Should We Offer Surgery for Biliary Atresia in Low-Resource Settings? Surgical Outcomes in Rwanda.

BACKGROUND: In many resource-limited settings, patients with biliary atresia present too late for surgical correction to be offered, and the diagnosis is fatal. As pediatric surgical and anesthesia capabilities have improved, patients in Rwanda have been offered surgical exploration. This study explores initial outcomes. METHODS: Patients presenting with direct hyperbilirubinemia and clinical suspicion of biliary atresia were identified at the main university teaching hospital in Kigali, Rwanda, from January 2016 to June 2019. Patient demographics, referral history, geographic location, preoperative imaging, preoperative laboratory studies, operative details, postoperative laboratory studies, in-hospital complications, length of stay, and survival were abstracted from retrospective chart review. Descriptive analysis was performed, and univariate analysis evaluated survival and complications. RESULTS: Seventeen patients were identified with biliary atresia, and thirteen were offered surgery. The median age of admission was 77 d (interquartile range [IQR] 63-92 d), with the median time wait for the operation being 19 d (IQR 9-27 d). The median age at operation was 93 d (IQR 76-123 d). In-hospital postoperative mortality was 15.4% (n = 2) and postoperative complications occurred in 46.2% (n = 6). Eleven patients survived to hospital discharge (84.6%), with a median length of stay of 8 d (IQR 6-13 d). CONCLUSIONS: While future studies are needed to evaluate the long-term outcomes, this series shows that surgical treatment of biliary atresia can be safely performed in Rwanda. Early referral of direct hyperbilirubinemia is essential, particularly as limited resources and personnel may impact the time from diagnosis to operation.

Prognostic Factors for Survival of Patients with Biliary Atresia Following Kasai Surgery.

Biliary atresia (BA) is a progressive obstruction and fibro-obliteration of the extrahepatic and intrahepatic biliary tract that causes cholestatic jaundice in infants, resulting in biliary cirrhosis and even death in the first year of life if the Kasai procedure is not performed at an earlier age. There are many prognostic factors that could affect the survival of patients with BA after Kasai surgery, however results still show some conflicting findings. A retrospective study was conducted using medical records of patients with BA who underwent Kasai surgery at Dr. Sardjito Hospital, Yogyakarta, Indonesia from June 2012 to April 2018. Twenty-nine BA patients were involved in our study, with 16 males and 13 females. Log-rank analysis showed a significant association between survival rate of BA patients with albumin level 1 month and 3 months after Kasai surgery, with p-values of 0.043 and 0.016, respectively. Interestingly, multivariate analysis revealed that cholangitis tended to have an association with BA patients' survival (p=0.09). In conclusion, the BA patients' survival might be affected by the presence of cholangitis after Kasai surgery. Further multicenter studies with a larger sample size are important to verify our results.

Perioperative Complications after Kasai Hepatoportoenterostomy: Data from the Swiss National Biliary Atresia Registry.

INTRODUCTION: Hepatoportoenterostomy (HPE) is the first-line treatment for biliary atresia (BA) patients. This study aims to describe perioperative complications after HPE and to analyze their impact on outcome. MATERIALS AND METHODS: Patients with HPE (Swiss National Biliary Atresia Registry, 1994-2017) were retrospectively analyzed. Perioperative complications were defined as complications occurring up to 30 days after surgery. Surgical complications were defined as directly related to the surgical act; medical complications were defined as any other deviation from the uneventful postoperative course. RESULTS: Sixty-two patients were included. Median age at HPE was 63 days (18-126). Twenty six patients out of 62 (42%) had ≥ 1 complications: 6/62 (10%) surgical, 24/62 (39%) medical, that is, we observed 7 surgical and 28 medical complications. As for medical complications, cholangitis was the most frequent: 19/28 (68%). Lower gestational age at birth correlated with more overall complications (p = 0.02). Age, weight at HPE, syndromic BA, and postoperative steroid administration were not significantly correlated. There were no perioperative deaths. Perioperative complications did not correlate with overall survival (p = 0.14) and survival with native liver (p = 0.55). CONCLUSION: HPE is often associated with perioperative medical complications. Lower gestational age at birth was significantly associated with more complications. Perioperative complications had no impact on overall outcome.

MULTIVARIATE ANALYSIS OF BILIARY FLOW-RELATED FACTORS AND POST-KASAI SURVIVAL IN BILIARY ATRESIA PATIENTS.

BACKGROUND: Biliary atresia represents the most common surgically treatable cause of cholestasis in newborns. If not corrected, secondary biliary cirrhosis invariably results. OBJECTIVE: To evaluate, through multivariate analysis, the prognostic factors associated with the presence of biliary flow and survival with the native liver following Kasai portoenterostomy. METHODS: The study analyzed data from 117 biliary atresia patients who underwent portoenterostomy and had suitable histological material for evaluation. A logistic regression model was used to assess the presence of biliary flow. Survival was investigated through Kaplan-Meier curves and Cox-adjusted models. RESULTS: One third of patients achieved biliary flow and the median age at surgery was 81 days. Age at surgery, albumin, postoperative complications, biliary atresia structural malformation (BASM), liver architecture, larger duct diameter at porta hepatis, and cirrhosis (Ishak score) were the initial variables for the multivariate analysis. Age at surgery >90 days was the only variable associated with the absence of biliary drainage. Survival analysis revealed that the absence of biliary flow (P<0.0001), age at surgery >90 days (P=0.035), and the presence of BASM (P<0.0001), alone, could predict death or need for liver transplantation. Multivariate analysis demonstrated that the absence of biliary flow (P<0.0001 hazard ratio [HR] 6.25, 95% confidence interval [CI] 3.19-12.22) and the presence of BASM (P=0.014 HR 2.16, 95% CI 1.17-3.99) were associated with lowest survival with the native liver. CONCLUSION: Age at surgery >90 days was associated with absence of biliary flow. The presence of biliary drainage and the absence of structural malformations are cornerstone features for higher survival rates with the native liver.

Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center.

BACKGROUND/AIMS: The present study aimed at investigating the long-term outcomes and prognostic factors of patients with biliary atresia (BA) diagnosed and followed at a single center. MATERIALS AND METHODS: Patients with BA treated during 1994-2014 at a large-volume pediatric tertiary referral center were reviewed retrospectively with regard to demographic, clinical, laboratory, and diagnostic characteristics for identifying the prognostic factors and long-term clinical outcomes. RESULTS: Overall, 81 patients (49 males, 32 females) were included. Mean age at diagnosis was 73.1±4.7 (median: 64) days. Of the patients included, 78 patients (96%) underwent a portoenterostomy procedure. Mean age at operation was 76.8±4.7 (median: 72) days. The surgical success rate was 64.8%. A younger age (either at diagnosis or surgery) was the only determinant of surgical success. The 2-, 5-, and 10-year overall survival (OS) rates, including all patients with or without liver transplantation, were 75%, 73%, and 71% respectively, whereas the 2-, 5-, and 10-year survival rates with native liver (SNL) were 69%, 61%, and 57%, respectively. Mean follow-up duration was 9.4±7.5 years. Successful surgery, presence of fibrosis and/or cirrhosis on the liver pathology, and prothrombin time [international normalized ratio (INR)] at presentation were independent prognostic factors for both OS and SNL. CONCLUSION: A younger age at diagnosis is strongly associated with surgical success in BA. Surgical success, the prothrombin time (INR) at presentation, and liver pathology are independent prognostic factors affecting the long-term outcomes in patients with BA. Therefore, timely diagnosis and early referral to experienced surgical centers are crucial for optimal management and favorable long-term results in BA.

Predictors of Need for Liver Transplantation in Children Undergoing Hepatoportoenterostomy for Biliary Atresia.

PURPOSE: The purpose of this study was to determine perioperative risk factors for need of liver transplantation following hepatoportoenterostomy. METHODS: A retrospective review of patients undergoing hepatoportoenterostomy for biliary atresia at our institution from 1990 to 2016 was completed. RESULTS: A total of 81 patients were identified with a median age of 51 days (IQR: 33-68) at hepatoportoenterostomy and a median follow-up time of 5.7 years (IQR: 1-11.6). Ten-year overall survival was 93% (95% CI: 84-97). Thirty-six patients (44%) ultimately required transplantation at a median time from hepatoportoenterostomy of 8.9 months (IQR: 5.2-19). The 10-year transplant-free survival was 36% (95%CI: 24-49). Steroid use (N=42) was not associated with improved 10-yr transplant-free survival (33% vs. 38%, p=0.690). Age at hepatoportoenterostomy was not significantly associated with the need for transplantation. Multivariable logistic regression analysis demonstrated that total bilirubin >2mg/dL (OR: 97, p<0.001) and albumin < 3.5g/dL (OR: 24, p=0.027) at 3 months after surgery were independent predictors of the need for transplantation, while adjusting for age, sex, prematurity, and steroid use. CONCLUSION: Overall survival for children with biliary atresia is excellent, although most patients will ultimately require liver transplantation. Total bilirubin and albumin level at 3 months following hepatoportoenterostomy are predictive of the need for transplantation. Steroid use is not associated with improved outcomes.

The effects of Kasai procedure on living donor liver transplantation for children with biliary atresia.

OBJECTIVE: To evaluate the effects of Kasai procedure (hepatic portoenterostomy) on living donor liver transplantation (LDLT) for children with biliary atresia (BA). METHODS: From January 2006 to January 2014, 150 children with BA were treated with LDLT in China. The children were categorized into pre-Kasai and non-Kasai groups, based on whether they had previously undergone Kasai procedure. Clinical data were retrospectively analyzed, and the difference in postoperative survival was compared between the groups. Preoperative data, including height, weight, serum bilirubin, and pediatric end-stage liver disease score, and perioperative blood loss, operation duration, incidence of postoperative surgical complications including vascular complications, bile duct complications, lymphatic fluid leakage, and digestive tract fistula were compared between the groups. RESULTS: In total, 89 and 61 children were categorized in the pre-Kasai and non-Kasai groups, respectively. The 1-, 6-, and 12-month survival was 97.8%, 95.4%, and 95.4% for the Kasai group, and 98.4%, 96.7%, and 96.7% for the non-Kasai group, respectively (P > 0.05). The differences in mean operation duration and mean blood loss, and the incidences of outflow tract obstruction, portal vein stenosis, hepatic artery thrombosis, bile duct complications, lymphatic fluid leakage, and digestive tract fistula were not statistically significant between the groups (P > 0.05). CONCLUSION: Kasai procedure could effectively delay the requirement of liver transplantation. In light of previous findings that Kasai procedure could significantly improve the liver transplantation-free survival of children with BA, we suggest that Kasai procedure should be used as a first-line treatment method for this condition. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level III.

Inflammation, Active Fibroplasia, and End-stage Fibrosis in 172 Biliary Atresia Remnants Correlate Poorly With Age at Kasai Portoenterostomy, Visceral Heterotaxy, and Outcome.

Published histologic studies of the hilar plate or entire biliary remnant at the time of Kasai portoenterostomy (KHPE) have not provided deep insight into the pathogenesis of biliary atresia, relation to age at surgery, prognosis or the basis for successful drainage. We report detailed histologic findings in 172 centrally reviewed biliary remnants with an average of 6 sections per subject. Active lesions were classified as either necroinflammatory (rare/clustered in a few subjects) or active concentric fibroplasia with or without inflammation (common). Inactive lesions showed bland replacement by collagen and fibrous cords with little or no inflammation. Heterogeneity was common within a given remnant; however, relatively homogenous histologic patterns, defined as 3 or more inactive or active levels in the hepatic ducts levels, characterized most remnants. Homogeneity did not correlate with age at KHPE, presence/absence of congenital anomalies at laparotomy indicative of heterotaxy and outcome. Remnants from youngest subjects were more likely than older subjects to be homogenously inactive suggesting significantly earlier onset in the youngest subset. Conversely remnants from the oldest subjects were often homogenously active suggesting later onset or slower progression. More data are needed in remnants from subjects <30 days old at KHPE and in those with visceral anomalies. Prevalence of partially preserved epithelium in active fibroplastic biliary atresia lesions at all ages suggests that epithelial regression or injury may not be a primary event or that reepithelialization is already underway at the time of KHPE. We hypothesize that outcome after KHPE results from competition between active fibroplasia and reepithelialization of retained, collapsed but not obliterated lumens. The driver of active fibroplasia is unknown.

Current management of biliary atresia based on 35 years of experience at a single center.

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

Liver transplant score for prediction of biliary atresia patients' survival following Kasai procedure.

OBJECTIVES: Recently, a scoring system has been developed to predict which patients with biliary atresia (BA) who underwent a Kasai procedure should be considered for liver transplant. Here, we applied the scoring system to predict the survival of BA patients following the Kasai procedure at Dr. Sardjito Hospital, Yogyakarta, Indonesia from January 2012 to January 2016. RESULTS: There were 26 patients, of whom 14 were males and 12 females. Outcomes of BA patients after the Kasai surgery were 15 survived and 11 died. There were significant associations between ascites and sepsis with the liver transplant score of ≥ 8 (p value = 0.006 and 0.014, respectively), whereas post-operative bilirubin level, ALT level, prothrombin time, cirrhosis, esophageal varices, portal hypertension, and cholangitis did not significantly correlate to the score. The patients with a score ≥ 8 have a relatively greater risk by 3.5-fold to die compared with patients with a score < 8, but it did not reach a significant level (p value = 0.13). In conclusions, the incidence of ascites and sepsis might predict the poor prognosis of BA patients following the Kasai procedure. Moreover, patients with a score ≥ 8 are prone to die after the Kasai surgery if they do not undergo a liver transplant.

Significance and indications for reoperative portoenterostomy in biliary atresia in light of long-term outcome.

BACKGROUND: Among biliary atresia (BA) patients undergoing revision following failed portoenterostomy, long-term native liver survival (NLS), physical condition, and indications for revision were explored. METHODS: From 1977-2015, 33 of 95 BA patients (35%) at our institution underwent revision. Ten- and 20-year NLS rates (NLSRs) and biochemical statuses of 20-year-old native liver survivors were compared between post-portoenterostomy BA patients with and without history of revision. Factors associated with 10-year NLS following revision and optimal cut-offs for potential predictors were analyzed. RESULTS: Overall 10- and 20-year NLSRs were 57% and 54%, respectively. Ten- and 20-year NLSRs were 49% and 45% in the revision group and 63% and 61% in the non-revision group, respectively. Among 20-year-old native liver survivors, differences in admission rates between ages 10-20 years were not significant for cholangitis (revision, 46%; non-revision, 40%; P = 0.30) or portal hypertension (revision, 38%; non-revision, 15%; P = 0.21). Differences in hepatobiliary function-associated blood tests between these groups at 20 years old were only significant for aspartate aminotransferase (P = 0.02) and gamma-glutamyl transpeptidase (P = 0.047). Among potential predictors of 10-year NLS that we investigated, rate of change in total bilirubin (TB) over the first month post-portoenterostomy was best (P = 0.0019), and the receiver operating characteristic curve revealed an optimal cut-off for this first-month change in TB of -3.7 mg/dl (area under the curve, 0.85; sensitivity, 0.79; specificity, 0.83). CONCLUSIONS: In approximately half of the patients, revision provided 10- and 20-year NLS, and biochemical status at 20 years old was comparable across revision and non-revision patients. Rate of change in TB during the first month post-portoenterostomy offers a sensitive predictor of revision.

Current management of biliary atresia based on 35 years of experience at a single center

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

Molecular signature of active fibrogenesis prevails in biliary atresia after successful portoenterostomy.

BACKGROUND: In biliary atresia mechanisms of progressive liver injury leading to need of liver transplantation after successful portoenterostomy remain unknown. A better understanding is a prerequisite for development of novel therapies to extend native liver survival, and we aimed to unravel molecular characteristics of liver injury after successful portoenterostomy. METHODS: Liver biopsies obtained from 28 biliary atresia children during successful portoenterostomy and at median age 3.0 years were studied. Biopsies were analyzed for histology and immunohistochemical expression of collagen 1, myofibroblast marker α-smooth muscle actin, and cytokeratin-7 positive ductal reactions. Hepatic ribonucleic acid (RNA) expression of growth factors and inflammatory cytokines was evaluated. Intestinal failure patients with comparable liver fibrosis and nonfibrotic gallstone patients and donor livers were controls. RESULTS: After successful portoenterostomy, histologic cholestasis resolved and portal inflammation reduced, while fibrosis along with ductal reactions and overexpression of collagen and α-smooth muscle actin persisted. At follow-up, liver RNA expression of collagen and platelet-derived growth factor was increased, whereas RNA expression of various inflammatory cytokines remained low. Disappearance of periductal α-smooth muscle actin expression after successful portoenterostomy (36% of patients) associated with contracted ductal reactions and reduced progression of fibrosis, collagen accumulation, platelet-derived growth factor RNA expression, and serum levels of bile acids and bilirubin. Fibrosis progressed less rapidly in syndromic than in isolated biliary atresia patients. CONCLUSION: These findings suggest that instead of inflammation, molecular signature of active fibrogenesis in association with ductal reactions prevails in long-term native liver survivors with biliary atresia. Patients should be stratified for isolated and syndromic disease forms in interventional studies.

Outcome of Biliary Atresia After Kasai's Portoenterostomy: A 15-year Experience.

BACKGROUND: To study the outcome of Biliary atresia after Kasai's portoenterostomy and clinical and biochemical factors affecting the outcome. METHODS: Medical record review of patients of biliary atresia operated from January 2000 to December 2014. The following data were collected and analyzed - sex, age at surgery, liver function tests, associated congenital anomalies, and clearance of jaundice (at 3 months). Final outcome was classified as alive, dead, or jaundice-free at last follow-up (minimum 1 year). RESULTS: 121 patients (61.9% males) were included; 32 (26.5%) were lost to follow-up at 1 year. At last follow-up, out of the 89, 42 (47.2%) were alive, 29 (32.6%) were jaundice-free, and 47 (52.8%) had died. The native liver survival rate at last follow up was 43.8%. 42 (47.2%) patients had complete clearance of jaundice at 3 months post-procedure. Jaundice-clearance rate was significantly high in patients alive (83.3% vs 16.7%, P<0.001)) as compared to those who died later. CONCLUSION: Jaundice clearance at 3 months post surgery is a good early indicator of long term success.

Long-term outcomes after revision of Kasai portoenterostomy for biliary atresia.

BACKGROUND: The indications for and efficacy of revision of portoenterostomy (PE) for biliary atresia (BA) needs to be reassessed in an era of liver transplantation. We therefore reviewed the long-term outcomes following revision of PE. METHODS: This was a retrospective study of the medical records of patients with BA who underwent PE and revision of PE. We investigated the role of revision on outcomes of jaundice-free native liver survival (approval number: 2015-0094). RESULTS: Portoenterostomy was performed in 76 patients, among whom 22 underwent revision. Revision for recurrent jaundice was performed for four of 51 patients, who were transiently jaundice free after initial PE, but only one achieved native liver survival. Revision for repeated cholangitis in two patients achieved native liver survival over 10 years. Revision was performed in 16 of the 25 patients in whom initial PE failed; of these, four survived with their native liver (ages 3, 12, 12, and 14 years). The PE revision did not significantly affect liver transplantation duration and survival outcome. CONCLUSIONS: Revision of PE was suitable for repeated cholangitis. Revision for recurrent jaundice, regardless of whether the initial PE was successful, could have a limited but positive effect in preventing long-term progressive liver failure.

Prognostic values of serum bilirubin at 7th day post-Kasai for survival with native livers in patients with biliary atresia.

BACKGROUND: Biliary atresia (BA) is a serious liver disease with uncertain prognosis. The objective of this study was to investigate prognostic values of the >20 % decrease in serum total bilirubin (TB) at 7th day post-op regarding early outcome and 5-year survival with native liver in BA. METHODS: Biliary atresia patients undergoing Kasai operation between 2000 and 2014 were reviewed. The ratio of serum TB at 7th day post-op to pre-op TB levels (TB7/TB0) was calculated for every patient. TB7/TB0 ratio of <0.8 indicated the >20 % decrease in serum TB. At 6th month following Kasai operation, outcome of BA patients were categorized into good outcome (TB < 2 mg % or clinically jaundice free) and poor outcome (TB > 2 mg % or clinically jaundice). For outcome analysis, logistic regression was used. For survival analysis, Cox regression was applied. RESULTS: There were 133 BA patients (M:F = 68:65) undergoing Kasai operation. Median age at surgery was 79 days. BA patients with TB7/TB0 ratio of <0.8 were found in 38 %. Outcome at 6-month post-op could be evaluated in 126 patients (good: poor = 68:58). The 1-, 3- and 5-year survival rates with native livers were 85, 70 and 65 %, respectively. The median overall survival with native livers was 164 months. Median follow-up time was 87 months. Logistic regression showed that gender and age at operation were not significant factors impacting on early outcome (p > 0.05). However, TB7/TB0 ratio of <0.8 was an independent factor for good outcome (Odds ratio = 3.0, p = 0.006). Cox regression analysis demonstrated that 5-year survival rate was significantly correlated with TB7/TB0 ratio of <0.8 (HR = 0.46, 95 % CI 0.23-0.91, p = 0.025) and outcome at 6th month post-op (HR = 0.05, 95 % CI 0.01-0.15, p < 0.001). CONCLUSIONS: The >20 % decrease in serum TB at 7th day post-Kasai is a predictor for good outcome. BA patients with TB7/TB0 of <0.8 had 5-year survival with native livers significantly higher than those with the ratio of >0.8.

Long-term results of biliary atresia in the era of liver transplantation.

PURPOSE: The purpose of this study was to analyze the survival of patients with biliary atresia (BA) after Kasai operation and liver transplantation (LT) and to analyze the factors affecting survival. METHODS: Seventy-two patients diagnosed with BA were operated on between April 1995 and December 2009 and retrospectively analyzed. RESULTS: Out of the 72 patients, 59 received Kasai operation and 13 received LT without prior Kasai operation. Twenty-seven patients received LT after Kasai operation. Survival with native liver was 39 % at 10 years. With the application of LT, overall 10-year survival for patients with BA was 94.9 %. Among patients alive with native livers after Kasai operation, 14 patients (58.3 %) have at least one complication associated with biliary cirrhosis and portal hypertension. Age at which Kasai operation was performed (60 days) and postoperative normalization of bilirubin were independent risk factors for survival with the native liver, according to multivariate analysis (HR 2.90, p = 0.033 and HR 9.89, p = 0.002). CONCLUSIONS: Survival of BA patients has greatly increased in the era of LT. However, many patients surviving with native livers after Kasai operation continue to have signs of biliary cirrhosis and abnormal liver function.

Effect of repeat Kasai hepatic portoenterostomy on pediatric live-donor liver graft for biliary atresia.

OBJECTIVES: Treatment for patients with biliary atresia is a Kasai hepatic portoenterostomy; however, the efficacy of repeat Kasai hepatic portoenterostomy is unclear. This study sought to examine the effect of a prior Kasai hepatic portoenterostomy, especially a repeat Kasai hepatic portoenterostomy, on the outcomes of living-donor liver transplant. MATERIALS AND METHODS: One hundred twenty-six of 170 children that underwent a living-donor liver transplant between May 2001, and March 2010, received a living-donor liver transplant for biliary atresia. These patients were divided into 2 groups according to the number of previous portoenterostomies: 1 (group A, n=100) or 2 or more Kasai hepatic portoenterostomies (group B, n=26). Portoenterostomy was performed twice in 24 patients in group B, 3 times in 1, and 4 times in 1. Preoperative, operative factors, mortality, morbidity, and survival rates were examined and compared between groups. RESULTS: The surgical factors such as operative time, blood loss per weight, cold ischemia time, and weight of the native liver were significantly greater in group B than they were in group A. The patient survival rates were comparable in the 2 groups (94.5% in group A and 93.3% in group B), and the difference was not statistically significant. No statistically significant difference was observed between the groups with regard to vascular complications, biliary complications, and other factors including postoperative variables. Bowel perforation requiring surgical repair was more frequent in group B than it was in group A. CONCLUSIONS: Repeat Kasai hepatic portoenterostomy might have a negative effect on patients who undergo living-donor liver transplant for biliary atresia patients with potential lethal complications such as bowel perforation. More biliary atresia patients could have a liver transplant, with improved survival and better life expectancy, if they have inadequate biliary drainage after the initial Kasai hepatic portoenterostomy.

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.

Survival with the native liver after laparoscopic versus conventional kasai portoenterostomy in infants with biliary atresia: a prospective trial.

OBJECTIVE: A prospective study to compare survival with own liver in laparoscopic versus conventional Kasai portoenterostomy in patients with biliary atresia. BACKGROUND: Available studies on laparoscopic versus conventional Kasai portoenterostomy focus on short-term results, include small numbers of patients and have design limitations. METHODS: A consecutive series of patients underwent laparoscopic Kasai procedure from 2006 to 2007. Conventionally operated control patients consisted of a consecutive series of infants with biliary atresia operated from August 2003 to 2006. All data were ascertained prospectively using the European Biliary Atresia Registry/EBAR registration forms. Primary outcome measure was survival with own liver 6 months after Kasai without being listed for liver transplantation. An interim analysis was planned after data became available for the first 12 patients, who underwent the laparoscopic Kasai procedure. In case of a significantly different interim outcome, the follow-up period should be extended to 24 months until a final decision can be made. RESULTS: Twelve infants underwent laparoscopic Kasai procedure without conversion or revision and there was no revision in the control group of 28 conventionally operated patients. Six months after operation, 5 of 12 laparoscopically operated patients (42%) survived with own liver, compared with 23 of 28 (82%) controls (P < 0.01). The study was stopped due to the significantly higher rate of liver transplantation after laparoscopic operation. Ten patients (83%) after laparoscopic Kasai versus 18 (64%) conventionally operated patients were transplanted after 24 months (P < 0.05) and survival rates with own liver and serum bilirubin <20 µmol/L were 1 (8%) versus 8 (29%), respectively (P < 0.05). CONCLUSIONS: This prospective study shows that the laparoscopic Kasai procedure for biliary atresia is technically feasible. However, the study was stopped after inclusion of 12 laparoscopically operated infants due to a lower survival with the native liver after laparoscopic versus conventional Kasai operation. Superior results after conventional operation were confirmed at follow-up after 24 months. Study registration ID: EBAR 9260/NCT01063699.