Kasai Portoenterostomy Research Articles

Expression of activin A in liver tissue and the outcome of patients with biliary atresia

Biliary atresia (BA) is a rare disease of unknown etiology which leads to cirrhosis and death if left untreated. The standard of care is an early hepatoportoenterostomy (HPE). Long-term follow-up is mandatory, during which most patients will require a liver transplant. Activin A belongs to the transforming growth factor-β (TGF-β) superfamily. TGF-β is a central regulator in chronic liver disease. We have studied the expression of activin A in liver tissue collected intraoperatively during the HPE. We included patients who underwent HPE in a single medical center. Clinical, ultrasonographic, and pathohistological data were collected. Activin A immunostaining was performed. Expression in the bile duct epithelium and hepatocytes was scored as either weakly positive, moderately positive, or strongly positive. Patients were then divided into three groups accordingly. We observed the outcome after the HPE at 3 months, 2 years, and at the end of follow-up. The study encompassed 37 patients. At 3 months after HPE, 92.3% of those with a weakly positive activin A reaction (group A) achieved good jaundice clearance, whereas only 44.4% of those with a moderately (group B) and 40% of those with a strongly positive reaction (group C) achieved good jaundice clearance (p = 0.008). Furthermore, 2 years after the HPE, 92.3% of those in group A survived with native liver (SNL), but only 33.3% of those in group B and 46.7% of those in group C had SNL (p = 0.007). At the end of follow-up, 83.3% of those in group A survived with native liver, as did 33.3% in group B and 40% in group C. Activin A is a valuable pathohistological predictor of the outcome of BA after an HPE.

Comparison between robotic-assisted Kasai portoenterostomy and open Kasai portoenterostomy in patients with biliary atresia.

Robotic-assisted Kasai portoenterostomy (RAKPE) is regarded as a treatment option for patients with biliary atresia (BA). We aimed to compare the clinical outcomes of RAKPE to the open Kasai portoenterostomy (OKPE). A retrospective review was conducted on a total of 74 type III BA patients who underwent RAKPE (RA group, n = 36) or OKPE (OP group, n = 38) from January 2018 to December 2022. The study analyzed demographic characteristics, intraoperative findings, and postoperative outcomes. The operative time in the RA group was significantly longer than that in the OP group (218.61 ± 31.70min vs 178.50 ± 27.90min, P < 0.05). The estimated blood loss in RA group was significantly lower than that in the OP group (8.65 ± 2.30mL vs 17.55 ± 3.60mL, P < 0.05). The recovery time of bowel sounds was significantly shorter in RA group than in the OP group (0.96 ± 0.12days vs 2.84 ± 0.37days, P < 0.05). All patients had bile-stained stools within 1-3days after surgery. The clearance rate of jaundice at 3 and 6months were significantly higher in RA group than in OP group (69.44% vs 60.53%, 75.00% vs 68.42%, P < 0.05). The rate of cholangitis in RA group was similar to that in OP group (50.00% vs 52.63%, P > 0.05). The native liver survival rate during the follow-up period was comparable between two groups (66.67% vs 63.16%, P > 0.05). Robotic-assisted Kasai surgery offers advantages in short-term outcomes and the clearance of jaundice in patients with type III BA.

Case Reports of Type 1 Cystic Biliary Atresia- A rare variant of Biliary atresia along with review of literature

Abstract Cystic biliary atresia is a rare (5-10%) variant of type I biliary atresia. The present report intends to share our experience with cystic variant of biliary atresia along with a review of pertinent literature. The first case was a 3-month-old infant with cholestatic jaundice with preoperative diagnosis of an infantile type of choledochal cyst. The second case was a 4-month-old twin infant with cholestatic jaundice and with preoperative differential diagnoses of either cystic biliary atresia or an infantile type of choledochal cyst (cyst located within the hepatic hilum). Cystic biliary atresia was confirmed intra-operatively in both the patients. In first infant, Kasai portoenterostomy procedure was performed, while in the second patient hepaticoduodenostomy procedure was performed. At 6 months follow-up, there was no episode of cholangitis in first and two episodes in second case. In both patients’ total bilirubin was trending down on follow-up. Cystic biliary atresia presents with cystic dilatation of proximal biliary tract. Type I cystic biliary atresia may masquerade as infantile variant of choledochal cyst. The Kasai portoenterostomy procedure is a standard palliative surgical procedure for patients with biliary atresia to restore bile drainage. A high index of suspicion with early operative intervention is recommended to palliate cholestasis in the infant with cystic variant of biliary atresia.

Primary antibiotic prophylaxis in biliary atresia did not demonstrate decreased infection rate: Multi-centre retrospective study.

This retrospective study aimed to assess the efficacy of prophylactic antibiotics in preventing ascending cholangitis following Kasai portoenterostomy (KPE). Data from 72 patients treated across four tertiary centres in Israel from 2008 to 2018 were analysed. Clinical and laboratory data were collected from biliary atresia (BA) diagnosis until liver transplantation (LT) or study completion. Median age at KPE was 58.5 days. Successful KPE was achieved in 23 (32%) patients. Ascending cholangitis occurred in 6/23 (26%) successful KPE cases and 15/45 (33%) unsuccessful cases. Primary antibiotic prophylaxis (49% of patients) was associated with earlier onset of cholangitis (median 77 vs 239 days, p = 0.016). During follow-up, 39% underwent LT, with a 5-year survival with native liver (SNL) of 54%. Prophylactic antibiotics did not reduce cholangitis rates post-KPE in our cohort. Further research is essential to optimise management strategies for infants with BA.

The association of human milk intake and outcomes in biliary atresia.

Human milk intake has many benefits which could influence outcomes in biliary atresia (BA). However, the role of human milk in BA has not been examined. We hypothesized that human milk intake would be associated with improved outcomes in BA. We assessed the impact of any human milk (AHM) as compared to formula only (FO) intake before Kasai portoenterostomy (KP) on outcomes in 447 infants with BA using the PROBE database (NCT00061828) post hoc. The primary outcome was clearance of jaundice (COJ = total bilirubin (TB) < 2 mg/dL by 3 months post-KP). Secondary outcomes included 2-year native liver survival (SNL), bilirubin levels, cholangitis, ascites, and growth. We assessed the fecal microbiome (n = 8) comparing AHM versus FO. At baseline, 211 infants received AHM and 215 received FO. 53.9% of AHM and 50.5% of FO achieved COJ (p = NS). SNL was insignificantly increased in AHM (odds ratio = 1.47, 95% confidence interval: 1.00-2.12, p = 0.053). TB decreased in AHM from 4 weeks to 3 months post-KP [4.8-4.0 mg/dL (p = 0.01)] unlike the FO group (4.9-4.9 mg/dL, p = 0.4). At 3 months post-KP, AHM infants had greater weight gain (1.88 ± 0.66 vs. 1.57 ± 0.73 kg, p < 0.001) and mid-upper arm circumference (12.9 ± 1.4 vs. 12.2 ± 1.7 cm, p < 0.001). Other secondary outcomes were not different. Microbiome differences were seen between AHM and FO. Human milk intake in infants with BA did not significantly improve COJ or SNL. However, growth parameters were improved, and TB 3 months post-KP was decreased. Thus, human milk intake should not be discouraged. Prospective studies with detailed assessment of human milk intake are needed.

Role of percutaneous transhepatic biliary drainage for managing bile lake formation after Kasai portoenterostomy.

Bile lake (BL) formation following Kasai portoenterostomy (KPE) can complicate the prognosis of biliary atresia (BA). Percutaneous transhepatic biliary drainage (PTBD) performed under fluoroscopic/ultrasonographic (US) guidance is discussed for the management of BL. A retrospective review of 64 BA patients treated by KPE (open = 31, laparoscopic = 33) at a single center (2004-2023) identified 9 BL cases (9/64; 14.1%). PTBD was indicated for jaundice or cholangitis refractory to antibiotic therapy. All BL were asymptomatic, diagnosed after an episode of postoperative cholangitis. KPE type was not correlated with BL incidence; 2/31 (6.5%) for open and 7/33 (21.2%) for laparoscopic; p = 0.15. Median onset was postoperative day 273 (IQR: 170-920). One case resolved with antibiotics while another case required early liver transplantation (LTx) due to advanced hepatic dysfunction unrelated to BL. All remaining cases (7/9) had PTBD at a median of 14.3months (IQR: 7.3-34.7) post-KPE, with successful resolution in 6/7; one case required two PTBD procedures. Post-PTBD biliary peritonitis (n = 3) was resolved by abdominal lavage (laparoscopic = 2; open = 1). Screening for BL is advisable in all postoperative BA patients especially when cholangitis occurs. Although the risk for biliary peritonitis warrants caution, PTBD seems a viable option for managing BL formation after KPE.

Enhanced lymphangiogenesis in the left lateral segment of a biopsied liver during portoenterostomy for biliary atresia

PurposeWe investigate the histopathology of the portal vein branches and lymphatic vessels to elucidate the mechanism of atrophy of the left lateral segment (LLS) of the liver in biliary atresia (BA).MethodsLLS and right anterior segment (RAS) liver biopsy samples obtained during Kasai portoenterostomy (KPE) from ten consecutive patients with BA underwent histopathological investigation of the portal vein and lymphatic vessels using double chromogenic immunostaining for CD31/D2-40 and the hepatitis-like findings (HLF) score. Each parameter and clinical data were compared between prognostic groups.ResultsHLF scores in the LLS were always higher than those in the RAS. There was no difference in portal vein and lymphatic vascular morphology, whereas the number of lymphatic vessels was correlated with the fibrotic area of all specimen areas. Left-to-right ratio of the number of lymphatic vessels was correlated with the age at KPE (r = 0.784, p = 0.007) and the pre-KPE CRP value (r = 0.723, p = 0.018).ConclusionsLymphangiogenesis on the LLS compared to the RAS was significantly correlated with the degree of fibrosis and the age at KPE. Further investigation is warranted to clarify the causes of LLS atrophy and lymphangiogenesis relevant to immune dysregulation.

The Assessment and Management of Biliary Atresia in Hawai'i, 2009-2023.

Although biliary atresia (BA) is a rare neonatal disorder, it remains the leading cause of pediatric end-stage liver disease. Early diagnosis of BA and treatment with the Kasai procedure can significantly reduce the need for pediatric liver transplant. Current data suggests that performing the Kasai procedure at 30-45 days of life is associated with longer native liver survival rates and reduction of the need for liver transplant. The incidence rate of BA in the state of Hawai'i is nearly double the incidence rate in the continental US. International studies have demonstrated that screening programs for BA reduce the age at diagnosis and treatment. However, there has been no statewide analysis on the ages at diagnosis or at Kasai, nor does a statewide screening program for BA exist. The purpose of this study is to review the age of diagnosis and treatment of BA to determine if the current practice in Hawai'i is in line with the published data. A retrospective chart review of all patients diagnosed with BA at the state's primary children's hospital was performed (2009-2023) and 19 patients who underwent the Kasai procedure were identified. The mean age at diagnosis is 71.4 days (n=19) and the mean age at Kasai procedure is 72.0 days (n=19). Both the average age at diagnosis and treatment for BA in Hawai'i is significantly higher than published data suggesting best outcomes at 30-45 days of life. This review suggests that the implementation of a statewide screening program for BA in Hawai'i is warranted.

Experience and Results of Liver Transplantation in Patients With Alagille Syndrome at Our Center.

Alagille syndrome is an autosomal recessive disorder with multisystemic involvement. Patients with Alagille syndrome have many accompanying anomalies such as atypical facial appearance, posterior embryotoxon, butterfly vertebra, cardiac anomalies, and biliary problems. Liver and heart complications can determine the mortality and morbidity of patients with Alagille syndrome. The scarcity of intrahepatic bile ducts leads to cholestatic liver damage. In the heart, although the disease most commonly causes peripheral pulmonary stenosis, patients can present with various structural heart diseases. These cardiac anomalies make intraoperative and postoperative management difficult in patients who need liver transplant. Here, we present 10 cases of liver transplant due to Alagille syndrome. From 1988 to the present, our center performed 724 liver transplants, with 366 being pediatric cases; among these, 10 were due to Alagille syndrome. We retrospectively examined patient morphological findings, cardiac pathology, and perioperative complications. Average weight and age of patients were 11 kg and 4.9 years, respectively. Eight patients had atypical facial appearance, 5 had posterior embryotoxon, and 6 had butterfly vertebra. Portoenterostomy (Kasai procedure) was performed in 3 patients because of suspicion of early biliary atresia. Cardiac pathology was present in 8 patients, with peripheral pulmonary stenosis being the most common (present in 8 patients). One patient had cardiac catheterization, with right ventricular and pulmonary artery pressures measured at 110 and 37 mm Hg, respectively. Two patients required preoperative pulmonary angioplasty. Two patients had double superior vena cava. No complications occurred in any patient during the anhepatic phase with appropriate volume replacement. No patients had early mortality. Cardiac anomalies should be identified in patients with Alagille syndrome to minimize complications before liver transplant. If necessary, cardiac catheterization should be performed for pulmonary stenosis. Peripheral pulmonary stenosis does not constitute an absolute contraindication to liver transplant.

Newborn screening for biliary atresia using direct bilirubin: An implementation science study.

Biliary atresia (BA) is a liver disease of infancy characterized by obstruction of the biliary tree. Infants with BA have the best outcomes when identified early and the Kasai portoenterostomy is performed before 45 days of life (DoL). In our hospital system, the average age at Kasai was 60 DoL. To address the problem of late presentation, we implemented a two-stage BA screening strategy utilizing direct bilirubin (DB). New institutional policies were established that all newborns were tested at 24-48 h of life, and those with levels ≥0.5 mg/dL were followed further. The infant's primary care provider was contacted to recommend a repeat DB at 2 weeks of life. If the repeat DB was ≥1.0 mg/dL, the patient was evaluated by gastroenterology. Over the 16 months, 3880 infants were born and 3861 (99.5%) were screened; 53 infants (1.3%) had DB levels ≥0.5 mg/dL initially. Upon repeat testing at 2 weeks, there were three groups of infants: not retested (n = 1), retested <1.0 mg/dL (n = 40), and retested ≥1.0 mg/dL (n = 12). The average time to be seen by gastroenterology was 4.3 days or 18.3 DoL. The screening included a series of steps that needed to be implemented effectively. Screening had a net false positive rate of 0.3% (12 out of 3861) and identified causes of cholestasis other than BA. BA was excluded by 28 DoL on average. Our results can provide a template for other institutions interested in implementing a BA screening protocol in their practice.

Identification of Novel Biomarkers for Post-Kasai Portoenterostomy in Biliary Atresia through Shotgun Proteomics Analysis

Introduction: Biliary Atresia (BA) causes neonatal cholestasis jaundice. The primary therapeutic treatment for BA is the Kasai portoenterostomy. Current diagnostic approaches for BA are imprecise and time-consuming, making early diagnosis crucial for successful treatment outcomes. Objective: This study aims to analyze proteins from Peripheral Blood Mononuclear Cells (PBMCs) obtained from children with BA compared with healthy children Methods and Study Design: We employed a large-scale, total shotgun quantitative serum proteomics approach to analyze the protein from PBMC samples from a discovery cohort. This approach allowed for the simultaneous identification and quantification of multiple proteins, enabling the detection of disease-specific protein expression patterns. The study is proteomic-based study. Results: We identified 24 proteins, by Liquid Chromatography-Mass Spectrometry (LC-MS) analysis that exhibited high discriminatory power for five subjects with BA post-Kasai operation compared to ten healthy controls. ATP2A3, LIN28B, SLC25A3, ITGB3, COX5A, and HLA-B identified proteins of upregulation were predicted to associate with BA post-Kasai operation. Discussion: Our findings highlight the utility of proteomic techniques in BA research. The identified proteomic markers offer promise for improving BA diagnostic accuracy and timeliness, leading to enhanced treatment outcomes for affected children. Conclusion: Proteomic analysis revealed a set of potential biomarkers for early and accurate diagnosis of biliary atresia. These biomarkers hold significant clinical value and have the potential to transform the management of biliary atresia by facilitating timely intervention and improving patient outcomes.

Calcification of the mesenteric veins suspected of idiopathic mesenteric phlebosclerosis associated with long‐term herbal medicine ‘inchinkoto’ prescribed for biliary atresia: A multicenter study

AbstractAimInchinkoto (ICKT) is administered to patients with biliary atresia (BA) after surgery. Gardeniae fructus which is contained in ICKT is reported to be associated with idiopathic mesenteric phlebosclerosis (IMP). We investigated the influence of ICKT on calcification of the mesenteric veins suspected of IMP in BA patients.MethodsAll BA patients with native liver survival who received ICKT after Kasai portoenterostomy were reviewed. All patients underwent abdominal computed tomography (CT) during treatment. Calcification of the mesenteric veins on the most recent CT scan after the initiation of ICKT was assessed.ResultsTwenty patients (6 males and 14 females) were enrolled in this study. Of these patients, 17 (85.0%) and 10 (50%) patients had used ICKT for ≥5 years and &gt;10 years, respectively. Calcification of the mesenteric veins was detected in one patient, who was a 14 year old girl. The total dosage of Gardeniae fructus was 21 800 g.ConclusionsThe incidence of calcification of the mesenteric veins in BA patients receiving ICKT was low. However, it is necessary to be aware that ICKT containing Gardeniae fructus is associated with the risk of developing calcification of the mesenteric veins suspected of IMP.

A case series of prenatal hepatic hilar cyst in the presence of a gallbladder - navigating the dilemma between biliary atresia and choledochal cyst

BackgroundPrenatally diagnosed hepatic hilar cysts are a challenging finding for the clinician. They can either be a sign of cystic biliary atresia (BA) or a choledochal cyst (CC), two diagnoses with different postnatal management and prognosis. Based on a case report of four patients, we aim to propose a management algorithm for prenatally diagnosed “hepatic hilar cysts”.Case presentationA hepatic hilar cyst, ranging from 5 to 25 mm, was detected prenatally in all four girls confirmed postnatally along with the presence of a gallbladder. Stool color was normal until two weeks of life at which time the stool color became lighter, and the patients developed cholestasis. All were operated before seven weeks of life: Case 1 had a CC with patent but irregular intrahepatic bile ducts at intraoperative cholangiogram, and no communication with the duodenum. A Roux-en-Y bilioenteric anastomosis was performed. The cyst showed complete epithelial lining loss, and liver pathology showed BA features. Case 2 had the final diagnosis of cystic BA with patent but abnormal intrahepatic bile ducts. She underwent two operations: the first operation at four weeks as described for case 1, since intraoperative findings were similar, as was histology. As cholestasis increased postoperatively, she underwent a Kasai hepato-porto-enterostomy six weeks later, where distinct BA findings were found with complete scarring of the hilar plate. Case 3 had a cystic BA with the cyst located within the common bile duct and atretic bile ducts proximal to the porta hepatis. It exhibited no communication with the liver or duodenum. A Kasai operation was performed, with histology showing complete epithelial loss within the cyst wall and scarring of the hilar plate. Case 4 had a cystic BA presenting a completely obliterated hepatic duct with the cyst lying within the common bile duct. A Kasai procedure was performed. Histology showed a common bile duct with a residual lumen of 0.1 mm.ConclusionsThe spectrum of disease from CC to BA in the setting of a prenatally discovered hepatic hilar cyst is emphasized. Even if cholangiogram differentiates most patients with BA from those with CC, caution is advised for transitional types.

The Predictive Value of Gamma-Glutamyl Transferase on Jaundice Clearance in Biliary Atresia: An Evidence-Based Case Report and Meta-Analysis

Background: Biliary atresia (BA) is a neonatal condition characterized by the obstruction of extrahepatic bile ducts. Case: A 2-year-old female with BA underwent a Kasai procedure at 3 months of age. Despite the surgery, she developed persistent jaundice and abdominal enlargement. Preoperative laboratory findings revealed elevated liver enzymes, GGT, and bilirubin levels. This case raises the clinical question of whether preoperative GGT levels can predict JC after the Kasai procedure, in addition to its role in diagnosis. Methods: A systematic literature search was performed on 4 databases (PubMed, Scopus, Cochrane, and Proquest) by using relevant keywords and identified seven high quality studies. Results: Five studies found higher GGT levels in patients who achieved JC following Kasai surgery, compared to those with unsuccessful jaundice clearance (JUC). However, two studies reported conflicting findings. A pooled analysis indicated a non-significant trend toward higher GGT levels in the JC group compared to the JUC group (WMD: 65.6, 95% CI: -58.6; 189.8). Conclusion: Elevated preoperative GGT levels may be associated with improved JC in BA patients. However, further investigation to validate these findings, determine the optimal role of GGT in assessing prognosis, and later, guiding treatment decisions in BA patients.

Morio Kasai Corrects the Uncorrectable: Hepatic Portoenterostomy for Biliary Atresia

One of the fundamental innovations of pediatric surgery is hepatic portoenterostomy for biliary atresia, widely known as the Kasai procedure for its originator, Morio Kasai (1922–2008), of Sendai, Japan. It was the first effective operation for the “uncorrectable” form of biliary atresia, where death within months from biliary cirrhosis was certain. His radical premise was that microscopic ductules present in the fibrous tissue of the porta hepatis, when transected, drained enough bile to relieve cholestatic jaundice. His first operations from 1955 to 1961 had a jaundice-free survival rate of only 8 percent, but the cures were durable. By 1982–1986 survival reached 55 percent.Adoption of his procedure in the West came slowly. The original operation in 1955 was reported in the Japanese literature in 1959, but the first English language communication came in 1968. Surgeons disbelieved his results. Scientific chauvinism held that advances in pediatric surgery came from America and Europe; Japan had little to add.Surgeons visited Sendai to learn from Kasai, notably John Lilly and R. Peter Altman, then of Washington, DC, Jacques Valayer in Paris, and Edward Howard in London. At first, mortality rates in their hands was high. As expertise developed in the West with the technically demanding operation, mortality fell and approached those reported by Kasai. Successes in America, France, and England proved Kasai's operation as effective and one of signal achievements in pediatric surgery.

Eicosapentaenoic acid administration ameliorates the progression of liver fibrosis after laparoscopic Kasai portoenterostomy.

Biliary atresia (BA) poses a persistent challenge characterized by ongoing liver inflammation and subsequent fibrosis even after the clearance of jaundice (COJ). This study aimed to evaluate the therapeutic potential of eicosapentaenoic acid (EPA) in alleviating liver inflammation and limiting fibrosis during the post-COJ phase of BA. Among the BA patients undergoing laparoscopic Kasai portoenterostomy (lapKP) between December 2016 and October 2021, EPA (20-40mg/kg/day) was administered orally to those whose parents consented. The study included patients from January 2014 to October 2021, classifying them into two groups: EPA-treated (Group E) and untreated (Group N). Their liver fibrosis and clinical course at 1 and 2years post-lapKP were compared. Group E consisted of 25 patients, while Group N comprised 32 patients. Twenty-one patients in Group E and 25 patients in Group N achieved COJ (p = 0.74). Among jaundice-free patients at 1 and 2years post-lapKP, Group E exhibited significantly lower M2BPGi levels and platelet counts, and Group E showed a significant reduction in Aminotransferase-to-Platelet Ratio Index (APRI) at 2years post-lapKP. Although EPA administration did not improve COJ, it attenuated the progression of liver fibrosis during the 2years following lapKP in jaundice-free patients. (200/200Words).

Factors predicting the need for liver transplantation in biliary atresia patients after 18years of age.

We aimed to identify factors predicting the need for future liver transplantation (LT) at 18years of age in patients with biliary atresia (BA). BA patients with native liver survival at > 18years of age were retrospectively reviewed. The clinical characteristics, outcomes, hepatobiliary function, and liver fibrosis markers of native liver survivors (NLS group) were compared with patients who subsequently underwent LT (LT group). The study population included 48 patients (NLS, n = 34; LT, n = 14). The male-to-female ratio, age at Kasai procedure, and type of BA in the two groups did not differ to a statistically significant extent. There was no significant difference in the MELD scores between the groups at 18years of age. The aspartate aminotransferase-to-platelet ratio index (APRI), albumin-bilirubin (ALBI), and BA liver fibrosis (BALF) scores at 18years of age were significantly higher in the LT group. The AUCs for APRI, ALBI, and BALF were 0.91, 0.79, and 0.85, respectively. Adult BA patients have limited options for LT owing to the lack of donor candidates and the low prevalence of deceased donors. The elucidation of prognostic factors for LT in adulthood is important. APRI was the most useful marker in this study.

Early predictors of successful kasai portoenterostomy

Early predictors of successful kasai portoenterostomy

Pediatric portal hypertensive enteropathy after Kasai surgery

BackgroundPortal hypertensive enteropathy (PHE) can cause chronic or acute gastrointestinal (GI) bleeding. Although there is a consensus on the diagnosis and treatment guidelines for PHE in adults, there are few reports on children. We aimed to report the clinical progress and outcomes of children with PHE treated with Kasai surgery and compare them with those of adults with PHE. MethodsIn total, 309 patients who underwent postoperative endoscopy were enrolled among 336 patients who underwent Kasai surgery for biliary atresia at Severance Children's Hospital between May 2005 and March 2022. ResultsThe PHE diagnosis rate was 15.2 % (n = 47). PHE was not diagnosed until 2012; however, the diagnosis rates significantly increased over time (13.3 % and 36.1 % between 2013 and 2017 and 2018–2022, respectively). Of the 47 patients diagnosed with PHE, 14 (29.8 %) had GI bleeding caused solely by PHE. None of the PHE patients required ICU care at the time of bleeding, compared to 32 (24.1 %) in the gastroesophageal varix (GEV) group. After initial management, the rates of re-bleeding, refractory bleeding, and mortality were 78.6 %, 28.6 %, and 0.0 %, respectively, in the PHE group. ConclusionsPediatric PHE diagnosis rates after Kasai surgery have increased since clinicians have become aware of the existence of the disease. Pediatric PHE has a high re-bleeding rate (78.6 %), although fatal GI bleeding is rare compared to GEV. Diagnosis and treatment of pediatric PHE can be performed according to adult guidelines. Level of evidenceIII.

Comparison of Da Vinci Robotic-assisted With Open Kasai Portoenterostomy for Biliary Atresia

BackgroundRobotic-assisted Kasai portoenterostomy (RAKPE) has been used to treat biliary atresia (BA). This study aimed to compare the efficacy of RAKPE and open Kasai portoenterostomy (OKPE) for BA. MethodsThirty-one children with type III BA who underwent surgical treatment in two centers from January 2022 to December 2023 were retrospectively collected. According to the operative techniques, the participants were divided into the RAKPE group (13 cases) and the OKPE group (18 cases). The operative time, jaundice clearance (JC) rate, and incidence of cholangitis were analyzed. ResultsThe operative time in the RAKPE group (204.3 ± 19.9 min) was significantly longer than that in the OKPE group (186.2 ± 22.2 min), P < 0.05. However, the blood loss (8.1 ± 2.5 ml) in the RAKPE group was significantly decreased compared with the OKPE group (13.6 ± 4.8 ml), and 15.4% patient need blood transfusion in RAKEP group was litter than that 55.6% in the OKPE group, P < 0.05. The time to oral feeding (2.8 ± 0.4 days vs. 4.3 ± 0.7 days) and the time to pass ICG-positive stools (3.6 ± 0.6 days vs. 4.7 ± 0.9 days) in the RAKPE group were significantly shorter than those in the OKPE group, P < 0.05. No significant differences were observed in the bile excretion rate, hospital stay time, and JC rate. The incidence of cholangitis in the RAKPE group was significantly lower than that in the OKPE group during short follow-up. ConclusionRAKPE may be associated with lower intraoperative blood loss, decrease need for postoperative transfusions and decreased rate of postoperative cholangitis compared to OKPE. Level of evidenceIII