首页> 外文期刊>Hong Kong Journal of Paediatrics >A Retrospective Study Between Type I Cystic Biliary Atresia and Infantile Choledochal Cyst at a Tertiary Centre
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A Retrospective Study Between Type I Cystic Biliary Atresia and Infantile Choledochal Cyst at a Tertiary Centre

机译:第三中心I型囊性胆道闭锁与小儿胆总管囊肿的回顾性研究

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Purpose : Type I cystic biliary atresia (CBA) is an uncommon variant of biliary atresia (BA) which is liable to misdiagnosis as infantile choledochal cyst (ICC). We assessed our experience with type I CBA and compared with that of ICC. Methods: Seven patients (4 males and 3 females) with type I CBA, receiving liver function detection, radiological imaging studies, liver/cyst histological examinations and radical surgery, were compared with 23 ICC patients (14 males and 9 females) admitted in our division during a 6-year period. Findings: The mean levels of total bilirubin (T-Bil), direct bilirubin (D-Bil), alanine aminotransferase (ALT), aspartate aminotransferase (AST) and alkaline phosphatase (AKP) in the type I CBA group were significantly higher than those in the ICC group (p<0.01). The mean diameter of the cyst in the type I CBA group was significantly smaller than that in the ICC group (p<0.01). All 7 patients in the type I CBA group were accurately diagnosed by magnetic resonance cholangiopancreatography (MRCP) preoperatively, being in accordance with the results of intraoperative cholangiography and liver/cyst histology. Six cysts (85.7%) in the type I CBA group had a fibroinflammatory wall with no biliary epithelial lining while all 23 (100%) cysts in the ICC group had normal biliary epithelial lining. Six infants (85.7%) with type I CBA cleared their jaundice (total bilirubin ≤20 μmol/L) within 6 months after a Kasai portoenterostomy or hepaticojejunostomy procedure. All patients with ICC were in good condition with no jaundice and cholangitis after the operation. Conclusions: While elevated T-BIL/D-BIL and AST/ALT/AKP as well as smaller gallbladder/extrahepatic cyst imply type I CBA, MRCP can provide further effective assistance in making a relatively accurate diagnosis before operation. Either hepaticojejunostomy or Kasai portoenterostomy could be chosen as an optimal procedure in the treatment of type I CBA.
机译:目的:I型胆囊闭锁症(CBA)是胆道闭锁症(BA)的罕见变体,容易被误诊为婴儿胆总管囊肿(ICC)。我们评估了我们使用I型CBA的经验,并与ICC进行了比较。方法:将接受肝功能检测,放射影像学检查,肝/囊肿组织学检查和根治性手术的7例I型CBA患者(男4例,女3例)与我院收治的23例ICC的患者(男14例,女9例)进行比较。在六年的时间里划分。结果:I型CBA组的总胆红素(T-Bil),直接胆红素(D-Bil),丙氨酸氨基转移酶(ALT),天冬氨酸氨基转移酶(AST)和碱性磷酸酶(AKP)的平均水平显着高于那些在ICC组中(p <0.01)。 I型CBA组的囊肿平均直径明显小于ICC组(p <0.01)。根据术中胆管造影和肝/囊肿组织学检查的结果,术前通过磁共振胰胆管造影(MRCP)对I型CBA组的所有7例患者进行了准确诊断。 I型CBA组中有6个囊肿(占85.7%)具有纤维性炎症壁,无胆管上皮衬里,而ICC组中所有23个(100%)囊肿均具有正常的胆管上皮衬里。在进行开赛肠胃肠吻合术或肝空肠吻合术后的6个月内,有6名IBA婴儿(85.7%)清除了黄疸(总胆红素≤20μmol/ L)。所有ICC患者术后状况良好,无黄疸和胆管炎。结论:虽然T-BIL / D-BIL和AST / ALT / AKP升高,以及胆囊/肝腹囊肿变小,提示I型CBA,但MRCP可以为手术前的相对准确诊断提供进一步的有效帮助。肝空肠造口术或开赛肠胃造口术可以选择作为治疗I型CBA的最佳方法。

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