新生儿肝外胆管囊性病变的临床与病理特征

Clinical and pathological features of cystic lesions of extrahepatic bile duct in neonates

目的 了解新生儿不同类型肝外胆道囊性病变的临床表现与病理特征。方法 对 2 2例新生儿肝外胆道囊性病变 [Ⅰ型胆道闭锁 (BA) 4例 ,Ⅲ型BA 1例 ,胆总管囊肿 (CC) 17例 ]患儿临床表现、肝功能、B超、CT、光镜及电镜特征进行比较。结果 BA与新生儿CC均好发于女童 ,以梗阻性黄疸为主要表现 ,肝功能检查结果符合梗阻性黄疸的肝功能改变。二者肝脏组织学改变 (光镜与电镜 )也可能相似。但B超与CT检查发现 ,BA组囊肿与胆囊大小均小于CC组 (P均 <0 .0 5 )。术中胆道造影显示ⅢBA型组肝外胆管囊肿终止于肝门处 ,肝内胆管树无法显示 ;Ⅰ型BA组肝内胆管树变形、连续性中断且有左右肝管狭窄 ;而CC组肝内胆管树形态正常。结论 新生儿肝外胆道囊性病变可能是多种胆道疾病的局部表现 ,术中造影可有效鉴别这些疾病 。

Objective To investigate the difference of clinical and pathological manifestations between typeⅠand typeⅢ of biliary atresia(BA) and choledochal cyst(CC) in neonates.Methods Symptoms,signs,hepatic function,B ultrasound,computed tomography(CT), intraoperative cholangiograhy and pathologic features were all analysed in 5 neonates with BA and 17 neonates with CC.Results Malformations occurred more commonly in girls, and the obstructive jaundice was the main manifestation in all groups. All of the indexes of hepatic function were similar between patients with BA and CC. No significant difference between BA and many cases of CC were seen either in light microscopy or in transmission electron microscopy. But the length and width of the cyst and the gallbladder in neonates with BA were all shorter than those in CC found by B ultrasound and CT (P<0.05). Intraoperative cholangiography showed that the biliary tree within liver could not be demonstrated in type Ⅲ BA, and the deformation of biliary tree within liver and the stricture of the portal bile duct were the predominant features in type Ⅰ BA, while the bile duct within liver was normal or dilated in neonates with CC.Conclusions The cystic lesions of extrahepatic bile duct may be common manifestation of different causes,and intraoperative cholangiography can give the precise and effective differential diagnosis and guide the rational way of treatment.