儿童先天性胆管扩张症的诊断与治疗

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.

Diagnosis and treatment of congenital biliary dilatation in children

Objective: To explore the clinical, pathological and imaging characteristics of congenital biliary dilatation(CBD) in children and its management.Methods: The clinical data of 58 pediatric patients with CBD, which was indentified and confirmed byimaging examinations, surgical and pathological findings, were retrospectively analyzed.Results: All the 58 patients exhibited clinical symptoms that mainly were abdominal pain, jaundice andabdominal mass, and some of them were combined with fever and vomiting. All the results of CT (performedin 45 cases), ultrasonography (performed in 40 cases) and magnetic resonance cholangiopancreatography(MRCP) (performed in 17 cases) revealed bile duct dilatation, and 11 cases were diagnosed withpancreaticobiliary maljunction (PBM) by MRCP. Intraoperative cholangiography was successfully performed