Congenital esophageal stenosis associated with tracheoesophageal fistula

Two patients with congenital esophageal stenosis associated with an H-type tracheoesophageal fistula in one and esophageal atresia with distal fistula in the other are presented. In both patients the correct preoperative diagnosis was made by esophagram. Satisfactory results were obtained with surgical excision. Histologic studies revealed fibromuscular stenosis and membranous esophageal mucosa, respectively.     

Intramural tracheobronchial remnants associated with esophageal atresia: diagnostic aids

A patient with isolated esophageal atresia and congenital esophageal stenosis resulting from intramural tracheobronchial remnants (TBR) in the distal esophagus is presented. The esophagographic appearance and operative findings suggesting the presence of TBR are discussed.     

Congenital cystic adenomatoid malformation of the lung associated with esophageal atresia and tracheoesophageal fistula

Bronchopulmonary malformations associated with esophageal atresia (EA) and tracheoesophageal fistula (TEF) are extremely rare. The authors describe a case of type II congenital cystic adenomatoid malformation (CCAM) of the right lower lobe associated with EA and TEF (Vogt-Gross type C) in a full-term female infant. The CCAM presented as an incidental radiologic finding, and a contralateral tension pneumothorax developed shortly after surgical repair of the EA. Early recognition of this rare association is essential for correct operative management. Accepted: 26 May 1998     

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.     

Congenital diaphragmatic hernia associated with esophageal atresia, tracheoesophageal fistula, and truncus arteriosus in a premature newborn

The occurrence of coexisting congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with distal tracheoesophageal fistula (TEF) is extremely rare and is considered highly lethal. The combination of CDH with EA/TEF and truncus arteriosus communis (TAC) has not been reported in the literature to date. The authors describe a premature neonate with this association.     

Concurrent right diaphragmatic hernia and esophageal atresia

The occurrence of a coexisting congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) with distal tracheoesophageal fistula is extremely rare and is considered highly lethal. Only 19 cases of CDH with EA have been reported in the world literature to date. This is a very challenging clinical problem, and the neonate is likely to deteriorate rapidly. Such a case is reported with a successful outcome, probably the first survivor with a right CDH and EA. Management guidelines for such a case are discussed. Accepted: 8 October 1997     

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.     

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     

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     

Bilateral bronchopulmonary foregut malformation associated with esophageal atresia

A case of esophageal atresia associated with bilateral accessory lung tissue communicating with a short lower double esophagus opening into trachea at the level of the carina is presented. The infant also had a right-sided aorta, a hiatal herniation of the stomach, and possibly situs inversus abdominis. Such a complex, hitherto undescribed anomaly is likely to add to our understanding of the embryogenesis of bronchopulmonary foregut malformations.     

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.     

Coexisting left diaphragmatic hernia and esophageal atresia

Coexisting left diaphragmatic hernia (DH) and esophageal atresia, a rare phenomenon, is described in two newborns, one with tracheoesophageal fistula (TEF) and the other without. The coexistence of DH and TEF in the one resulted in uncontrolled gastrointestinal distention with severe respiratory distress. Transabdominal division of the TEF was feasible and was performed concurrently with the diaphragmatic repair. Severe hypoplastic lungs and persistent pulmonary hypertension resulted in early postoperative death in both cases. Offprint requests to: R. Udassin     

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.     

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.     

Postoperative morbidity in patients with esophageal atresia

A retrospective study was carried out in 110 patients with esophageal atresia seen at the Sophia Children's Hospital, Rotterdam, from 1975 to 1984. Special attention was paid to post-operative morbidity in view of the lack of detailed information concerning this aspect in the literature. Of the 87 surviving patients, 77 had a primary end-to-end anastomosis. The experience with these 77 patients is reviewed and discussed with reference to the literature. Gastroesophageal reflux seems to be the major postoperative complication and a contributing factor to other serious conditions. Early detection and treatment of gastroesophageal reflux may prevent some of these conditions. Offprint requests to: J. C. Molenaar     

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.     

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。     

Visualization of the intestinal tract by amniography in a fetus with esophageal atresia

In pregnancies complicated by hydramnios, amniography can be used for the detection of fetal gastrointestinal obstruction. A case is presented in which the appearance of contrast material in the fetal gastrointestinal tract was noted in an infant who proved to have esophageal atresia and tracheoesophageal fistula. Presumably, the contrast reached the intestinal tract by aspiration and passage through the fistula.     

Congenital esophageal stenosis

A series of 20 patients with congenital esophageal stenoses (CES), including 4 with tracheobronchial remnants, 6 with membranous diaphragm, and 10 with fibromuscular stenosis, is presented. The experience acquired by treating these patients is compared to 95 cases from the literature and a definition of CES is proposed. The 35% association of CES woth other anomalies, especially esophageal atresia, is analyzed and some difficulties in the treatment of this rare anomaly are discussed. Offprint requests to: C. Nihoul-Fékété     

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