• Advances in pediatric surgery
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• v.17 no.1
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• pp.1-14
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• 2011

Biliary atresia (BA) is an infantile cholestatic disease of progressive obliterative cholangiopathy with varying degrees of damage to both extra and intrahepatic bile ducts due to unknown causes. The diagnostic studies should be done to diagnose or exclude BA without unnecessary delay. Kasai portoenterostomy is the first choice of treatment for bile drainage from microscopic bile ductules present in the portal fibrous mass. The medical management after Kasai portoenterostomy should be done carefully to maintain bile excretion and prevent and treat complications Including cholangitis, hepatic fibrosis, portal hypertension and nutritional problem. The reported five years-survival rates after Kasai portoenterostomy range from 30 to 60 %. About 20 % of all patients undergoing Kasai portoenterostomy during infancy survive into adulthood with their native liver. Even if Kasai portoenterostomy remains as the first line of treatment In BA, liver transplantation serves as a good salvage treatment when portoenterostomy fails or liver function gradually deteriorates after initially successful establishment of bile flow, Overall 5-year survival rate in BA is about 90 % in recent series.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.1014-1031
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• 2022

The Kasai portoenterostomy is the first-line treatment for the restoration of the flow of bile to the small intestine in patients with biliary atresia. Various complications can occur after Kasai portoenterostomy, including ascending cholangitis, biliary cirrhosis, and portal hypertension. Of these potential complications, ascending cholangitis in the most common. In cases of patients having uncontrolled complications due to progressive liver cirrhosis, portal hypertension, or progressive hyperbilirubinemia, liver transplantation is the indicated as treatment plan. Lifelong follow-up, particularly involving imaging studies, is important for the identification of various complications arising from biliary atresia after Kasai portoenterostomy. Additionally, imaging studies play a crucial role in the evaluation of potential liver donors and recipients. US is a key imaging modality utilized in the management of patients who undergo Kasai portoenterostomy, while CT and MRI are imperative to obtaining an accurate diagnosis.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.979-990
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• 2022

Biliary atresia is a progressive, idiopathic, obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction in the neonatal period. It is the most common indication for liver transplantation in children. If untreated, progressive liver cirrhosis leads to death by two years of age. Nowadays, more than 90% of biliary atresia patients survive into adulthood with the development of Kasai portoenterostomy and liver transplantation technology. Early diagnosis is critical since the success rate of the Kasai portoenterostomy decreases with time. This study comprehensively reviews the recent advances in the etiology, classification, prevalence, clinical manifestations, treatment, and prognosis of biliary atresia.

• Advances in pediatric surgery
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• v.12 no.2
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• pp.202-212
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• 2006

The prognostic factors for extrahepatic biliary atresia (EHBA) after Kasai portoenterostomy include the patient's age at portoenterostomy (age), size of bile duct in theporta hepatis (size), clearance of jaundice after operation (clearance) and the surgeon's experience. The aim of this study is to examine the most significant prognostic factor of EHBA after Kasai portoenterostomy. This retrospective study was done in 51 cases of EHBA that received Kasai portoenterostomy by one pediatric surgeon. For the statistical analysis, Kaplan-Meier method, Logrank test and Cox regression test were used. A p value of less than 0.05 was considered to be significant. Fifteen patients were regarded as dead in this study, including nine cases of liver transplantation. There was no significant difference of survival to age. The age is also not a significant risk factor for survival in this study (Cox Regression test; p = 0.63). There was no significant difference in survival in relation to the size of bile duct. However, bile duct size was a significant risk factor for survival (Cox Regression test; p = 0.002). There was a significant difference in relation to survival and clearance (Kaplan-Meier method; p = 0.02). The clearing was also a significant risk factor for survival (Cox Regression test; p = 0.001). The clearance of jaundice is the most significant prognostic factor of EHBA after Kasai portoenterostomy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.1
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• pp.74-80
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• 2011

Purpose: Based on previous research findings, it is well-known that the timing of surgery is generally considered the most important prognostic factor for a Kasai portoenterostomy, the primary treatment for biliary atresia. This research aimed to identify the optimal timing of a Kasai portoenterostomy and to verify if the proposed optimal timing corresponds to previous studies. All patients were classified by the timing of surgery, and pre- and post-operative fibrotic changes of the liver were measured with the elasticity value from fibroscans. Methods: The stiffness scores of the pre- and post-operative fibroscans in 34 patients who were treated by Kasai portoenterostomy from October 2007 to September 2010 in Severance children's hospital were reviewed. Results: The earlier the patients were treated by Kasai portoenterostomy, the lower the fibroscan scores. When the patients were treated prior to the 8th week, the post-operative scores of the fibroscans were significantly better than those patients who were treated after the 8th week, and some even recovered partially. Moreover, when operated before the 8th week, the differences between each pre- and postoperative fibroscan score also showed statistical relevance (p=0.0002). Conclusion: The earlier the patient was treated by Kasai portoenterostomy, the less liver fibrosis that developed, the lower the level of post-operative fibrosis, and the less the degree of fibrotic progress before and after the operation. Thus, this research proposal reconfirms once more that the 8th week is the optimal timing for a Kasai portoenterostomy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.3
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• pp.266-275
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• 2020

Purpose: The outcome predictors of Kasai portoenterostomy (KPE) for biliary atresia (BA) are controversial. This study aimed to identify possible short-term outcome predictors of KPE for BA in infants. Methods: This retrospective study included infants with BA who underwent KPE between January 2015 and December 2017 and were followed up for at least 6 months after surgery at the Pediatric Hepatology Unit, Cairo University Pediatric Hospital, Egypt. The short-term outcome was jaundice clearance within 6 months following surgery. All data were compared between the jaundice free group and those with persistent jaundice to identify the predictors of jaundice clearance. Results: The study included 75 infants. The mean age at the time of surgery was 82.43±22.77 days (range, 37-150 days), and 28 (37.3%) infants cleared their jaundice within 6 months postoperative. Age at surgery did not significantly affect the outcome (p=0.518). Infants with persistent jaundice had significantly higher pre-operative levels of aspartate aminotransferase (AST) than those who were jaundice free (p=0.041). Receiver operating characteristic curve analysis showed that preoperative AST ≤180 IU/L was predictive of a successful KPE, with sensitivity 74.5% and specificity 60.7%. Infants with bile plugs in liver biopsy had a 6-fold higher risk of persistent jaundice than those without bile plugs (95% confidence interval: 1.59-20.75, p=0.008). Conclusion: Jaundice clearance after KPE for BA can be predicted using preoperative AST and presence of bile plugs in liver biopsy.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.156-160
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• 2002

When jaundice persists for more than 14 days postnatally, the early diagnosis of surgical jaundice is important for the prognosis in extrahepatic biliary atresia after draining procedure. The role of diagnostic laparoscopy to differenctiate medical causes of jaundice from biliary atresia is evaluated in this report. Four patients with prolonged jaundice have been included in this study. When the gallbladder was not visualized we proceeded to laparotomy. In patients with enlarged gallbladder visualized at laparoscopy, laparoscopic guided cholangiogram was performed, and laparoscopic liver biopsy was done for those who had a patent biliary tree. Two patients had small atretic gallbladder and underwent a Kasai hepato-portoenterostomy. One patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a Kasai hepatic-portoenterostomy. One patient showed an enlarged gallbladder and laparoscopic-guided cholangiogram were normal. Laparoscopic liver biopsy was performed. There were no complications. Laparoscopy with laparoscopic-guided cholangiogram may be a valuable method in accurate and earlier diagnosis in an infant with prolonged jaundice.

• Advances in pediatric surgery
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• v.18 no.1
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• pp.12-17
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• 2012

The purpose of this study is to analyse clinical impact of specific MRI findings in liver in patients of long-term survivors after Kasai portoenterostomy (KPE). Twenty-eight patients who were underwent KPE were followed up more than 5 years. Macro-regenerative nodule (MRN) and beaded-duct dilatation (BDD) were considered as important findings in liver MRI. The association between these findings in MRI and clinical indicator, serum bilirubin level and history of cholangitis were evaluated. Sixteen patients (57.1%) were shown MRN in liver MRI. Therewere 14 patients(50%) whose MRI showed BDD. Serum total and direct bilirubin were 3.6 mg/dL and 1.8 mg/dL respectively in positive MRN group whereas 1.4 mg/dL and 0.7 mg/dL in negative MRN group (p = 0.427). Serum total and direct bilirubin level were 4.2 mg/dL and 2.1 mg/dL in patients with BDD negative group compare to 1.1 mg/dL and 0.5 mg/dL in BDD positive group (p = 0.281). The odds ratio to have cholangitis in the patient with MRN was 2.3 and 0.53 in patient with BDD in their MRI findings. MRN in liver MRI may suggest high bilirubin level and more chance to have cholangitis, but the findings of BDD may related to low bilirubin level and less change to have cholangitis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.sup2
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• pp.35-43
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• 2008

Jaundice is common in breast-fed infants. Any infant noted to be jaundiced at 2 weeks of age need to be evaluated for cholestasis with measurement of total and direct serum bilirubin. The most common causes of cholestatic jaundice in infants are biliary atresia and neonatal hepatitis. Genetic causes of the neonatal hepatitis syndrome are increasingly recognized and idiopathic neonatal hepatitis is decreasing. Cholestasis should be investigated using a structured protocol. Early detection and timely, accurate diagnosis is important for successful treatment and a favorable prognosis. In particular, a Kasai portoenterostomy for biliary atresia has the best outcome if performed before the infant is 8 weeks of age. The management of cholestasis is mainly supportive, including nutritional support and alleviation of symptoms to improve the quality of life. Specific treatments are available for some causes of neonatal hepatitis syndrome and should be started as soon as possible. For decompensated liver disease, liver transplantation yields a better outcome.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.126-129
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• 1999

A 6 and a half year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst ($4.5{\times}4.0$ cm in size) in the left hepatic lobe and another tubular dilatation ($2.0{\times}0.8$ cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangiodrainage(PTCD) and cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring $6.6{\times}5.0$ cm in size. Following drainage and administration of parenteral antibiotics she became afebrile and anicteric. However she continued to drain 45-150 cc of bile per day via the tube over the next 2 weeks. The patient successfully underwent intrahepatic cystojejunostomy with intraoperative ultrasonographic guidance. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, successfully managed with PTCD followed by an internal drainage procedure.