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Background: The aim of the present study was to compare the outcomes of pediatric LT with left liver grafts, obtained either from a living donor (LD) or a split deceased donor (sDD). Methods: Retrospective single‐center study from 2002 to 2022. All pediatric LT with left liver grafts (not including middle hepatic vein) from LD or sDD were included. Reduced grafts were not included. Results: A total of 112 pediatric LT were performed: 58 with LD grafts and 54 with sDD grafts (17 split ex situ and 37 in situ). Donor characteristics were similar, apart from donor age (33 years in LD vs. 30 years in sDD, p = 0.03). Indications were similar with 55% biliary atresia in each group. Retransplantation was more frequently performed in the sDD group (2% vs. 15%, p = 0.01). Recipient age, weight, and PELD score at transplant were not significantly different between groups. Cold ischemia time was longer for sDD (158 min in LD vs. 390 min in sDD; p < 0.0001). Posttransplant peak ALT was higher with sDD grafts (1470 vs. 1063, p = 0.018), and hospital stay was longer with sDD grafts (27 vs. 21 days, p = 0.005). However, there was no difference between groups in terms of major morbidity (Dindo‐Clavien grade ≥3), vascular and biliary complications, and 90‐day mortality. Patient survival at 10 years was 93.1% for LD and 92.8% for sDD (p = 0.807). Graft survival at 10 years was 89.7% for LD and 83.1% for sDD (p = 0.813). Conclusions: Technically similar LD and sDD grafts achieve very similar postoperative and long‐term outcomes with excellent patient and graft survival. [ABSTRACT FROM AUTHOR]

Background: Kinesin family member 12 (KIF12) mutation‐related cholestatic disorder represents a rare subtype of progressive familial intrahepatic cholestasis (PFIC), referred to as PFIC Type 8, with only 21 reported cases globally to date. Methods: Here, we present a unique case of a 6‐month‐old boy diagnosed with homozygous KIF12 gene mutation, who successfully underwent a living donor liver transplant at our center for end‐stage liver disease. Results: This case marks the youngest patient of KIF12‐related cholestatic disorder necessitating a liver transplant to date. The child initially presented with neonatal cholestasis and then developed infantile hepatic decompensation. Our report discusses the diagnostic process and management strategies employed. It underscores the importance of prompt diagnosis through clinical suspicion, biochemical parameters, and genetic testing, as well as the adoption of suitable management strategies, including the early contemplation of liver transplant in such exceptional and rare cases of genetic intrahepatic cholestasis. Conclusion: KIF12‐related genetic disease should be considered in neonatal cholestasis cases with high gamma glutamyl transpeptidase to differentiate from conditions like biliary atresia. Favorable outcomes post liver transplant stress the importance of early genetic testing and referral to liver transplant centers for unresponsive patients, potentially saving lives. [ABSTRACT FROM AUTHOR]

Background: Sir Roy Calne in 1976 described "Biliary reconstruction is the Achilles heel of liver transplantation," and it remains true. In some patients, such as those with short‐gut syndrome and concomitant biliary atresia, neither duct to duct nor Roux biliary reconstruction is feasible. Methods: We present a case of child's third liver transplant (LT), where an innovative extra‐anatomical biliary bypass was created using a sleeve from greater curvature of the stomach. Results: The patient is well nearly 10 years following the LT. Conclusions: This technique could prove to be an important addition to the armamentarium of a surgeon in difficult retransplants and in patients with short‐gut syndrome as it provides a viable option with good long‐term outcome. [ABSTRACT FROM AUTHOR]

Introduction: Acute‐on‐chronic liver failure (ACLF) is associated with increased mortality and morbidity in patients with biliary atresia (BA). Data on impact of ACLF on postoperative outcomes, however, are sparse. Method: We performed a retrospective analysis of patients with BA aged <18 years who underwent LT between 2011 and 2021 at our institution. ACLF was defined using the pediatric ACLF criteria: ≥1 extra‐hepatic organ failure in children with decompensated cirrhosis. Results: Of 107 patients (65% female; median age 14 [9–31] months) who received a LT, 13 (12%) had ACLF during the index admission prior to LT. Two (15%) had Grade 1; 4 (30%) had Grade 2; and 7 (55%) had Grade ≥3 ACLF. ACLF cohort was younger at time of listing (5 [4–8] vs. 9 [6–24] months; p <.001) and at LT (8 [8–11] vs. 16 [10–40] months, p <.001) compared to no‐ACLF group. Intraoperatively, ACLF patients had higher blood loss (40 [20–53] vs. 10 [6–19] mL/kg; p <.001) and blood transfusion requirements (33 [21–69] vs. 18 [7–25] mL/kg; p =.004). Postoperatively, they needed higher vasopressor support (31% vs. 10.6%; p =.04) and had higher total hospital length of stay (106 [45–151] vs. 13 [7–30] days; p =.023). Rate of return to the operating room, hospital readmission rates, and 1‐year post‐LT survival rates were comparable between the groups. Conclusion: Despite higher perioperative complications, survival outcomes for ACLF in BA after LT are favorable and comparable to those without ACLF. These encouraging data reiterate prioritization during organ allocation of these critically ill children for LT. [ABSTRACT FROM AUTHOR]

Background: Portal vein thrombosis is a potentially devastating complication following pediatric liver transplantation. In rare instances of complete portomesenteric thrombosis, cavoportal hemitransposition may provide graft inflow. Here we describe long‐term results following a case of pediatric cavoportal hemitransposition during liver transplantation and review the current pediatric literature. Methods: A 9‐month‐old female with a history of biliary atresia and failed Kasai portoenterostomy underwent living donor liver transplantation, which was complicated by portomesenteric venous thrombosis. The patient underwent retransplantation with cavoportal hemitransposition on postoperative day 12. Outcome: The patient recovered without further complication, and 10 years later, she continues to do well, with normal graft function and no clinical sequelae of portal hypertension. CT scan with 3‐D vascular reconstruction demonstrated recanalization of the splanchnic system, with systemic drainage to the inferior vena cava via an inferior mesenteric vein shunt. The cavoportal anastomosis remains patent with hepatopetal flow. Of the 12 previously reported cases of pediatric cavoportal hemitransposition as portal inflow in liver transplantation, this is the longest‐known follow‐up with a viable allograft. Notably, sequelae of portal hypertension were also rare in the 12 previously reported cases, with no cases of long‐term renal dysfunction, lower extremity edema, or ascites. Conclusions: Long‐term survival beyond 10 years with normal graft function is feasible following pediatric cavoportal hemitransposition. Complications related to portal hypertension were generally short‐lived, likely due to the development of robust collateral circulation. Additional reports of long‐term outcomes are necessary to facilitate informed decision making when considering pediatric cavoportal hemitransposition for liver graft inflow. [ABSTRACT FROM AUTHOR]

Background: Small‐for‐size syndrome (SFSS) in pediatric liver transplant recipients, particularly those weighing less than 10 kg, is rare. This report describes a case of a 15‐month‐old whole liver transplant recipient who suffered SFSS, and systematic literature review was performed to identify outcomes of such cases and potential risk factors for SFSS. Case Presentation: A 15‐month‐old toddler with a history of biliary atresia underwent a deceased donor whole liver transplant. The graft weighed 160 g, resulting in a graft‐to‐recipient weight ratio (GRWR) of 1.6%. The post‐operative course was complicated by SFSS, characterized by massive ascites causing hemodynamic instability and compromised hepatic artery flow. Pharmacological intervention with octreotide was initiated, and the patient eventually recovered. Conclusion: In small pediatric recipients, especially those weighing less than 10 kg, the native liver body weight ratio (LBWR) is significantly higher. When selecting an appropriately sized graft for these recipients, this higher ratio should be taken into consideration. The literature review suggests that a GRWR of less than 2% is associated with a higher incidence of small‐for‐size syndrome in small pediatric recipients weighing less than 10 kg. [ABSTRACT FROM AUTHOR]

Background: Pediatric liver transplantations generally represent advanced surgery for selected patients. In case of acute or chronic graft failure, biliary or vessel complications, a retransplantation (reLT) can be necessary. In these situations massive adhesions, critical patient condition or lack of good vessels for anastomosis often are problematic. Methods: Between 2008 and 2021, 208 pediatric patients received a liver transplantation at our center. Retrospectively, all cases with at least one retransplantation were identified and stored in a database. Indication, intra‐ and postoperative course and overall survival (OS) were analyzed. Results: Altogether 31 patients (14.9%) received a reLT. In 22 cases only one reLT was done, 8 patients received 2 reLTs and 1 patient needed a fourth graft. Median age for primary transplantation, first, second and third reLT was 14 (range: 1–192 months), 60.5 (range: 1–215 months), 58.5 (range: 14–131 months) and 67 months, respectively. Although biliary atresia (42%) and acute liver failure (23%) represented the main indications for the primary liver transplantation, acute and chronic graft failure (1st reLT: 36%, 2nd reLT: 38%), hepatic artery thrombosis (1st reLT: 29%, 2nd reLT: 25%, 3rd reLT: 100%) and biliary complications (1st reLT: 26%, 2nd reLT: 37%) were the most frequent indications for reLT. OS was 81.8% for patients with 1 reLT, 87.5% with 2 reLTs and 100% with 3 reLTs. Conclusion: Pediatric liver retransplantation is possible with a good outcome even after multiple retransplantations in specialized centers. Nevertheless, careful patient and graft selection, as well as good preoperative conditioning, are essential. [ABSTRACT FROM AUTHOR]

Background: Liver transplant is the cure for children with liver failure. Sri Lanka is a lower‐middle‐income country with a predominant free, state health system. Pediatric liver transplant program in Sri Lanka is still in the budding state where the initial experience of the program is yet to be documented. Methods: A retrospective review was performed including the clinical characteristics of all pediatric liver transplant recipients of Colombo North Centre for Liver Diseases since the inception of the program from June 2020 to May 2023. Results: There were 14 PLT performed in 3 years. The median recipient age and weight were 8 years (6 months–15 years) and 23.3 kg (6.4–49.2), respectively. The majority were boys (64%). All were from low‐income backgrounds. Indications for LT were acute liver failure (5/14), decompensated chronic liver disease (5/14), and acute on chronic liver failure (4/14). Underlying liver diseases were Wilson disease (6/14), autoimmune liver disease (3/14), biliary atresia (2/14) and progressive familial intrahepatic cholestasis type 3 (1/14), and unknown etiology (2/14). The majority were living donor liver transplants (86%). Of the living donors, 42% (5/12) were Buddhist priests. There were three immediate deaths and two late deaths. The 3‐month survival was 78%, and overall survival was 64%. Living donor transplants carried a higher success rate (92%) compared to diseased donor transplants (0%; 2/2). Conclusions: Initial experience of pediatric liver transplant program of Sri Lanka is promising despite being established in a free healthcare system amidst the crisis circumstances. Initial experience of pediatric liver transplant program of Sri Lanka is promising despite being established in a free healthcare system amidst the crisis circumstances. There were 14 PLT performed in 3 years. [ABSTRACT FROM AUTHOR]

Introduction: Liver transplantation (LT) has emerged as a lifesaving modality for many liver diseases in children. Pediatric LT is an established treatment in the Western world but is relatively a new procedure in resource‐limited countries like Pakistan. The study aims to highlight the outcomes and survival of pediatric recipients from the first pediatric liver transplant center in Pakistan. Method: A retrospective analysis of pediatric LT was done from 2012 to 2019. The study was conducted in the Hepatobiliary and liver transplant department of Shifa International Hospital (SIH), Islamabad. A detailed analysis for indications for pediatric LT, survival, and complications was done. Results: Forty‐five patients under 18 years of age underwent Living donor liver transplant (LDLT) in SIH. Median age was 9 years and M:F of 2:1. Cryptogenic liver disease followed by Wilson disease were the two most common indications of LT. The majority of patients had chronic liver disease 34 (75%) while 11 (24%) had acute liver failure. The right lobe graft was the most common type of graft 19 (42.2%). Thirty days, 1‐year, 3‐year, and 5‐year survival was 77.8%, 75.6%, 73.3%, and 60.6% respectively. Mortality was highest in patients with biliary atresia 4 (33%). Causes of death included pulmonary embolism, sepsis, surgical complications, and acute kidney injury. Mean survival was 88.850 months (±7.899) (CI 73.369–104.331). Conclusion: Pediatric LDLT has offered disease‐free survival for patients. Survival can improve further with nutritional rehabilitation and anticipation and management of post‐operative complications. [ABSTRACT FROM AUTHOR]

Orthotopic liver transplantation (OLT) in the care of children with inborn errors of metabolism (IEM) is well established and represent the second most common indication for pediatric liver transplantation in most centers worldwide, behind biliary atresia. OLT offers cure of disease when a metabolic defect is confined to the liver, but may still be transformative on a patient's quality of life reducing the chance of metabolic crises causing neurological damage in children be with extrahepatic involvement and no "functional cure." Outcomes post‐OLT for inborn errors of metabolism are generally excellent. However, this benefit must be balanced with consideration of a composite risk of morbidity, and commitment to a lifetime of post‐transplant chronic disease management. An increasing number of transplant referrals for children with IEM has contributed to strain on graft access in many parts of the world. Pragmatic evaluation of IEM referrals is essential, particularly pertinent in cases where progression of extra‐hepatic disease is anticipated, with long‐term outcome expected to be poor. Decision to proceed with liver transplantation is highly individualized based on the child's dynamic risk‐benefit profile, their family unit, and their treating multidisciplinary team. Also to be considered is the chance of future treatments, such as gene therapies, emerging in the medium term. [ABSTRACT FROM AUTHOR]

Background: Liver transplantation (LT) is a serious cardiovascular stressor for patients with end‐stage liver disease (ESLD). Data on the effects of cardiovascular diseases on pediatric LT is limited. No study on LT for pediatric patients with ESLD combined with congenital heart disease (CHD) has been reported from mainland China. Methods: A total of 1005 patients were included in this study. The Kaplan–Meier method with log‐rank testing was used to evaluate survival outcomes between groups. Univariable and multivariable Cox regression models were used to determine the risk factors for patient and graft survival. Results: The most common indication for LT was biliary atresia (BA 90.3%). The prevalence of CHD was 3.8% (38). 42 CHD were found in 38 patients. The incidence of death and graft loss was more common in the CHD group than in the no‐CHD group (13.2% vs. 5.0%, p =.045 and 15.8% vs. 6.2%, p =.019, respectively). The 5‐year patient survival and graft survival in the CHD group versus the no‐CHD group was 86.8% versus 94.7% (log‐rank p =.022) and 84.2% versus 93.5% (log‐rank p =.015), respectively. No significant differences were observed in re‐transplantation, hepatic artery thrombosis (HAT), and portal vein thrombosis (PVT). After adjusting for age, BMI, etiology of LT, and other confounding factors, we can still find that the presence of CHD was associated with patient and graft survival after LT. Conclusion: The presence of CHD was associated with higher mortality and lower graft survival after LT. If possible, the cardiac defects should be addressed prior to LT. [ABSTRACT FROM AUTHOR]

Liver transplantation (LT) has been indicated for smaller and more clinically severe patients in recent years. Small biliary atresia (BA) patients often show portal hypoplasia and sclerotic portal vein (PV), which may make PV reconstruction more difficult during the operation. Among PV complications, intraoperative PV thrombosis can be considered a disaster, and it is important to prevent this catastrophic event by the precise assessment of the PV structure and PVF using radiological imaging before and during LT. However, there are no objective parameters to indicate whether sufficient PVF can be obtained. PV pressure (PVP) and PV flow (PVF) have mainly been studied in adult living donor LT, for the purpose of preventing small‐for‐size syndrome, and PVP has been considered an objective parameter of graft inflow modulation (GIM). In the setting of pediatric LT, GIM is mainly performed to prevent hypoperfusion, and it must be performed before graft implantation. GIM to maximize the PVF of pediatric patients with potentially low PVF in LT consists of the interruption of collateral vessels, the assessment of the usability of the native PV, and technical modifications in PV reconstruction. Reliable objective parameters that represent sufficient PVF before graft implantation are desired. Our recent study proposed that a PVP of ≥25 mmHg before graft implantation can be considered an objective parameter to obtain sufficient PVF (cutoff value: 50 mL/min/100 g of graft weight). Further investigation is needed to determine the best strategy for successful PV reconstruction in pediatric LT. [ABSTRACT FROM AUTHOR]

Background: Several studies describe poorer motor developmental motor outcomes post‐liver transplant (LT) in younger children. Limited studies examine physical function in older children and adolescents pre‐ and post‐LT. Methods: Retrospective review of standard of care physical function outcome measures pre‐ and 1‐year post‐LT in children ≥6 years at LT. Measures include: 6‐minute walk test (6MWT), grip strength, Bruininks‐Oseretsky Test of Motor Proficiency‐2 (BOT‐2) components, Physical Activity Questionnaire (PAQ), and Paediatric Quality of Life Multidimensional Fatigue Scale. Association of medical variables with outcomes was explored. Results: The study cohort included 23 (8 male, median (interquartile range) age 11.67 (8.25, 13.92) years at LT) participants. Top two primary diagnoses included biliary atresia (30.4%) and fulminant hepatic failure (21.7%). At 1‐year post‐LT, over one‐third (36%) were overweight or obese. Compared with healthy norms, children had significantly lower pre‐LT PAQ scores (p =.002), pre‐ and post‐6MWT scores (p <.001) and post‐LT BOT‐2 strength and agility scores (p <.001). Pre‐LT, lower balance scores were associated with abdominal distention/ascites (p =.009) and splenomegaly (p =.017). Lower pre‐LT platelet count correlated with poorer balance (r =.532, p =.017) and lower strength and agility scores (r =.446, p =.043). Significant moderate inverse correlations were found between weight/body mass index z‐scores and BOT‐2 components. Post‐LT children continue to demonstrate decreased levels of motor proficiency and functional capacity but report less fatigue and increased physical activity. Conclusions: Older children and adolescents undergoing LT are at risk of decreased physical function, highlighting the need for pre‐ and post‐LT rehabilitation to optimize long term outcomes. [ABSTRACT FROM AUTHOR]

Background: Kaposi sarcoma (KS) is an endothelial cell tumor, rare in children. It is 200 times more frequent after solid organ transplantation than in the general population. Methods: We report three cases of pediatric patients who developed KS after liver transplantation (LT). Results: Case 1, a 4‐year‐old boy undergoing LT due to familial intrahepatic cholestasis. Five months after LT, he presented with fever, dyspnea, and cough with enlarged lymph nodes and splenomegaly, anemia, thrombocytopenia, elevated liver enzymes, and positive EBV viral load. Lymph node biopsy diagnosed KS with an elevated HHV8 viral load. Case 2, a 4‐year‐old boy who underwent LT due to secondary biliary cirrhosis resulting from extrahepatic biliary atresia. Two years later, graft dysfunction was noticed with positive EBV viral load, thrombocytopenia, massive cervical lymph node enlargement, and splenomegaly. Lymph node biopsy diagnosed KS, Castleman's disease, and plasmablastic lymphoma related to HHV8 infection. Case 3, a 15‐month‐old girl, who received two LT due to biliary cirrhosis. Six months later, she presented with diarrhea, abdominal distension, anemia, thrombocytopenia, enlarged lymph nodes, splenomegaly, and positive CMV viral load. Axillary lymph node biopsy diagnosed KS and HHV8 infection was confirmed. In all three cases, tacrolimus was discontinued and, after diagnosis, sirolimus was started. All recovered without relapse and have a good graft function. Conclusions: Kaposi sarcoma is a rare disease post‐LT in children. Recognizing keywords and early diagnosis is crucial for timely treatment and survival. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia (BA) is the most common cause of neonatal cholestatic syndrome. The true incidence of BA in Pakistan is largely unknown. Aim: This study aimed to report the clinical features, age at diagnosis and outcomes of biliary atresia from the first pediatric liver transplant center in Pakistan. Methods: The study was done in Shifa International hospital from 2013 to 2020. All babies who had biliary atresia confirmed by laboratory investigation were included. Demographic data, age of presentation, clinical presentation, supporting investigations like liver function tests, ultrasound abdomen, HIDA scan and liver biopsy were noted. Outcome related to Kasai portoenterostomy, liver transplant, complications and immunosuppressant agents were noted. Result: A total of 42 children were included, 23 (54.7%) males and 19 (45.2%) were females. Jaundice was seen in all patients (100%) followed by acholic stools (81%). Associated malformations were noted in 6 (14.2%) patients. Liver function tests confirmed obstructive cholestasis (p 0.04). Kasai was done in 19 (45%) patients only, living donor liver transplant was performed in 6 (14%) patients. Age range of transplant patients was from 3 months to 1 year. Indication for liver transplant was failed Kasai in 1(16.7%) patient and chronic liver disease in 5 (83.3%) patients. LDLT survivors were 10 months to 1 year of age at the time of transplant, mean age was 10.6 months. Maximum survival noted so far is 7 years. Acute complications seen post‐transplant were sepsis (three patients), surgical site infections (two patients), biliary leaks and acute cellular rejection in one patient each. Chronic graft rejection, portal vein stricture needing stenting was done in one patient. Discussion: All patients underwent LDLT from related donors wih no donor related mortality. All are deceased patients were yonger and had advanced disease. BA remains third most commo indication of transplant in our center. Conclusion: Liver transplant is the only lifesaving procedure after failed Kasai or as primary liver transplant due to advance liver disease. The advent of liver transplantation services offers survival and improving outlook of the disease. [ABSTRACT FROM AUTHOR]

Background: Repeat liver transplantation (LT) for patients with the liver graft failure who underwent metallic stent placement in the previous graft hepatic vein (HV) for HV complications can be very difficult. We retrospectively reviewed the safer surgical procedures during repeat LT for patients with a metallic stent in the graft HV. Case Reports: Patient 1 with biliary atresia who was treated with metallic stent placement for HV stenosis underwent a third LT form a deceased donor at the age 17 years. Patient 2 with ornithine transcarbamylase deficiency who was treated with metallic stent placement for refractory HV stenosis underwent a second LT form a deceased donor at age 9 years. In both patients, transection of the previous graft HV through an intraabdominal approach was difficult during repeat LT, and a supradiaphragmatic inferior vena cava (IVC) approach was introduced. Using a midline incision of the diaphragm, the pericardium was incised and the supradiaphragmatic IVC was encircled. After clamping the supradiaphragmatic IVC, graft hepatectomy was performed. The metallic stent was successfully removed breaking, and HV reconstruction was performed on the suprahepatic IVC. Both patients did well without serious HV complications after repeat LT. Conclusions: The surgical technique for the supradiaphragmatic IVC approach is useful to decrease the risk of fatal operative complications during repeat LT for patients with a metallic stent in the graft HV. [ABSTRACT FROM AUTHOR]

Background: Several surgical strategies, including split donor transplantation and living donor transplantation, have been used to increase the donor liver pool. This report focuses on the effects of whole, split, and LDLT on recipient outcomes. Methods: We retrospectively analyzed the records of all patients with biliary atresia at Tianjin First Central Hospital between April 2013 and December 2019. Results: A total of 882 patients were included and divided into three groups by graft type, with 198 in the whole‐liver‐transplantation group, 78 in the split liver transplantation group, and 606 in the LDLT group. The median follow‐up time was 39 months, patient survival rates of three groups were 94.4%, 88.5%, and 95.0%, respectively, and graft survival rates were 90.2%, 83.3%, and 94.7%, respectively. We divided the split liver transplantation group into two subgroups according to the donor's age, and patient survival rates exhibited a significant difference only in the group whose donor age was over 45 years. The postoperative complication rates were significantly higher with respect to hepatic artery thrombosis, portal stenosis, and AR; and lower in hepatic venous stenosis, PTLDs, CMV virus, and EBV infection in the WLT group. Our multivariate model showed that donor age ≥45 years, RBC transfusion, pneumonia, and HAT were the independent predictors of allograft loss. Conclusions: The survival of split liver transplantation group was slightly lower. The types of complications are different from different graft types. Therefore, postoperative monitoring and treatment need to be adjusted according to the different graft types used. [ABSTRACT FROM AUTHOR]

Background: Portal vein complications (PVCs) after pediatric liver transplantation (LT) are sometimes asymptomatic, especially in the early phase, and can threaten both the graft and patient's survival. Therefore, the purpose of this study is to analyze the risk factors for portal vein thrombosis (PVT) and portal vein stenosis (PVS) after pediatric LT. Methods: All pediatric patients (n = 115) who underwent primary LT at Regensburg University Hospital between January 2010 and April 2017 were included in this study. The pre‐, intra‐, and postoperative parameters of all patients were retrospectively reviewed and risk factors for both PVT and PVS were analyzed. Results: Of the 115 patients, living donor LT was performed on 57 (49.5%) patients, and biliary atresia was the primary diagnosis in 65 patients (56%). After pediatric LT, 9% of patients developed PVT, and 16.5% developed PVS. Patient weight ≤7 kg [odds ratio (OR) 9.35, 95% confidence interval (CI) 1.03–84.9, p =.04] and GRWR >3% (OR 15.4, 95% CI 1.98–129.5, p =.01) were the independent risk factors for the development of PVT and PVS, respectively upon multivariate analysis. The overall patient survival rates at 1, 3, and 5 years were 91%, 90%, and 89%, respectively, and there was no difference in patient survival among those with and without PVCs. Conclusions: Pediatric patients with body weight <7 kg and/or receiving a graft with GRWR >3% may develop PVCs and so require certain surgical modifications, close follow‐up, and prophylactic anticoagulant therapy following transplant. [ABSTRACT FROM AUTHOR]

Aim: Infants with biliary atresia (BA) generally have chronic malnutrition. However, the best anthropometric measure to assess malnutrition and its correlation with disease severity is unknown. We aimed to assess correlations of various anthropometric measurements, including air displacement plethysmography (ADP), with laboratory parameters and with the pediatric end‐stage liver disease (PELD) score in infants with BA. Methods: Infants with BA were followed at a pediatric liver transplantation center during 2014–2018. Follow‐up comprised laboratory tests and nutritional assessment by a dietitian including dietary intake, weight, height, mid‐upper arm circumference (MUAC), and skin‐fold thickness. Fat‐free mass (FFM) and fat mass (FM) were measured by ADP. Results: Forty‐three nutritional evaluations were performed in 28 infants with BA (13 boys, 44.4%). The median age was 20.7 weeks (IQR: 13–25.9). Based on the various anthropometry modalities, infants with BA were found to be malnourished on most of the visits; 63% had a MUAC‐Z score lower than −2 standard deviations. High serum bilirubin level predicted lower weight for age, length for age, and MUAC‐Z. Lower MUAC‐Z was associated with a higher PELD score. Neither FM mass nor FFM correlated with PELD or with serum bilirubin level. However, FM correlated with skin‐fold thickness‐Z and was low in most patients. Conclusions: The majority of BA infants suffer from malnutrition as assessed by most anthropometrics modalities; low MUAC correlated best with disease severity and serum bilirubin level. Further studies are warranted to determine the contribution of FM measurement by ADP to the anthropometric assessment of infants with BA. [ABSTRACT FROM AUTHOR]

Background: Pediatric patients with biliary atresia (BA) often present liver cirrhosis‐associated portal hypertension and portal vein (PV) hypoplasia. For successful liver transplantation (LT), it is essential to maintain sufficient PV inflow through stenosis‐free PV reconstruction with effective ligation of collateral veins. The aim of this study was to assess the clinical usability of intraoperative cine‐portogram (IOCP) in young pediatric patients who underwent LT for BA. Methods: Medical records of pediatric patients younger than 10 years who underwent primary LT for BA from 2018 to 2020 were reviewed. Results: A total of 31 patients had undergone Kasai portoenterostomy soon after birth. Their median ages at Kasai portoenterostomy and LT were 1 and 11 months, respectively. Types of LT were living‐donor LT in 13, deceased‐donor split LT in 15, and deceased‐donor whole LT in three patients. PV interposition using an iliac vein homograft was performed in 28 patients receiving partial liver grafts. Side‐to‐side PV unification venoplasty was performed in three patients undergoing whole LT. All patients underwent ligation of collateral veins. IOCP was performed in 6 (19.4%) patients. Four showed no or faint residual venous collaterals. Collateral vein embolization and endovascular stenting were performed in one patient each. PV insufficiency‐free survival rate was 100% at 1 year and 93.8% at 3 years. All patients are currently alive with a median follow‐up period of 23 months. Conclusions: Intraoperative cine‐portogram can be a useful method for identification and embolization of residual portosystemic collateral veins in young pediatric patients who undergo LT for biliary atresia. [ABSTRACT FROM AUTHOR]

Background: Motor skill acquisition plays an important role in physical activity participation and overall social and physical health. Limited studies have examined motor development in children pre‐and post‐liver transplant (LT). Methods: Retrospective review of motor outcomes in children <6 years old with cholestatic liver disease assessed pre‐and 1‐year post‐isolated LT. Measures include Alberta Infant Motor Scale and Peabody Developmental Motor Scales (gross motor quotient (GMQ), fine motor quotient (FMQ), and total motor quotient (TMQ)). Association of medical variables with motor outcomes was explored. Results: Participants included 33 (58% male) children with diagnoses of biliary atresia (70%), Alagille syndrome (21%), and others (9%). Median age at LT was 10 (IQR 7.0–20.5) months. Pre‐LT >75% of children were at risk for motor delay (≤10th percentile on AIMS/ ≥1SD below mean GMQ). Post‐LT, 52% scored ≥1 SD below the mean GMQ compared with 22% FMQ. Children at risk/delayed pre‐LT had an increased risk of motor delay on GMQ post‐LT (odds ratio 11.43, 95% CI 1.12–116.7, p =.017). Higher INR pre‐LT correlated with lower TMQ post‐LT (r = −.51, p =.003). Longer waitlist time correlated with lower FMQ post‐LT (r =.41, p =.03). GMQ post‐LT and height z‐scores pre‐LT (r =.46, p =.02) and post‐LT (r =.45, p <.01) were positively correlated. There was no correlation with presence of ascites, weight z‐score, length of hospitalization, and age at LT. Conclusions: Young children have increased risk of motor delay pre‐LT, which may persist post‐LT. Severity of liver disease and growth delays may impact motor development, highlighting the need for ongoing rehabilitation pre‐ and post‐LT. [ABSTRACT FROM AUTHOR]

Background: The native liver of patients with maple syrup urine disease (MSUD) (1st recipients) can be used as a graft for non‐MSUD patients with end‐stage liver disease (2nd recipients). This study aimed to demonstrate the optimal operational procedures and the long‐term outcomes of 2nd recipients. Methods: Six 2nd recipients of living donor domino liver transplantation (LD‐DLT) (age: 42.5 [22–169] months at DLT) received a native liver as a graft from an MSUD patient at our hospital between June 2014 and April 2020. We reviewed the operational procedures and outcomes of 2nd recipients after LD‐DLT. Results: The 2nd recipients' original diseases included biliary atresia, congenital hepatic fibrosis, congenital protein C deficiency, familial hypercholesterolemia, hepatoblastoma, and mitochondrial hepatopathy. Five of the six recipients had a whole liver and one had a right lobe graft. The site at which the vessels of the MSUD liver were dissected prioritized the safety of the 1st recipient. At the end of follow‐up, all recipients were doing well without surgical complications. The mean serum amino acid values of the 2nd recipients did not exceed the upper limit of the reference values during the long‐term observation period. All patients showed normal growth while maintaining the same z‐score of height and weight after LD‐DLT as the preoperative level. Conclusion: The liver of patients with MSUD can be used safely without concern regarding long‐term complications or de novo MSUD development. LD‐DLT using the MSUD liver can expand the donor pool as an alternative graft in pediatric LT. [ABSTRACT FROM AUTHOR]

Background: Endoscopic and PTB interventions are common nonsurgical interventions for biliary anastomotic strictures that occur after liver transplantation. When these nonsurgical interventions fail, surgical re‐anastomosis is considered; however, this is quite invasive and can cause additional injury that may lead to graft loss. We report a case in which conventional nonsurgical interventions failed, but a new method that involve the use of a transseptal needle—a device to create a transseptal left‐heart access during cardiac catheter interventions—was successfully used in recanalization of the hepaticojejunal anastomotic obstruction. Case: A 21‐year‐old man, who had received living‐donor liver transplantation for biliary atresia at the age of 23 months presented with recurrent cholangitis and liver dysfunction due to a biliary anastomotic stricture of the hepaticojejunostomy. Therapeutic interventions for biliary stricture, including the PTB approach, double‐balloon enteroscopic approach, and rendezvous approach failed. We then performed needle puncture of the anastomotic obstruction using a transseptal needle and succeeded in recanalizing the complete anastomotic obstruction. To perform the procedures safely, we evaluated the organ and needle positions using biplane fluoroscopy and placed a balloon in the afferent jejunal limb as a target for puncture. The 12 Fr catheter via the biliary route was removed 7 months after the procedure, without using a catheter, there was no recurrent stricture or cholangitis for 26 months. Conclusion: Using a transseptal needle to manage hepaticojejunal anastomotic obstruction can reduce the number of patients who need surgical re‐anastomosis. [ABSTRACT FROM AUTHOR]

Background: LT for infants less than 5 kg remains a challenge with high technical complication rates, which is further compounded by large‐for‐size grafts requiring hyper‐reduction. The benefits of MIDH especially for standard left lateral segment (LLS) resection have been unequivocally demonstrated. However, given the fine margins of error, the highly challenging technical aspects of anatomical graft reduction test the limits of safety and may not be routinely feasible with the conventional laparoscopic approach. Case Report: A 14‐month‐old girl weighing 4.4 kg with extrahepatic biliary atresia was referred to our unit for an LT. Her mother volunteered to donate and the calculated volume of the LLS was 342 ml, with an estimated GRWR of 7.6. Given the extremely high GRWR, a segment II monosegment graft was planned. A RMDH was performed, with a final GRWR of 4. The donor and recipient were discharged on the 5th and 12th post‐operative days, respectively. Conclusion: We present the first‐ever report of an RMDH. Our report highlights the fact that robotic surgery can safely replicate a highly precise surgical operation, thereby safely pushing the limits of MIDH. [ABSTRACT FROM AUTHOR]

Background: Immunoadsorption (IA) plasmapheresis is standard modality for pretransplant desensitization in ABO‐incompatible solid organ transplants though technically challenging when considered for an infant or a child less than 10 kg due to non‐availability of pediatric immunoadsorption (IA) columns. The major challenge is to maintain hemodynamic stability considering the large extracorporeal circuit volume meant for adults. To our best knowledge after extensive search in acclaimed global medical journals, this is the first successful attempt in an underweight (6 kg) infant of less than 1 year of age using adult size IA Column thus making it a reality. Case characteristics: We report an 8‐month‐old male infant (A positive) of 6 kg with decompensated liver disease secondary to extrahepatic biliary atresia requiring urgent live donor liver transplantation with AB positive donor with significantly elevated pretransplant anti‐B IgG/ IgM antibody titers >1:1024. Baby underwent multiple sessions of anti‐B immunoadsorption plasmapheresis to lower anti‐B IgM / IgG titers using available adult anti‐B immunoadsorption column. Postprocedure, the antibody titers reduced to 1:8 (anti‐IgG) 1:16 (anti‐IgM) followed by successful ABO‐incompatible live donor liver transplant (LDLT). Outcome: Anti‐B titers remained in normal range in the immediate and post‐transplant period with satisfactory liver functions and no rejection. Conclusion: Immunoadsorption plasmapheresis for ABO‐incompatible solid organ transplantation in infants gives desirable results and can be offered to small sized infants using currently available adult sized IA columns when conducted with adequate technical expertise. [ABSTRACT FROM AUTHOR]

Impact of pre-transplant liver hemodynamics and portal reconstruction techniques on post-transplant portal vein complications in pediatric liver transplantation: a retrospective analysis in 197 recipients. Using an hypoplastic fibrotic portal vein increases the risk of low portal flow at unclamping, graft portal hypoperfusion, portal thrombosis and ultimately graft loss. [Extracted from the article]

Sarcopenia is common in cirrhotic adults and associated with waitlist mortality and worse outcome after liver transplantation. Psoas muscle mass has been used to define sarcopenia. Therefore, we aimed to determine the association between psoas muscle mass and waitlist mortality as well as post‐transplant outcome in children with end‐stage liver disease. Medical records and abdominal imaging of pediatric liver transplant candidates during 2010–2019 were reviewed. A subset of images was measured by two radiologists to determine inter‐rater reliability. Psoas muscle surface area was determined at intervertebral lumbar disk 3–4 (L3‐4) and 4–5 (L4‐5) levels. PMI was calculated by psoas muscle surface area divided by height squared. We included 105 children, most with biliary atresia (84%). Patients with waitlist mortality had lower PMI compared to the ones who survived to transplantation (PMI at L3‐4 levels 352.8 ± 162.5 vs. 416.8 ± 136.2 mm2/m2 and at L4‐5 levels 497.3 ± 167.8 vs. 571.4 ± 163.4 mm2/m2, both p =.04), but not in the multivariate analyses. For transplanted patients (n = 75), a higher rate of re‐operation (39% vs. 15%, p =.03) and longer hospital stay (53 vs. 45 days, p =.02) were found in patients with lower PMI. Lower PMI is associated with higher re‐operation rate and longer hospital stay following transplantation, but not waitlist mortality. PMI may be taken into consideration with other biomarkers to predict post‐transplant complications. [ABSTRACT FROM AUTHOR]

The orifice size of the LHV trunk in LLS grafts is often too small for direct anastomosis. Several methods were developed to enlarge the graft and recipient hepatic vein orifices. This study described our surgical techniques to secure hepatic vein reconstruction in infant recipients and analyzed the patency outcomes. Twelve infants undergoing pediatric LDLT were selected during 2‐year study period between January 2018 and December 2019. Surgical techniques and vascular complications of graft hepatic vein outflow were analyzed. The mean recipient age was 12.5 ± 4.5 months; mean body weight was 9.4 ± 1.0 Kg; and mean graft‐recipient weight ratio was 2.8 ± 0.6%. Primary diseases were biliary atresia in six patients, metabolic diseases in two, hepatoblastoma in two, and acute liver failure in two. Eight LLS grafts were recovered through an open method, and four LLS grafts were recovered through a laparoscopic method. A small superficial LHV branch was present in five of 12 LLS grafts, which was opened to widen the graft hepatic vein orifice. Incision‐and‐patch venoplasty was performed in 10, unification venoplasty in 1 and no venoplasty in 1. All four LLS grafts recovered through a laparoscopic approach required circumferential vein patch because of very short hepatic vein stump. No patient experienced graft hepatic vein‐associated vascular complications during the follow‐up period of 19.3 ± 9.3 months. Our surgical techniques with incision‐and‐patch venoplasty for LLS grafts is beneficial to reduce the risk of hepatic vein outflow obstruction in recipients receiving LLS grafts. [ABSTRACT FROM AUTHOR]

While sarcopenia is an important predictor of LT outcomes in adults, few studies have examined the association of sarcopenia with LT outcomes in pediatric patients. We investigated the clinical influence of sarcopenia on the post‐transplant outcomes in infants with BA. To define sarcopenia in infants, the cross‐sectional area of the tPMA in 93 healthy control infants was measured by computed tomography. Sarcopenia was defined as a tPMA lower than two standard deviations below the mean of healthy control infants. Eighty‐nine infants with BA with a median age at LT of 7.6 months old were enrolled. The clinical characteristics and outcomes of LT were verified in the sarcopenia group (n = 21) and non‐sarcopenia group (n = 68). The sarcopenia group had a significantly longer operation time and greater blood loss during LT than the non‐sarcopenia group (P =.03 and 0.02). The incidence of portal vein stenosis and post‐operative bloodstream infection was also significantly higher in the sarcopenia group than in the non‐sarcopenia group (23.8% vs 4.4%, P =.02 and 28.6% vs 10.3%, P =.04, respectively). The total length of hospital stay did not differ significantly. The 1‐year patient and graft survival rates tended to be lower in the sarcopenia group than in the non‐sarcopenia group (90.5% vs 98.5%, P =.07 and 85.7% vs 97.1%, P =.05, respectively). Sarcopenia in infants with BA may be associated with the patient survival and serve as an effective marker for post‐operative outcomes of LT. [ABSTRACT FROM AUTHOR]

Dexmedetomidine, an α2‐agonist, is used in the PICU for its sedative properties as it minimally affects respiratory status. However, hemodynamic instability is one of its known side effects. There is limited published experience with its use in pediatric liver transplant. We present a case of a 9‐month‐old infant who received a deceased donor liver transplantation for biliary atresia and received an IV dexmedetomidine infusion for sedation starting at 20 hours post‐operatively. The patient received an IV bolus of 0.08 mcg/kg followed by an increase to 1 mcg/kg/hour. She was also receiving a fentanyl infusion for sedation at the time of dexmedetomidine initiation. Approximately 3 hours after initiation, she developed bradycardia as low as 30 beats‐per‐minute with an associated sinus pause of 7 seconds. She was given chest compressions by the bedside nurse briefly before arousing and becoming agitated. Evaluation of other etiologies for the patient's bradycardia was unrevealing. Thus, bradycardia was attributed to dexmedetomidine therapy which was discontinued without recurrence. Hemodynamic instability, specifically bradycardia, is known to occur with dexmedetomidine administration. As this medication is primarily metabolized by the liver, its use immediately after transplantation, when liver function is still recovering, may be associated with an increased risk of side effects. Understanding risk factors for bradycardia and hemodynamic instability early after liver transplantation, particularly with dexmedetomidine, is critical to allow clinicians to identify the patients for higher risk for dexmedetomidine side effects. [ABSTRACT FROM AUTHOR]

In this study, possible risk factors of gastrointestinal perforations (GIP) that increase mortality after liver transplantation in children were investigated. One hundred and thirty‐one pediatric patients who underwent 139 liver transplants between January 2016 and February 2020 were evaluated retrospectively based on preoperative and surgical data. Furthermore, cases with biliary atresia, which constitute 26.7% (35) of the patients, were compared within themselves and with other groups. It was found that the cases that developed perforations were younger, lower in weight, and had higher number of surgeries than those who did not, while the mortality and morbidity rates were higher in these patients. When cases with biliary atresia were analyzed within themselves, no significant difference was found between perforated biliary atresia and non‐perforated cases in terms of age, weight, and previous surgery. When biliary atresia and other etiologies were compared, biliary atresia cases were found to be transplanted at a younger age, at a lower weight, and this group had a higher risk for perforation. Early laparotomy should be performed in order to reduce mortality in GIPs. Patients that are younger, underweight, previously operated, and using mesh must be closely monitored. [ABSTRACT FROM AUTHOR]

Living donor liver transplantation (LDLT) in infants for congenital biliary atresia (BA) poses various challenges nowadays. We aim to investigate independent preoperative risk factors for LDLT in infants. We retrospectively analyzed medical records of infant patients who underwent LDLT surgery for BA from 1 July 2014 to 31 December 2016. Cox regression was used to explore risk factors. The Kaplan‐Meier method was used to calculate the recipient and graft survival, and subgroup analysis was then applied according to the risk factors. Independent t test or Mann‐Whitney U test was applied for comparison of certain factors between survival patients and death. A total of 345 infant LDLT for BA were included in the analysis. In the multivariate Cox‐regression model, 3 factors were determined as independent risk factors for recipient and graft survival, there were neutrophil‐lymphocyte ratio (NLR), pediatric end‐stage liver disease (PELD), and recipient age. The HR (95% CI) of baseline NLR for recipient and graft survival were 1.25 (1.12‐1.38) and 1.25 (1.13‐1.39), with all P <.0001. Kaplan‐Meier curves for NLR using different cut‐offs (1.5; 1, 2) suggested that higher baseline NLR was significantly associated with recipient and graft survival. The subgroup analysis indicated that for infants with elevated NLR, the recipient survival was significantly lower when their age >6 months or PELD >20. Our results indicate that infants with higher baseline NLR value may have lower survival rate 3 years after transplantation. Further investigations about broaden the application of pre‐ and post‐transplant NLR to guide nutrition intervention and immunosuppression therapy are necessary. [ABSTRACT FROM AUTHOR]

Young children listed for liver transplant have high waitlist mortality (WL), which is not fully predicted by the PELD score. SRTR database was queried for children < 2 years listed for initial LT during 2002‐17 (n = 4973). Subjects were divided into three outcome groups: bad (death or removal for too sick to transplant), good (spontaneous improvement), and transplant. Demographic, clinical, listing history, and laboratory variables at the time of listing (baseline variables), and changes in variables between listing and prior to outcome (trajectory variables) were analyzed using random forest (RF) analysis. 81.5% candidates underwent LT, and 12.3% had bad outcome. RF model including both baseline and trajectory variables improved prediction compared to model using baseline variables alone. RF analyses identified change in serum creatinine and listing status as the most predictive variables. 80% of subjects listed with a PELD score at time of listing and outcome underwent LT, while ~70% of subjects in both bad and good outcome groups were listed with either Status 1 (A or B) prior to an outcome, regardless of initial listing status. Increase in creatinine on LT waitlist was predictive of bad outcome. Longer time spent on WL was predictive of good outcome. Subjects with biliary atresia, liver tumors, and metabolic disease had LT rate >85%, while >20% of subjects with acute liver failure had a bad outcome. Change in creatinine, listing status, need for RRT, time spent on LT waitlist, and diagnoses were the most predictive variables. [ABSTRACT FROM AUTHOR]

Recipient cava may be unavailable for outflow reconstruction in some children undergoing liver transplantation (PLT) due to caval agenesis, tumor, or fibrotic caval occlusion. Non‐standard hepatic venous reconstruction (NHVR) with a direct veno‐caval anastomosis or neo‐cava reconstruction is necessary in such cases. Retrospective review of all PLT needing NHVR performed in our unit from January 2010 to September 2019 was performed. Outcomes of this group were compared to a 2:1 matched control group who underwent transplantation with standard piggyback technique. Fifteen children (4.9%) of 304 PLT recipients underwent NHVR. Caval agenesis in biliary atresia (n = 5, 33%) and hepatoblastoma infiltrating the cava (n = 4, 27%) were the commonest indications. Ten children had neo‐cava reconstruction, while 5 had direct anastomosis to the supra‐hepatic caval cuff or right atrium. One child had developed neo‐cava thrombosis without graft venous outflow obstruction in the post‐operative period. There was no significant difference in major morbidity, need for re‐operation (20% vs 16.7%; P = 1.00), hospital stay (24 days, vs 21 days; P =.32), graft & patient survival among the study and control groups. Absent or inadequate recipient cava during PLT with a partial liver graft can be safely managed with technical modifications. Results equivalent to standard piggyback implantation can be achieved. [ABSTRACT FROM AUTHOR]

Background: Cryptosporidium enteritis can be devastating in the immunocompromised host. In pediatric liver transplant recipients, infection may be complicated by prolonged carriage of the parasite, rejection, and biliary tree damage and fibrosis. Herein, we report on six patients and their long‐term outcomes following cryptosporidiosis. Methods: We reviewed all cases of cryptosporidiosis in a pediatric liver transplant population over a 17‐year period at a single center. Six patients with infection were identified, and their outcomes were analyzed. Results: Infection was associated with significant diarrhea and dehydration in all cases, and led to hospitalization in one‐half of patients. Four of the six patients developed biopsy‐proven rejection following infection, with three of those patients developing rejection that was recalcitrant to intravenous steroid treatment. Additionally, three patients developed biliary tree abnormalities with similarity to sclerosing cholangitis. In one patient, those biliary changes led to repeated need for biliary drain placement and advancing fibrotic liver allograft changes. Conclusions: Cryptosporidiosis in pediatric liver transplant recipients may lead to significant complications, including recalcitrant episodes of rejection and detrimental biliary tree changes. We advocate for increased awareness of this cause of diarrheal disease and the allograft injuries that may accompany infection. [ABSTRACT FROM AUTHOR]

Amidst the coronavirus (COVID‐19) pandemic, the American Society for Transplant Surgeons has recommended that only urgent liver transplant with deceased donors should occur. However, young pediatric candidates rely on living donors for lifesaving transplant. We present a case of non‐directed left lateral lobe living liver donor transplant for a 7‐month‐old child with biliary atresia experiencing repeated life‐threatening episodes of sepsis and cholangitis from infected bile lakes. Using careful preoperative planning among the entire multidisciplinary team, paying meticulous attention to infection control pre‐ and post‐operatively, and taking advantage of robust telehealth technology both in and out of the hospital, a successful transplant was achieved. Amidst the COVID pandemic, non‐directed liver transplantation can be safely achieved for pediatric recipients. [ABSTRACT FROM AUTHOR]

Background: To investigate the role of two‐dimensional shear wave elastography (2D‐SWE) in the preoperative evaluation of pediatric patients with biliary atresia awaiting liver transplantation. Methods: Among a total of 152 pediatric patients enrolled in this single‐institution prospective study between March 2018 and August 2019, 143 patients (age range, 4‐97 months; median age, 7 months; 84 males, 59 females) who underwent successful routine ultrasound examination, SWE examination, and blood test before liver transplantation were included in the final analysis. The values of liver stiffness measured by SWE were compared with ultrasound and blood test parameters by Spearman's correlation analysis. Results: The overall median liver stiffness with 2D‐SWE was 29.0 ± 10.9 kPa, with a range of 9.0‐53.3 kPa. The success rate of 2D‐SWE measurements was 98.0% (149/152). Liver stiffness measurement (LSMs) had no significant correlation with gender, age, weight, and height of the pediatric recipients. LSMs were correlated with ultrasound parameters including portal vein (PV) maximum velocity, PV direction, hepatic artery resistance index (HARI), spleen diameter, ascites, and blood test parameters (albumin level, platelet count level, and international normalized ratio). In the pediatric recipients with hepatofugal PV flow, high HARI (HARI ≧ 0.90), and ascites, or without Kasai operation, LSMs were significantly higher (P <.05). Conclusions: SWE is feasible and valuable for assessing liver damage in children with biliary atresia awaiting liver transplantation and might be used as selection criteria for children in need of priority access to liver transplantation. [ABSTRACT FROM AUTHOR]

This retrospective study was conducted to examine the development and current status of pediatric liver transplantation (LT) in western China. Clinical, demographic, morbidity, and mortality data were collected to analyze. It included 260 consecutive pediatric LTs performed at three centers in western China between January 2000 and May 2019. Kaplan‐Meier graft survival rates at 1, 3, 5, and 10 years were 82.1%, 77.2%, 76.6%, and 76.6%, respectively; corresponding patient survival rates were 84.7%, 80.7%, 80.0%, and 80.0%, respectively. More patients underwent living donor liver transplantation (LDLT; n = 188 (73.4%)) than deceased‐donor liver transplantation (DDLT; n = 68 (26.6%)). Survival was better after LDLT (91.5%, 86.6%, and 80.6% at 1, 3, and 5 years, respectively) than after DDLT (80.9%, 72.4%, and 63.9%, respectively; P <.05). Biliary atresia was the leading LT indication (n = 141 (55.1%)), followed by metabolic disease (n = 36 (14.1%)), which was associated with the best recipient survival (88.5% at 5 years). The transplant era and graft‐to‐recipient body weight ratio (GRWR) also significantly predicted overall survival. Survival rates at 5 years were worst in 2000‐2005 (54.5%) and best for GRWRs of 0.8%‐4% (80.4%). The development of pediatric LT in western China began slowly, but the quantity and quality of pediatric LT has progressed in recent years. This procedure is now a promising and reliable treatment for children with end‐stage liver disease in western China. [ABSTRACT FROM AUTHOR]

We present a case of a pediatric liver transplant recipient diagnosed with severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection four days after receiving a living donor liver allograft from her mother. The recipient was a 6‐month‐old with end‐stage liver disease due to biliary atresia and failed Kasai. The infant had an uncomplicated implantation, excellent graft function and down‐trending liver enzymes until developing fevers, diarrhea, and moderate respiratory distress requiring non‐invasive respiratory support. SARS‐CoV‐2 testing (nasal swab Polymerase Chain Reaction) was positive on post‐operative day (POD) 4. Liver enzymes peaked ~1000 U/L (5‐fold higher than the previous day) on POD 6. Histology demonstrated a mixed picture of moderate acute hepatitis and classical elements of mild to moderate acute cellular rejection. Her hepatitis and respiratory symptoms improved coincident with completing treatment with hydroxychloroquine, reduced immunosuppression, and intravenous gamma globulin (IVIG). [ABSTRACT FROM AUTHOR]

This case report describes a 13‐year 10‐month‐old girl who underwent a deceased‐donor split LT for primary diagnosis of biliary atresia at the age of 12 months, who presented with a lower GI bleed. Ultrasound and CT revealed a venous vascular anomaly involving the cecum and ascending colon, with communication of the SMV and pelvic veins consistent with a CEPS. Associated varices were noted in the pelvis along the uterus and urinary bladder. These findings were confirmed by trans‐hepatic porto‐venography, which was diagnostic and therapeutic as a successful embolization of the CEPS was performed using micro‐coils. There were no complications following the procedure and no further GI bleeding occurred, illustrating the efficacy of this treatment option for CEPS. We discuss the literature regarding the presenting complaint of GI bleeding post–LT, CEPS as a rare cause of GI bleeding and its association with PV, and the classification and treatment of CEPS. [ABSTRACT FROM AUTHOR]

To measure HU and HRQOL in pediatric liver transplant (LT) recipients, a cross‐sectional study of patient‐parent dyads was conducted. Direct HU were assessed in 48 adolescents ≥12 years using SG and TTO techniques. Indirect HU were measured by Health Utility Index 2 and HUI3 for subjects ≥12 years and CHU9D for ≥7 years. Patients reported HRQOL using PedsQL™ GC and PedsQL™ TM. A total of 108 dyads participated (55.6% female; 73.2% Caucasian; 42.6% biliary atresia; 35.2% living donor; 37.0% Medicaid). Mean age at survey was 13.6 ± 3.5 years, and time from LT was 8.9 ± 4.9 years. 61.2% were on monotherapy, 25 (23.2%) had acute rejection within 3 years, and 15 (13.9%) had a biliary obstruction within 5 years. Mean indirect HU and HRQOL scores by child report were lower than norms (P <.001). LRD recipients had higher PedsQL™ GC, PedsQL™ TM, and HUI3 scores (P <.01). HU in pediatric LT recipients are lower than norms. Availability of HU scores for post‐transplant health states will enable measurement of quality‐adjusted life years for future comparative effectiveness studies. [ABSTRACT FROM AUTHOR]

Background: Biliary atresia is the number one cause of cirrhosis and liver transplantation in children. Hyponatremia is the most important electrolytic disturbance observed in decompensated cirrhosis. Studies of hyponatremia in cirrhotic children are scarce and those that exist have defined hyponatremia as serum sodium < 130 mEq/L lasting for at least 7 days. Methods: We evaluated transplant‐free survival (Kaplan‐Meier) of children with cirrhosis due to biliary atresia and serum sodium < 130 mEq/L persisting for 1, 2‐6, and ≥7 days. This was a single‐center, historical cohort that included all patients aged ≤ 18 years on a liver transplantation waiting list. Results: We studied 128 patients. The overall frequency of hyponatremia was 30.5% (39/128). Thirteen patients (10.2%) had hyponatremia when put on the list, and 20.3% developed it during follow‐up. The Kaplan‐Meier overall transplant‐free survival rate was 83.3%. Patients with persistent hyponatremia for at least two days had the lowest transplant‐free survival. Glomerular filtration rate (P =.00, RR = 0.96, IC 95% = 0.94‐0.99), BMI/age Z‐score (P =.02, RR = 0.59, IC 95% = 0.39‐0.91), INR (P =.00, RR = 1.43, IC 95% = 1.17‐1.74), and serum sodium (P =.04, RR = 0.91, IC 95% = 0.84‐0.99) were independently associated with transplant‐free survival. We did not observe any difference in mortality prediction after adding sodium to the original PELD score. Conclusions: We conclude that persistent hyponatremia lasting at least two days may herald poor prognosis for children with cirrhosis due to biliary atresia. [ABSTRACT FROM AUTHOR]

Children who undergo liver transplantation and subsequently develop BSI are at risk for adverse outcomes. Research from high‐income settings contrasts the dearth of information from transplant centers in low‐ and middle‐income countries, such as South Africa. Therefore, this study from Johannesburg aimed to describe the clinical and demographic profile of children undergoing liver transplantation, and determine the incidence and pattern of BSI and associated risk factors for BSI during the first year after liver transplant. Pediatric liver transplants performed from 2005 to 2014 were reviewed. Descriptive analyses summarized donor, recipient, and post‐transplant infection characteristics. Association between BSI and sex, cause of liver failure, age, nutritional status, PELD/MELD score, graft type, biliary complications, and acute rejection was determined by Fisher's exact test; and association with length of stay by Cox proportional hazards regression analysis. Survival estimates were determined by the Kaplan‐Meier method. Sixty‐five children received one transplant and four had repeat transplants, totaling 69 procedures. Twenty‐nine BSI occurred in 19/69 (28%) procedures, mostly due to gram‐negative organisms, namely Klebsiella species. Risk for BSI was independently associated with biliary atresia (44% BSI in BA compared to 17% in non‐BA transplants; P =.014) and post‐operative biliary complications (55% BSI in transplants with biliary complications compared to 15% in those without; P =.0013). One‐year recipient and graft survival was 78% (CI 67%‐86%) and 77% (CI 65%‐85%), respectively. In Johannesburg, incident BSI, mostly from gram‐negative bacteria, were associated with biliary atresia and post‐operative biliary complications in children undergoing liver transplantation. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the effect of heparin and argatroban on coagulation function and vascular thrombosis in the early period after pediatric LRDLT. Method: Eighty‐four congenital biliary atresia pediatric patients who had undergone LRDLT were studied. Patients were divided into two groups according to the method of anticoagulation (heparin or argatroban). AT‐Ⅲ activity, APTT, and INR of the two groups were measured in the first 5 days after LRDLT. Vascular thrombosis was investigated by Doppler ultrasound daily. Results: There were no significant differences in gender, age, weight, graft‐recipient weight ratio, and Kasai procedure between the two groups. The AT‐Ⅲ activity of the two groups was low and increased gradually after surgery, with no significant difference between the two groups. There was no significant difference of APTT between the two groups immediately after and in the first day after surgery. After anticoagulation treatment, a significant difference in APTT between the two groups was observed. The incidences of vascular thrombosis were 4.76% (3/63) and 0% (0/21) in the heparin and argatroban groups, respectively, with no significant difference between the two groups. During the treatment, no serious complications such as active hemorrhage or drug allergy were observed in the two groups. Conclusion: Argatroban is a direct anticoagulant, which is independent of AT‐Ⅲ activity. Argatroban might be an alternative to heparin in uncomplicated LRDLT with recovered hepatic and coagulation function. [ABSTRACT FROM AUTHOR]

Patient and graft survival were comparable between TV grafts and whole organ graft recipients. An appraisal of technical variant grafts compared to whole liver grafts in pediatric liver transplant recipients: multicenter analysis from the Society of Pediatric Liver Transplantation (SPLIT) registry. Abbreviations OPTN Organ Procurement and Transplantation Network SPLIT Society of Pediatric Liver Transplantation TV Technical Variant In this issue, McElroy and colleagues report an important reappraisal of the outcomes of technical variant (TV) liver grafts in the multicenter Society of Pediatric Liver Transplantation (SPLIT) database.[1] A total of 1839 children were analyzed for the report of which 18% received living donor grafts, 26% received deceased donor TV grafts, and 56% received whole grafts over the 8 year study period. [Extracted from the article]

Portal vein malformations might occur during the embryonic period, as a consequence of abnormal remodeling of vitelline veins during embryonic life. Patients suffering from biliary atresia are particularly prone to have vascular malformations; although being the most frequent indication for liver transplantation in the pediatric age, portal vein duplication has not been so far associated with biliary atresia, and to the best of our knowledge, there is no‐written evidence describing how to manage it when it is first diagnosed while performing a pediatric liver transplant. Therefore, we present a recent case from our group, describing the intraoperative diagnosis of a double portal system in a patient with biliary atresia and failed Kasai. We aim to describe its surgical management, understanding that it is a real challenge to find them unexpectedly during the surgical procedure in the setting of cirrhosis and portal hypertension, particularly in small patients; therefore, by reporting this case, we aim to make readers aware about the chance of finding it, and how to managed it, to include this approach as part of the surgical armamentarium. [ABSTRACT FROM AUTHOR]

Objectives: Acute‐on‐chronic liver failure (ACLF) is an acute decompensation of cirrhosis complicated by other organ failure and is associated with increased mortality and morbidity. ACLF has not been studied in children with biliary atresia (BA), which is the commonest indication for pediatric liver transplantation (LT) worldwide. This study aims to evaluate ACLF and outcomes in children with BA while awaiting deceased donor LT. Methods: This was a subanalysis of the dataset from a prospective cohort study of patients aged 0‐18 years who underwent portoenterostomy for BA and were listed for LT at King's College Hospital, London, between 1999 and 2003. Outcomes included the development of ACLF, mortality, and complications. Results: Ninety‐nine (41 male) children were included, and follow‐up was 10 [6.0‐15.0] years. A total of 20/99 children developed ACLF. ACLF was associated with increased mortality while awaiting LT (20% vs 4%; P = 0.03). There were no associations between biochemical parameters at listing and death. Increased bilirubin levels 3 months post‐portoenterostomy was predictive of development of ACLF (AUROC = 0.72, P < 0.01). Age at LT and time on the waiting list in the ACLF subgroup were both lower compared to the non‐ACLF group (P > 0.05). Sepsis and gastrointestinal bleeding were the commonest precipitants of ACLF. Complications included ascites, hepatic encephalopathy, and hepatorenal syndrome; the ACLF subgroup required multisystem support and longer intensive care unit stay. Conclusions: ACLF in children with BA awaiting deceased donor LT carries increased mortality and morbidity. This warrants stratification of patients for earlier wait‐listing and prioritization for LT. [ABSTRACT FROM AUTHOR]

Abstract: The aim of our study was to determine the impact of initial orientation for medical and surgical care of children with BA on procedures and outcomes of the first LT. We retrospectively analyzed charts of children with BA who underwent first LT between 2006 and 2015. Patients were divided into two groups for comparison: a single‐center management group (from diagnosis to transplantation) and a secondarily referred group (children referred after failure of KP). We focused analysis on disease severity at transplantation, blood transfusion, and overall survival. One hundred and eighty‐five children were included. The median delay between pretransplant check‐up and transplantation was shorter in patients secondarily referred. A severe undernutrition was observed in 23.7% of children secondarily referred compared to 11.1% in children with a single‐center management (P = .024). At transplantation, INR and factor V level were higher in single‐center group patients (respectively, 67% vs 55%, P < .001 and 61% vs 49%, P = .002). The total of red blood cell and fresh frozen plasma administrated during procedure was two times higher in patients secondarily referred. Finally, patients with a single‐center management had a higher overall 12 months of survival rate (92.1% vs 83.1%, P = .033). In a country without low‐density population issues, the authors advocate an early referring to transplant center to further improving LT outcomes in children with BA. [ABSTRACT FROM AUTHOR]

Abstract: Severe portopulmonary hypertension (POPH) is a contraindication for liver transplantation (LT) because of the high risk of postoperative heart failure. The early detection of POPH is important for patients with biliary atresia (BA). Brain natriuretic peptide (BNP) is known to be correlated with liver fibrosis in patients with liver cirrhosis. The aim of this study was to elucidate the efficacy of BNP measurement for the follow‐up of patients with BA. Thirty‐two patients with BA were identified from September 2011 to December 2016. As indices of liver fibrosis/cirrhosis, APRI (P < .0001), FIB‐4 (P < .0001), Child‐Pugh score (P < .0001), IV collagen (P = .0005), and hyaluronic acid (P = .0291) had high or moderate correlations with BNP. Patients with splenomegaly, esophageal varices, liver fibrosis, and collateral veins had significantly higher BNP levels than those without. Patients diagnosed with POPH had significantly higher BNP levels in comparison with those patients without (P = .0068). In contrast, PELD/MELD scores showed an almost negligible correlation with the BNP level. LT was successful in 3 asymptomatic BA patients with POPH who had high BNP levels despite the low PELD/MELD scores. In conclusion, routine serum BNP surveillance can be easy to predict asymptomatic POPH. This may help to identify POPH before it reaches a stage that would contraindicate LT. [ABSTRACT FROM AUTHOR]