Primary liver transplantation vs transplant after Kasai portoenterostomy in children with biliary atresia: A retrospective Brazilian single-center cohort

BACKGROUND Biliary atresia(BA)is the most common indication for pediatric liver transplantation,although portoenterostomy is usually performed first.However,due to the high failure rate of portoenterostomy,liver transplantation has been advocated as the primary procedure for patients with BA.It is still unclear if a previous portoenterostomy has a negative impact on liver transplantation outcomes.AIM To investigate the effect of prior portoenterostomy in infants un-dergoing liver transplantation for BA.METHODS This was a retrospective cohort study of 42 pediatric patients with BA who underwent primary liver transplantation from 2013 to 2023 at a single tertiary center in Brazil.Patients with BA were divided into two groups:Those undergoing primary liver transplantation without portoenterostomy and those undergoing liver transplantation with prior portoenterostomy.Continuous variables were compared using the Student’s t-test or the Kruskal-Wallis test,and categorical variables were compared using theχ2 or Fisher’s exact test,as appropriate.Multivariable Cox regression analysis was performed to determine risk factors for portal vein thrombosis.Patient and graft survival analyses were conducted with the Kaplan–Meier product-limit estimator,and patient subgroups were compared using the two-sided log-rank test.RESULTS Forty-two patients were included in the study(25[60%]girls),23 undergoing liver transplantation without prior portoenterostomy,and 19 undergoing liver transplantation with prior portoenterostomy.Patients with prior portoenterostomy were older(12 vs 8 months;P=0.02)at the time of liver transplantation and had lower Pediatric End-Stage Liver Disease scores(13.2 vs 21.4;P=0.01).The majority of the patients(35/42,83%)underwent livingdonor liver transplantation.The group of patients without prior portoenterostomy appeared to have a higher incidence of portal vein thrombosis(39 vs 11%),but this result did not reach statistical significance.Prior portoenterostomy was not a protective factor against portal vein thrombosis in the multivariable analysis after adjusting for age at liver transplantation,graft-to-recipient weight ratio,and use of vascular grafts.Finally,the groups did not significantly differ in terms of post-transplant survival.CONCLUSION In our study,prior portoenterostomy did not significantly affect the outcomes of liver transplantation.

Congenital biliary atresia caused by GPC1 gene mutation in Chinese siblings:A case report

BACKGROUND Congenital biliary atresia(CBA)is a serious hepatobiliary disease in children with unknown etiology.Its outcome is often liver transplantation or death.Clarifying the etiology of CBA is of great significance for prognosis,treatment,and genetic counseling.CASE SUMMARY A male Chinese infant at an age of 6 mo and 24 d was hospitalized because of"yellow skin for more than 6 mo".Soon after birth,the patient developed jaundice,which then progressively intensified.A"laparoscopic exploration"indicated"biliary atresia".After coming to our hospital,genetic testing suggested a GPC1mutation[loss 1(exons 6-7)].The patient recovered and was discharged after living donor liver transplantation.After discharge,the patient was followed up.The condition was controlled by oral drugs,and the patient’s condition was stable.CONCLUSION CBA is a complex disease with a complex etiology.Clarifying the etiology is of great clinical importance for treatment and prognosis.This case reports CBA caused by a GPC1 mutation,which enriches the genetic etiology of biliary atresia.However,its specific mechanism needs to be confirmed by further research.

Clinical Assessment of Differential Diagnostic Methods in Infants with Cholestasis due to Biliary Atresia or Non-Biliary Atresia

The different methods in differentiating biliary atresia（BA） from non-BA-related cholestasis were evaluated in order to provide a practical basis for a rapid,early and accurate differential diagnosis of the diseases.396 infants with cholestatic jaundice were studied prospectively during the period of May 2007 to June 2011.The liver function in all subjects was tested.All cases underwent abdominal ultrasonography and duodenal fluid examination.Most cases were subjected to hepatobiliary scintigraphy,magnetic resonance cholangiopancreatography（MRCP） and a percutaneous liver biopsy.The diagnosis of BA was finally made by cholangiography or histopathologic examination.The accuracy,sensitivity,specificity and predictive values of these various methods were compared.178 patients（108 males and 70 females with a mean age of 58±30 days） were diagnosed as having BA.218 patients（136 males and 82 females with a mean age of 61±24 days） were diagnosed as having non-BA etiologies of cholestasis jaundice during the follow-up period in which jaundice faded after treatment with medical therapy.For diagnosis of BA,clinical evaluation,hepatomegaly,stool color,serum gamma-glutamyltranspeptidase（GGT）,duodenal juice color,bile acid in duodenal juice,ultrasonography（gallbladder）,ultrasonography（griangular cord or strip-apparent hyperechoic foci）,hepatobiliary scintigraphy,MRCP,liver biopsy had an accuracy of 76.0%,51.8%,84.3%,70.0%,92.4%,98.0%,90.4%,67.2%,85.3%,83.2% and 96.6%,a sensitivity of 83.1%,87.6%,96.1%,73.7%,90.4%,100%,92.7%,27.5%,100%,89.0% and 97.4%,a specificity of 70.2%,77.5%,74.8%,67.0%,94.0%,96.3%,88.5%,99.5%,73.3%,75.4% and 94.3%,a positive predictive value of 69.0%,72.6%,75.7%,64.6%,92.5%,95.7%,86.8%,98.0%,75.4%,82.6% and 98.0%,and a negative predictive value of 83.6%,8.5%,95.9%,75.7%,92.3%,100%,84.2%,93.7%,100%,84.0% and 92.6%,respectively.It was concluded that all the differential diagnosis methods are useful.The test for duodenal drainage and elements is fast and accurate.It is helpful in the differential diagnosis of BA and non-BA etiologies of cholestasis.It shows good practical value clinically.Key words

The diagnostic value of high-frequency ultrasonography in biliary atresia

BACKGROUND: It is a globally challenging problem to differentially diagnose biliary atresia (BA) from other disease processes causing infantile cholestatic jaundice. The high frequency ultrasonography (HUS) yields much improved spatial resolution and therefore, might show better image in BA diagnostic examination. The present study was to evaluate the HUS on the diagnosis of BA in infants with jaundice. METHODS: Fifty-one infants with neonatal jaundice were scanned with ultrasonography. Images included gallbladder, bile duct, right hepatic artery (RHA), portal vein (PV) and triangular cord (TC) sign, magnetic resonance imaging and additionally laboratory tests and histopathology reports were assessed. RESULTS: Twenty-three BA and 28 non-BA cases were con firmed. The sensitivity, specificity, and accuracy of HUS were 91.3%, 92.9%, and 92.2%, respectively. All of these indices were significantly higher than those of conventional ultrasonography (P【0.01) and MR cholangiopancreatography (P【0.05). The HUS features, included a positive TC sign, an increased RHA diameter and RHA-diameter to portal-vein-diameter ratio (RHA/PV) and abnormal gallbladder, were important in the diagnosis of BA. CONCLUSION: HUS provided better imaging of BA and should be considered as a primary modality in the differential diagnosis of infantile jaundice.

Outcomes in children with biliary atresia following liver transplantation

BACKGROUND: Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death. The present study aimed to evaluate the outcomes of orthotopic liver transplantation in children with biliary atresia. METHOD: We retrospectively analyzed 45 patients with biliary atresia who had undergone orthotopic liver transplantation from September 2006 to August 2012. RESULTS: The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively. CONCLUSION: With advances in surgical techniques and management, children with biliary atresia after liver transplantation can achieve satisfactory survival in China, although there remains a high risk of complications in the early postoperative period.

The pre-Kasai procedure in living donor liver transplantation for children with biliary atresia

BACKGROUND:Biliary atresia(BA) is a major cause of chronic cholestasis,a fatal disorder in infants.This study was undertaken to evaluate the safety and effectiveness of primary living donor liver transplantation(LDLT) in comparison with the traditional first-line treatment,the Kasai procedure.METHODS:We assessed 28 children with BA at age of less than two years(3-21.3 months) who had undergone LDLT in two hospitals in Southwest China during the period of 2008-2011.Eighteen children who had had primary LDLT were included in a primary LDLT group,and ten children who had undergone the Kasai operation in a pre-Kasai group.All patients were followed up after discharge from the hospital.The records of the BA patients and donors were reviewed.RESULTS:The time of follow-up ranged 12-44.5 months with a median of 31 months.The 30-day and 1-year survival rates were 85.7% and 78.6%,respectively.There was no significant difference in the 30-day or 1-year survival between the two groups(83.3% vs 90% and 77.8% vs 80%,P】0.05).The main cause of death was hepatic artery thrombosis.There were more patients with complications who required intensive medical care or re-operation in the pre-Kasai group(8,80%) than in the primary LDLT group(9,50%)(P=0.226).But no significant differences were observed in operating time(9.3 vs 8.9 hours,P=0.77),intraoperative blood loss(208.6 vs 197.0 mL,P=0.84) and blood transfusion(105.6 vs 100.0 mL,P=0.91) between the two groups.The durations of ICU and hospital stay in the primary LDLT group and pre-Kasai group were 180.4 vs 157.7 hours(P=0.18) and 27 vs 29 days(P=0.29),respectively.CONCLUSIONS:Primary LDLT is a safe and efficient management for young pediatric patients with BA.Compared with the outcome of LDLT for patients receiving a previous Kasai operation,a similar survival rate and a low rate of re-operation and intensive medical care for patients with BA can be obtained.

Use of shear wave elastography for the diagnosis and follow-up of biliary atresia: A meta-analysis

BACKGROUND Timely differentiation of biliary atresia(BA)from other infantile cholestatic diseases can impact patient outcomes.Additionally,non-invasive staging of fibrosis after Kasai hepatoportoenterostomy has not been widely standardized.Shear wave elastography is an ultrasound modality that detects changes in tissue stiffness.The authors propose that the utility of elastography in BA can be elucidated through meta-analysis of existing studies.AIM To assess the utility of elastography in:(1)BA diagnosis,and(2)post-Kasai fibrosis surveillance.METHODS A literature search identified articles that evaluated elastography for BA diagnosis and for post-Kasai follow-up.Twenty studies met criteria for meta-analysis:Eleven for diagnosis and nine for follow-up post-Kasai.Estimated diagnostic odds ratio(DOR),sensitivity,and specificity of elastography were calculated through a random-effects model using Meta-DiSc software.RESULTS Mean liver stiffness in BA infants at diagnosis was significantly higher than in non-BA,with overall DOR 24.61,sensitivity 83%,and specificity 79%.Post-Kasai,mean liver stiffness was significantly higher in BA patients with varices than in patients without,with DOR 16.36,sensitivity 85%,and specificity 76%.Elastography differentiated stage F4 fibrosis from F0-F3 with DOR of 70.03,sensitivity 96%,and specificity 89%.Elastography also differentiated F3-F4 fibrosis from F0-F2 with DOR of 24.68,sensitivity 85%,and specificity 81%.CONCLUSION Elastography has potential as a non-invasive modality for BA diagnosis and surveillance post-Kasai.This paper’s limitations include inter-study method heterogeneity and small sample sizes.Future,standardized,multi-center studies are recommended.

SOX9 in biliary atresia: New insight for fibrosis progression

Background:Liver fibrosis is a hallmark determinant of morbidity in biliary atresia(BA)even in successfully operated cases.Responsible factors for this rapid progression of fibrosis are not completely defined.Aberrant expression of the transcription factor SOX9 and hepatic progenitor cells(HPCs)proliferation have roles in fibrogenesis in cholestatic disorders.However,they were not investigated sufficiently in BA.We aimed to delineate the relation of SOX9 and HPCs to fibrosis and its progression in BA.Methods:Forty-eight patients with BA who underwent an initial diagnostic liver biopsy(LB)and consequent intraoperative LB were recruited and compared to 28 cases with non-BA cholestasis that had an LB in their diagnostic workup.Liver fibrosis,tissue SOX9 and HPC expressions were studied in both BA and non-BA-cholestasis cases.Liver fibrosis,SOX9,and HPCs’dynamic changes in BA cases were assessed.Relation of fibrosis and its progression to SOX9 and HPCs in BA was assessed.Results:SOX9 and HPCs in ductular reaction(DR)form were expressed in 100%of BA and their grades increased significantly in the second biopsy.The rapidly progressive fibrosis in BA,represented by fibrosis grade of the intraoperative LB,correlated significantly to SOX9-DR and HPC-DR at the diagnostic(r=0.420,P=0.003 and r=0.405,P=0.004,respectively)and the intraoperative(r=0.460,P=0.001 and r=0.467,P=0.001,respectively)biopsy.On the other hand,fibrosis,SOX9-DR,and HPC-DR were significantly lower in non-BA cases at a comparable age(P<0.001,P=0.006,and P=0.014,respectively).Conclusions:Fibrosis in BA is rapidly progressive within a short time and is significantly correlated to SOX9 and HPCs.Assessment of targeting SOX9 and HPCs on fibrosis progression is warranted.

MR Cholangiography and Dynamic Examination of Duodenal Fluid inthe Differential Diagnosis between Extrahepatic Biliary Atresia and Infantile Hepatitis Syndrome

In order to evaluate the value of magnetic resonance cholangiography （MRC） and dynamic examination of duodenal fluid in the differential diagnosis between extrahepatic biliary atresia （EHBA） and infantile hepatitis syndrome （IHS）, 52 patients with infantile cholestatic jaundice were examined by MRC and duodenal fluid examination. Original interpretations were compared with clinical outcome. Calculated sensitivity of duodenal fluid examination in diagnosis of EHBA was 100 %, and specificity was 91.1%. Sensitivity of MRC in the diagnosis of EHBA was 94.4 % and specificity 88.24 %. The sensitivity of MRC and examination of duodenal fluid combined in diagnosis of EHBA was 94.4 % and specificity 97.06 %. We are led to conclude that MRC and dynamic examination of duodenal fluid are useful in the differential diagnosis between IHS and EHBA and the combined use of the two techniques yield better resutls.

Diagnosis and treatment of biliary atresia: a retrospective study

BACKGROUND: Biliary atresia (BA) is the end results of an inflammatory process, which affect the intrahepatic and extrahepatic bile ducts, leading to fibrosis and obliteration of the biliary tract with the development of biliary cirrho- sis. Early diagnosis of BA is difficult, and there is no speci- fic therapy for BA at present. The purpose of this study was to investigate the diagnosis, treatment and postoperative outcome of BA and to explore new diagnostic and thera- peutic strategies. METHODS; Thirty-one children with biliary atresia from our hospital and 201 children with such disease from other hospitals in China were reviewed retrospectively in terms of diagnostic modalities, operative age, operative methods and long-term survival rates after operation. RESULTS : The operative age of the 31 patients varied from 13 to 270 days. It was <60 days in 6 children (19% ), 61-90 days in 14 (45% ), 91-120 days in 8 (26% ), and >120 days in 3 ( 10% ). No children underwent transplantation for BA. Their 1-6 year survival rate on average was 32. 3% (10/31). The early operative rate in China was 12.9% (30/ 232). One child received liver transplantation with a long- term survival rate of 31.9%. CONCLUSIONS; Early recognition of babies with BA is critical for optimal intervention for preventing progressive fibrosis. Clinical presentation, imaging and laparoscopy are helpful in enhancing the early diagnostic rate of BA pa- tients. The long-term survival rates can be obtained after the improvement of operative methods, liver transplanta- tion for children with advanced BA, and development of other medical strategies.

Living donor liver transplantation in 43 children with biliary atresia: a single-center experience from the mainland of China

BACKGROUND: There is no large-cohort report on living donor liver transplantation (LDLT) for biliary atresia (BA) patients from the mainland of China. This single-center study describes our initial experience with 43 LDLTs for BA patients aged two years or younger. METHODS: In this study, the eligibility criteria were BA as the primary diagnosis and two years of age or younger. From October 2006 to December 2010, the clinical data of 43 LDLTs, including pre-operative evaluations, surgical techniques, postoperative complications and outcomes of donors and recipients, were retrospectively analyzed. RESULTS: Donor graft type was the left lateral segment with compatible ABO blood groups. Forty-three recipients were selected in this study. The median patient age at operation was 9 months (range 6-24), and the median body weight was 8 kg (range 5.7-12.5). Fourteen (32.6%) recipients received Kasai operations before liver transplantation. The overall one- and two-year cumulative survival rates for grafts and recipients were 81%, 81% and 76%, 76%, respectively. No donor mortality was encountered, with a minimal morbidity and no long- term sequelae. Nine out of 43 recipients died. Postoperative complications of recipients were biliary leakage and refluxing cholangitis (11/43, 25.6%), hepatic artery thrombosis (4, 9.3%), pulmonary infections (4, 9.3%), portal vein thrombosis (3, 7.0%), wound disruption (3, 7.0%), acute rejection (3, 7.0%), cytomegalovirus infection (2, 4.7%), and intra-abdominal bleeding (1, 2.3%).CONCLUSION: Despite the relatively low survival rates due to lack of experience initially, LDLT still provides encouraging outcomes for pediatric recipients with BA, even small children under two years old.

Preoperative hepatic hemodynamics in the prediction of early portal vein thrombosis after liver transplantation in pediatric patients with biliary atresia

BACKGROUND： Portal vein thrombosis （PVT） is one of the main vascular complications after liver transplantation （LT）, especially in pediatric patients with biliary atresia （BA）. This study aimed to assess the preoperative hepatic hemodynamics in pediatric patients with BA using Doppler ultrasound and determine whether ultrasonographic parameters may predict early PVT after LT.METHODS： One hundred and twenty-eight pediatric patients with BA younger than 3 years of age underwent Doppler ultra- sound within seven days before LT, between October 2006 and June 2013. The preoperative hepatic hemodynamic parameters were then compared between patients with early PVT （within 1 month following LT） and those without PVT. Receiver oper- ating characteristic analysis was performed to determine the optimal cutoff value for predicting early PVT. RESULTS： Of the 128 transplant recipients, 41 （32.03%） had a hypoplastic portal vein （PV）, 52 （40.63%） had hepatofugal PV flow and 40 （31.25%） had a high hepatic artery resistance index （HARI） of 〉1. Nine cases （7.03%） experienced early PVT. A PV diameter 〈4 mm （sensitivity 88.89%, specificity 72.27%）, and a hepatofugal PV flow （sensitivity 77.78%, specificity 62.18%） with a high HARI ≥1 （sensitivity 77.78%, specificity 72.27%） were hepatic hemodynamic risk factors for early PVT.（ CONCLUSIONS： Hepatic hemodynamic disturbances in pediatric recipients with BA were more common. Small PV diameter （≤4 mm） and hepatofugal PV flow combined with high HARI （≥1） are strong warning signs of early PVT after LT in pediatric patients with BA. Intense monitoring of vascular patency and prophylactic thrombolytic therapy should be considered in pediatric patients undergoing LT for BA.

Laparoscopic Kasai portoenterostomy can be a standard surgical procedure for treatment of biliary atresia

BACKGROUND Biliary atresia(BA)is a rare pediatric disease.AIM To compare the outcomes of laparoscopic portoenterostomy(Lap-PE)with those of laparotomy(Open-PE)at a single institution.METHODS The surgical outcomes of PE were retrospectively analyzed for patients with a non-correctable type of BA from 2003 to 2020.RESULTS Throughout the assessment period,119 patients received PE for BA treatment,including 66 Open-PE and 53 Lap-PE cases.Although the operation duration was longer(medians:for Open-PE,242 min;for Lap-PE,341 min;P<0.001),blood loss was considerably less(medians:for Open-PE,52 mL;for Lap-PE,24 mL;P<0.001)in the Lap-PE group than in the Open-PE group.The postoperative recovery of the Lap-PE group was more favorable;specifically,both times to resume oral intake and drain removal were significantly shorter in the Lap-PE group.Complete resolution of jaundice was observed in 45 Open-PE cases and 42 Lap-PE cases,with no statistically significant difference(P=0.176).Native liver survival rates were>80%for both groups for the first half year post surgery,followed by a gradual decrease with time;there were no statistically significant differences in the native liver survival rates for any durations assessed.CONCLUSION Lap-PE could be a standard therapy for BA.

Living related liver transplantation for an infant with biliary atresia

Objective: To sum up the preliminary experience in living related liver transplantation (LRLT). Methods: A 9-month-old male infant with biliary atresia (BA) who had undergone an unsuccessful Kasai operation was defined as a candidate for LR- LT. The donor was his 30-year-old mother. Her la- teral lobe of the left liver was transplanted into the infant's body as the graft. The left branches of the portal vein, left hepatic artery and left hepatic vein of the graft were end-to-end anastomosed to the por- tal vein, hepatic artery proper and hepatic vein of the recipient respectively. Biliary drainage was re- established via Roux-en-Y operation. Results: The donor retained her liver function within 2 weeks after the operation. Steroid and FK506 were prescribed in immunosuppressive therapy for the re- cipient. The blood bilirubin level of the infant de- creased to normal 2 weeks after operation. No acute rejection occurred. Biliary leakage in the early peri- od after the transplantation was controlled by drain- age, and E. coli infection was effectively treated with antibiotics. The donor and recipient are in satisfacto- ry condition to the present. Conclusion: LRLT is advisable for children with bili- ary atresia.

Application of narrative nursing in the families of children with biliary atresia:A retrospective study

BACKGROUND Narrative nursing is an important clinical nursing intervention model.It is the practice of patient storytelling to share the essence of nursing.The current clinical intervention for biliary atresia(BA)mainly focuses on disease treatment and does not pay enough attention to the psychological state of family members.AIM To explore the application value of narrative nursing in the families of children with BA.METHODS Sixty-four family members of children with BA in our hospital from December 2017 to October 2020 were retrospectively included and were divided into a study group(n=32)and a control group(n=32).The control group was provided with routine nursing,while the study group was given narrative nursing on the basis of the control group.The scores of mood state(depression and anxiety),family members’nursing ability,perceived stress,and nursing job satisfaction of the children’s families were calculated before and after the intervention.RESULTS Before intervention,there was no significant difference in the self-rating anxiety scale and self-rating depression scale scores between groups(P>0.05).After intervention,the self-rating anxiety scale and self-rating depression scale scores in the study group were lower than those in the control group(both P=0.000).Before intervention,the study group adjusted life to meet care needs,evaluated family members and social resources,dealt with personal emotions,responded to needs,and provided assistance,and the adaptive care role scores were not significantly different from those in the control group(P=0.802,0.819,0.694,0.796,and 0.686,respectively).After intervention,all scores were significantly lower in the study group than in the control group(all P<0.0001).Before intervention,there was no significant difference in the child post-traumatic stress disorder symptom score(CPSS)score between groups(P=0.615).After intervention,the CPSS scores were significantly lower than those before intervention in both groups and lower in the study group than in the control group(P<0.0001).Nursing job satisfaction of the family members of the study group(93.75%)was higher than that of the control group(75.00%)(P=0.039).CONCLUSION Narrative nursing with family members of children with BA can effectively alleviate negative emotions,reduce perceptual pressure,and improve nursing ability.Additionally,family members are more satisfied with nursing work.

Management of biliary atresia:To transplant or not to transplant

Kasai procedure(KP)and liver transplantation(LT)represent the only therapeutic options for patients with biliary atresia(BA),the most common indication for LT in the pediatric population.However,KP represents by no means a radical option but rather a bridging one,as nearly all patients will finally require a liver graft.More and more experts in the field of transplant surgery propose that maybe it is time for a paradigm change in BA treatment and abandon KP as transplantation seems inevitable.Inadequacy of organs yet makes this option currently not feasible,so it seems useful to find ways to maximize the efficacy of KP.In previous decades,multiple studies tried to identify these factors which opt for better results,but in general,outcomes of KP have not improved to the level that was anticipated.This review provides the framework of conditions which favor native liver survival after KP and the ones which optimize a positive LT outcome.Strategies of transition of care at the right time are also presented,as transplantation plays a key role in the surgical treatment of BA.Future studies and further organization in the transplant field will allow for greater organ availability and better outcomes to be achieved for BA patients.

Neonatal biliary atresia combined with preduodenal portal vein:A case report

BACKGROUND Congenital biliary atresia is a type of obstruction of the bile ducts inside and outside the liver,which can lead to cholestatic liver cirrhosis and eventually liver failure.The preduodenal portal vein(PD-PV)is a rare developmental malformation of the PV.The PV courses in front of the duodenum.However,very few cases of neonatal biliary atresia combined with PD-PV have been reported in the scientific literature.CASE SUMMARY A 1-mo-and-4-d-old child was admitted to the hospital in January because of yellowish skin.After surgical consultation,surgical intervention was recommended.The child underwent Hilar-jejunal anastomosis,duodenal rhomboid anastomosis,and abdominal drainage under general anesthesia.During the operation,the PV was located at the anterior edge of the duodenum.CONCLUSION Diagnoses:(1)Congenital biliary atresia;(2)PD-PV;and(3)Congenital cardiovascular malformations.Outcomes:Recommendation for liver transplantation.Lessons:The choice of treatment options for neonatal biliary atresia combined with PD-PV.

Liver Transplantation in a Patient with Situs inversus Associated with Biliary Atresia, Polysplenia and Absence of Inferior Vena Cava: A Case Report

Biliary atresia (BA) is the result of a process of unknown etiology which can occur as an isolated lesion or in association with several congenital abnormalities and remains the main indications of liver transplantation among infants. One of the malformations associated with biliary atresia is Situs inversus totalis, which is a rare congenital abnormality found in 10% - 20% of infants with biliary atresia and presents an incidence varying from 1:5000 to 1:20,000. This case report aims to present an extremely rare case of a patient with Situs inversus associated with biliary atresia, polysplenia and abscence of inferior vena cava that underwent liver transplantation. Case Report: A 10-month-old boy was referred to our hospital with the diagnosis of cirrhosis, due to biliary atresia. Abdominal ultrasound and Doppler scan showed the liver in the left upper quadrant as well as signs of chronic liver disease. In addition, we could notice that the spleen had several lobes, suggesting polysplenia, and was positioned in the right upper quadrant. The patient underwent living donor liver transplant. His mother was the donor and the graft, segment III, was positioned in the left upper quadrant associated with a Y en Roux reconstruction of the biliary tract. During the first post-operatory day, the patient presented signs of disseminated sepsis. Laboratory and imaging diagnostic tests were performed presenting a small intestinal malrotation associated with diffuse ischemia. The patient died at the third day of PO due to complications related to this condition. Conclusion: Any patient with Situs inversus with acute abdomen should be aggressively investigated for other structural and biochemical abnormalities. If liver transplant is indicated, the procedure can be successfully performed.

Forty-four living donor liver transplantations for children with biliary atresia

Objective To observe the outcomes of living donor liver transplantation ( LDLT) for children with biliary atresia ( BA) and to summarize clinical experiences. Methods Forty - four BA patients ( 26 boys and 18 girls) underwent LDLT between October 2006 and December 2010. Mean ( SD) and median ( range) age

The abnormal expression of E-cadherin in intrahepatic bile duct epithelia cells in biliary atresia and its relationship with apoptosis

To explore the relationship between the expression of E-cadherin and the apoptosis in intrahepatic bile duct epithelial cells in biliary atresia (BA).Methods The E-cadherin expression was demonstrated by immunohistochemical staining for the liver specimens from 38 children with BA and 16 normal children.The apoptotic intrahepatic bile duct epithelial cells in these specimens were visualized by TdT-mediated dUTP-digoxigenin nick end labeling (TUNEL) assay,and the apoptotic index (AI) was calculated from the percentage of apoptotic cells in total cells.Results The intensity of E-cadherin expression in bile duct epithelial cells in BA group was lower than that in the normal control group (0.33±0.12 vs 0.62±0.20,P<0.01).On the other hand,the AI in BA group was significant higher than that in control group (51.74±19.93 vs 12.34±19.32,P<0.01).An inverse correlation was detected between the intensity of E-cadherin and the AI in the liver from children with BA.Conclusion The abnormal decrease of E-cadherin may lead to an increase of the apoptosis of intrahepatic bile epithelial cells in BA,resulting in developmental disorder of intrahepatic bile duct and ductal plate malformation in the liver.12 refs,4 figs,1 tab.