X Wang, Q Qin, Y Lou

Abstract

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.

目的：I型膽道閉鎖是膽道閉鎖中不常見的類型，其較易誤診為小兒先天性膽總管囊腫，故我們對兩者的診治經驗進行評估。方法：在6年間收集7例I型膽道閉鎖患者（男性4例，女性3例），病童進行了肝臟功能檢測、影像學研究、肝臟／膽道的病理檢查以及外科根治手術；並同時與23例小兒先天性膽總管囊腫患者（男性14例，女性9例）進行比較。結果：I型膽道閉鎖組中總膽紅素、直接膽紅素、穀丙轉氨酶、穀草轉氨酶和鹼性磷酸酶的平均值顯著高於先天性膽總管囊腫組（p<0.01）。所有7例I型膽道閉鎖患者均在通過磁共振膽管胰造影術前，已有明確的診斷，並與術中病理檢查一致。6例膽道閉鎖患者（85.7%）發現膽道纖維炎性內膜而無膽道上皮內膜層，但所有23例膽總管囊腫患者（100%）具有正常的膽道上皮內膜層。6例膽道閉鎖患者（85.7%）通過Kasai膽腸吻合術或肝管空腸吻合術後，在6個月內黃疸消失（總膽紅素≤20 μmol/L）。所有膽總管囊腫患者術後預後良好，均無黃疸和膽管炎發生。結論：總膽紅素/直接膽紅素以及穀丙轉氨酶、穀草轉氨酶和鹼性磷酸酶的增高，並伴隨肝外小膽管和小膽囊，已提示病童可能出現I型膽道閉鎖。磁共振膽管胰造影術能夠提供急性期術前有效明確的診斷。Kasai膽腸吻合術或肝管空腸吻合術均是有效治療I型膽道閉鎖的方法。