• 한국간담췌외과학회지
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• 제27권4호
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• pp.433-436
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• 2023

Forty-five adults with type IV-A choledochal cysts (CDC) who underwent extrahepatic cyst excision from January 2013 to December 2021 were followed up for a median interval of 25 months (range, 2 to 10 years) to observe the long-term complications in the remaining intrahepatic cyst. Late complications in varying combinations were seen in 10 patients, which included cholangitis and/or intrahepatic stones in 9 patients, intrahepatic bile duct stenosis with stones in 2 patients, anastomotic stricture in 6 patients, and left lobar atrophy with intrahepatic stones in 3 patients. Out of 6 patients who required re-do hepaticojejunostomy (HJ), three patients had left lobe atrophy with patent HJ anastomosis and a recurrent attack of cholangitis on follow-up at 3, 8, and 10 years. Complications occur frequently after extrahepatic cyst excision for type IV-A CDC and require a long-term follow-up.

• Advances in pediatric surgery
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• 제10권2호
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• pp.136-141
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• 2004

1988년 9월부터 1999년 4월까지 가톨릭 대학교 의과대학 부속병원 외과에서 황달, 무담즙성 변 및 복부 종괴를 주소로 내원하여 간 외 담관낭이 동반된 담도폐쇄증으로 진단받은 7예의 환자의 임상상을 검토하였다. 전체 선천성 담도폐쇄증 환아 중 9.6 %에서 간 외 담관낭을 동반한 III 형의 담도폐쇄증을 보였다. 남여 성비는 2:5로 여아에서 높았다. 모두 카사이 수술을 시행하였으며 수술 시 평균연령은 57일이었다. 7예 중 3예 (42.9 %)에서 긴 생존기간을 보였다. 예후에 미치는 인자에 대해서는 지속적인 연구관찰이 필요할 것으로 사료된다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제2권2호
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• pp.262-266
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• 1999

Obstruction of the extrahepatic bile ducts is the most common cause of conjugated hyperbilirubinemia in early infancy. More than 90% of such obstructive lesions are accounted for by extrahepatic biliary atresia. A rare lesion is obstruction of the common duct by impacted, thickened secretions and bile. Bile plug syndrome is defined as extrahepatic obstruction of the bile ducts by bile sludge in term infants without anatomic abnormalities, congenital chemical defects of bile, or hepatocellular lesions. Obstruction of extrahepatic ducts by plugs of biliary material apperas to be due to the inspissation and precipitation of bile and mucus within the lumen of the ducts. Cholestasis and precipitation of bile develop in association with abnormal composition of bile in cystic fibrosis, hepatocellular damage, prolonged erythroblastic jaundice, altered biliary dynamics with total parenteral nutrition, gut dysfunction, diuretic therapy, exchange transfusions and perinatal hemolysis. In those cases, the term inspissated bile syndrome is used. The clinical and laboratory findings in bile plug syndrome are identical to those observed in biliary atresia and choledochal cyst. The diagnosis can be suspected based on the findings of clinical and laboratory examinations together with hepatobiliary imaging, ultrasonography, radionuclide scan and liver biopsy. We experienced a case of spontaneous resolution of bile plug syndrome in a 4-year-old girl. We report this case with brief review related literatures.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제5권2호
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• pp.186-191
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• 2002

구토, 심한 보챔, 고열, 복수를 동반한 복부 팽만 등 급성 복막염의 양상을 보인 10개월 된 여아에서 복부 천자 후 담즙성 복막염 의심하에 $^{99m}Tc$ DISIDA 간담도 스캔을 시행하여 진단된 총 담관낭의 자연 천공에 의한 담즙성 복막염 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제59권5호
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• pp.239-241
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• 2016

Choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts. Postnatally, ultrasonography is the initial diagnostic modality of choice, allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge. Symptoms depend on the age at presentation. Common bile duct malformations should be considered as a differential diagnosis of a cystic mass regardless of the cyst's size or patient's age, especially in children presenting with abdominal pain, jaundice, and palpable mass. To the best of our knowledge, we report the largest choledochal cyst in infancy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권2호
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• pp.114-123
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• 2017

Purpose: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. Methods: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. Results: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. Conclusion: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.