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目的:探讨儿童先天性胆管扩张症(CBD)的临床、病理、影像特点及治疗。方法:回顾性分析58例经影像学检查、手术和病理证实CBD患儿的临床资料。结果:58例均出现临床症状,主要表现为腹痛、黄疸、腹部肿块,部分患者合并发热、呕吐症状。CT(45例)、超声(40例)、MRCP(17例)检查均显示胆管扩张;11例MRCP诊断为胰胆管合流异常(PBM)。57例成功进行术中胆道造影,影像和手术分型:Ⅰ型43例,其中囊状35例,8例表现为梭型或柱形;Ⅱ型2例;Ⅳ型13例;未发现Ⅲ型患者。58例患儿术后病理显示CBD囊壁纤维组织增生,囊肿壁内衬上皮部分或全部脱落,40例囊壁伴有炎症,39例慢性胆囊炎。53例行一次性囊肿切除肝管空肠Roux-en-Y吻合术,5例先行胆道外引流后二次肝管空肠Roux-en-Y吻合术,术后短期均无严重并发症发生。随访1~9年,患儿无胆道梗阻、胆管炎、肠梗阻和胆道肿瘤发生。结论:超声检查、术中造影、CT和MRCP均对儿童CBD诊断有一定价值;MRCP为CBD合并PBM首选诊断方法;儿童CBD的治疗应早期采用肝管空肠Roux-en-Y吻合术。
Objective: To investigate the clinical, pathological and imaging features and treatment of children with congenital bile duct dilatation (CBD). Methods: The clinical data of 58 children with CBD confirmed by imaging examination, surgery and pathology were retrospectively analyzed. Results: All of the 58 patients had clinical symptoms. The main symptoms were abdominal pain, jaundice and abdominal mass. Some patients had fever and vomiting. CT (45 cases), ultrasound (40 cases) and MRCP (17 cases) showed bile duct dilatation. MRCP was diagnosed as pancreaticobiliary duct abnormalities (PBM) in 11 cases. Fifty-seven cases were successfully performed intraoperative cholangiography, imaging and surgical typing: 43 cases were type Ⅰ, 35 cases were cystic, 8 cases were shuttle or column, 2 cases were type Ⅱ and 13 cases were type Ⅳ. No type Ⅲ patient. Postoperative pathology of 58 cases showed that the proliferation of CBD fibrous tissue in the CBD, partial or complete loss of epithelial lining of the cyst wall, inflammation of the cystic wall in 40 cases and chronic cholecystitis in 40 cases. Fifty-three consecutive Roux-en-Y anastomosis was performed in 53 cases of primary hepaticojejunostomy and 5 cases of primary hepaticojejunostomy after Roux-en-Y anastomosis. No serious complications occurred in short-term postoperatively. Follow-up 1-9 years, children without biliary obstruction, cholangitis, intestinal obstruction and biliary tumors. Conclusion: Ultrasonography, intraoperative angiography, CT and MRCP are valuable for the diagnosis of children with CBD. MRCP is the first choice of the diagnosis of CBD combined with PBM. Roux-en-Y hepaticojejunostomy should be used early in the treatment of children with CBD.