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目的探讨肝外囊肿型胆道闭锁的诊治过程及发病机制。方法 2010年4月至2012年4月期间,我院诊治的3例肝外囊肿型胆道闭锁患者,对其病例进行回顾性分析。结果患儿多因面部、躯干皮肤黄染,大便色浅入院。入院查体:全身皮肤、巩膜黄染,腹胀,可触及肝脏。B超:胆总管囊肿,以及肝实质弥漫性病变。实验室检查:γ谷氨酰转肽酶、总胆红素、直接和间接胆红素明显升高。全麻状态下,行腹腔探查术,肝脏、脾脏增大,质地中等或者变硬、表面布满结节,胆总管扩张,胆总管及胆囊内为白色黏液,术中进行胆道造影,初步诊断为肝外囊肿型胆道闭锁。行胆囊切除术,术后逐步改善,术后6个月,基本恢复正常。术中快速肝脏病理显示,肝细胞浊肿,肝细胞内淤胆,形成胆管内胆栓,汇管区纤维组织增生,并有炎性细胞浸润。术后随访6个月,患儿肝功能基本正常,没有再出现黄染现象。结论了解肝外囊肿型胆道闭锁的发病机制,以及临床特点,提高检出率,有利于早期治疗,提高患儿的预后质量。
Objective To investigate the diagnosis and treatment of extrahepatic cystoid biliary atresia and its pathogenesis. Methods From April 2010 to April 2012, 3 cases of extrahepatic cystic biliary atresia treated in our hospital were retrospectively analyzed. The results of children with facial and torso skin yellow dye, pale stool admission. Admission examination: systemic skin, scleral yellow dye, bloating, palpable liver. B ultrasound: common bile duct cysts, and diffuse lesions of the liver. Laboratory tests: γ-glutamyl transpeptidase, total bilirubin, direct and indirect bilirubin were significantly higher. Under general anesthesia, underwent abdominal exploration, liver, spleen increased, medium texture or hardened, the surface covered with nodules, common bile duct dilatation, common bile duct and gallbladder white mucus, intraoperative cholangiography, the initial diagnosis of Extrahepatic cyst type biliary atresia. Cholecystectomy, and gradually improved after 6 months, basically returned to normal. Intraoperative rapid liver pathology showed hepatocellular turbidity, cholestasis in hepatocytes, the formation of bile duct cholecystitis, portal area fibrous tissue proliferation, and inflammatory cell infiltration. After 6 months of follow-up, children with normal liver function, there is no recurrence of yellow dye phenomenon. Conclusion To understand the pathogenesis of extrahepatic cyst-type biliary atresia, as well as clinical features, to improve the detection rate, is conducive to early treatment and improve the prognosis of children with quality.