新生儿先天性支气管胆管瘘并胆道闭锁1例及文献复习

A Case of Neonatal Congenital Broncho-Biliary Fistula with Biliary Atresia and Literature Review

目的探讨新生儿先天性支气管胆管瘘(CBBF)的流行病学特征、临床表现和诊疗要点。方法整理郑州大学第三附属医院收治的1例新生儿CBBF合并胆道闭锁的临床资料。以“新生儿”“婴儿”“先天性支气管胆管瘘”“胆道闭锁”“neonate”“infant”“congenital bronchobiliary fistula”“biliary atresia”为关键词,对中国生物医学文献数据库、中国知网、万方数据库、维普中文科技期刊全文数据库、PubMed、Embase及Web of Science数据库自建库至2021年4月30日收录的文献进行检索。分析归纳CBBF的流行病学特征、临床表现和诊疗要点。结果本例患儿为足月女婴,临床表现为进行性加重呼吸困难,气管插管后发现气管导管内有大量胆汁样痰液,行CT气道重建及仿真内窥镜检查确诊为支气管胆管瘘,肝胆超声及痰液中检测出胆红素可协助诊断。术中胆道造影明确诊断胆道闭锁,手术切除瘘管并接受Roux-en-Y胆囊-空肠吻合术,术后患儿恢复良好,随防至2岁8个月,患儿生长发育正常。文献检索出新生儿期确诊者20例,包括本病例共21例。其中女15例(71.43%),男6例(28.57%);合并胆管发育不良8例,胆道闭锁3例,右侧先天性膈疝1例。死亡3例,存活18例(85.7%)且预后良好。结论新生儿CBBF系罕见疾病,女性多见,常合并胆道发育畸形,若能尽早诊断,预后良好。

Objective To study the epidemiology,clinical manifestation and key points of diagnosis and treatment of neonatal congenital broncho-biliary fistula(CBBF).Methods The data of one neonate with CBBF admitted to the Third Affiliated Hospital of Zhengzhou University was retrospectively analyzed.Databases were searched including China Biology Medicine disc,CNKI,Wanfang,VIP,PubMed,Embase and Web of Science starting from the database established until Apr 30,2021.Literatures were retrieved using the following key words:“neonate”,“infant”,“congenital bronchobiliary fistula”,“biliary atresia”.The epidemiology,clinical manifestation and key points of diagnosis and treatment were summarized from literature review.Results The clinical manifestation of our case was a full-term female with progressively worsening dyspnea,and a large amount of bile-like sputum in the tracheal tube after tracheal intubation.Computed-temography airway reconstruction and virtual endoscopy were performed to diagnose bronchobiliary fistula.Hepatobiliary ultrasound and bilirubin detection in sputum can assist in the diagnosis.Intraoperative cholangiography clearly diagnoses biliary atresia.The bronchobiliary fistulous tract was resected and the Roux-en-Y cholecysto-jejunostomy was performed.The neonate recovered well after the operation,and the overall growth and development were normal at 2 years old and 8 months on follow-up.Literature search collected a total of 20 cases diagnosed in the neonatal period,and there are a total of 21 cases including this case.Among them,6 were male(28.57%),15 were female(71.43%),a total of 8 cases were complicated with biliary dysplasia,a total of 3 cases were with biliary atresia,and 1 case was with right congenital diaphragmatic hernia.There were 3 cases died and 18 cases own good prognosis after surgery(85.7%).Conclusion Neonatal CBBF is a rare disease,which is common in women.It is often complicated with biliary tract malformations.If diagnosed as early as possible,the prognosis is good.