Congenital bile duct dilatation (CBD) that developed in a parent and son is presented. Familial occurrence of CBD is rare, with only a few male cases having been reported. Since the initial report of CBD occurring in ...Congenital bile duct dilatation (CBD) that developed in a parent and son is presented. Familial occurrence of CBD is rare, with only a few male cases having been reported. Since the initial report of CBD occurring in siblings in 1981, a total of 20 cases (10 pairs) have been published as of 2007. Clinical and genetic features of CBD are discussed.展开更多
Congenital cystic dilatations of the bile duct are rare malformations representing 1 per 13,000 births with a female predominance. Diagnosis of this pathology is based on radiological imaging especially radiological i...Congenital cystic dilatations of the bile duct are rare malformations representing 1 per 13,000 births with a female predominance. Diagnosis of this pathology is based on radiological imaging especially radiological investigations conducted on an ultrasound coupled with Computed tomography (CT) scan and especially a bili-MRI which allows the analysis of the bile duct malformations and the anomalies of the pancreaticobiliary junction. We will discuss in this clinical case the various radiological aspects of cystic dilatations of the biliary duct.展开更多
文摘Congenital bile duct dilatation (CBD) that developed in a parent and son is presented. Familial occurrence of CBD is rare, with only a few male cases having been reported. Since the initial report of CBD occurring in siblings in 1981, a total of 20 cases (10 pairs) have been published as of 2007. Clinical and genetic features of CBD are discussed.
文摘Congenital cystic dilatations of the bile duct are rare malformations representing 1 per 13,000 births with a female predominance. Diagnosis of this pathology is based on radiological imaging especially radiological investigations conducted on an ultrasound coupled with Computed tomography (CT) scan and especially a bili-MRI which allows the analysis of the bile duct malformations and the anomalies of the pancreaticobiliary junction. We will discuss in this clinical case the various radiological aspects of cystic dilatations of the biliary duct.
文摘目的:探讨内镜逆行胰胆管造影(endoscopic retrograde cholangiopancreatography, ERCP)在诊断和治疗小儿胰胆管汇合异常(pancreaticobiliary maljunction, PBM)中的安全性和有效性。方法:回顾性分析本院ERCP确诊为PBM的74例患儿,分析临床资料和术后随访。结果 :按照日本胆胰管合流异常研究组(Japanese Study Group on Pancreaticobiliary Maljunction, JSGPM)分型,A型32例,B型16例,C型23例,D型3例。MRCP检查的阳性诊断率仅8%。本研究所有患儿均行乳头肌切开和鼻胆管引流。其中69例(93.4%)行取石/蛋白酶栓,2例术后留置胰管支架。7例(9.5%)术后出现ERCP后轻型胰腺炎,无术后出血、贲门撕裂及穿孔等严重并发症发生。除7例(9.5%)PBM不合并胆道扩张的患儿外,其余术后均行肝外胆管切除+肝管空肠Roux-en-Y吻合术。结论:ERCP在小儿PBM的诊治过程中具有诊断效力强、疗效好、安全性高的优点,对早期确诊并干预PBM的病理进程具有重要意义。
