The spectrum of bronchopulmonary foregut malformations

Bronchogenic cysts and pulmonary sequestrations have in the past been defined as separate congenital pathological entities. The association of both in one mediastinal lesion is very rare. Such a case is reported, and lends support to the concept that bronchogenic cysts and pulmonary sequestrations belong in a spectrum of bronchopulmonary malformations due to abnormal ventral budding of the primitive foregut. Their exact position in the spectrum is probably dependent upon several independent key embryological events. Offprint requests to: M. Kent     

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.     

Duodenal duplication cyst associated with duodenal atresia

A 2-day-old premature infant with a duodenal duplication cyst situated between the two blind ends of a duodenal atresia is presented. This entity has not been reported previously in the literature. This rare association of a duplication cyst and atresia may occur when recanalization is interrupted, with failure to form a single duodenal lumen.     

Gastric duplication cyst as a differential for an intra-thoracic cystic mass

We report a case of a neonate who presented with respiratory distress initially managed for a suspected pneumothorax before being transferred to a tertiary centre where he had a thoracotomy. A large cystic structure was excised later histologically confirmed to be a gastric duplication cyst. We discuss its management.     

Esophageal atresia with proximal pouch tracheoesophageal fistula and noncommunicating esophageal duplication

This report describes a case of esophageal atresia with proximal-pouch tracheoesophageal fistula and a noncommunicating esophageal duplication. Until now this variation has not appeared in the literature.     

VATER syndrome and esophageal foregut duplication

A case of incomplete VATER syndrome (esophageal atresia, anorectal malformation, vertebral anomalies, horseshoe kidney) associated with a foregut duplication of the esophagus is described. A review of the literature is presented.     

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.     

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.     

Oesophageal duplication cyst: Another cause of neonatal respiratory distress

Oesophageal duplication cysts in infants (usually males) should be recognized as a cause of respiratory distress which worsens during the neonatal period, and which is associated with feeding difficulties. Their radiological appearance is that of a right-sided posterior mediastinal mass, which is shown to be cystic on ultrasound. Excision is curative.     

Congenital esophageal stenosis associated with esophageal atresia: new concepts

Congenital esophageal stenosis (CES) is suspected by a fixed intrinsic narrowing of the esophagus that affects the normal swallowing mechanism. The diagnosis is only confirmed by histopathologic picture, which may show fibromuscular disease (FMD) or tracheobronchial remnants (TBR). The latter involves ciliated pseudo stratified columnar epithelium, seromucous glands or cartilage each alone or in combination. The aim of this study is to document the usefulness of histologic picture of surgical specimens obtained from the lower esophageal pouch (LEP) during primary repair in detecting cases of CES associated with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF). Over an 8-year period, 57 consecutive cases operated upon for EA with or without TEF were subjected for histologic examination of surgical specimens obtained from the tip of the LEP. Cases that histologically showed FMD or TBR were included. The usefulness of this histologic picture as a diagnostic and therapeutic aid is assessed. Methods of treatment and outcome were also reviewed. Eight patients out of 57 (14%) had a histologic picture suggestive of CES, two with FMD, four with TBR without cartilage and two with cartilage. Out of 57 patients, 23 developed strictures, six of them had positive biopsies suggestive of CES. One patient with TBR without cartilage did not have stricture. Another case of pure atresia had LEP resection and gastric pull up showed cartilage involving the whole lower esophagus. Excluding the case of pure EA with gastric pull up, all patients suffered from feeding problems and recurrent aspiration. Fluoroscopic barium studies showed late onset minor dysmotility in five patients and late onset major dysmotility in two. All cases studied showed significant gastro-esophageal reflux (GER). Stricture was seen at the anastomotic site extending distally in the two fibromuscular cases and one case with cartilage, at the anastomotic site in three cases with TBR without cartilage. Anti reflux surgical procedures were performed in four patients without benefit in two patients with major dysmotility. Dilatation was successful in the three patients with TBR without cartilage. One patient with cartilage had resection of the anastomotic site and required frequent dilatations and is now doing well. A case of FMD did not improve after frequent dilatations and myotomy together with Nissen’s fundoplication and required resection while the other case of FMD responded partially to dilatations. Cartilage in cases of CES requires surgical resection. Those with TBR without cartilage may not develop stricture. If stricture develops, it responds well to dilatation and patients have good clinical outcomes. Unlike isolated CES, GER is a significant feature in CES with EA. Anti reflux procedures should be avoided before definitive surgery for the stricture and if necessary a partial wrap with gastrostomy is recommended. CES should be considered in the etiology of anastomotic stricture. Taking a surgical specimen routinely from the tip of the LEP during primary esophageal repair for histologic studies is highly recommended.     

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.     

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.     

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     

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.     

Respiratory distress due to a posterior mediastinal mass following failed repair of esophageal atresia

Respiratory distress due to masses in the posterior mediastinum have been extensively described, including duplication cysts of the esophagus. Retained esophagus following failed repair of esophageal atresia (EA) and esophageal replacement was described only once. Double atresia and midportion esophageal cyst is also a rare entity. We describe a baby with respiratory distress following esophageal replacement for failed repair of EA with tracheoesophageal fistula who was cured by the removal of an overlooked mediastinal cystic mass of esophageal origin at the age of 16 months.     

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     

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     

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.