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病因的胚胎学探讨

先天性食管闭锁并气管食管瘘(esophageal atresia and tracheoeso phageal fistula，EA-TEF)是一种严重的新生儿消化道发育畸形，在活产儿中的发病率为1：3000～4000。近年来，随着阿霉素诱导EA-TEF动物模型的研制成功，使对于该畸形的胚胎学研究再一次成为热点。但是对于正常气管食管如何分隔、食管闭锁如何形成、远端食管的起源等问题各家莫衷一是，各自进行了一系列的研究，下面就这些问题作一简述。     

先天性食管闭锁伴气管瘘基础问题研究进展

先天性食管闭锁 (ｅｓｏｐｈａｇｅａｌａｔｒｅｓｉａ,ＥＡ)是一种严重的新生儿消化道发育畸形 ,发病率为 2 .2 /万～ 2 .8/万[1] ,常伴发气管食管瘘 (ｔｒａｃｈｅｏｅｓｏｐｈａｇｅａｌｆｉｓｔｕｌａ,ＴＥＦ)。近年来 ,随着阿霉素诱导ＥＡ ＴＥＦ动物模型的研制成功 ,作者围绕该畸形的胚胎形成及食管动力学功能异常等基础问题作一简述。一、ＥＡ ＴＥＦ动物模型的制作先天性食管闭锁伴气管食管瘘动物模型的制作始终是困扰医学界对该畸形的胚胎形成及手术前后各类并发症的病因学深入认识的障碍 ,国外曾经有人尝试通过给某一敏感株的大鼠…     

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。     

先天性食管闭锁和气管食管瘘疗效探讨

目的探讨先天性食管闭锁和气管食管瘘的治疗效果。方法对1994年1月-2006年12月治疗的107例先天性食管闭锁和气管食管瘘进行了回顾分析。结果成活85例,成活率为79.44%;放弃治疗13例,死亡9例,死亡率8.41%;术后发生吻合口瘘29例,吻合口狭窄4例。结论提高食管闭锁和气管食管瘘患儿成活率的关键是早期诊断,减少吻合口瘘的发生。     

胸腔镜与开胸手术治疗先天性食管闭锁并食管气管瘘的对比研究

目的 比较胸腔镜手术与开胸手术治疗先天性食管闭锁并食管气管瘘的疗效差异.方法 回顾性分析50例Ⅲ型先天性食管闭锁并食管气管瘘的临床资料,其中25例为江西省儿童医院2012年12月至2014年1月收治,采用胸腔镜治疗;另25例为首都儿科研究所2008年6月至2014年4月收治,采用开胸治疗.男27例,女23例,术前均明确诊断并完成手术治疗.胸腔镜组采用3孔法,经胸行食管气管瘘结扎,食管吻合术.开胸组采用经胸膜外完成手术.结果 胸腔镜组25例均一期完成食管吻合术,无中转,术后2例死亡.开胸组25例中23例完成经胸膜外食管吻合术,2例因食管盲端距离过长行食管气管瘘结扎,胃造瘘术,术后放弃治疗.手术时间胸腔镜组平均为127min(110～160min),开放组平均133min(105～170min);呼吸机使用时间胸腔镜组平均为2.6 d(1～13 d),开放组平均2.3 d(1～11 d),两组对比差异均无统计学意义.胸腔镜组25例中2例吻合口漏(8％),7例狭窄(28％),2例食管气食管瘘复发(8％).开放组25例中2例吻合口漏(8％),4例狭窄(16％),无食管气管瘘复发,两组比较差异均无统计学意义.结论 胸腔镜手术治疗先天性食管闭锁并食管气管瘘是安全可行的,但要求术者具备熟练的微创手术技术和相关的手术技巧.     

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     

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。     

先天性食管闭锁／食管气管瘘合并食管狭窄的治疗分析

目的探讨先天性食管狭窄并食管气管瘘／食管闭锁的临床特征、分型及治疗方法,以减少误诊误治。方法通过2例先天性食管狭窄并食管气管瘘／食管闭锁患儿的病例报告,分析影像学资料及临床分型、随访结果。结果1例患儿在食管闭锁手术中得到了确诊并切除了隔膜,术后接受了食管扩张术;1例患儿术后造影发现了食管狭窄,仅行保守治疗。2例患儿随访效果良好。结论食管闭锁手术中有必要对远端食管进行探查,排除合并食管狭窄畸形。     

食管闭锁术后食管气管瘘复发的治疗进展

食管闭锁是一种常见的先天性消化系统畸形,手术治疗是唯一有效的治疗方式.但术后常发生一系列并发症,其中以食管气管瘘复发最为棘手.目前食管气管瘘复发的治疗以外科手术为主,然后围手术期的管理及二次手术的困难一直是小儿外科医师所面临的一项挑战.基于近年来国内外对于食管闭锁术后食管气管瘘复发的研究,本文介绍了食管气管瘘复发的围手术期管理技巧,简述了在外科手术方面提高食管气管瘘复发治愈率的要点及进展.同时也回顾了近年来内镜下治疗食管气管瘘复发的各种方法,分析了其优缺点及适应证.指出目前外科治疗治愈率已明显提高,内镜治疗也日益完善,并对将来的治疗研究方向进行展望.     

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。     

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.     

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.     

Sonic hedgehog and Glis famliy expression in the human neonatal of congenital esophageal atresia and tracheoesophageal fistula

目的 研究Sonic hedgehog基因及Gli家族在人类先天性食管闭锁并气管食管瘘(esophageal atresia and tracheoesophageal fistula,EA-TEF)的表达特点,探讨EA-TEF病因及发病机制的可能影响因素.方法 食管吻合术中留取22例EA-TEF患儿近端食管盲端及远端气管食管瘘管组织,7例行HE染色,10例行real-time RT-PCR处理,5例行免疫荧光染色处理.观察食管盲端及气管食管瘘管形态上的变化及各指标的差异.结果 ①形态学:瘘管组织内皮下可见粘液腺体,肌层稀疏且肌肉组织结构紊乱;②Shh表达:食管盲端组织中可见表达,瘘管组织中未有表达;③Glis表达:Gli-1、Gli-3mRNA表达无差异,Gli-2mRNA表达差异有统计学意义(P＜0.05),瘘管中表达低于食管盲袋.结论 气管食管瘘组织具有气管源性特征.EA-TEF的发生可能与Shh信号通路表达下调有关.Gli-2的功能缺失在EA-TEF的发生中可能发挥重要作用.     

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.     

Routine gastrostomy is contraindicated in esophageal atresia

Between 1980 and 1993, 62 cases of esophageal atresia were treated by the authors with a survival rate of 81%. Of the 17 Waterston A patients, primary anastomosis was done in 12 cases. A simultaneous gastrostomy was performed in the first 2. Of the 15 Waterston B₁ and C₁ patients, 14 were treated primary anastomosis without gastronomy. Of the 30 Waterston B₂ and C₂ patients, a preliminary gastrostomy was done in 11 cases for severe pneumonitis or long-gap atresia. The remaining 19 cases were treated by primary anastomosis without gastrostomy. Among the 45 patients treated by primary anastomosis (2 with and 43 without a gastrostomy), there were 6 mortalities, with pneumonia being the cause of death in only 1. We conclude that gastrostomy is contraindicated when primary anastomosis is feasible; primary anastomosis can be done safely in low-birth-weight infants; and the preoperative pulmonary condition can frequently be improved without a decompression gastrostomy     

食管闭锁相关致病机制研究进展

食管闭锁是以食管的连续性中断为特点的先天性畸形,伴或不伴气管食管瘘,严重威胁新生儿的生命,是一种多因素参与的复杂疾病,其具体病因仍不明确,遗传机制复杂,本文将就其遗传相关致病因素进行综述,为进一步的研究提供参考.     

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