囊肿型胆道闭锁的产前产后一体化鉴别诊断  

Integrated pre-and post-natal differential diagnosis of cystic biliary atresia

作　　者：童明欣 谢华[2] 唐维兵[2] 刘翔 杨合英[4] 李英超[5] 潘永康[6] 詹江华[7] 吕志宝[8] 任红霞[9] 高志刚 冯杰雄[11] 陈琦[12] 虞梅 张志波[1] Tong Mingxin;Xie Hua;Tang Weibing;Liu Xiang;Yang Heying;Li Yingchao;Pan Yongkang;Zhan Jianghua;Lyu Zhibao;Ren Hongxia;Gao Zhigang;Feng Jiexiong;Chen Qi;Yu Mei;Zhang Zhibo(Department of Pediatric Surgery,Shengjing Hospital of China Medical University,Shenyang 110003,China;Department of Pediatric Surgery,Nanjing Children’s Hospital,Nanjing 210000,China;Department of Pediatric Surgery,Children’s Hospital of Anhui Province,Hefei 230000,China;Department of Pediatric Surgery,1st Affiliated Hospital of Zhengzhou University,Zhengzhou 461499,China;Department of Pediatric Surgery,2nd Affiliated Hospital of Hebei Medical University,Shijiazhuang 050005,China;Department of Pediatric Surgery,Children’s Hospital of Xian,Xian 710018,China;Department of Pediatric Surgery,Children’s Hospital of Tianjin,Tianjin 300074,China;Department of Pediatric Surgery,Children’s Hospital of Shanghai,200062,China;Department of Pediatric Surgery,Children’s Hospital of Shanxi Province,Taiyuan 030032,China;Department of Pediatric Slurgery,Cildre's Hopital of Zhejiang Unicersity,Hangzhou 310000,China;Dpartmend of Pediatric Surgery,Tongi Hoptad of Houazhong University of Science and Tehology,Wuhan 430071,China;Depurtment of Pedicric Surgerny,3rd Aflisted Hospsial of Zhengzhou Uinicersity,Zhengzhou 450012,Chinn;Depenrment of Pedictrie Surgery,Guishou Prorineiad Pesple's Hapinl,Guiyang 550499,China)

机构地区：[1]中国医科大学附属盛京医院小儿外科,沈阳110003 [2]南京市儿童医院小儿外科,南京210000 [3]安徽省儿童医院小儿外科,合肥230000 [4]郑州大学第一附属医院小儿外科,郑州461499 [5]河北医科大学第二附属医院小儿外科,石家庄050005 [6]西安市儿童医院小儿外科,西安710018 [7]天津市儿童医院小儿外科,天津300074 [8]上海市儿童医院小儿外科,上海200062 [9]山西省儿童医院小儿外科,太原030032 [10]浙江大学附属儿童医院小儿外科,杭州310006 [11]华中科技大学附属同济医院小儿外科,武汉430071 [12]郑州大学附属第三医院小儿外科,郑州450012 [13]贵州省人民医院小儿外科,贵阳550499

出　　处：《临床小儿外科杂志》2024年第12期1125-1132,共8页Journal of Clinical Pediatric Surgery

基　　金：国家自然科学基金(82170529)。

摘　　要：目的囊肿型胆道闭锁(cystic biliary atresia,CBA)是产前通过超声检查可发现的特殊类型胆道闭锁(biliary atresia,BA),容易与胆总管囊肿(choledochal cyst,CC)相混淆,二者早期鉴别诊断存在一定困难。本研究旨在探讨CBA与CC患儿产前超声影像特征、出生后2周内结合胆红素(conjugated bilirubin,CB)及总胆汁酸(total bile acid,TBA)水平对于CBA和CC的鉴别诊断价值。方法本研究为多中心回顾性研究。收集2020年10月至2021年12月中国内地13个儿科研究中心产前发现的肝门区囊肿新生儿作为研究对象,收集所有入选新生儿的产前超声检查结果,并于出生后2周内每周检测血CB和TBA水平各1次。根据手术结果或最终临床结局将患儿分为CBA组和CC组,对比两组产前超声影像特征以及出生后2周内CB和TBA的差异,并通过统计分析评估上述指标对于CBA和CC的鉴别诊断效能及临界值。结果共143例产前发现肝门区囊肿的患儿纳入研究,其中CBA组37例,CC组106例,初始诊断时间为孕(25.29±6.07)周。两组囊肿初始体积和直径差异无统计学意义(P>0.05)。CC组囊肿体积增长速度显著高于CBA组,至产前末次超声检查时,CC组囊肿体积平均增长8.34倍,而CBA组仅增长1.48倍。当囊肿体积增长临界值设为3.90倍时,诊断CBA的AUC、灵敏度分别为0.828(95%CI:0.728~0.929)、83.3%。CBA组患儿的CB和TBA水平自出生后第1周开始显著升高,第2周继续升高。当出生后第1周CB(CB1)临界值为24μmol/L、出生后第2周TBA(TBA2)临界值为20.9μmol/L时,鉴别诊断CBA和CC的约登指数达到最大值,分别为0.880和0.826,对应AUC分别为0.902和0.860。结论经系列产前超声检查提示囊肿体积增加倍数<3.90的新生儿患CBA的风险较高。胆囊形态异常对于CBA的产前诊断有重要意义。出生后2周内CB和TBA水平可用于鉴别诊断CBA和CC,当CB1≥24μmol/L和TBA2≥20.9μmol/L时,提示CBA的患病风险较高。Objective Cystic Biliary Atresia(CBA)is a special type of Biliary Atresia(BA)that can be detected prenatally through ultrasound examination.It is easily confused with Choledochal Cyst(CC),and there is a certain challenge in differentiating between the two in early diagnosis.The purpose of this study is to explore the differences in prenatal ultrasonic imaging characteristics between CBA and CC cases,and to evaluate the value of conjugated bilirubin(CB)and total bile acid(TBA)levels in the early postnatal period(i.e.,within the first 2 weeks after birth)for the differential diagnosis of these two conditions.Methods This is a retrospective multicenter clinical study.The patients were divided into CBA group and CC group according to surgical outcome or final clinical outcome.The prenatal ultrasound image characteristics and the differences in CB and TBA values between the two groups were compared,and the diagnostic efficacy and cut-off value of these indicators in differentiating CBA and CC were evaluated by statistical analysis.Results A total of 143 children with hepatic portal cysts were included in this study,including 37 in the CBA group and 106 in the CC group.The mean time of initial diagnosis was(25.29±6.07)weeks of gestation.There was no difference in the initial volume and diameter of cysts between the two groups.However,the growth rate of cysts in the CC group was significantly higher than that in the CBA group.The average growth rate of cysts in the CC group was 8.34 times at the last prenatal ultrasound examination,while that in the CBA group was only 1.48 times.When the critical value of cyst volume growth was set to 3.90 times,the AUC and sensitivity for diagnosing CBA were 0.828(95%CI,0.728-0.929)and 83.3%,respectively.In addition,CB and TBA levels in the CBA group increased significantly from the first week after birth and continued to increase at the second week.When the critical value of CB1 was 24μmol/L,and the critical value of TBA2 was 20.9μmol/L,the Jorden index of CBA and CC reached the ma

关 键 词：囊肿型胆道闭锁 胆总管囊肿 诊断 鉴别 产前诊断 超声检查 产前 产后期 诊断 结合胆红素 总胆汁酸 婴儿 新生 

分 类 号：G84[文化科学—体育训练]