Imvrios George, Papanikolaou Vasilios, Lalountas Miltiadis, Patsiaoura Kaliopi, Giakoustidis Dimitrios, Fouzas Ioannis, Anagnostara Eva, Antoniadis Nikolaos, Takoudas Dimitrios
Department of Transplant Surgery, Aristotle University, Hippokration Hospital, Thessaloniki, Greece.
Liver Transpl. 2007 Jul;13(7):1045-8. doi: 10.1002/lt.21207.
Approximately 60 cases of biliary papillomatosis have been reported in the world literature, while only 6 cases have been reported to be treated with liver transplantation. This rare disease, which is characterized by relapsing episodes of obstructive jaundice and cholangitis that lead to secondary cirrhosis and death from sepsis or liver failure, it is also considered premalignant because of its frequent malignant transformation (25-50%). We present a case of a 43-year-old white man with papillomatosis of intra- and extrahepatic biliary tree who sought care for repeated episodes of obstructive jaundice and cholangitis. The diagnosis was suspected after endoscopic retrograde cholangiopancreatography and confirmed by liver and common bile duct biopsies. The patient underwent orthotopic liver transplantation with Roux-en-Y hepatico-jejunostomy to treat end-stage liver cirrhosis. Fifteen months' follow-up revealed a patient with normal graft function and with no clinically or laboratory findings of disease recurrence or cancer development.
世界文献中报道了约60例胆管乳头状瘤病病例，而仅有6例报道采用肝移植治疗。这种罕见疾病的特征是梗阻性黄疸和胆管炎反复发作，进而导致继发性肝硬化，并因败血症或肝衰竭死亡，由于其频繁发生恶变（25%-50%），它也被认为是癌前病变。我们报告一例43岁白人男性，患有肝内和肝外胆管乳头状瘤病，因梗阻性黄疸和胆管炎反复发作前来就医。经内镜逆行胰胆管造影术后怀疑诊断，并经肝脏和胆总管活检确诊。该患者接受了原位肝移植及 Roux-en-Y 肝空肠吻合术以治疗终末期肝硬化。15个月的随访显示，患者移植肝功能正常，无疾病复发或癌症进展的临床及实验室检查结果。
