Liver Transplantation In Infants Research Articles

Does body weight below 6 kg modify survival of infants after liver transplant? Complete National Transplant Registry Data.

To determine the impact of infant recipient body weight at primary liver transplantation (LT) on both recipient and graft survival rates in complete national data from Poland. We conducted a single-center, retrospective cohort study including 142 LT recipients below 1 year of age with body weights below 10 kg who received primary and isolated LT between 2001 and 2017. Patients were divided into two study groups according to body weight at the time of LT: (1) Group I (≤6.0 kg, 32 patients) and (2) Group II (6.1-9.9 kg, 110 patients). Independent impact of body weight on patient and graft survival were assessed using survival curves and a multivariable Cox regression analysis. The univariate predictors of mortality or retransplantation at 1 year post-LT were recipient body weight of ≤6 kg at transplantation, pediatric end-stage liver disease score, urgent LT, graft from deceased donor, cold ischemia time, post-LT hepatic artery thrombosis, and post-LT dialysis. No statistically significant impact of body weight ≤6 kg on 1-year failure-free survival was found based on the multivariable analysis (p = 0.063). Body weight ≤6 kg was associated with longer post-LT intensive care unit and post-LT hospital stays (p = 0.013 and 0.025, respectively). Since no evidence of independent negative impact of recipient body weight ≤6 kg on failure-free survival 1 year post-LT was found, LT in infants with end-stage liver disease in Poland should be performed according to medical indications and urgency when an appropriate donor is available.

Acute kidney injury after living‐related liver transplantation in infants with biliary atresia: A retrospective study

AbstractAlthough acute kidney injury (AKI) is a frequent postoperative complication after liver transplantation, renal function following living‐related liver transplantation (LRLT) in infants remains understudied. The aim of this study was to identify the prevalence of AKI and the impact of AKI on outcomes in infant recipients with biliary atresia. We retrospectively reviewed infants who underwent LRLT surgery between January 2018 and January 2021. The primary outcome was the risk of postoperative AKI occurrence in the first 7 postoperative days. A multivariate regression analysis model was used to investigate risk factors for AKI, and the incidence and impacts of AKI on outcomes were analyzed. A total of 98 patients were included in the analysis. AKI occurred in 59 patients (60.2%) within 7 days after surgery. Logistic regression analysis revealed that preoperative transfusion (p < 0.05) and lower serum creatinine (SCr) (p < 0.05) were independent risk factors for AKI. The incidence of serious complications was significantly higher in the AKI group than in the non‐AKI group (p < 0.05). The postoperative mechanical ventilation time (p < 0.05) and hospital stay (p = 0.019) were significantly longer in the AKI group. There was no evidence of chronic kidney disease (CKD) in any surviving infants within 1 year after surgery. In conclusion, AKI is common in infant LRLT (60.2%), and preoperative transfusion and lower SCr levels were independently associated with AKI. AKI may be associated with the incidence of serious complications, prolonged use of ventilators, and hospital stays. No CKD occurred within 1 year.

Reduced-Size Left Lateral Segment Grafts in Infants Weighing

Live donor liver transplantation for infants weighing <10 kg has unique complexities, as patient/graft size discrepancies may cause vascular perfusion deficiencies. Failure of the abdominal closure further complicates this already challenging procedure. To overcome these potential problems, several techniques for graft size reduction-either anatomic or nonanatomic-have been proposed in the literature. Technically easier and less time-consuming, nonanatomic size reductions have the advantage of avoiding the risk of injury to the portal pedicle. This study aimed to evaluate and compare the effects of nonanatomic graft size reduction in infants weighing <10 kg with a large estimated preoperative graft recipient weight ratio. We enrolled 106 infants weighing <10 kg. Of these infants, 50 received reduced-size grafts. The outcomes were compared between the groups. No difference was observed between the groups according to survival and vascular or biliary complications. None of the patients required an open abdomen or mesh closure. Nonanatomic size reduction of left lateral segment grafts can be safely applied without compromising vascular supply, graft function, and patient survival with comparable vascular and biliary complication rates. This technique is safe and efficient in overcoming the complications caused by large-for-size syndrome in infants weighing <10 kg.

Comparative analysis of whole vs. split liver transplantation in infants.

Liver transplantation (LT) in infants can be challenging due to their small size and small vasculature. Although both whole LT (WLT) and split LT (SLT) have been described in infants, the head-to-head comparison of these techniques in this population is sparse. We retrospectively analyzed the records of all patients with age ≤1 year at Indiana University between 2016 and 2022. All SLT were left lateral segment grafts split in situ. A total of 24 infants were transplanted, with 11 SLT and 13 WLT. The median follow-up time was 52.1 months. Donor and recipient characteristics were comparable except for donor age (19 years vs. 2 years; p < .01) and weight (64 kg vs. 14.2 kg; p < .01). Early allograft dysfunction, primary nonfunction, and hepatic artery thrombosis developed more frequently in the WLT group. There were no biliary complications. There were two early deaths (2 and 4 days) in the WLT group. One-year graft survival (100% vs. 77%; p = .10) and patient survival (100% vs. 85%; p = .18) were numerically higher in the SLT group. SLT with LLS offers a safe and viable option for liver transplantation in infants and is associated with a trend toward superior outcomes. SLT should be considered as a strategy to reduce waitlist times for infants in the absence of small, deceased donors for WLT.

Biliary drainage surgery before or after 3 months of life versus primary liver transplantation in children with biliary atresia: comparative cohort study.

Early biliary drainage surgery (BDS; Kasai) is associated with longer transplant-free survival in biliary atresia. However, evidence is lacking on whether an age limit can be established at which liver transplantation should be performed as first-line treatment for children with a delayed diagnosis of biliary atresia. The aim of the current study was to compare the outcome of a large cohort of children with biliary atresia who underwent BDS after 90 days of life with those who underwent early BDS (before 90 days) and those who did not receive BDS and were directly referred for primary liver transplantation. All patients with biliary atresia treated at Bicêtre, Paris-Saclay University Hospital between 1995 and 2017 were analysed in this STROBE-compliant study. Three groups were defined: BDS before 90 days of life (early BDS); BDS after 90 days of life (late BDS); and patients without BDS who were referred for primary liver transplantation (no BDS). Patient characteristics, overall survival, and native liver survival were compared. Of 424 children with biliary atresia, 69 patients (16 per cent) were older than 90 days when they underwent BDS. Twenty-five patients had no BDS and were referred for primary liver transplantation (6 per cent). The main reason for not performing BDS was manifest portal hypertension (18/25). Two- and 5-year transplant-free survival were significantly higher in patients with late BDS compared with no BDS (53.5 versus 12.0 per cent respectively for 2-year data and 30.4 versus 4.0 per cent respectively for 5-year data, P < 0.001). Five- and 10-year overall survival did not differ between early BDS (92 and 91 per cent respectively), late BDS (88 and 83 per cent respectively) and no BDS (80 and 80 per cent respectively, P = 0.061). Age alone should not routinely be considered a contraindication to BDS in patients older than 90 days. Liver transplantation in infancy (less than 12 months) could be avoided in 88 per cent of cases with late diagnosis of biliary atresia and is delayed significantly even when BDS is performed after 3 months. Overall survival is at least equal to patients who are referred for primary liver transplantation.

Impact of muscle mass on the prognosis of liver transplantation for infants with biliary atresia.

Sarcopenia in adult cirrhotic patients is associated with increased morbidity and mortality whereas in children it is still being studied. Anthropometric variables in cirrhotic children are not reliable for assessing muscle mass as they may be altered by ascites, edema, and organomegaly. Measuring the area of the psoas showed good correlation with muscle mass in adults. We aimed to study in cirrhotic infants undergoing liver transplantation the association of the psoas area with liver transplant prognosis as well as with several analytical and anthropometric parameters used to evaluate nutritional status. Retrospective cohort of 29 infants with cirrhosis due to biliary atresia who underwent abdominal CT scan as a pre-transplant study. We measured the psoas muscle index (PMI) at L4-L5 since it best correlates with muscle mass in pediatric patients. As there are no validated cut-off points to define sarcopenia in children under one year of age, PMI was recorded as a continuous variable and correlated with different prognostic, clinical, and analytical variables. The SPSS 17.0 package was used for statistical analysis and a P < 0.05 was considered significant. 29 infants (10 boys, 19 girls) were studied. 62% were Caucasian and the rest were South American. The mean age at CT scan was 8.5 months (range 3-15 months). There was a negative correlation between PMI and days of admission prior to liver transplant, previous infections, and bone fractures. Among the analytical parameters, cholinesterase, albumin, and prealbumin correlated positively with PMI (P < 0.05). No relationship was observed with anthropometric parameters: weight, height, BMI, brachial perimeter, or bioimpedance. During surgery, patients with lower PMI had a greater need for plasma transfusion, and in the immediate postoperative period, there was a longer stay in intensive care, more days of mechanical ventilation, and more days of hospital admission (P < 0.05). On the contrary, no relationship was found with other complications: bleeding, re-interventions, biliary leaks, rejection, thrombosis, re-transplantation, or infections. The decrease in muscle mass is associated with increased morbidity in infants with biliary atresia undergoing liver transplantation. Muscle mass in these patients cannot be adequately assessed with anthropometric measurements commonly used in the clinic.

Doppler evaluation of hepatic hemodynamics after living donor liver transplantation in infants.

Objective: The aim of this study was to explore the hemodynamic changes of hepatic artery and portal vein detected by Doppler ultrasound (DU) in infants who underwent living donor liver transplantation (LDLT). Methods: The data of 41 infant patients (22 Males, 19 Females, median age of 5 months) were collected in the Children’s Hospital affiliated to the Chongqing Medical University from May 2018 to December 2019. The patients underwent left lateral segment LDLT (LLS -LDLT) because of biliary atresia (BA). Hemodynamic parameters, including the peak systolic velocity (PSV), resistivity index (RI) of the hepatic artery (HA), portal vein velocity (PVV), and portal vein flow (PVF) were recorded from Doppler ultrasound on the day before the operation, and on the 1st, the 7th, the 14th and the 30th day after LDLT procedures. The changes of PSVHA, RIHA, PVV and PVF before and on the 1st day after transplantation were analyzed by paired t-test. The comparison of the data between different postoperative time points were assessed by ANOVA. Results: Compared with the parameters measured before LDLT, PSVHA, and RIHA decreased, and PVV and PVF increased significantly (p < 0.001) on the 1st day after LLS-LDLT. As for PSV, there was no significant difference between the 7th day and the 1st day after transplantation (POD7 VS POD1, p = 0.167) while there was a substantial difference between the 14th, 30th and 1st day after LT (POD14 vs. POD1, p = 0.003) (POD30 vs. POD1, p < 0.001). And there was a significant difference between the 14th, 30th, and 7th days after LT (POD14 vs. POD7, p = 0.014) (POD30 vs. POD7, p < 0.001). There was no significant difference between 30th and 14th after transplantation (POD30 vs. POD14, p = 0.092). As for RIHA and PVV, the decrease was slow within the first month after the operation, and there was no significant difference at different times. Conclusion: We have identified major hepatic flow changes that occurred in 41 infants who underwent LLS -LDLT due to BA. The data could be used for future studies of LDLT in infants including hemodynamic modeling, liver regeneration and clinical management.

Whole liver transplantation in children under 10 kg: How to minimize the high risks of a still challenging procedure.

Whole liver transplantation in infants <10kg is a rare procedure with moderate outcomes (67%-79% graft survival at 1year) and high rates of vascular complications (hepatic artery thrombosis 5-26%). Retrospective single-center analysis of whole liver transplantation in infants <10kg and systematic review of the literature focused on survival rates and surgical complications. Between January 2005 and December 2020, 175liver transplantations in 173 children were performed at our center. A total of 92 (53%) children weighed less than 10kg; 19 (21%) of them underwent WLT and constitute the study population. Median age of the recipients was 10months (21days-24months) and median body weight 6.5 (3.1-9.8) kg. Median age of the donors was 5 (1-84) months and median body weight 6.1 (4-21) kg. Median donor-to-recipient body weight ratio was 1.2 (range: 0.6-4.5). Postoperatively, neither hepatic artery nor portal vein thrombosis occurred. A biliary complication occurred in 4 cases: 1 bile leak (early), 3 anastomotic stenoses (1 delayed and 2late), and 1 non-anastomotic stenosis (late). Patient survival rate at 1, 5, and 10years was 100%, 92%, and 92%, respectively. Overall, death-censored graft survival after 1, 5, and 10 was 100%. Our results are excellent in terms of complications and graft and patient survival. This involves not only high-end surgical performance but also close interdisciplinary perioperative cooperation based on strong standard operating procedures and mainly focused on fluid management, hemostasiology, and strict monitoring of vessel patency.

Intellectual development of patients with biliary atresia who underwent living donor liver transplantation in infancy.

The impact of pediatric liver transplantation on intellectual development has yet to be determined. We investigated the intellectual outcomes of school-aged patients after living donor liver transplantation for biliary atresia in infancy. The Wechsler Intelligence Scale for Children-fourth edition test was administered to 20 patients who survived [Formula: see text] 5years after living donor liver transplantation. Borderline full scale intelligence quotient was defined as ≤ 85. Pre-, peri-, and postoperative data were compared between patients with > 85 and ≤ 85 to identify predictive factors of borderline performance. The one-sample t test demonstrated that the mean full scale intelligence quotient of patients after transplantation for biliary atresia was significantly lower than that of the general population (91.8 vs. 100.0, p = 0.026) and 7 (35%) were classified as intellectual borderline functioning. Multivariable logistic regression models were unable to identify any factors predictive of full scale intelligence quotients of ≤ 85. This is the first study to indicate that the mean full scale intelligence quotient among school-aged patients who underwent living donor liver transplantation for biliary atresia in infancy is significantly lower than that of the general population.

Acute respiratory distress after liver transplantation in infants-looking beyond infection and hepatopulmonary syndrome: A brief report.

Acute respiratory distress after liver transplantation in infants-looking beyond infection and hepatopulmonary syndrome: A brief report.

Third retransplantation using a whole liver graft for late graft failure from hepatic vein stent stenosis in a pediatric patient who underwent split liver retransplantation

We present a case of third retransplantation using a whole liver graft in a 13-year-old girl who suffered graft failure and hepatopulmonary syndrome following split liver retransplantation with endovascular stenting of the hepatic and portal veins as an infant. She was diagnosed with biliary atresia-polysplenia syndrome, and thus underwent living donor liver transplantation from her mother at 9 months of age. The first liver graft failed due to stenosis of the portal vein. She underwent the second liver transplantation with a split left lateral section graft. Endovascular stenting was performed to the portal vein stenosis 2 months and hepatic vein stenosis 9 months after transplantation. During the next 9 years, 11 sessions of balloon angioplasty for hepatic vein stent stenosis were performed. Ten years after the second transplantation, she underwent third transplantation using a whole liver graft recovered from a 12-year-old-girl. The double inferior vena cava technique was used for outflow vein reconstruction. The graft portal vein was anastomosed with the stent-containing portal vein stump because it was not possible to remove the stent and the inner diameter of the portal vein stent was large enough. An aorto-hepatic jump graft was used for arterial reconstruction. The patient recovered slowly and is doing well for 6 months posttransplant. In conclusion, because stenting of the hepatic vein or portal vein can induce graft failure leading to late retransplantation, we emphasize secure vascular reconstruction to prevent endovascular stenting during liver transplantation in infants.

SCYL1 disease and liver transplantation diagnosed by reanalysis of exome sequencing and deletion/duplication analysis of SCYL1

SCYL1 disease results from biallelic pathogenic variants in SCYL1. We report two new patients with severe hepatic phenotype requiring liver transplantation. Patient charts reviewed. DNA samples and skin fibroblasts were utilized. Literature was reviewed. 13-year-old boy and 9-year-old girl siblings had acute liver insufficiency and underwent living related donor liver transplantation in infancy with no genetic diagnosis. Both had tremor, global developmental delay, and cognitive dysfunction during their follow-up in the medical genetic clinic for diagnostic investigations after their liver transplantation. Exome sequencing identified a likely pathogenic variant (c.399delC; p.Asn133Lysfs*136) in SCYL1. Deletion/duplication analysis of SCYL1 identified deletions of exons 7-8 in Patient 1. Both variants were confirmed in Patient 2 and the diagnosis of SCYL1 disease was confirmed in both patients at the age of 13 and 9 years, respectively. SCYL1 protein was not expressed in both patients' fibroblast using western blot analysis. Sixteen patients with SCYL1 disease reported in the literature. Liver phenotype (n = 16), neurological phenotype (n = 13) and skeletal phenotype (n = 11) were present. Both siblings required liver transplantation in infancy and had variable phenotypes. Exome sequencing may miss the diagnosis and phenotyping of patients can help to diagnose patients.

Impact of prolonged clamping of the portal vein during liver transplantation in infants with biliary atresia: renewed interest in a long-standing issue.

Various approaches to portal vein (PV) reconstruction have been described; however, there is still scope to improve the safety and efficiency of how PV anastomosis is performed in infants. We analyzed, retrospectively, the predictive factors for PV reanastomosis during living donor liver transplantation (LDLT) in 117 infants (< 1year old) with biliary atresia (BA). Twenty-six infants required PV reanastomosis because of intraoperative PV thrombosis in 21 and insufficient PV flow in 5. Univariate analysis identified multiple previous laparotomies and a prolonged anhepatic phase (AHP) as significant risk factors for repeated PV anastomosis. The AHP cutoff value for identifying patients requiring PV reanastomosis was 134min. Multivariate analysis revealed prolonged AHP > 134min (odds ratio, 15.98; 95% confidence interval, 5.05-50.6; P < 0.001) as an independent risk factor for repeated PV anastomosis. The plasma D-dimer and fibrinogen degradation product concentration just after reperfusion were significantly higher in patients requiring PV reanastomosis (P < 0.001). Patients requiring reanastomosis of the PV had a higher incidence of PV complications after LDLT than those who did not need PV reanastomosis (P = 0.01). Attempting to minimize the AHP is indispensable for successful LDLT in infants with BA.

Quantification of Serum Matrix Metallopeptide 7 Levels May Assist in the Diagnosis and Predict the Outcome for Patients with Biliary Atresia

To assess the diagnostic and prognostic usefulness of the serum matrix metallopeptidase-7 (MMP-7) level for biliary atresia in infants with cholestasis after hepatoportoenterostomy. We enrolled 100 infants with cholestasis (age, 43.56±1.97days; 62 males) with a direct bilirubin level of >1mg/dL, of whom 36 (36%) were diagnosed with biliary atresisa. The MMP-7 levels in serum samples collected during the cholestasis workup and 6months after hepatoportoenterostomy were assessed by enzyme-linked immunosorbent assay. We quantified liver fibrosis by Picro Sirius red staining of collagen in specimens from the 81 infants with cholestasis. Infants with biliary atresisa had a significantly higher serum MMP-7 level than that of non-biliary atresisa infants with cholestasis of equivalent age (P<.0001). Receiver operating characteristic analysis showed that a serum MMP-7 level of >1.43ng/mL was predictive of biliary atresisa in infants with cholestasis (diagnostic accuracy, 88%). There was a positive correlation between the serum MMP-7 level and the severity of liver fibrosis (P=.0002). Survival analysis showed that the frequency of liver transplantation was significantly higher in infants with biliary atresisa with a serum MMP-7 level of >10.30ng/mL compared with a serum MMP-7 level of ≤10.30ng/mL after hepatoportoenterostomy (hazard ratio, 4.22; P=.02). The serum MMP-7 level, which reflects the severity of liver fibrosis and can be determined noninvasively, may facilitate the diagnosis of biliary atresisa among infants with cholestasis. Moreover, the serum MMP-7 level after hepatoportoenterostomy is associated with a need for liver transplantation in infants with biliary atresisa.

Long-term outcome and necessity of liver transplantation in infants with biliary atresia are independent of cytokine milieu in native liver and serum

PurposeBiliary atresia (BA) is a rare disease of unknown pathogenesis in infants characterized by an inflammatory, progressive destruction of the biliary system and deterioration of liver function. The standard treatment for BA is a Kasai-hepatoportoenterostomy (KPE). However, liver transplantation (LTX) becomes necessary in about 50–80% of cases. Therefore, some authors advocate for primary LTX in BA, but this would require early markers to predict which children would benefit from KPE or to show rapid progression to liver cirrhosis (RLC) instead. MethodsSnap-frozen liver biopsies and sera samples of 57 infants with BA were collected during KPE. Clinical and follow-up data were assessed via the biliary atresia and related diseases registry (BARD-online.com). Protein-levels of 25 pro- and anti-inflammatory mediators of 49 infants were assessed via multiplex protein-immunoassay and analyzed by t-test as well as multidimensional principal component analysis. Results22 different immunomodulatory mediators were detectable in livers of children with BA, while serum protein levels were very low to undetectable. Following KPE, 33 BA patients showed RLC that required early LTX, while 24 had favorable course of disease with long-term survival with native liver (SNL). There were no significant differences between RLC and SNL in terms of local (from liver samples) nor systemic (from sera) immunomodulatory mediators. Protein levels were much lower in sera than in livers without statistical correlation. ConclusionOur data suggest that local or systemic immunomodulatory mediators are unsuitable for predicting the disease course of BA. Thus, no deduction for optimal treatment strategy can be drawn. Collectively, we conclude that in BA, the degree of inflammation and protein microenvironment in the liver at the time-point of KPE are dismissible factors for the future course of disease.

Computed tomography donor liver volumetry before liver transplantation in infants ≤10 kg: does the estimated graft diameter affect the outcome?

Aim of the studyLiving donor liver transplantation (LDLT) is regularly performed in small-sized infants. Computed tomography (CT)-based donor liver volumetry is used to estimate the graft size. The aim of our study was to assess the results of CT liver volumetry and their impact on the clinical outcome after LDLT in extremely small-sized infants.Patients and methodsIn this study, we included all patients with a body weight of ≤10 kg who underwent living related liver transplantation at our centre between January 2004 and December 2014. In all cases of LDLT, a preoperative CT scan of the donor liver was performed, and the total liver and graft volumes were calculated. The graft shape was estimated by measuring the ventro-dorsal (thickness), cranio-caudal, and transversal (width) diameter of segment II/III. We assessed the impact of CT donor liver volumetry and other risk factors on the outcome, defined as patient and graft survival.ResultsIn the study period, a total of 48 living related liver transplantations were performed at our centre in infants ≤10 kg [20 male (42%), 28 female (58%)]. The mean weight was 7.3 kg (range 4.4–10 kg). Among the recipients, 33 (69%) received primary abdominal closure and 15 (31%) had temporary abdominal closure. The patient and graft survival rates were 85% and 81%, respectively. In CT volumetry, the mean estimated graft volume was 255 mL (range 140–485 mL) and the actual measured mean graft weight was 307 g (range 127–463 g). The mean ventro-dorsal diameter of segment II/III was 6.9 cm (range 4.3–11.2 cm), the mean cranio-caudal diameter was 9 cm (range 5–14 cm), and the mean width was 10.5 cm (range 6–14.7 cm). The mean graft-body weight ratio (GBWR) was 4.38% (range 1.41–8.04%). A high graft weight, a GBWR >4%, and a large ventro-dorsal diameter of segment II/III were risk factors for poorer patient survival.ConclusionPreoperative assessment of the graft size is a crucial investigation before LDLT. For extremely small-sized recipients, not only the graft weight but also the graft shape seems to affect the outcome.

Transient elastography is useful in diagnosing biliary atresia and predicting prognosis after hepatoportoenterostomy.

LSM assessment during the workup of cholestatic infants may facilitate the diagnosis of BA. LSM post-HPE may predict complications and the need for early LT in infants with BA. (Hepatology 2018).

Effect of Preoperative Growth Status on Clinical Outcomes After Living-Donor Liver Transplantation in Infants

ObjectiveThe aim of this study was to evaluate the impact of preoperative growth status on clinical outcomes of infantile living-donor liver transplantation (LDLT). MethodsFrom 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. ResultsBaseline 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/m2 vs 14.8 kg/m2; 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. ConclusionsPreoperative 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.

ABO incompatible living related liver transplantation in infants: a report of three cases

ABO incompatible living related liver transplantation in infants: a report of three cases

The Vascular Reconstruction for Post-Kasai Biliary Atresia Splenic Malformation in Living Donor Liver Transplantation.

Aims: Biliary atresia (BA) is a major indication for liver transplantation in infants. These infants have a high frequency of associated malformations known as biliary atresia splenic malformation (BASM) syndrome. Anatomical abnormalities in patients with BA often include polysplenia, preduodenal portal vein, interrupted retrohepatic inferior vena cava, cardiac abnormalities and situs inversus. In living donor liver transplant (LDLT) patients who had congenital vascular anomalies that compounded the technical hazards of the operation, additional surgical modifications for the vascular reconstruction are sometimes necessary to achieve satisfactory liver perfusion. We aimed to evaluate outcomes of patients with BASM syndrome. Methods: A retrospective analysis was performed on medical records of LDLT patients. Patients with a diagnosis of BA were identified. Results: Between 1991 and 2013, 82 LDLTs for post-Kasai BA were performed at Tohoku University Hospital. There were 49 female (59.8%) and 33 male (40.2%) pediatric recipients. Mean age at transplant was 1.1±9.6 years (5 months-44 years), with 50 children (61%) being less than 2 years of age at LDLT. The overall 1-, 5-, and 10-year patient survival in these 82 LDLT patients with BA was 90.2%, 89.0%, and 87.4%, respectively. Of these, 3 patients (3.7%) had BASM syndrome: absence of retrohepatic vena cava (3), intestinal malrotation (2), preduodenal portal vein (1), hepatic artery anomalies (0), cardiac anomalies (1), and situs inversus (1). LDLTs were performed at the patient age of 8 months to 10 years. A right liver lobe without middle hepatic vein was used in 1 case and a left liver lobe with MHV was used in 2 cases. A superficial femoral vein as a vascular graft was obtained from living donor and used for middle and inferior hepatic veins reconstruction in 1 case. In a left liver graft, we created single larger orifice using recipient's LHV, MHV, and RHV, and then reconstructed the LHV and MHV of the donor graft directly to this orifice without vascular conduits. There were no vascular complications after transplantation. All patients were alive and well at a median follow-up of 57 months. Conclusions: LDLT can be successfully performed in pediatric patients with BASM syndrome. Complex vascular anomalies associated with the use of partial liver grafts obtained from living donors are not associated with an increased occurrence of vascular complications.