• Advances in pediatric surgery
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• 제17권1호
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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.

• 대한영상의학회지
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• 제83권5호
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• pp.979-990
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• 2022

담도폐쇄증은 간외 담도의 진행성, 특발성, 섬유 폐쇄성 염증질환으로 신생아기에 담도 폐쇄가 나타나고 소아 간이식의 가장 흔한 적응증이다. 조기에 치료하지 않으면 간경화가 진행되어 2세 이전에 사망하게 된다. 오늘날 Kasai portoenterostomy와 간이식 수술기법의 발달로 담도폐쇄증 환자의 90% 이상이 성인기까지 생존한다. 따라서 Kasai portoenterostomy의 성공 가능성은 시간이 지날수록 줄어들기 때문에 조기 진단이 매우 중요하다. 이 고찰은 담도폐쇄증의 원인, 분류, 유병률, 임상양상, 치료와 예후를 최신 지견을 중심으로 종합적으로 검토하고자 한다.

• 대한영상의학회지
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• 제83권5호
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• pp.1014-1031
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• 2022

신생아 황달의 주요 원인인 담도폐쇄증은 Kasai portoenterostomy가 기본적인 치료 술식이다. 수술 이후 다양한 합병증이 발생할 수 있는데 portoenterostomy를 통해 발생하는 상행성 담도염이 가장 흔하다. 성공적인 Kasai portoenterostomy 후에도 간 섬유화가 진행할 수 있어 이로 인한 간경화, 문맥 고혈압으로 인한 합병증이 많은 경우에서 발생하게 된다. 합병증이 조절되지 않거나 진행성 고빌리루빈혈증이 있는 경우 간이식을 시행하게 된다. Kasai portoenterostomy 이후 합병증 확인 및 장기 생존 환자의 추적 검사로 다양한 영상 진단이 주요 역할을 하고 있다. 또한 간이식이 필요한 경우 이식 전후로 공여자 및 수혜자 모두에서 수술 금기 확인 및 합병증 조기 발견을 위해 영상 검사들이 중요한 역할을 한다. 초음파 검사가 추적 관찰에 가장 먼저 사용되는 유용한 검사이며 추가적으로 CT 및 MRI를 통해 진단에 도움을 받을 수 있다.

• Advances in pediatric surgery
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• 제8권2호
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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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• 제5권2호
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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.

• Korean Journal of Radiology
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• 제24권5호
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• pp.465-475
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• 2023

Objective: To evaluate the feasibility of ultrasound shear wave elastography (SWE) for predicting hepatic fibrosis and native liver outcomes in patients with biliary atresia. Materials and Methods: This prospective study included 33 consecutive patients with biliary atresia (median age, 8 weeks [interquartile range, 6-10 weeks]; male:female ratio, 15:18) from Severance Children's Hospital between May 2019 and February 2022. Preoperative (within 1 week from surgery) and immediate postoperative (on postoperative days [PODs] 3, 5, and 7) ultrasonographic findings were obtained and analyzed, including the SWE of the liver and spleen. Hepatic fibrosis, according to the METAVIR score at the time of Kasai portoenterostomy and native liver outcomes during postsurgical follow-up, were compared and correlated with imaging and laboratory findings. Poor outcomes were defined as intractable cholangitis or liver transplantation. The diagnostic performance of SWE in predicting METAVIR F3-F4 and poor hepatic outcomes was analyzed using receiver operating characteristic (ROC) analyses. Results: All patients were analyzed without exclusion. Perioperative advanced hepatic fibrosis (F3-F4) was associated with older age and higher preoperative direct bilirubin and SWE values in the liver and spleen. Preoperative liver SWE showed a ROC area of 0.806 and 63.6% (7/11) sensitivity and 86.4% (19/22) specificity at a cutoff of 17.5 kPa for diagnosing F3-F4. The poor outcome group included five patients with intractable cholangitis and three undergoing liver transplantation who showed high postoperative liver SWE values. Liver SWE on PODs 3-7 showed ROC areas of 0.783-0.891 for predicting poor outcomes, and a cutoff value of 10.3 kPa for SWE on POD 3 had 100% (8/8) sensitivity and 73.9% (17/23) specificity. Conclusion: Preoperative liver SWE can predict advanced hepatic fibrosis, and immediate postoperative liver SWE can predict poor native liver outcomes in patients with biliary atresia.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권3호
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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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• 제5권2호
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• pp.111-115
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• 1999

The results of hepatic portojejunostomy in 34 patients with biliary atresia operated upon by one surgeon between May 1989 and December 1997 were analyzed. Eleven (32.3 %) patients were 60 days or younger, 14 patient (41.2 %) were between 60 and 90 days, and 9 (26.5 %) were over 90 days of age. Jaundice cleared in 20 cases (58.8 %). Three patients died of liver insufficiency, 2 were anicteric but died from esophageal variceal bleeding. Three patients died as a result of sepsis, heart failure and left kidney agenesis. Five patients were lost to follow-up. The five-year survival rate was 73.8 %. Two patients over 90 days of age, survived more than 5 years. Survival rates were not significantly related to the age at operation. We conclude that hepatic portojejunostomy should be considered as a primary surgical modality for biliary atresia, even at age 90 days or more. Early detection of esophageal varices and sclerotherapy may be necessary. Liver transplantation is necessary if hepatic failure develops.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제27권4호
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• pp.224-235
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• 2024

Purpose: Biliary atresia (BA) is the leading cause of neonatal cholestasis (25-45%). The primary treatment is hepatic portoenterostomy (Kasai procedure), but only 20-40% provide long-term benefits. This study aimed to develop a predictive model for surgical efficacy by comparing preoperative and early postoperative indicators in infants with different outcomes. Methods: We enrolled 166 infants with BA (93 girls, 73 boys) who underwent the Kasai procedure between September 2002 and December 2021, dividing them into favorable or adverse outcome groups. Over 40 parameters were measured, and the diagnostic significance of the prognostic model was evaluated. Results: Kasai surgery was efficacious in 69 patients (42%) and non-efficacious in 97 (58%). Our model assesses efficacy by day 14 after surgery, improving on the <34 µmol/L direct bilirubin threshold established for 3-6 months after the procedure. Including the Desmet fibrosis score refined the model. Conclusion: Blood cholesterol below 5.41 mmol/L, direct bilirubin below 56.3 µmol/L on postoperative days 14±3, and a low Desmet score indicate a high probability of efficacious Kasai surgery in infants with BA.

• 대한영상의학회지
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• 제83권5호
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• pp.1003-1013
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• 2022

담도폐쇄증은 원인은 알 수 없으나 간외 담도의 섬유화로 내강이 막히면서 간문부에 '섬유성 담도잔유물'의 형태로 남아있는 질환이다. 'Triangular cord' (이하 TC) sign은 간문부에 남아있는 섬유성 담도잔여물을 초음파 검사에서 나타내는 소견이다. 본 종설에서는 담도폐쇄증의 수술 및 초음파 소견의 비교, TC sign의 정의, 객관적인 진단기준, 진단의 정확도 및 감별진단에 대하여 기술하고자 한다.