• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.18 no.3
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• pp.175-179
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• 2015

Purpose: Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as biliary atresia and subsequently undergo Kasai operation. The purpose of this study was to investigate the outcome of patients with AGS who had previously received Kasai operation during infancy. Methods: This retrospective study was conducted at the Department of Pediatrics, Samsung Medical Center. We compared the prognosis and mortality between those who had undergone Kasai operation during infancy (Kasai group) and those who had not (non-Kasai group). Results: Among the 15 children with AGS, five had received Kasai operation, while 10 had not. All subjects in the Kasai group revealed neonatal cholestasis, while 70% of the non-Kasai group showed neonatal cholestasis. Liver transplantation was performed in 100% (5/5) among the Kasai group, and 20.0% (2/10) among the non-Kasai group (p=0.007). Mortality was observed in 60.0% (3/5) among the Kasai group, and 10.0% (1/10) among the non-Kasai group (p=0.077). Conclusion: Although overall mortality rate did not significantly differ between the two groups, the proportion of patients receiving liver transplantation was significantly higher in the non-Kasai group. The relatively worse outcome in AGS patients who had received Kasai operation may be due to the unfavorable influences of Kasai operation on the clinical course of AGS, or maybe due to neonatal cholestasis, irrespective of the Kasai operation.

• 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.

• 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.

• Advances in pediatric surgery
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• v.14 no.2
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• pp.125-133
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• 2008

To evaluate the long-term prognosis of biliary atresia after Kasai operation, a total of 14 patients (of the 41 patients operated upon from 1982 to 1997), who had been followed up for more than 10 years, were included in this retrospective study. Eleven out of 14 patients survived with their native livers, and their data analyzed for age at operation, clearing time of jaundice, histological outcome, postoperative complications, effectiveness after the application of an intussusception anti-reflex valve, and quality of life. Average age at surgery was 62.8 days. Serum bilirubin was normalized within three months in all patients. Six among the eleven long-term survivors had ascending cholangitis as one of the postoperative complications. The application of an intussusception anti-reflux valve did not show any statistical significance in long-term survival. Most of long-term survivors appeared to enjoy good quality of life. Kasai operation might not be the definitive treatment for biliary atresia; however, Kasai operation made it possible to achieve long-term survival for patients with biliary atresia when the patients were detected and treated as early as possible.

• Journal of the Korea Academia-Industrial cooperation Society
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• v.12 no.5
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• pp.2261-2266
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• 2011

Being the most widely used in ultrasound Doppler color flow mapping, the Kasai algorithm, also known as lag-1 autocorrelation method, is capable of estimating the Doppler mean frequency relatively accurately with a modest amount of computation. Particularly in the case of imaging deep lying areas, however, its performance suffers due to low signal-to-noise ratios. The purpose of this paper is to propose a dealiased lag-2 autocorrelation method which is superior to the Kasai algorithm even at low signal-to-noise ratios and to compare their performances through simulations. The proposed algorithm is found to be better by about 2 to 3 dB than the Kasai algorithm in terms of Doppler mean frequency estimation error in the presence of measurement noise.

• 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.

• Advances in pediatric surgery
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• v.13 no.2
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• pp.135-143
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• 2007

Biliary atresia (BA) is an uncommon neonatal surgical disease that has a fatal outcome if not properly treated. The survival rates of the patients with native liver after Kasai's operation in countries outside Japan are not so good. We reviewed the results of 22 cases of biliary atresia treated in Kosin University Hospital between October 1987 and March 2001. There were 13 males and 9 females aged from 21 to 106 days (mean 52 days). There were 3 cases of Type I (13.6%), and 3 of Type II (13.6%), and 16 Type III (72.7%). The operative methods were resection of the common bile duct remnant and cyst followed by Roux-en-Y hepaticojejunostomy in 3 cases for Type I BA; Kasai I in 15 cases, Kasai II in 1 case, and Ueda's operation in 3 cases for Types II and III BA. There was no death within the first 30 days after operation. We were able to follow 21 of the 22 patients (95.4%) for more than 5 years. The actual 5 year survival rate (YSR) was 40.9%. One Type I case received a living-related liver transplantation at 6 years of age because of the multiple intrahepatic stones and liver cirrhosis. Five YSR after biliostomy group (Kasai II and Ueda op.) was 75 % (3/4) while that of Kasai I was 20% (3/15). One case had no bile duct in the resected fibrotic plaque on microscopic review and died 8 months after Kasai I operation, would have been a strong candidate for early liver transplantation. From the above result, our conclusions are as follows; (1) early liver transplantation should be considered for cases of no bile duct after pathologic examination of the resected specimen, (2) measures to prevent postoperative cholangitis and prevention of postoperative liver cirrhosis are needed, (3) liver transplantation program should be available for failed cases.

• 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.

• Advances in pediatric surgery
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• v.13 no.1
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• pp.1-12
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• 2007

Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.

• 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.