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Mucin-Producting Biliary Papillomatosis Associated with a Choledochodiodenal Fistula: Report of a Case

黏液分泌性膽道乳頭狀瘤合併膽管十二指腸瘻管:一病例報告

摘要


黏液分泌性膽道乳頭狀瘤是一種很少見但有惡性腫瘤傾向的膽道疾病。疾病初期通常沒有症狀;但膽道阻塞時,病人會有腹痛、黃疸、膽管炎、甚至造成肝衰竭等併發症,因此早期診斷並治療是非常重要的。膽道乳頭狀瘤引起的瘻管更罕見,文獻上只報導過一位病人合併膽管胃瘻管,臨床上無黃疸及膽管炎的症狀。而我們的病人接受膽管鏡檢查證實膽道乳頭狀瘤合併膽管十二指腸瘻管,臨床上僅有黃疸而無腹痛及膽管炎的情形,其原因可能為部份膽汁經由膽管引流至十二指腸瘻管減壓而讓感染不易發生。這位病人因為年齡及病灶範圍廣大,無法肝移植或手術完整切除,故改以5-FU化療合併體外膽汁引流治療。三個月後門診追蹤發現,膽道乳頭狀瘤明顯減小且黃疸指數正常。這是文獻上第一例黏液分泌性膽道乳頭狀瘤合併膽管十二指腸瘻管的案例報告。

並列摘要


We report a 75-year-old Taiwanese man who presented with the symptoms and signs of obstructive jaundice and was found on imaging studies to have intraductal papillomas in the biliary tract. Subsequent workup with endoscopic retrograde cholangiography revealed a choledochoduodenal fistula proximal to the major papilla with abundant mucin and several nodular lesions in the orifice of the fistula leading to the diagnosis of mucin-producing biliary papillomatosis (BP) with a choledochoduodenal fistula. Mucin-producing BP associated with gastrobiliary fistula has been reported once in the past. To our knowledge, this is the first case reported associated with a choledochoduodenal fistula. Although mucin-producing BP may be associated with Charcot's triad, the patient only presented with obstructive jaundice without abdominal pain or cholangitis. This was probably because the mucinous bile could be excreted into the duodenum through the choledochoduodenal fistula. We assume that this extremely rare ”drainage route” prevented the development of cholangitis. He was subsequently treated with percutaneous biliary drainage and weekly 5-FU with reduction in the papillomas as well as the bilirubin levels.

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