Okada T, Itoh T, Sasaki F, Cho K, Honda S, Todo S
Department of Pediatric Surgery, Hokkaido University Graduate School of Medicine, Sapporo, Japan.
Eur J Pediatr Surg. 2007 Feb;17(1):6-11. doi: 10.1055/s-2007-964948.
Because it is difficult to distinguish preoperatively between a prenatally diagnosed choledochal cyst (CC) and type-1 cystic biliary atresia (BA) by ultrasound scanning or magnetic resonance imaging, some mode of discriminating between the 2 entities is required. The aim of this study was to investigate the immunohistological differences between prenatally diagnosed CC and type-1 cystic BA, using liver biopsy specimens immunostained for CD56. Five children with prenatally diagnosed CC and two children with prenatally diagnosed type-1 cystic BA were identified by fetal ultrasonography between 1985 and 2004. The control group included two children who were operated on at an earlier period due to postnatally diagnosed BA. Liver wedge biopsy in the right lobe was performed at the time of the radical operation. Histological findings of the CD56-stained liver biopsy specimens were classified into 4 categories each, with particular focus on staining distribution and intensity. The staining distribution was classified according to the scale 0 = no staining; 1 = some staining of bile ducts/ductules but staining in less than one-third of portal tracts; 2 = staining in one-third to less than two-thirds of portal tracts; and 3 = staining in more than two-thirds of portal tracts. Staining intensity was scored as follows: 0 = no staining, 1 = weak staining, 2 = moderate staining, and 3 = strong staining. The staining intensity and distribution in the CC group was zero in all 5 cases. The type-1 cystic BA group consisted of patients with scale 1 or 3 staining distribution and score 1 or 2 staining intensity. In the control group, staining distribution was 1 or 3, and staining intensity was 1 or 3. These results indicate that CD56-positive biliary duct cells are present in prenatally diagnosed type-1 cystic BA. The authors suggest that exploratory laparotomy might be avoided and, instead, immunohistological examination using liver biopsy specimens may be a reliable test for the differential diagnosis of CC and type-1 cystic BA in prenatally diagnosed neonates.
由于通过超声扫描或磁共振成像在术前难以区分产前诊断的胆总管囊肿（CC）和1型囊性胆道闭锁（BA），因此需要某种区分这两种实体的方法。本研究的目的是利用对CD56进行免疫染色的肝活检标本，研究产前诊断的CC和1型囊性BA之间的免疫组织学差异。1985年至2004年间，通过胎儿超声检查确定了5例产前诊断为CC的儿童和2例产前诊断为1型囊性BA的儿童。对照组包括2例因出生后诊断为BA而在早期接受手术的儿童。在根治性手术时进行右叶肝楔形活检。对CD56染色的肝活检标本的组织学结果各分为4类，特别关注染色分布和强度。染色分布根据以下标准分类：0 = 无染色；1 = 胆管/小胆管有一些染色，但染色的门静脉区域不到三分之一；2 = 门静脉区域三分之一至不到三分之二有染色；3 = 门静脉区域三分之二以上有染色。染色强度评分如下：0 = 无染色，1 = 弱染色，2 = 中度染色，3 = 强染色。CC组的所有5例病例的染色强度和分布均为零。1型囊性BA组由染色分布为1或3级且染色强度评分为1或2级的患者组成。对照组的染色分布为1或3级，染色强度为1或3级。这些结果表明，产前诊断的1型囊性BA中存在CD56阳性胆管细胞。作者建议，对于产前诊断的新生儿，可能可以避免剖腹探查，取而代之的是，使用肝活检标本进行免疫组织学检查可能是CC和1型囊性BA鉴别诊断的可靠检测方法。
