先天性食管闭锁合并气管软骨食管异位一例并文献回顾  

Congenital esophageal atresia associated with tracheobronchial remnant: a case report and literature review

作　　者：吴雨昊 车思艺 周悦航[1] 金鑫[1] 代江涛[1] 李勇刚[1] 吴春[1] Wu Yuhao;Che Siyi;Zhou Yuehang;Jin Xin;Dai Jiangtao;Li Yonggang;Wu Chun(Department of Cardiothoracic Surgery, Children’s Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing 400014, China;Department of Pulmonary, Children’s Hospital of Chongqing Medical University, Chongqing 400014, China)

机构地区：[1]重庆医科大学附属儿童医院胸心外科儿童发育疾病研究教育部重点实验室儿童发育重大疾病国家国际科技合作基地儿科学重庆市重点实验室,400014 [2]重庆医科大学附属儿童医院呼吸内科,400014

出　　处：《中华小儿外科杂志》2019年第6期503-506,共4页Chinese Journal of Pediatric Surgery

摘　　要：目的探讨先天性食管闭锁/气管食管瘘合并气管软骨食管异位的诊治,以提高临床上对此类疾病的认识。方法回顾性分析一例先天性食管闭锁/气管食管瘘合并气管软骨食管异位患儿的发病、诊断及治疗过程,并通过PubMed、Embase、Scopus等英文数据库及CNKI、万方、维普等中文数据库对相关文献进行检索,总结该病的临床表现和诊治要点。英文文献检索关键词为"(congenital tracheobronchial cartilage remnants或tracheobronchial remnants或cartilaginous tracheobronchial remnants)和(congenital esophageal atresia)",中文文献检索关键词为"(气管软骨食管异位或气管软骨异位)和(先天性食管闭锁)"。结果本例患儿因食管闭锁术后出现进食固体食物呕吐入院,食管吞钡造影提示距贲门2~3 cm处食管狭窄,且该处狭窄为突然变细,未见移行段。术后食管狭窄段病理检查提示气管软骨食管异位。先天性食管闭锁合并气管软骨食管异位十分罕见,此次共检索到6篇21例英文报道。其中18例通过食管闭锁术后食管吞钡造影及病理检查诊断,另3例于行食管闭锁根治术时诊断。手术方式主要为狭窄处切除及食管吻合,或加行胃底折叠术。结论先天性食管闭锁合并气管软骨食管异位较为罕见,行食管闭锁根治术后出现的进食固体食物呕吐是其主要表现。该疾病易误诊为食管闭锁术后的吻合口狭窄,诊断主要依靠食管吞钡造影及术后病理活检。手术疗效可靠,但术后可能出现食管狭窄等并发症。Objective To report one rare case of esophageal atresia/tracheo-esophageal fistula (EA-TEF) associated with tracheobronchial remnant (TBR) and review the relevant literature. Methods The clinical manifestations, diagnosis and treatment of one patient with EA-TEF associated with TBR were reviewed. And a systematic review of Pubmed, Embase, Scopus, CNKI, Wanfang and VIP databases was performed for searching for the relevant studies and summarizing the clinical and diagnostic characteristics of TBR-associated EA, cartilaginous tracheobronchial remnants and congenital esophageal atresia. Results TBR-associated EA is rather rare in the literature. Only 6 English studies involving 21 cases have been reported so far. After EA repairing, vomiting of solid food is a major manifestation. And it may be easily misdiagnosed as anastomotic stricture. The major diagnostic mode is composed of barium contrast esophagogram and postoperative pathologic examination. Surgery has a definite efficacy. However, such postoperative complications as anastomotic stricture may be observed. Conclusions EA associated with TBR is rare. However, its clinical outcomes are satisfactory if appropriate diagnosis and treatment are performed.

关 键 词：食管闭锁 气管软骨 食管异位软骨 

分 类 号：R726.5[医药卫生—儿科]