先天性胆管扩张症亦称先天性胆管囊肿，其病因目前认为主要是胰胆管合流异常，是一种比较少见的临床疾病，典型临床表现为黄疸、腹痛、腹部包块，常伴有胆管炎、胰腺炎、癌变等并发症。超声诊断通常作为首选检查方法，磁共振胰胆管成像（MRCP）结合CT检查是目前最为常用的诊断和术前评估方法。囊肿彻底切除+肝管空肠Roux-en-Y吻合术是目前最为推崇的手术方式，但对于肝内胆管的囊肿，目前认为肝移植可能是最终有效的选择。

Evolvement of diagnosis and treatment in congenital biliary dilatation *PENG Shu-you, WANG Xu-an. *Department of Surgery, the Second Affiliated Hospital of College of Medicine, Zhejiang University, Hangzhou310009, China
Corresponding author: WANG Xu-an, E-mail: doctor.wxa@163.com
Abstract    Congenital biliary dilatation (CBD) is a group of rare conditions, commonly associated with the presence of pancreaticobiliary maljunction. The main clinical symptoms are jaundice, abdominal pain and abdominal mass with complications of cholangitis, acute pancreatitis and canceration. Ultrasonic diagnosis is regarded as the first examination. Magnetic resonance cholangiopancreatography & CT scan is the most commonly used preoperative imaging study to assess cyst anatomy and to classify the disease according to the standard Todani classification currently. Excision of the dilated bile duct with Roux-en-Y hepaticojejunostomy is strongly recommended treatment. But the optimal treatment of intrahepatic bile duct dilatations remains controversial.