经胸腔镜手术治疗先天性食管闭锁并食管气管瘘

先天性食管闭锁是严重先天性消化道畸形之一,发病率约为1／3000,根据Gross五型分类法,食管闭锁并食管气管瘘约占所有病例的85％,手术是唯一有效的治疗方法,传统手术方法为取侧胸沿肋间隙开胸手术,无论经胸膜外或经胸膜腔途径,术中为显露术野均需对切口两侧胸壁做较大幅度撑开,加上术毕关胸缝合肋骨,对胸壁损伤较大,术后遗有胸壁畸形的可能。随着微创技术的提高及更加精致器械的应用,使微创手术在新生儿、高难度复杂疾患中逐渐应用,经胸腔镜手术治疗先天性食管闭锁较传统开放手术而言,术野放大清晰,手术操作对肺组织的影响小,切口更美观,胸壁损伤小,不离断肋间肌肉,术后无遗留胸廓畸形后遗症的可能。     

胸腔镜治疗先天性食管闭锁

目的 阐述胸腔镜治疗先天性食管闭锁的经验体会.方法 回顾性分析了2006年8月至2011年5月期间共进行11例胸腔镜下食管闭锁手术,采用Styker公司的胸腔镜器械,进行胸腔镜手术操作,患儿年龄为3 d～2个月,男7例,女4例,体重2.1～3.6kg.术前均作碘油造影及胸腹部摄片,证实为食管闭锁,1例1型,10例为Ⅲ型,其中8例伴有先天性心脏病.结果 3例转为开胸,其中2例因手术野影响,另1例为术中出血;7例均在胸腔镜下Ⅰ期完成手术;1例采用胸腔镜分期手术.术中出血少,术后恢复平稳,有2例发生吻合口漏,经保守治疗后恢复好.术后7例作食管扩张各一次.结论 胸腔镜下食管闭锁手术有一定的优越性,但需要掌握一定的适应证,同时要求手术者有较熟练的腔镜技术,患儿的选择和手术者的技术是保证手术成功的关键.     

先天性食管闭锁并食管气管瘘胸腔镜手术探讨

目的 探讨胸腔镜下手术治疗先天性食管闭锁并食管气管瘘的方法和疗效.方法 回顾性分析本院2013年7月至2015年7月间经胸腔镜手术治疗54例先天性食管闭锁患儿的病例资料.其中男32例,女22例,患儿年龄1～12d,平均(5.17±2.32)d,患儿体重2.0～3.8kg,平均(2.85±0.44) kg.术前通过食管造影检查诊断先天性食管闭锁ⅢA型13例,ⅢB型41例.结果 51例患儿在胸腔镜下完成食管气管瘘结扎切断、食管端端吻合术,手术时间94～208min,平均(138±30) min.3例患儿中转开胸手术.2例患儿术中发现近端也合并有食管气管瘘,同时行近端瘘管结扎.3例患儿发现远端合并食管狭窄,术中同时行食管狭窄切开术.2例患儿术中发现合并右位主动脉弓畸形,行主动脉弓前食管吻合.2例患儿游离近端食管时气管破裂,予5-0可吸收线缝合修补治愈.术后9例发生吻合口漏,其中2例放弃治疗,2例因重症肺炎并呼吸衰竭死亡,1例食管气管瘘复发失访,其余4例患儿通过静脉营养支持治疗治愈.49例患儿治愈出院并获得随访4～28个月.5例患儿出现食管狭窄,在胃镜下行食管扩张治愈出院.结论 胸腔镜下手术治疗新生儿Ⅲ型先天性食管闭锁是一种安全有效的手术途径;术中视野清晰,手术操作方便,能同时处理合并的其他食管畸形;食管斜面裁剪食管吻合可降低术后食管狭窄的发生.胸腔镜下食管闭锁手术的疗效需要术者具备丰富的手术经验和娴熟的腔镜操作技术,麻醉的配合是手术顺利进行的重要因素.     

先天性食管闭锁并气管食管瘘的早期胚胎学研究

目的利用阿霉素诱导制作食管闭锁并气管食管瘘（esophageal atresia and tracheoesophageal fistula，EA-TEF）大鼠模型，探讨胚胎发育中气管、食管分化异常的发生机制及可能的影响因素。方法明确孕龄的SD雌性孕鼠9只，随机分为正常组2只及模型组7只，模型组于E6～E9d每日腹腔注射2mg／kg阿霉素，正常组不注射。正常组于E11．5、E12．5d，模型组于E11．5、E12．5、E13．5d取材。进行HE及PAS染色，成纤维细胞生长因子7、10（fibroblast growth factor7，FGF7，10）免疫组化染色。留1只模型组孕鼠于E21d处死，体式镜下解剖。结果①正常对照组：HE染色可见到11．5d肺芽萌出，12．5d时可见气管、食管分离，在气管、食管分离处的前肠壁内有大量的凋亡小体。PAS染色及FGF7、10免疫组化染色可见前肠背侧半及食管为阴性，前肠腹侧半及气管部分为阳性；②模型组：HE染色11．5d未见明显肺芽萌出，12．5d可见瘘管与两侧支气管类似三分叉结构，在几乎相同水平发出。PAS染色及FGF7、10免疫组化染色可见瘘管组织阳性染色，近段前肠可见喉气管憩室以远的腹侧半为阳性。结论对正常大鼠及阿霉素致畸动物模型早期胚胎学的观察得出以下结论：①正常气管、食管的分隔可能与局部细胞的凋亡有关；②气管、食管的正常分隔可能为前肠正常发育的必要条件；③FGF7、10在模型组TEF中表达，表明可能存在间充质细胞的异常，产生异常的信号因子导致TEF。     

先天性食管闭锁术后食管气管瘘复发诊治进展

复发性食管气管瘘是先天性食管闭锁术后常见且严重的并发症。瘘管修补术后再次复发仍是临床及护理工作中的重大挑战。随着微创和内镜技术的不断发展,近十年来不断涌现出更加精巧的治疗方式。本文基于既往研究报道,就先天性食管闭锁术后食管气管瘘复发进行综述。     

先天性双气管食管瘘的诊治一例并文献复习

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...     

超声诊断先天性食管闭锁并气管食管瘘的初步探讨

目的探讨超声对先天性食管闭锁并气管食管瘘的诊断价值。方法对10例临床疑先天性食管闭锁并气管食管瘘的患儿进行超声检查并与食管造影及手术对照,总结阳性病例的超声声像特点及超声扫查方法。结果本组病例超声检查3例阳性,其中2例食管造影阳性,1例多次检查可疑阳性;2例手术证实（含造影可疑阳性者）。超声报告阴性者食管造影均显示阴性。结论超声对先天性食管闭锁并气管食管瘘的筛查和诊断有一定的价值,可作为一种新的检查方法,值得进一步的研究。     

先天性食管闭锁并气管食管瘘的大鼠胚胎学研究

目的 利用阿霉素诱导食管闭锁并气管食管瘘大鼠胚胎模型(adriamyein-induced rat model,ARM)与正常大鼠胚胎作比较,研究气管、食管的正常与异常分化过程.探讨产牛食管闭锁并气管食管瘘(esophageal atresia and traeheoesophageal fistula,EA-TEF)的可能影响因素.方法 SD孕鼠12只.随机分为模犁组8只及正常组4只.模型组于E6～9 d每Et腹腔注射阿霉素2 mg/kg.正常组不注射.于E10.5～13.5 d每天取2只模型组孕鼠及1只正常组孕鼠.剖腹取胚胎.逐个石蜡包埋,做前肠横切片,进行HE染色观察形态学、免疫荧光检测Shh表达.结果 ①形态学:模型组胚胎发育较正常组晚.细胞增生较少;多存在异常脊索与前肠背侧接近、相连或融合;②Shh表达:正常组E10.5 d表达最明显.随着胚胎天数增大.表达量渐少,呈下降性表达.模型组表达比止常组弱.无下降性表达.但在有脊索异常分支处表达明显.同时前肠表达明显.结论 EA-TEF的发牛可能与脊索的异常相关.脊索异常可能使Shh表达部位及水平异常,在EA-TEF的发生中发挥重要作用.     

先天性食管闭锁并气管食管瘘婴儿行结肠代食管手术一例

患儿：女，3d。因出生后唾液过多、口鼻溢奶并气急紫绀3d入院。体检：体重2．7kg，体温36．2℃，呼吸56次／分，心率160次／min。经胃管注射碘水X线摄片提示：先天性食管闭锁，食管盲袋底部位于第一胸椎水平以上。入院后立即给予补液支持及抗感染治疗，并用软导管间歇吸引食道盲袋、咽部和口腔，入院2d后行右侧剖胸探查 胃造瘘     

Extrapleural thoracoscopic repair of esophageal atresia with tracheoesophageal fistula

Traditionally, open repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) required thoracotomy. Innovations in minimal access surgery have created a thoracoscopic technique resulting in violation of the pleural space. Most pediatric surgeons favor an extrapleural approach for open repair. We present a novel minimal access, extrapleural technique for repairing EA with TEF. A 2-day-old infant with EA and distal TEF underwent thoracoscopic extrapleural repair that utilized three ports. Initial creation of the extrapleural space was achieved through one of the port sites and was completed thoracoscopically. A thoracoscopic repair of EA with distal TEF was achieved within the extrapleural space. A small tear in the pleura was inadvertently created during the dissection. The child began feeding normally. At 1 year of age, the patient had dysphagia requiring a single esophageal dilatation. This is the first known report of an extrapleural thoracoscopic repair of EA with TEF. Although thoracoscopic repairs of EA/TEF have been previously reported, these were all done transpleurally. Many pediatric surgeons favor the extrapleural approach for two reasons: (1) containment of a potential leak within the extrapleural space, avoiding an empyema, and (2) easier transpleural access for future thoracic procedures.     

Near-missed upper tracheoesophageal fistula in esophageal atresia

Upper pouch tracheoesophageal fistula (TEF) accompanying esophageal atresia (EA) occurs in less than 1% of all EA/TEF variants and could be easily missed after birth. To confront such diagnostic inaccuracy, perioperative tracheobronchoscopy (TBS) and preoperative upper pouch esophagogram (UPEG) have been proposed but are still controversial. We describe the role of UPEG and TBS, used early after birth, in two cases of EA/TEF with upper pouch TE fistulas with unusual high location (one intrathoracic, one subglotic). These upper TE fistulas were almost missed but ultimately detected very early while employing both UPEG and TBS, wherein UPEG was for the diagnosis of TEF and TBS for both intraoperative diagnostic confirmation and aid in TEF identification. We conclude that UPEG and TBS are complementary in detecting near-missed upper TE fistula accompanying EA. Such approach ensures early and accurate diagnosis of EA/TEF variants, thus preventing the complications of a missed congenital upper pouch TE fistula.     

A new variant of esophageal atresia with tracheoesophageal fistula and duodenal atresia: a diagnostic dilemma

A new variant of esophageal atresia (EA) with tracheoesophageal fistula (TEF) associated with duodenal atresia is reported. The TEF was between the lower pouch and the trachea, with a cystic dilatation in the midportion. The tracheal end of the fistula was obstructed by a membranous septum at both ends of a cystic dilatation, leading to a diagnosis of pure EA (gasless abdomen). After the lower pouch was opened beyond the cystic dilatation, 100 ml nonbilious fluid was obtained. A laparotomy revealed a type III atresia of the first part of the duodenum.     

Azygos vein preservation in primary repair of esophageal atresia with tracheoesophageal fistula

The aim of this study is to report a series of patients with the Azygos vein preserved during the surgery for esophageal atresia with tracheoesophageal fistula (EA&TEF), highlighting the advantages in terms of survival and prevention of anastomotic leak. Ninety-six neonates with EA&TEF, admitted to the Department of Pediatric Surgery, King George Medical University between 2004 and 2006, were reviewed prospectively; the babies were randomly allocated to two groups: Group A (n = 46) in which the Azygos vein was preserved and Group B (n = 50), wherein it was ligated. The two groups were comparable in respect to sex, weight, prematurity, associated anomalies, Waterston classification, Spitz classification and distance between the pouches after mobilization. Anastomotic leak occurred in three cases (6%) in Group A and ten cases (20%) in Group B and was responsible for mortality in one (2%) case in Group A and six cases (12%) in Group B. Preservation of Azygos vein resulted in significant reduction in the number of anastomotic leaks. We propose that preservation of the Azygos vein prevents early postoperative edema of the esophageal anastomosis by maintaining the venous drainage and thus may form an additional protective factor against anastomotic leaks.     

Esophageal atresia and tracheoesophageal fistula: a review of 25 years' experience

During a 25-year period (1972–1996), 585 patients with esophageal atresia with or without tracheoesophageal fistula were treated at the Department of Pediatric Surgery, SMS Medical College, Jaipur, India. Increasing awareness of the anomaly has led to early detection and referral with fewer pulmonary complications. For purposes of analysis the period has been divided into five phases, with a steady decline in overall mortality observed from 95.4% in phase 1 to 41% in phase V. Although postoperative complications have also shown a declining trend, delay in diagnosis, prematurity, low birth weight, delayed arrival at the surgical centers, sepsis, pulmonary complications including pnuemonitis, and inadequate nursing care all continue to contribute substantially to lower the survival in developing countries such as ours. Accepted: 27 September 1999     

先天性食管闭锁食管肌层超微结构及神经递质表达的研究

目的对先天性食管闭锁患儿食管肌层进行电镜观察和免疫组化检测,研究其超微结构变化及神经递质表达的特点。方法食管闭锁组:2003年6月~2004年6月间新生儿先天性食管闭锁伴食管气管瘘远端食管肌层组织10例;对照组:非食管疾病死亡新生儿食管中段组织10例。分别进行HE染色光镜观察、透射电镜观察和NSE、SP、VIP和NOS的免疫组化检测。结果光镜下,食管闭锁组肌间神经丛分布稀疏,4.2±0.6/10×视野,神经节细胞减少,细胞核偏位,深染。电镜下,食管闭锁组平滑肌纤维线粒体肿胀,内质网扩张。线粒体细胞膜边缘现象明显。在肌间神经丛突触末梢中,核心小泡/清亮小泡较对照组明显增高(0.511±0.139vs0.192±0.020,P<0.05)。免疫组化染色食管闭锁肌间神经丛及神经节细胞的NSE,SP表达阳性率分别为20%和10%,明显低于对照组(90%和80%,P<0.05);VIP和NOS表达阳性率分别为90%和90%,明显高于对照组(30%和10%,P<0.05)。结论食管闭锁患儿食管肌层存在内源性神经结构缺陷和神经递质表达异常。     

Congenital esophageal atresia with tracheoesophageal fistula occurring in both members of dizygotic twins

The authors present a pair of dizygotic twins with congenital esophageal atresia with tracheoesophageal fistula who underwent successful single-stage surgical repair. To our knowledge, this is the second set of dizygotic twins with this congenital anomaly in the literature. Accepted: 26 February 1997     

Elective, postoperative ventilation in the management of esophageal atresia and tracheoesophageal fistula

The management of esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) has undergone many changes. As a result of recent advances in neonatal intensive care and pediatric anesthesia, the survival of infants with EA and TEF has improved markedly, but the occurrence of anastomosic complications has remained constant. To overcome this problem, various techniques and suture materials have been used. This review of 20 consecutive cases of EA/TEF stresses the importance and influence of non-reversal of anesthesia, paralysis, and elective ventilation for protection of the esophageal anastomosis following repair of EA and TEF.