Small Intestinal Adenocarcinoma Arising at the Anastomotic Site after Kasai Operation for Biliary Atresia: A Case Report and Literature Review.

Biliary atresia is an obliterative cholangiopathy of unknown etiology. Hepatic portoenterostomy, in which obliterated extrahepatic bile ducts are resected and bile flow is restored, known as Kasai operation, is performed within 3 months after birth. While this operation enhances long-term survival of patients, the occurrence of primary malignant hepatic tumors has been increasing. We report a case of small intestinal adenocarcinoma arising at the anastomotic site after Kasai operation. A 49-year-old man, who underwent Kasai operation for biliary atresia when he was 2 months old, experienced rapidly progressive jaundice and liver dysfunction. Deceased-donor liver transplantation was performed for liver failure. Macroscopically, there was a white-yellow tumor located at the anastomotic site of hepatic portoenterostomy of the resected liver. Pathological examination revealed a well-differentiated adenocarcinoma with some Paneth cells in the neoplastic lesion. Immunohistochemically, the tumor cells were negative for cytokeratin 7 (CK7) but positive for cytokeratin 20 (CK20) and a homeobox domain-containing transcription factor (CDX2). Mucin expression in tumor cells was negative for mucin 1 (MUC1) and mucin 6 (MUC6) and positive for mucin 2 (MUC2) and mucin 5AC (MUC5AC). The pathological diagnosis was small intestinal adenocarcinoma originating from the jejunum. The patient was discharged 48 days after the operation. The patient had not experienced recurrence at 10 months after the operation. This is the first report of small intestinal adenocarcinoma arising at the anastomotic site after Kasai operation for biliary atresia. Special care should be taken for the patients after Kasai operation with acute progressive jaundice and liver dysfunction because there is a possibility of malignancy in their native liver.

The AST-to-Platelet Ratio Index (APRi) at Kasai Portoenterostomy: Standing the Test of Time.

AIMS: The outcomes following surgical treatment of infants with biliary atresia (BA) varies across the world with many possible confounding factors. APRi (AST-to-platelet ratio index) is a simple surrogate marker of liver fibrosis and we sought to determine its long-term relationship (if any) with outcome post-Kasai portoenterostomy (KPE). METHODS: Prospectively acquired database (Jan 1998-Dec 2021). Clearance of jaundice was defined as achieving <20 umol/L post-KPE. Categorical and survival data were tested using Chi2 tests and a log rank test respectively. P ≤ 0.05 was regarded as significant. Data are quoted as median (interquartile range) unless otherwise stated. RESULTS: There were 473 infants with a calculated APRi at time of KPE [0.70 (IQR 0.45-1.2)] and known outcomes. There was significant but moderate correlation with age at KPE (rS = 0.43; P < 0.0001). APRi was divided into quartiles (1st 0.11-0.44, n = 120; 2nd 0.45-0.69, n = 120; 3rd 0.70-1.18, n = 115 and 4th 1.2-15.1; n = 118). There was a clear distinction in APRi levels between CMV + ve BA and the other groups (Syndromic BA, Cystic BA, Isolated BA), with an overrepresentation of CMV IgM + ve BA in the higher APRi quartiles (Χ2 = 26.6; P = 0.0002). Clearance of jaundice showed a stepwise decrease across the quartiles (67%; 58%; 55%; 49%; overall Χ2 = 7.8, P = 0.049 and P = 0.005 for trend). Decreasing native liver survival also showed a significant trend (P = 0.01). CONCLUSION: APRi appears to be of fundamental prognostic value in stratifying the BA population. In our series, CMV status was associated with higher APRi score and appears to be different. This simple variable offers an objective method of assessing the biological status of BA at presentation and variability between different series. LEVEL OF EVIDENCE: II (prospective comparison).

The composition of the gut microbiota is altered in biliary atresia with cholangitis.

Aim: To detect the composition of the gut microbiota in biliary atresia after Kasai surgery. Methods: Infants within six months after the Kasai operation who were diagnosed by cholangiography at Shanghai Children's Hospital were enrolled in the study. Fecal samples were collected from diapers, placed into sterile tubes in the inpatient department or outpatient department and frozen at -80°C within half an hour. The gut microbiota was detected by 16S rRNA sequences. Then, the patients that were followed up to one year after the Kasai operation who suffered from cholangitis at least one time were grouped into the BAcho group, and the others were grouped into the BAnoncho group. Results: Nine of 18 BA patients were grouped into the BAcho group, and the others were grouped into the BAnoncho group. In the BAcho group, AST, ALT and GGT were significantly increased compared to the BAnoncho group. The number of total OTUs (operational taxonomic units) in feces was more elevated in the BAnoncho group than in the BAcho group. In the BAnoncho group, the Chao index at the OTU level was significantly increased compared to that in the BAcho group (66.37 ± 21.5 vs. 45.64 ± 11.25, p = 0.02 < 0.05). Bifidobacterium was the most abundant genus in the BAnoncho group, accounting for 22.14%, and Klebsiella accounted for 22.74% in the BAcho group. Compared with the BAnoncho group, Bacteroides was significantly decreased in the BAcho group (p = 0.037). Conclusion: The composition of the gut microbiota was different between BA with cholangitis and BA without cholangitis.

Surgical and Medical Aspects of the Initial Treatment of Biliary Atresia: Position Paper.

Biliary atresia, a fibro-obliterative disease of the newborn, is usually initially treated by Kasai portoenterostomy, although there are many variations in technique and different options for post-operative adjuvant medical therapy. A questionnaire on such topics (e.g., open vs. laparoscopic; the need for liver mobilisation; use of post-operative steroids; use of post-operative anti-viral therapy, etc.) was circulated to delegates (n = 43) of an international webinar (Biliary Atresia and Related Diseases-BARD) held in June 2021. Respondents were mostly European, but included some from North America, and represented 18 different countries overall. The results of this survey are presented here, together with a commentary and review from an expert panel convened for the meeting on current trends in practice.

Relationship between the expression levels of CD4+ T cells, IL-6, IL-8 and IL-33 in the liver of biliary atresia and postoperative cholangitis, operative age and early jaundice clearance.

OBJECTIVE: To investigate the expression levels of CD4+ T cells, IL-6, IL-8 and IL-33 in liver tissue of BA, and the relationship with postoperative cholangitis, operative age and early jaundice clearance. METHODS: 45 cases of jaundice treated in the hospital from June 2018 to May 2020 were analyzed retrospectively. The expression and distribution of these factors were detected by HE staining and immunohistochemistry, the total bilirubin level and the incidence of cholangitis were recorded, and the relationship between liver inflammation level and the postoperative incidence of cholangitis, age of operation and early jaundice clearance were compared. RESULTS: Immunohistochemistry showed that the expression of CD4+ T cells, IL-6, IL-8 and IL-33 in the BA group were higher than those in the CBD group. ROC curve analysis showed the AUC of CD4+ T cells, IL-6 and IL-8 were 0.869, 0.886 and 0.838, respectively. The expression level of CD4+ T cells was negatively correlated with the decline rate of TBIL 3 months after operation, and the expressions of IL-8 and IL-33 were negatively correlated with the decline rate of TBIL 1 week after operation. CONCLUSION: The high expression of CD4+ T cells, IL-6, IL-8 and IL-33 in the BA liver tissue may lead to cholangitis and can be used as a predictor of early jaundice clearance. The degree of liver inflammation infiltration had nothing to do with the age of operation and is not a risk factor for postoperative cholangitis.

Autologous bone marrow mononuclear cell infusion for liver cirrhosis after the Kasai operation in children with biliary atresia.

AIM: To evaluate the safety and early outcomes of autologous bone marrow mononuclear cell (BMMNC) infusion for liver cirrhosis due to biliary atresia (BA) after Kasai operation. METHODS: An open-label clinical trial was performed from January 2017 to December 2019. Nineteen children with liver cirrhosis due to BA after Kasai operation were included. Bone marrow was harvested through anterior iliac crest puncture under general anesthesia. Mononuclear cells (MNCs) were isolated by Ficoll gradient centrifugation and then infused into the hepatic artery. The same procedure was repeated 6 months later. Serum bilirubin, albumin, alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, and prothrombin time were monitored at baseline, 3 months, 6 months, and 12 months after the first transplantation. Esophagoscopies and liver biopsies were performed in patients whose parents provided consent. Mixed-effect analysis was used to evaluate the changes in Pediatric End-Stage Liver Disease (PELD) scores. RESULTS: The average MNC and CD34+ cell counts per kg body weight were 50.1 ± 58.5 × 106/kg and 3.5 ± 2.8 × 106 for the first transplantation and 57.1 ± 42.0 × 106/kg and 3.7 ± 2.7 × 106 for the second transplantation. No severe adverse events associated with the cell therapy were observed in the patients. One patient died 5 months after the first infusion at a provincial hospital due to the rupture of esophageal varices, while 18 patients survived. Liver function was maintained or improved after infusion, as assessed by biochemical tests. The severity of the disease reduced markedly, with a significant reduction in PELD scores. CONCLUSION: Autologous BMMNC administration for liver cirrhosis due to BA is safe and may maintain or improve liver function. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT03468699. Name of the registry: Vinmec Research Institute of Stem Cell and Gene Technology. https://clinicaltrials.gov/ct2/show/NCT03468699?cond=biliary+atresia&cntry=VN&draw=2&rank=2 . Registered on March 16, 2018. The trial results will also be published according to the CONSORT statement at conferences and reported in peer-reviewed journals.

Biliary Atresia: Clinical Phenotypes and Aetiological Heterogeneity.

Biliary atresia (BA) is an obliterative condition of the biliary tract that presents with persistent jaundice and pale stools typically in the first few weeks of life. While this phenotypic signature may be broadly similar by the time of presentation, it is likely that this is only the final common pathway with a number of possible preceding causative factors and disparate pathogenic mechanisms-i.e., aetiological heterogeneity. Certainly, there are distinguishable variants which suggest a higher degree of aetiological homogeneity such as the syndromic variants of biliary atresia splenic malformation or cat-eye syndrome, which implicate an early developmental mechanism. In others, the presence of synchronous viral infection also make this plausible as an aetiological agent though it is likely that disease onset is from the perinatal period. In the majority of cases, currently termed isolated BA, there are still too few clues as to aetiology or indeed pathogenesis.

Case Report: Kasai Operation in Biliary Atresia After Extensive Bowel Resection.

Biliary atresia (BA) is a major and devastating cholestatic liver disease in infancy. The Kasai procedure is an operation to re-establish bile flow from the liver into the intestine that can prevent the young infant from progressing rapidly to cirrhosis. The standard Kasai procedure includes the removal of extrahepatic bile duct remnants and reconstruction. We report a case of BA with short bowel due to previous small intestinal volvulus. This full-term female infant received extensive small bowel resection after birth due to intestinal volvulus. The length of the residual small bowel was 55 cm with an intact ileocecal valve. Because of progressive cholestasis and clay stool, another laparotomy was performed under the diagnosis of BA on the 52 days old. After dissection of the hepatic portal area, a segment of the colon instead of intestine was used as a biliary conduit to avoid further shortening her small bowel. The patient recovered from the procedure uneventfully and the parenteral nutrition was discontinued 2 weeks later. Two episodes of cholangitis happened after discharge. She gradually resumed body weight gain and the bilirubin level returned to normal range 6 months after the operation. This unique case demonstrated successful use of this specific procedure in the patient with BA and short bowel that have never been reported in the literature.

Standard management protocol to improve the short-term outcome of biliary atresia.

AIM: This study compared the outcomes of patients with biliary atresia (BA) treated according to a standardised protocol with historical patients. METHODS: This is a single-centred retrospective study of BA patients treated from 1980 to 2016. A standardised treatment protocol was established since 2008 regarding peri-operative management. The outcomes being compared between the two groups (Groups I and II = before and after 2008, respectively) were jaundice clearance (JC), incidence of recurrent cholangitis, hospital admission and native liver survival (NLS). RESULTS: A total of 128 patients were included (Group I = 100, Group II = 28). The overall median follow-up period was 15.3 years (I vs. II = 20.6 years vs. 5.1 years, respectively). There was no significant difference in the JC at the sixth month between the two groups (I vs. II = 60.0 vs. 82.1%, respectively, P = 0.07). The incidence of recurrent cholangitis was similar between the two groups (I vs. II = 39 vs. 35.7%, respectively, P = 0.45), but the median hospital admission episode per patient was non-significantly higher in Group I (I vs. II = 4.2 vs. 2.7, respectively, P = 0.08). There was an improvement in the 1-year NLS rate in Group II (I vs. II = 69.0 vs. 85.7%, respectively, P = 0.05). CONCLUSIONS: The introduction of a standardised management protocol has improved the short-term outcome of BA patients, with a better 1-year NLS observed.

MicroRNA-499 rs3746444 polymorphism in Egyptian children with biliary atresia.

AIM OF THE STUDY: We aimed to evaluate the association of microRNA-499 rs3746444 polymorphism and biliary atresia (BA) risk and its correlation with clinic-pathologic features of BA. MATERIAL AND METHODS: This study was performed on 300 Egyptian children (100 BA cases, 100 cases with cholestatic liver diseases other than BA and 100 healthy controls). Routine laboratory investigations, clinical examination and abdominal ultrasound were done. All infants were genotyped for miR-499 single nucleotide polymorphisms (SNPs) (rs3746444 A>G) by real-time polymerase chain reaction (PCR) fluorescence detection on a Rotor Gene Real Time PCR System (QIAGEN, GmbH) using fluorescent labeled probes. RESULTS: The AG genotype was the most prevalent genotype of miR-499 rs3746444 among the studied groups. A significantly higher frequency of the rs3746444 G allele was found in the BA cases than the other groups (odds ratio = 1.62). This polymorphism was also correlated with the degree of fibrosis in BA cases (p < 0.05). The miR-499 rs3746444 polymorphism (GG genotype) was significantly associated with severe form of BA and bad prognosis after the Kasai operation (p < 0.05). miR-499 rs3746444 polymorphism had no effect on the clinic-pathological features or the liver function status in the non-BA group. CONCLUSIONS: There is an association between the miR-499 SNP genotypes and the occurrence of BA. The variant allele G is the predominant allele in the BA group and is associated with severe liver inflammation and bad prognosis after the Kasai operation.

Perihilar cholangiocarcinoma in an explanted liver after Kasai operation for biliary atresia: A case report and literature review.

Kasai operation is widely performed for biliary atresia (BA), as it improves the prognosis. Biliary tract cancer has rarely been reported as a complication after Kasai operation. A 17-year-old man underwent liver transplantation for progressive jaundice and liver dysfunction after Kasai operation for BA. A macroscopic examination of the explanted liver revealed a 3.6-cm-diameter mass in the hilus of the explanted liver. The tumor consisted of an atypical proliferation of glandular cells in the collagenous stroma. The differential diagnosis included a florid ductular reaction and primary adenocarcinoma. Immunohistochemistry revealed that the glandular cells were diffusely positive for IGF2BP3 and S100P, but negative for CDX2. A diagnosis of extrahepatic cholangiocarcinoma arising from the liver hilus was made. To our knowledge, this is the third case of perihilar cholangiocarcinoma after Kasai operation for BA. The previously reported cases had a poor prognosis, but the current case did not recur during a 15-year follow-up. Cholangiocarcinoma and hepatocellular carcinoma can occur as a late complication of BA after Kasai operation, and early detection and liver transplantation may improve the prognosis.

Incidence and Risk Factors of Acute Kidney Injury after Kasai Operation for Biliary Atresia: A Retrospective Study.

Background: Biliary atresia is a progressive, inflammatory, and destructive pathology of the bile ducts. Patients who undergo surgery for correction of biliary atresia (Kasai operation) are at risk of acute kidney injury (AKI) because of their young age at the time of surgery, long operation time, and liver fibrosis or failure as complication of biliary atresia. Conversely, AKI is associated with poor outcomes after surgery. This study therefore aimed to evaluate the incidence, risk factors, and outcomes of AKI after Kasai operation. Methods: All consecutive patients who underwent Kasai operation between March 2006 and December 2015 in a single tertiary-care university hospital were enrolled. AKI was defined based on the Acute Kidney Injury Network criteria. Multivariate logistic regression models were used to assess risk factors for AKI. Results: One hundred sixty-six patients received Kasai operation during study period. Of these, AKI occurred in 37 of 166 patients (22.3%). In multivariate logistic regression analysis, age older than 30 days, higher preoperative estimated glomerular filtration rate, and preoperative contrast use within 7 days were associated with the development of AKI. Perioperative packed red blood cells transfusion was related to reduced occurrence of AKI. AKI was associated with longer ICU stay (OR = 1.015, p = 0.016). More patients with AKI were also found to receive additional surgery except liver transplantation within 1 year compared to those without AKI (10.8 % vs. 2.3 %, p = 0.045). Conclusions: Increased age is strongly associated with the development of AKI after Kasai operation. These findings indicate a rational basis for early corrective surgery for biliary atresia, early screening for AKI, and intervention to improve the results of Kasai operation.

Error traps and culture of safety in biliary atresia.

Biliary atresia is a progressive inflammatory sclerosing disease of the bile ducts in the neonatal liver. Without surgical intervention these patients are destined to succumb to the disease. The development of the hepatoportoenterostomy in 1959 and liver transplantation in 1963 ushered a new era of success treating these patients. While many surgical modifications and adjuncts to treatment have been attempted over the last 50 years, the mainstay of treatment to give the child the best chance at prolonged survival with the native liver is a properly performed operation, in a timely fashion, with minimal post-operative complications. This review presents the authors current practice guideline to achieve these goals.

[Advantages of Kasai procedure through minimally invasive approach in children with biliary atresia (in Russian only)]. / Preimushchestva mini-dostupa pri vypolnenii operatsii Kasai u detei s biliarnoi atreziei.

AIM: To compare early and long-term results of different surgical interventions in children with biliary atresia. MATERIAL AND METHODS: Retrospective analysis included medical records of children with biliary atresia who were treated at the Filatov Munitsipal Children's Hospital and National Medical Research Center for Obstetrics, Gynecology and Perinatology from 2000 to 2018. There were 91 patients. All patients were divided into three groups. Group 1 - conventional Kasai procedure (n=24), group 2 - laparoscopic Kasai surgery (n=45), group 3 - Kasai procedure through minimally invasive approach (n=22). Groups were comparable. RESULTS: Duration of Kasai procedure through minimally invasive approach was 69±12,97 min that was significantly less than in groups 1 and 2 (p1,3=0,006085; p2,3=0,000024). ICU-stay was minimal in group 3 (1.27±0.55 days, p1,3<0,05; p2,3<0,05). Abdominal drainage time was maximal in group 2 (11.28±6.37 days) and minimal in group 3 (5.86±2.39 days, p2,3=0.0002). Early and 2-year postoperative surgical efficiency was similar in all groups. There were no surgical complications in group 3. In group 2 one child had gastrointestinal bleeding followed by successful medication. There were 3 surgical complications in group 3: adhesive intestinal obstruction, small and large intestine perforation and 2 cases of gastrointestinal bleeding. There was one lethal outcome in the first group. Overall annual survival in children with native liver was 81.8%, 2-year - 51.7%. CONCLUSION: Kasai procedure through minimally invasive approach is justified and rational method with certain benefits of open and laparoscopic interventions and can be considered as a method of choice in treatment of children with biliary atresia.

Predicting gastroesophageal varices through spleen magnetic resonance elastography in pediatric liver fibrosis.

BACKGROUND: A recent retrospective study confirmed that hepatic stiffness and splenic stiffness measured with magnetic resonance elastography (MRE) are strongly associated with the presence of esophageal varices. In addition, strong correlations have been reported between splenic stiffness values measured with MRE and hepatic venous pressure gradients in animal models. However, most studies have been conducted on adult populations, and previous pediatric MRE studies have only demonstrated the feasibility of MRE in pediatric populations, while the actual clinical application of spleen MRE has been limited. AIM: To assess the utility of splenic stiffness measurements by MRE to predict gastroesophageal varices in children. METHODS: We retrospectively reviewed abdominal MRE images taken on a 3T system in pediatric patients. Patients who had undergone Kasai operations for biliary atresia were selected for the Kasai group, and patients with normal livers and spleens were selected for the control group. Two-dimensional spin-echo echo-planar MRE acquisition centered on the liver, with a pneumatic driver at 60 Hz and a low amplitude, was performed to obtain hepatic and splenic stiffness values. Laboratory results for aspartate aminotransferase to platelet ratio index (APRI) were evaluated within six months of MRE, and the normalized spleen size ratio was determined with the upper normal size limit. All Kasai group patients underwent gastroesophageal endoscopy during routine follow-up. The Mann-Whitney U test, Kendall's tau b correlation and diagnostic performance analysis using the area under the curve (AUC) were performed for statistical analysis. RESULTS: The median spleen MRE value was 5.5 kPa in the control group (n = 9, age 9-18 years, range 4.7-6.4 kPa) and 8.6 kPa in the Kasai group (n = 22, age 4-18 years, range 5.0-17.8 kPa). In the Kasai group, the APRI, spleen size ratio and spleen MRE values were higher in patients with portal hypertension (n = 11) than in patients without (n = 11) (all P < 0.001) and in patients with gastroesophageal varices (n = 6) than in patients without (n = 16) (all P < 0.05), even though their liver MRE values were not different. The APRI (τ = 0.477, P = 0.007), spleen size ratio (τ = 0.401, P = 0.024) and spleen MRE values (τ = 0.426, P = 0.016) also correlated with varices grades. The AUC in predicting gastroesophageal varices was 0.844 at a cut-off of 0.65 (100% sensitivity and 75% specificity) for the APRI, and 0.844 at a cut-off of 9.9 kPa (83.3% sensitivity and 81.3% specificity) for spleen MRE values. CONCLUSION: At a cut-off of 9.9 kPa, spleen MRE values predicted gastroesophageal varices as well as the APRI and spleen size ratio in biliary atresia patients after the Kasai operation. However, liver MRE values were not useful for this purpose.

Vitamin A can ameliorate fibrosis of liver in an established rat model of biliary atresia and Kasai portoenterostomy.

BACKGROUND: Antifibrosis therapy may prevent progressive liver fibrosis after successful Kasai portoenterostomy (KPE) in biliary atresia (BA) patients. The aim of this study is to evaluate the efficacy of antifibrosis therapy in a rat model of BA and KPE. METHODS: BA model was created on three-week-old Sprague-Dawley rats by intrabiliary alcohol injection as previously described, and KPE was performed at postoperative week (POW) 5 by cystoenterostomy. Liver biopsies were performed at the time of BA creation, during KPE, POW 9, and at sacrifice (POW 17). Prednisolone (0.1 mg/100 g/day, group 1, n = 20), Vitamin A (0.5 mg/100 g/day, group 2, n = 20), and ursodeoxycholic acid (UDCA, 1.5 mg/100 g/day, group 3, n = 20) were respectively given to three groups after KPE and continued daily until sacrifice. Histological evaluation of fibrosis and immunohistochemistry stains for 8 fibrosis markers were compared to the control group (without medication, n = 10). RESULTS: Among the four markers, namely ɑ-smooth muscle actin (ɑ-SMA), glial fibrillary acidic protein (GFAP), tumor growth factor ß1 (TGFß1) receptors 1 and 2, which showed persistently high expression after successful KPE in the examined 8 markers, only the expression of ɑ-SMA was significantly reduced in all treatment groups at POW17. However, the fibrosis grade at POW 17 was only significantly reduced in group 2 in comparison with the control group (Vitamin A vs. control group, Ishak score 3 vs. 1.8, p < 0.05). CONCLUSION: In our rat model of BA with KPE, Vitamin A was effective in reducing liver fibrosis, and the mechanisms deserve further study. LEVEL OF EVIDENCE: Basic science.

Does the identity of the bacteria matter in post-Kasai cholangitis? A comparison between simple and intractable cholangitis.

PURPOSE: This study compared the bacteriology and clinical outcomes between simple (SC) and intractable cholangitis (IC) after Kasai operation. METHODS: Post-Kasai patients (n = 192) from 1980 to 2015 were retrospectively reviewed. The results of blood culture and clinical outcomes between the patients with SC and IC were compared. MAIN RESULTS: A total of 102 cholangitic episodes in 68 patients were analyzed (SC vs IC = 76 vs 26). There were more IC episodes within the first year of Kasai operation (SC vs IC = 36.8% vs 61.5%, p = 0.022). The most common bacteria identified in SC and IC groups were Escherichia Coli and Staphylococcus aureus. Until the latest follow up, the native liver survival rates in patients with SC and IC were 75.0% and 50.0% (p = 0.89). Among the patients with IC, the native liver survival rate was significantly better in those with a positive culture (100% vs 20%, p = 0.001). CONCLUSION: Intractable cholangitis is a common complication within the first year of Kasai operation and may be caused by a different spectrum of organisms. The identification of the bacteria by blood culturing may result in a better treatment outcome. LEVEL OF EVIDENCE: Level III.

Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis.

PURPOSE: Infants with Alagille syndrome (AGS) frequently develop neonatal cholestasis, and some AGS infants who suspected of biliary atresia subsequently undergo the Kasai operation with the diagnosis of biliary atresia. The aim of this study was to investigate the effect of the Kasai operation on liver and patient outcomes among AGS patients, using a meta-analysis. METHODS: A systematic review and meta-analysis of studies describing the outcomes of AGS patients with/without the Kasai operation were conducted. The analyzed outcomes were liver transplantation, not living with the native liver, and mortality for any reason. RESULTS: We identified 6 studies (394 AGS patients). All studies were retrospective cohort or case-control studies. The incidences of liver transplantation, not living with the native liver, and mortality were significantly higher in AGS patients who underwent the Kasai operation than in those who did not undergo the Kasai operation (odds ratio: 6.46, 95% CI 3.23-12.89, p < 0.00001; odds ratio: 25.88, 95% CI 2.83-236.84, p < 0.004; odds ratio: 15.05, 95% CI 2.70-83.93, p = 0.002, respectively). CONCLUSION: The Kasai operation was associated with poor outcomes in AGS patients. It remains unclear if the Kasai operation directly deteriorates liver and patient outcomes in AGS patients.

Reconsideration of Laparoscopic Kasai Operation for Biliary Atresia.

INTRODUCTION: Minimal invasive surgery for all kinds of surgical diseases had been practiced for years. The laparoscopic Kasai operation is one of the most challenging procedures and remains controversial for treating biliary atresia (BA). PURPOSE: This work presented the initial experience of our Institute and compared the outcomes of open and laparoscopic Kasai operations for BA. MATERIALS AND METHODS: Patients 18 years old and younger, and were operated in our Institute for BA from January 2011 to August 2017, were included in this study. General and operative data and outcomes from open and laparoscopic groups were retrospectively collected and analyzed. RESULTS: A total of 23 patients (13 for conventional open operation and 10 for the laparoscopic procedure) received Kasai portoenterostomy. The mean operative age and body weight in the open versus laparoscopic groups were 57.15 ± 20.14 days old and 4.03 ± 0.69 kg versus 57.70 ± 43.06 days old and 4.49 ± 1.48 kg, respectively, and no statistical difference was observed. The mean operative times were 209.62 ± 60.40 and 293.50 ± 39.09 minutes in the open and laparoscopic groups, respectively. The mean follow-up durations were 54.62 ± 22.00 and 23.30 ± 11.87 months for the open and laparoscopic patients, correspondingly. No statistically significant differences were observed for hospital stay and outcomes (including early jaundice clearance rate, episodes of cholangitis, and 2-year native liver survival rate) between the open and the laparoscopic Kasai operations. CONCLUSION: Experienced pediatric laparoscopic surgeons may reconsider the laparoscopic Kasai operation for application to BA treatment.

A review of long-term outcome and quality of life of patients after Kasai operation surviving with native livers.

Biliary atresia (BA) is a rare neonatal cholestatic disease which leads to progressive obliterative cholangiopathy, resulting in biliary obstruction and jaundice. The standard surgical treatment is hepatoportoenterostomy (Kasai operation). Although approximately 50% of the affected infants would require liver transplantation within the first 2 years of life, the other 50% of the patients can live for years with their native liver, despite the progression of cirrhosis and chronic liver disease. Many of these patients will be affected by long-term complications such as repeated cholangitis, portal hypertension, variceal bleeding, growth problems, biochemical abnormalities, and hepatic osteodystrophy. These morbidities impose a huge impact on the quality of life of the patients and their families. Herein, we performed a comprehensive review on the clinical status and quality of life of long-term survivors of biliary atresia with their native livers, to facilitate meticulous longitudinal follow-up of these patients, and alert caregivers the probable complications to be aware of.