled microvascular surgeons and this technique should be considered for selected cases where multiple tiny arteries supply the graft. [ABSTRACT FROM AUTHOR]

Objective The objective of this study was to compare the complications, outcomes, and survival prevalence in patients undergoing living donor liver transplantation due to biliary atresia (BA) or acute liver failure (ALF). Results In the period of June 1998–July 2016, 199 children underwent living transplantation due to BA or ALF. Of these 199, 184 were included in the analysis. The average age, weight, and body mass index of BA patients were lower than those of ALF ( P < .001). The chi-square test showed a higher prevalence of infection in transplant recipients due to BA ( P = .0001) and a higher prevalence of hepatic artery stenosis in those who underwent transplantation due to ALF ( P = .001). In the multivariate analysis, the infection remains statistically more prevalent in the BA group (95% confidence interval [CI], 0.20–0.60), while hepatic artery stenosis loses significance. The mortality rate was similar in both groups and the survival in 5 years also. The prevalence of hepatic artery thrombosis, portal vein thrombosis/stenosis, biliary stenosis, and acute and chronic cellular rejection showed no statistical difference between the two groups. Conclusion Living donor liver transplantation should be a valid option in cases of fulminant hepatitis with an indication for liver transplantation, especially in places where the number of cadaverous donors is low and the length of time on the waiting list is high. [ABSTRACT FROM AUTHOR]

Background and Purpose Small infants with biliary atresia and hypoplastic portal veins (PV) are at risk for portal vein thrombosis (PVT) after liver transplantation (LT), which can lead to graft loss and mortality. Extra-anatomical PV reconstruction techniques have been established for adult cases of PVT; however, they have not been widely accepted for infants. Methods Here, we report the successful use of an extra-anatomical meso-portal venous jump graft to treat early PVT after LT in a 6-month-old infant with biliary atresia and PV hypoplasia. At the time of LT, despite a reduced-sized left lateral graft, we had to create a temporary abdominal closure with silastic mesh. Findings On postoperative day 1, PVT was detected by Doppler ultrasound of the liver. Surgical thrombectomy was attempted. We removed the blood clots and reconstructed the PV using an interposition venous graft. As the PV flow was still not sufficient, we performed an extra-anatomical meso-portal venous jump graft procedure from the recipient superior mesenteric vein to the donor PV. This resulted in a significant improvement in PV flow. Conclusion For small infants at high risk for PVT, a detailed pretransplantation surgical plan and treatment options for possible early PVT are mandatory. An extra-anatomical meso-portal venous jump graft is a viable surgical technique for early PVT in infants. [ABSTRACT FROM AUTHOR]

Background Over the past few decades, reports have demonstrated the feasibility of liver transplantation in adult patients with situs inversus. However, this disease entity remains rare and experience remains limited in adult recipients with situs inversus undergoing transplantation. Methods A 23-year-old woman with situs inversus totalis and end-stage liver disease secondary to congenital biliary atresia was referred to our center and underwent a successful orthotopic liver transplantation. Results We report our experience and review the literature. We performed a modified piggy-back technique with cavo-cavostomy. Using a triangulated wide orifice, the suprahepatic cava was anastomosed in an end-to-side fashion. The patient underwent an uneventful hospitalization and recovery. Conclusion Situs inversus remains a rare condition. Careful perioperative planning, thorough anatomic knowledge of both donor and recipient liver, and use of a variety of different novel techniques can lead to successful outcomes. [ABSTRACT FROM AUTHOR]

Background Pediatric living donor liver transplant (LDLT) patients sometimes develop graft fibrosis after non-recurrent diseases such as biliary atresia (BA). Donor-specific antibodies (DSA) have recently been shown to play a possible role in graft damage after liver transplantation. We report the impact of DSA on pediatric LDLT for BA patients. Methods Patients under age 18 years who received LDLT for BA at our institution and who had at least 5 years' follow-up were identified, and 23 were eventually enrolled in this study. Pathological findings were assessed with the use of the last available biopsy. Patients were divided into 2 groups, DSA-positive and DSA-negative. Graft fibrosis after LDLT was assessed according to DSA groups. Results The mean patient age at transplant was 2.6 years. The mean time to the last available biopsy after LDLT was 8.2 years (4.8–15.6 years); 6 patients (26%) showed no fibrosis, whereas fibrosis was graded as F1, F2, or F3 in 8 patients (35%), 8 patients (35%), and 1 patient, respectively. DSA were observed in 12 patients (52%). Moderate graft fibrosis (F2 and F3) was found in 7 (58%) of the DSA-positive group, but only 2 (18%) of the DSA-negative group, showing a statistically significant difference ( P < .05). Pre-transplant cross-matching was performed in 17 patients. The 2 patients with a positive cross-match were DSA-positive. Six cross-match–negative patients developed de novo DSA after LDLT. Conclusions Graft fibrosis was observed after LDLT for BA during long-term follow-up, more commonly in DSA-positive patients. DSA may play a role in fibrosis formation. [ABSTRACT FROM AUTHOR]

Abstract: Background: To enhance the technical feasibility of portal vein (PV) interposition grafting for pediatric PV hypoplasia, we performed a computational simulation to establish a customized surgical technique allowing a secure anastomosis of an iliac vein graft to a severely hypoplastic PV stump. Methods: Based on the literature and on our own experience with reconstruction of PV hypoplasia, we devised three types of recipient PV stump preparations and three types of interposition vein grafts, yielding five technically feasible combinations. Results: The computational simulation model for PV reconstruction using an interposition vein graft revealed the most feasible combination to be a sequential inverted-T incision to the confluence of the superior mesenteric vein and splenic vein with a longitudinal slit in the transverse vein graft end, the technical feasibility was validated by an artificial suture model. This reconstruction was clinically applied to treat a 7.2-kg 10-month-old female patient with biliary atresia and a severely hypoplastic PV. The PV reconstruction was successful; the patient recovered uneventfully. Conclusions: We have presented a simplified surgical technique for PV interposition that is applicable to pediatric PV hypoplasia, which also appears to be a feasible option for pediatric liver transplantation. [Copyright &y& Elsevier]