先天性双气管食管瘘的诊治一例并文献复习

Congenital double tracheoesophageal fistulas:one case report with a literature review

目的报道一例先天性双气管食管瘘(congenital double tracheoesophageal fistulas,CDTEFs)患儿并结合相关文献总结其临床特征、诊断方法及治疗方案。方法收集1例CDTEFs患儿的临床资料及诊治方案,同时检索截至2020年8月PubMed、Ovid、Cochrane、万方、中国知网、维普及中国生物医学文献数据库中关于CDTEFs的相关文献。结果本例患儿因生后呛咳及反复肺炎入院,胸部CT及食管造影发现气管食管瘘(tracheoesophageal fistula,TEF),术前支气管镜检查发现气管下段后壁有2个异常开口,均有分泌物溢出,美蓝试验阳性。术中在支气管镜及胃镜协助下分别经2个瘘口放置导丝(直径0.036 cm,长300 cm),胸腔镜下完成双瘘修补,术后造影未见异常,经口顺利喂养。通过对国内外文献检索,共检索到10篇相关文献,均为个案报道。10例CDTEFs患儿中7例基本信息较完整。7例中有3例系死亡后尸检确诊CDTEFs;1例初诊为CDTEFs(手术情况和预后不详);3例初诊时仅发现远端TEF,其中仅1例患儿经一期开放手术(开胸+颈部同时修补)成功救治,术后3例均存活。结合上述文献,本例患儿是首次经胸腔镜手术成功治愈的案例。结论CDTEFs非常罕见,临床表现与先天性Ⅴ型食管闭锁类似,容易漏诊,对于初诊为Ⅴ型食管闭锁的患儿,支气管镜检查需注意气管壁是否存在其他异常开口,如患儿情况允许,胸腔镜一期手术修补CDTEFs是安全可行的。

Objective To report one rare case of congenital double tracheoesophageal fistulas and summarize its clinical features,diagnostic methods and treatment based on relevant literatures.Methods The clinical manifestations,diagnosis and treatment of one patient of CDTEFs were analyzed retrospectively.Literatures associated with CDTEFs were searched from PubMed,Ovid,Cochrane,Wanfang,CNKI,VIP and China Biomedical Literature Database up to August 2020.Results In this case,the child was admitted into the hospital due to the choking and cough and repeated pneumonia after birth,her chest CT and esophagography found the tracheoesophageal fistula(TEF),but preoperative bronchoscopy found two abnormal openings in the posterior wall of the lower trachea,all of which had secretion outflow and methylene blue test positive.During the operation,the guide wire(0.014 inches in diameter and 300 cm in length)was placed through two fistulas with the assistance of bronchoscope and gastroscope,and the double fistula repair was completed by thoracoscopic surgery.There was no abnormality in the postoperative angiography,and the oral feeding started successfully.A total of 10 relevant articles were retrieved through literature search,all of which were case reports.Seven of the 10 children with CDTEFs had relatively complete basic information.Among the 7 cases,CDTEFs were confirmed by postmortem examination in 3 cases;CDTEFs were confirmed in 1 case at initial diagnosis(surgery and prognosis were unknown),and in other 3 cases which were survived after surgical treatment only found distal TEF at initial diagnosis,and only 1 case was successfully treated by a one-stage open operation through neck and chest.Combined with the above mentioned literature,this is the first case successfully treated by thoracoscopic surgery.Conclusion CDTEFs are extremely rare.The clinical manifestations are similar to congenital tracheoesophageal fistula which lead to miss the diagnose easily.When patients are diagnosed with congenital tracheoesophageal fistula,bronchoscopy should pay attention to whether there are any other abnormal openings on tracheal posterior wall.If the patient's situation is good,it is safe and feasible for one-stage repair of CDTEFs by thoracoscopic surgery.