總膽管囊腫：家族性病例報告

Choledochal Cyst in Siblings: Report of Two Cases in a Family

總膽管囊腫為膽道少見的先天性病變，通常發生於嬰兒及幼童，為兒童時期黃疸的原因之一。以東方人及女性患者發生的比率較高，但發生於同一家族的病例極微少見。我們經歷一家庭中姊妹相繼發生總膽管囊腫的案例，她們在住院時的症狀除了輕微黃疸及不正常的大便顏色外無其他症狀，實驗室檢查發現為結合型黃疸合併肝功能異常，腹部超音波都顯示總膽管呈現囊狀或管狀擴張合併肝內膽管擴張。手術時將囊狀或管狀擴張的總膽管及膽囊切除並做肝臟空腸吻合手術，術後病程平順。我們將對總膽管囊腫的家族病例做文獻回顧及討論。

Choledochal cysts are known to be one of the causes of childhood jaundice. Reported familial cases are extremely rare. Choledochal cyst in siblings was experienced at our hospital. They were observed to be rather well except for mild jaundice and abnormal stool color after admission. Biochemical studies revealed conjugated hyperbilirubinemia and elevated liver function. Abdominal sonogram disclosed cystic mass or fusiform dilatation of common bile duct (CBD). Intrahepatic duct (IHD) dilatation was noted in both patients. Operative findings revealed cystic dilatation of CBD in one patient and fusiform dilatation of CBD in another. They both received excision of cyst with Roux-en-Y hepaticojejunostomy and cholecystectomy. The postoperative course was uneventful.