• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제19권4호
• /
• pp.286-290
• /
• 2016

Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a liver biopsy was suggestive of biliary atresia. This finding forced us to perform intraoperative cholangiography, which revealed a patent common bile duct with impacted thick bile. We performed normal saline irrigation and the symptom was improved, the final diagnosis was IBS. Thus, we herein report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.

• Parasites, Hosts and Diseases
• /
• 제57권5호
• /
• pp.517-520
• /
• 2019

Echinococcosis is a disease caused by the Echinococcus species that parasitizes in humans. Alveolar echinococcosis (AE) which is caused by Echinococcus multilocularis is harmful to humans. AE mainly occurs in the liver and can be transferred to retroperitoneal lymph nodes, lung, brain, bone, spleen and other organs through lymphatic and blood vessels. Cholangiocarcinoma can occur in of the intrahepatic and extrahepatic bile ducts and is more common in the hilar. We reported a case of hilar bile duct alveolar echinococcosis which was originally misdiagnosed an cholangiocarcinoma.

• 대한소화기학회지
• /
• 제72권6호
• /
• pp.318-321
• /
• 2018

Primary neuroendocrine tumors originating from the extrahepatic bile duct are rare. Among these tumors, large cell neuroendocrine carcinomas (NECs) are extremely rare. A 59-year-old man was admitted to Sanggye Paik Hospital with jaundice that started 10 days previously. He had a history of laparoscopic cholecystectomy, which he had undergone 12 years previously due to chronic calculous cholecystitis. Laboratory data showed abnormally elevated levels of total bilirubin 15.3 mg/dL (normal 0.2-1.2 mg/dL), AST 200 IU (normal 0-40 IU), ALT 390 IU (normal 0-40 IU), and gamma-glutamyl transferase 1,288 U/L (normal 0-60 U/L). Serum CEA was normal, but CA 19-9 was elevated 5,863 U/mL (normal 0-37 U/mL). Abdominal CT revealed a 4.5 cm sized mass involving the common bile duct and liver hilum and dilatation of both intrahepatic ducts. Percutaneous transhepatic drainage in the left hepatic duct was performed for preoperative biliary drainage. The patient underwent radical common bile duct and Roux-en-Y hepaticojejunostomy for histopathological diagnosis and surgical excision. On histopathological examination, the tumor exhibited large cell NEC (mitotic index >20/10 high-power field, Ki-67 index >20%, CD56 [+], synaptophysin [+], chromogranin [+]). Adjuvant concurrent chemotherapy and radiotherapy were started because the tumor had invaded the proximal resection margin. No recurrence was detected at 10 months by follow-up CT.

• 한국간담췌외과학회지
• /
• 제26권4호
• /
• pp.298-307
• /
• 2022

Portal cavernoma cholangiopathy is defined as an obstruction of the biliary system due to distended veins surrounding bile ducts that mainly occur in patients with extrahepatic portal venous obstruction. The periductal venous plexuses encircling the ducts can cause morphological changes which may or may not become symptomatic. Currently, non-invasive techniques such as ultrasonography, computed tomography, magnetic resonance cholangiopancreatography, and dynamic contrast enhanced magnetic resonance images are being used to diagnose this disorder. Only a few patients who have symptoms of biliary obstruction require drainage which might be accomplished using endoscopic stenting, decompression of the portal venous system usually via a lienorenal shunt, a difficult direct hepaticojejunostomy, and rarely a liver transplant.

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

• Advances in pediatric surgery
• /
• 제13권1호
• /
• pp.1-12
• /
• 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.

• Advances in pediatric surgery
• /
• 제8권2호
• /
• pp.156-160
• /
• 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.

• 대한소아치과학회지
• /
• 제31권3호
• /
• pp.400-405
• /
• 2004

담도폐쇄증은 간장외 담관의 부분적이거나 완전한 파괴 또는 부재로 인한 담즙흐름의 완전한 폐쇄로 정의된다. 이 질환은 대략 10,000명당 1명 정도 비율로 발생하며 여성에서 약간높은 빈도를 보인다. 원인인자는 명확히 밝혀지지 않았으며 증상은 과빌리루빈혈증과 함께 황달, 점토색변, 지방설사, 어두운 갈색뇨, 간비대 등을 보인다. 최근의 담도폐쇄증의 치료는 간문장문합술 단독 혹은 간이식술을 병행하는 경향이다. 본 증례의 환자에서는 다양한 정도의 치아변색을 보였으며 색상은 노란색에서부터 짙은 초록색까지 다양했다. 법랑질의 저형성증이 모든 치아에 나타났고 구강위생이 좋지 않았으며 다발성 우식증을 가지고 있었다.

• Radiation Oncology Journal
• /
• 제14권2호
• /
• pp.129-136
• /
• 1996

목적 : 담낭과 간외 담도를 포함한 간외 담도계암에 대한 방사선치료의 효과를 평가하고 방사선치료의 역할에 관하여 알아보고자 함이 본 연구의 목적이다. 대상 및 방법 : 1986년 4월에서 1994년 7월까지 본원 치료방사선과에서 외부 방사선치료를 받은 43명의 담낭 및 간외 담도계암 환자를 후향적으로 분석하였다. 23명은 담낭암이었고 20명은 간외 담도암이었다. 23명의 담낭암 환자중에서 제 2병기는 2명, 제 3 병기는 12명, 제 4병기는 9명이었다. 남녀비는 11명대 12명이었다. 15명은 근치적 절제술을 시행하였고, 8명은 조직생검과 우회수술만을 시행하였다. 3명을 제외한 모든 환자에서 수술후 4500 cGy 이상의 방사선을 조사하였으며, 추적기간은 11-82개월 이었다. 20명의 간외 담도암 환자중에서 제 1병기는 1명, 제 2병기는 2명, 제 3병기는 10명, 제 4병기는 7명이었다. 남녀비는 16명대 4명이었다. 16명이 근치적 목적으로 위플씨 수술 또는 절제와 배액술을 시행하였고, 나머지 4명은 우회수술만을 시행하였다. 16명은 수술후 방사선 치료로 4500 cGy 이상 조사하였고, 4명은 3180-4140cGy를 조사하였다. 추적기간은 8-34개월 이었다. 결과 : 담낭암 환자의 전체 중앙 생존기간은 11개월이었다. 제 3병기와 제 4병기의 중앙생존기간은 14개월과 5개월이었으며, 2년 생존율은 각각 $36\%$(4/11)와 $13\%$(1/8) 이었다. 근치적 목적으로 수술을 받은 환자들이 우회수술 만으로 치료받은 환자들보다 통계적으로 유의하게 생존율이 높았다. ($67\%$ : $13\%$ 12개월) 치료와 연관된 합병증으로 사망한 환자는 없었다. 20명의 간외 담도암 환자의 전체 중앙 생존기간은 10개월이었다. 제 3병기 10명과 제 4병기 7명의 중앙 생존기간은 각각 10개월과 8개월이었다. 위플씨 수술을 시행한 2명은 각각 11개월과 14개월간 생존하였으며, 절제와 배액술로 치료한 환자들의 중앙 생존기간은 10개월이었다. 결론 : 간외 담도계암 환자에서 수술후 외부 방사선치료에 의한 합병증은 미미하였으며, 특히 수술후 현미경적 또는 육안적 잔여병변이 있는 환자에서 방사선 치료에 의해 생존율이 증가하는 양상을 나타내었다.

• 대한영상의학회지
• /
• 제83권5호
• /
• pp.966-978
• /
• 2022

신생아와 유소아 황달은 다양한 원인에 의하여 발생하며, 특히 생리적 황달의 경우는 만삭아의 반수 이상에서 관찰되는 매우 흔한 질환이다. 생후 2주 이후 황달이 지속되거나 새로이 발생하는 경우, 담즙 정체의 가능성을 고려하여 총/직접 빌리루빈을 측정하게 되며, 담즙 정체로 판단되는 경우 외과적 치료를 요하는 간외 담도 폐쇄 질환을 감별하려는 목적으로 영상검사가 의뢰된다. 본 종설에서는 신생아 및 유소아에서 황달을 발생시킬 수 있는 질환들을 분류하고, 질환들을 진단하기 위한 여러 영상 검사들의 종류와 방법 및 정상 소견들을 기술하며, 연령별로 흔히 발생하는 각 질환에 대한 영상 소견들을 소개하고자 한다.