Inflammation patterns in early post-operative cholangitis predict long-term outcomes in biliary atresia: a potential role of non-suppurative cholangitis.

PURPOSE: Frequent post-operative cholangitis in biliary atresia (BA) affects the long-term native liver survival. This study assessed the characteristics of early cholangitis and their influence on the prognosis. METHODS: Forty-three patients with BA who underwent surgery between 2000 and 2020 were analyzed for routine inflammatory markers. Early cholangitis characteristics were compared between native liver survivor (NLS) and living donor liver transplant (LDLT) patients. RESULTS: Among the 43 patients, 30 (69.8%) experienced 130 episodes of cholangitis. In the area under the receiver operating characteristics curve (AUROC) analysis, the cutoff value of the total cholangitis episodes was 3, with an area under the AUROC curve of 0.695 (95% confidence interval 0.522-0.868). Before 3 years old, 113 episodes (86.9%) of cholangitis were observed. The white blood cell, C-reactive protein, and alanine aminotransferase values at cholangitis onset did not markedly differ between the LDLT and NLS groups. Conversely, the neutrophil-to-lymphocyte ratio in the NLS group was significantly lower than in the LDLT group (0.85 vs. 1.63, p < 0.001). CONCLUSIONS: Cholangitis in the NLS group was lymphocyte-dominant and atypical in its pathogenesis. Lymphocyte-dominant cholangitis is non-suppurative, and future research should clarify its pathogenesis to improve the treatment and prognosis of BA.

Kasai Portoenterostomy, Successful Liver Transplantation, and Immunosuppressive Therapy for Biliary Atresia in a Female Baby: A Case Report.

Background: Biliary atresia (BA) is a severe neonatal progressive cholangiopathy of unknown etiology. A timely Kasai portoenterostomy (KPE) improves survival of the native liver in patients with BA, although liver transplantation remains the ultimate treatment for most (60%-80%) patients. However, postoperative adverse effects of liver transplantation may be significant. In addition, patients require lifelong immunosuppressive therapy after liver transplantation. Case Summary: Here, we report a case of a newborn female baby (birthday: 10-03-2018) with congenital BA (confirmed at 76 days of life) who survived KPE (first surgery at 85 days of life) and underwent successful living-related liver transplantation (LRLT) (second surgery at 194 days of life). Additionally, we reviewed the existing literature on BA. After KPE (at 85 days of life), the liver function of the baby did not improve, and the indicators of liver and kidney function showed a trend of aggravation, indicating that the liver function had been seriously damaged before KPE (at 85 days of life), demonstrating the urgent need for liver transplantation surgery. The female baby survived after part of her father's liver was successfully transplanted into her body (at 194 days of life). The patient recovered successfully. No other diseases were found at the 4-year follow-up, and all indices of liver and kidney functions tended to be normal. Conclusion: This case highlights the following. Postoperative alkaline phosphatase was consistently above the normal range, although the reason for this was unclear; neither tacrolimus nor cyclosporine A has formulations designed specifically for infants, which does not meet the needs of clinical individualized medication, suggesting that these anti-rejection drugs are future development directions. Only one case of congenital BA has been found thus far in Hefei, and this case has extremely important reference significance for the prevention, treatment, and diagnosis of BA in Hefei, Anhui province.

Cholestasis after Kasai operation predicts portal hypertension in native liver survivors of biliary atresia: a multicenter study.

PURPOSE: This study evaluated portal hypertension (PHT) and its predictors among native liver survivors (NLS) of biliary atresia (BA) after Kasai portoenterostomy (KPE). METHODS: This was a multicenter study using prospectively collected data. The subjects were patients who remained transplant-free for 5 years after KPE. Their status of PHT was evaluated and variables that predicted PHT were determined by regression analysis and receiver operating characteristic (ROC) curve. RESULTS: Six centers from East Asia participated in this study and 320 subjects with KPE between 1980 to 2018 were analyzed. The mean follow-up period was 10.6 ± 6.2 years. At the 5th year after KPE, PHT was found in 37.8% of the subjects (n = 121). Patients with KPE done before day 41 of life had the lowest percentage of PHT compared to operation at older age. At 12 months after KPE, PHT + ve subjects had a higher bilirubin level (27.1 ± 11.7 vs 12.3 ± 7.9 µmol/L, p = 0.000) and persistent jaundice conferred a higher risk for PHT (OR = 12.9 [9.2-15.4], p = 0.000). ROC analysis demonstrated that a bilirubin level above 38 µmol/L at 12 months after KPE predicted PHT development (sensitivity: 78%, specificity: 60%, AUROC: 0.75). CONCLUSIONS: In BA, early KPE protects against the development of PHT among NLSs. Patients with persistent cholestasis at one year after KPE are at a higher risk of this complication. They should receive a more vigilant follow-up. LEVEL OF EVIDENCE: Level III.

Delphi Method Analysis and Consensus of Prevalent Distinctive Practices for Biliary Atresia Management after Kasai Portoenterostomy.

Background: Extrahepatic biliary atresia (BA) is seen in infants, with an incidence of 1 in 15,000 live births. The presentation is progressive jaundice, dark-colored urine, and clay-colored stools. Kasai portoenterostomy (KPE) is the commonly performed surgical procedure in these patients. Postoperatively, phenobarbitone, ursodeoxycholic acid (UDCA), steroids, and other drugs are given to improve bile drainage and prevent inflammation and fibrosis. However, a definitive protocol regarding the need for different drugs, dosage, and duration varies across individual surgeons and centers. No universally accepted protocol exists for postoperative management after KPE. Aim: The aim of this study was to know the prevailing postoperative management of BA by subject experts and use the Delphi process to know if the experts want to change their practice based on the results from the survey. Material and Methods: A questionnaire was made after discussing with two experts in the field of BA. The questionnaire was mailed to 25 subject experts. The first survey data were analyzed and shared with all responders. In the second survey, change in the management based on the results from the first survey was assessed. Results: The Delphi questionnaire was answered by 17 experts. Postoperatively, prophylactic antibiotics are prescribed for 6-12 weeks by around 40% and >12 weeks by 30% of respondents. Phenobarbitone is prescribed for <3 months by nearly 50%. UDCA is prescribed for <3 months, ≤6 months, and 6 months-1 year by 47.1%, 23.5%, and 23.5% responders, respectively. Nearly 50% prescribe steroids (mostly prednisolone), and among them, two-thirds prescribe it for 6-12 weeks. Approximately 60% give antiviral drugs to children who are cytomegalovirus immunoglobulin M positive. In our survey, 50% of experts perform 5-10 KPE per year, and 25% each perform 10-15 and >15 KPE per year. The second survey noted that a significant percentage of responders want to change their practice according to consensus. Conclusion: From our Delphi survey, an overview of the postoperative management of BA could be made. However, multicentric studies are required for uniform protocol on the postoperative management of BA.

Biliary atresia-splenic malformation (BASM) syndrome: A case report.

INTRODUCTION AND IMPORTANCE: Biliary atresia (BA) is characterized by the presence of persistent cholestatic jaundice during the neonatal period. This group of patients is at higher risk for liver disease and/or portal hypertension compared with other chronic liver diseases. CASE PRESENTATION: We present a newborn patient who had biliary stools early postnatally, but her gallbladder was not seen on radiological examination and was referred to our clinic. On examination, polysplenia was seen, and the inferior vena cava was not seen. The patient's stool color changed to a creamy stool without bile three weeks postnatally. The neonate was diagnosed with biliary atresia and splenic malformation (BASM) syndrome. The patient underwent a Kasai portoenterostomy in the 3rd week of age and was preoperatively diagnosed with malrotation and treated with a Ladd procedure at the same time. The patient had a postoperative stool with bile and decreased bilirubinemia with a normal defecation period. CLINICAL DISCUSSION: BASM syndrome, clinically has different causes and a worse prognosis than isolated BA. The syndrome can lead to end-stage cirrhosis and liver failure if left untreated. Although timely Kasai surgery is the standard treatment for BA, the age of the patient at the time of the surgery is different depending on BA groups. CONCLUSION: The most important prognostic factors for BASM syndrome are early diagnosis and treatment. Despite this, the patients need careful follow-up. Postoperative adjuvant steroid therapy may have a good impact on the outcomes.

"Where Did This Come From?": Antibiotic Prophylaxis in Biliary Atresia After Kasai Procedure.

For patients with Biliary atresia, antibiotic prophylaxis after Kasai portoenterostomy is a common practice. Societal guidelines often cite one reference as supportive evidence for this practice. In this paper, we go back to review the quality of this evidence and suggest more research is required to demonstrate the efficacy of antibiotic prophylaxis in this population.

Primary Liver Transplant in Biliary Atresia: The Case for and Against.

The role of liver transplantation as a primary procedure in biliary atresia has been argued over for at least 40 years, indeed since the coming of age of safe liver transplantation during the 1980s. Yet, it is not a common option in most series (usually ≤5%) and typically reserved for those with late presentations (arguably >100 days) with established cirrhosis. This review presents the pros and cons of primary liver transplant. The pros are based upon the observation that at best a Kasai portoenterostomy (KPE) is simply palliative in most, and at worse has no effect whatsoever on restoration of bile flow and is therefore pointless. Set against this are the cons: there is a dearth of prognostic tests (clinical, biochemical, or histological) at the time of presentation which may predict inevitable failure; the possibility of long-term native liver survival to adulthood in a proportion (albeit a minority); and the implied increased need for donor organs suitable for infants - a stressor for an already overstressed system. Improving results from KPE in terms of increasing the proportions clearing their jaundice and minimizing the effects of chronic liver fibrosis and cirrhosis would surely limit the siren calls for primary transplants but the key must be better discrimination at presentation with the use of biomarkers (circulatory or histological, individually or together) to enable better decision making.

Biliary atresia in preterm infants: a single center experience and review of literature.

Introduction: The diagnosis of biliary atresia (BA) remains challenging, and there is still uncertainty regarding the optimal time to perform a Kasai portoenterostomy (KPE). Little is known about the difficulties in the diagnosis and outcomes of BA in preterm infants (PBA). This study, which represents the first Italian report of preterm infants with BA, aims to describe a single-center experience of BA in preterm newborns. Methods: We retrospectively reviewed all infants consecutively diagnosed with BA who underwent a Kasai procedure at the Bambino Gesù Children's Hospital between January 1998 and December 2021. Prematurity was defined as a gestational age (GA) of <37 weeks. Demographic, laboratory, and histology data were recorded, and the main outcomes considered were clearance of jaundice (COJ), native liver survival, and mortality. Results: A total of 21 PBA were compared with 117 term BA controls (TBA). The median GA of PBA was 35.1 (32-36.1) weeks, with a mean birth weight of 2,100 (1,897-2,800) g. Age at first presentation was significantly lower in PBA patients: 46 (22-68) vs. 61 (44-72) days; p = 0.02. The median age at KPE was similar between the two groups: 70 days (33 corrected) for PBA vs. 67 in TBA; p = 0.8. At the time of surgery, median serum bilirubin was lower in the PBA group (7.7 vs. 8.6 mg/dl, p = 0.04). Similarly, the median APRi at the time of KPE was lower but not significant in the PBA group: 1.09 vs. 1.16; p = 0.8. No differences were found in terms of COJ between the PBA and TBA groups: n = 9 (43%) vs. 34 (35%); p = 0.2. Overall native liver survival was similar between the two groups: 8.6 (4.8-12.2) for the PBA group vs. 7.6 (5.6-9.5) years for the TBA group with no significant differences; p = 0.45. Post-KPE native liver survival was similar between the two groups: 38% vs. 52% at 5 years for the TBA and PBA groups, respectively; p = 0.54. Conclusion: The PBA and TBA groups appear to have similar outcomes in terms of COJ, overall native liver survival, and 5-year liver survival. Considering the corrected GA, early KPE is related to lower cholestatic damage. Further multicenter studies are required.

A Randomized Control Trial to Evaluate the Effect of Local Instillation of Mitomycin-C at the Porta after Kasai Portoenterostomy in Patients of Biliary Atresia.

Background: Kasai portoenterostomy (KPE) is the initial treatment for biliary atresia (BA). Even after initial jaundice clearance, a significant number of children presented with the reappearance of symptoms due to ongoing fibrosis involving porta and intrahepatic ducts. Mitomycin-C (MMC) is an antifibrotic agent, and the study hypothesized that local application of MMC at porta can decrease fibrosis, which can improve jaundice clearance and lead to better native liver survival (NLS). Materials and Methods: This prospective randomized control trial included children with BA, who were allocated to groups A or B. The patients in both groups underwent standard KPE; in addition, a 5 French infant feeding tube (IFT) was placed near the porta through the Roux limb in Group B children. During the postoperative period, MMC was locally instilled over the porta in Group B children through IFT. Postoperative jaundice clearance and NLS were assessed and compared. Results: A total of 27 children were enrolled in the study, 16 in Group A and 11 in Group B. Both groups were comparable preoperatively. Although the NLS was not statistically significant in Group B, the survival was quite higher, that was 91%, 81%, and 73% at 6 months, 1 year, and 2 years, respectively, compared to 63%, 50%, and 38% in Group A. Conclusion: Children in Group B clinically showed an early jaundice clearance and a better trend of serial bilirubin levels as well as longer NLS than Group A, but it was not statistically significant. The procedure was technically easy, and no complication was encountered related to surgical technique or MMC instillation.

Predictive model containing gene signature and shear wave elastography to predict patient outcomes after Kasai surgery in biliary atresia.

AIMS: Infants with biliary atresia (BA) are treated with Kasai portoenterostomy (KPE) surgery, but many BA patients need subsequent salvage liver transplants. The aim of this study is to develop a comprehensive gene-clinical model based on two-dimensional shear wave elastography (2DSWE), liver gene expression, and other clinical parameters to predict response to KPE for BA patients. METHODS: Differentially expressed gene patterns between liver samples of BA (n = 102) and non-BA control (n = 14) were identified using RNA sequencing analysis. Biliary atresia patients were then randomly assigned to training and validation cohorts. Gene classifier based on the differentially expressed genes was built in the training cohort. Nomogram models with and without gene classifier were further constructed and validated for predicting native liver survival of BA patients. The utility of the nomograms was compared by C-index. RESULTS: Using the least absolute shrinkage and selection operator model, we generated a nine-gene prognostic classifier. The nomogram based on the nine-gene classifier, age, preoperative 2DSWE, and albumin had the better C-index compared to gene classifier alone in the training cohort (0.83 [0.76-0.90] vs. 0.69 [0.61-0.77], p = 0.003) and the validation cohort (0.74 [0.67-0.82] vs. 0.62 [0.55-0.70], p = 0.001). Using risk scores developed from the nomogram, the 12-month survival rates of BA patients with native liver were 35.7% (95% confidence interval [CI], 22.7-56.3) in the high-risk group and 80.8% (95% CI, 63.4-100.0) in the low-risk group in the validation cohort. CONCLUSIONS: The comprehensive genetic-clinical nomogram based on preoperative 2DSWE, liver gene expression, and other clinical parameters can accurately predict response to KPE.

Laparoscopic versus open portoenterostomy for treatment of biliary atresia: a meta-analysis.

OBJECTIVE: Our goal was to compare laparoscopic portoenterostomy versus open portoenterostomy for the treatment of biliary atresia. MATERIALS AND METHODS: Using the databases EMBASE, PubMed, and Cochrane, we carried out a thorough literature search up to 2022. Studies comparing laparoscopic and open surgery for the treatment of biliary atresia were included. RESULTS: Twenty-three studies comparing laparoscopic portoenterostomy (LPE) (n = 689) and open portoenterostomy (OPE) (n = 818) were considered appropriate for meta-analysis. Age at surgery time was lower in the LPE group than OPE group (I2 = 84%), (WMD - 4.70, 95% CI - 9.14 to - 0.26; P = 0.04). Significantly decreased blood loss (I2 = 94%), (WMD - 17.85, 95% CI - 23.67 to - 12.02; P < 0.00001) and time to feed were found in the laparoscopic group (I2 = 97%), (WMD - 2.88, 95% CI - 4.71 to - 1.04; P = 0.002). Significantly decreased operative time was found in the open group (I2 = 85%), (WMD 32.52, 95% CI 15.65-49.39; P = 0.0002). Weight, transfusion rate, overall complication rate, cholangitis, time to drain removal, length of stay, jaundice clearance, and two-year transplant-free survival were not significantly different across the groups. CONCLUSIONS: Laparoscopic portoenterostomy provides advantages regarding operative bleeding and the time to begin feeding. No differences in remain characteristics. Based on the data presented to us by this meta-analysis, LPE is not superior to OPE in terms of overall results.

Questioning Diagnostic Value of Serum Matrix Metalloproteinase 7 for Biliary Atresia.

Background: Matrix metalloproteinase 7 (MMP7) has been suggested as a promising biomarker in diagnosing biliary atresia (BA). This study aimed to assess the diagnostic accuracy of serum MMP7 in BA in the Middle Eastern population. Methods and materials: In this cross-sectional study, neonates and infants with direct hyperbilirubinemia admitted to Namazi referral hospital, Shiraz, Iran, were studied. Baseline demographic and clinical characteristics and blood samples were obtained on admission. MMP7 serum concentration was measured using an enzyme-linked immunosorbent assay (ZellBio GmbH, Ulm, Germany). Results: 44 infants with a mean age of 65.59 days were studied. Of these patients, 13 cases were diagnosed with BA, and 31 cases' cholestasis related to other etiologies. Serum MMP7 concertation was 2.13 ng/mL in the BA group and 1.85 ng/mL in the non-BA group. MMP7 was significantly higher in those presented with either dark urine or acholic stool. The predictive performance capability of the MMP7 was not significant in the discrimination of BA from the non-BA group based on receiver operating characteristic curve analysis (area under curve: 0.6, 95% confidence interval: 0.45-0.75). In the optimal cut of point 1.9, the sensitivity and specificity were 84.6% and 45.1%, respectively. Further combination of MMP7 with Gamma-glutamyl transferase (GGT), alkaline phosphatase, direct and total bilirubin, and dark urine or acholic stool was not remarkably boosted the diagnostic accuracy of the test. Interestingly, GGT at a cut-off point of 230 U/L was 84.6% sensitive and 90.3% specific for BA. Conclusion: Our results are not consistent with previous studies on this subject. Considering more conventional and available tests like GGT besides conducting future studies with greater samples and different geographical areas is recommended.

Incidence and antiviral treatment of cytomegalovirus infection in infants with biliary atresia.

PURPOSE: Patients with biliary atresia (BA) and cytomegalovirus (CMV) infection may have poorer outcomes after Kasai portoenterostomy (KPE) than uninfected patients, suggesting a rationale for antiviral treatment (AVT). We aimed to describe the incidence of CMV infection and of AVT in BA patients, and to detect any differences between infected and uninfected patients to conclude if AVT is of use. METHODS: Data on BA patients who underwent KPE 2004-2020 were retrospectively collected, and the outcome was analyzed with regard to CMV status. RESULTS: Fifteen out of forty-six (33%) BA patients had signs of ongoing CMV infection. They did not differ significantly from the CMV-negative patients regarding rate of prematurity, birth weight, or biochemical markers but were slightly older at KPE. All patients received steroids postoperatively and all patients with ongoing CMV infection received AVT with very good effect on viremia and without major side effects. The AVT consisted of oral valganciclovir (10-40 (- 58) mg/kg/d) or intravenous ganciclovir (5.3-11 mg/kg/d). CONCLUSION: Ongoing CMV infection is common in this group of patients. The viremia can effectively be treated with AVT without any major side effects. Larger, randomized studies are needed to clarify the possible effect on clinical outcome.

Prognostic factors and outcomes of Kasai portoenterostomy (KPE): nine-year experience from a lower-middle income country.

PURPOSE: Outcome data after Kasai portoenterostomy (KPE) reported worldwide show considerable regional and institutional variation. It is not known whether the same standards of outcomes reported in western world can be replicated in resource-poor countries. METHODS: We reviewed 79 patients of which 43 had completed a 2-year minimum follow-up. Two cohorts were based on age at KPE. The median age at surgery was 60 days. RESULTS: Clearance of jaundice (COJ) at 3 months was 20.93% and was not affected by age at surgery (p = 0.295). Four patients (9.3%) received liver transplant and 16 patients (37.21%) were recorded dead at a median age of 7 months. Native liver survival (NLS) was 53.49% and overall survival (OS) was 62.79%. Kaplan-Meier estimated 4- and 6-year NLS were 55.8% and 49.6%, respectively. There was a significant difference in the NLS between early and late surgery groups. CONCLUSION: While causes for low COJ need to be explored, these data reaffirm that early surgery has a significant favorable effect on survival. NLS was comparable with data from the developed world, whereas low OS is explained by limited access to transplant. Thus, where the survival depends on native liver longevity, emphasis should be on as early KPE as possible.

Prognostic Factors Related to In-hospital Death in Children with Biliary Atresia: Analysis of a Nationwide Inpatient Database.

Background and Aims: Patients with biliary atresia (BA) are prone to hepatic decompensation, which might eventually lead to death. This study aimed to identify the possible risk factors affecting in-hospital death in BA patients in China. Methods: We collected data from the Hospital Quality Monitoring System, a national inpatient database. All patients aged up to 2 years old with a diagnosis of BA were included. The subjects were divided to three groups, including Kasai portoenterostomy (KP), liver transplantation (LT), and no surgery. Logistic regression with Firth's method was performed to identify potential influencing variables associated with in-hospital death. Results: During the year 2013 to 2017, there were 14,038 pediatric admissions with a diagnosis of BA. The proportion of in-hospital death in pediatric BA admissions was 1.08%. Compared with patients under six months, there was a higher risk of in-hospital death for children aged six months to 1 year and 1-2 years old. Clinical signs, including cirrhosis, variceal bleeding, and hepatic encephalopathy, were significantly associated with the risk of in-hospital death. In no surgery group, compared to those in Beijing and Shanghai, BA patients admitted in other districts had a lower risk of in-hospital death (OR=0.39, 95% CI: 0.21, 0.70). However, in the LT group, patients admitted in other districts had a higher risk of in-hospital death (OR=9.13, 95% CI: 3.99, 20.87). Conclusions: In-hospital survival remains unsatisfactory for pediatric BA patients with severe complications. Furthermore, more resources and training for BA treatment, especially LT, are essential for districts with poor medical care in the future.

Robotic-assisted Kasai portoenterostomy for biliary atresia.

BACKGROUND: Robotic-assisted Kasai portoenterostomy (RAKPE) has been utilized to treat biliary atresia (BA). However, RAKPE is not widely performed and its efficacy remains unknown. We summarized the experience of RAKPE for BA and determined its efficacy. MATERIALS AND METHODS: We retrospectively analyzed 25 consecutive infants with non-syndromic type III BA who received RAKPE in our center from January 2020 to July 2021. RAKPE is a three-arm setup and four-trocar operation. Bipolar coagulation was used to dissect the small blood vessels at the hepatic portal. The fibrous cone was shallowly transected with bending electric scissors, followed by gelatin sponge compression to staunch the hemorrhage. Finally, a wide anastomosis was accurately constructed. Demographics and outcomes were recorded. RESULTS: The mean operative time was 211.64 ± 18.93 min. No conversion to laparotomy or intraoperative complications occurred. The mean estimated blood loss was 7.64 ± 2.43 mL. Enteral feeding was resumed after 3.44 ± 1.23 days. All patients achieved bile excretion postoperatively, and dark green bile-stained stools were passed 1.50 days (range 1.00-3.00 days) after surgery. The average postoperative length of hospital stay was 10.32 ± 2.59 days. The jaundice clearance (JC) rate was 76.00% within 6 months after surgery and the incidence of cholangitis was 48.00% within 1 year following surgery. The survival with native liver (SNL) rate was 80.00% at 1 year and 66.67% at 2 years. CONCLUSION: RAKPE can be regarded as a treatment option for patients with BA due to the good outcomes reported. However, long-term studies comparing open or laparoscopic approaches are needed.

Peri-Kasai portoenterostomy sutures anchoring the jejunal loop to Glisson capsule: A novel modification to reduce the incidence of cholangitis.

BACKGROUND: Kasai portoenterostomy (KPE) is the standard surgical management for biliary atresia (BA). To improve the outcome these infants were operated on within the first two months of life. The success of the procedure is reflected by clearance of jaundice and either absence or occurrence of fewer attacks of cholangitis. The failure of the procedure indicates liver transplantation (LT). OBJECTIVE: to reduce the incidence of the recurrent attacks of cholangitis by peri-KPE sutures anchoring the jejunal loop to the Glisson capsule. METHODS: It is a retrospective study that included 45 infants diagnosed with BA and who were operated on at an age younger than 60 days. They were categorized into two groups, Group A (n = 23) included infants treated with the classic KPE, and Group B (n = 22) included infants treated in the same way plus peri KPE sutures anchoring the jejunal loop to the Glisson capsule. RESULTS: The mean operative time in Group A was 149.3 min versus 164.8 min in Group B (p-value 0.039). The mean level of bilirubin was 2.2 versus 2.1 in Group A and Group B respectively at two years follow up. The total attacks of cholangitis per patient were significantly lower in Group B than in Group A (cutoff value = 3), which was reflected by the significant reduction of the incidence of LT in Group B. CONCLUSION: peri KPE sutures anchoring the jejunal loop to the Glisson capsule significantly reduced the incidence of recurrent attacks of cholangitis and subsequently decreased the requirement of LT on the short-term follow-up.

Benefit of epidural analgesia for postoperative pain control after a Kasai Portoenterostomy: A ten-year retrospective cohort study.

INTRODUCTION: Biliary atresia is a rare obstructive cholangiopathy that presents in infants. The Kasai portoenterostomy procedure, which reestablishes biliary drainage into the intestine, is a surgical procedure that has been found to improve survival with the native liver. The options for postoperative analgesia include systemic opioids and epidural analgesia. The primary objective of this study was to compare the postoperative systemic opioids used in morphine equivalents (mg/kg) on postoperative days 0 through 3 between patients who underwent a Kasai portoenterostomy and received a thoracic epidural infusion to those without thoracic epidural analgesia. METHODS: We conducted a retrospective cohort study of 91 infants with biliary atresia undergoing a Kasai portoenterostomy between January 1, 2009, and September 1, 2019, at the Children's Hospital of Philadelphia. RESULTS: Sixty-three of the 91 patients (69%) had a continuous epidural catheter placed intraoperatively for postoperative analgesia. The total opioid requirement (morphine equivalents) for the first 72 h in the epidural group of (Mean (95% confidence interval): 0.52 mg/kg (0.38, 0.67 mg/kg) was lower than the non-epidural group (Mean (95% confidence interval): 1.15 mg/kg (0.8, 1.48 mg//kg) for a difference in mean opioid requirement (95% confidence interval) of 0.63 mg/kg (0.32, 0.94 mg/kg). Patients in the non-epidural group had higher rates of unplanned ICU admissions (36% non-epidural group vs. 3.3% epidural group, difference in proportion (95% confidence interval) 32.7% (13, 52%), p < .01). A higher percentage of patients in the non-epidural group had a postoperative oxygen requirement (32.1% vs. 11.3%, difference in proportion (95% confidence interval) 21% (2, 40%), p = .02). CONCLUSION: In our cohort study, continuous thoracic epidural analgesia in patients undergoing a Kasai portoenterostomy was associated with lower postoperative opioid use. We also observed that the epidural group had a lower ICU admission rate and a lower rate of postoperative supplemental oxygen requirement over the first three postoperative days.

The outcome of Kasai portoenterostomy after day 70 of life.

Background: The age at Kasai portoenterostomy (KPE) was reported to correlate with the prognosis of patients with biliary atresia (BA) and that a late KPE is bounded to be failure. Herewith, we reported the outcome of patients receiving KPE after day 70 of life. In addition, the prognostic indicators were evaluated. Materials and Methods: This was a retrospective analysis and all BA patients receiving KPE after day 70 of life in a tertiary centre between 1980 and 2018 were evaluated. Results: A total of 164 KPE procedures were performed during the study period and 62 cases were done after day 70 of life which were included in this study. The median follow up period of these patients was 10.6 years (range: 4.5 to 41.5 years). Thirty-nine patients (62.9%) patients were able to achieve jaundice clearance at 6 months after KPE. The NLS rate was 53.2% (n = 33) as recorded at the time of writing. There was no statistical difference in the age at KPE between native liver survivors and patients requiring liver transplant. For complications among the native liver survivors (n = 33), portal hypertension and recurrent cholangitis were found in 63.6% and 30.3% of these patients. There was also no significant difference in the age at KPE between those who developed portal hypertension and recurrent cholangitis (p = 0.451 and p = 0.173 respectively). Regarding the prognostic indicators in predicting NLS, pre-KPE bilirubin, alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT) were significantly higher among patients requiring liver transplant (p = 0.012, =0.011 and =0.017 respectively). The bilirubin level at 6 months after KPE was also higher among patients who required liver transplant (p = 0.016). Conclusion: More than half of the BA patients can survive for 10 years with their native liver despite KPE was performed after day 70 of life. However, they have a higher chance to develop BA-related complications. The level of pre-KPE bilirubin and ductal enzymes as well as post-KPE bilirubin are prognostic indicators to predict NLS.