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.     

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.     

Esophageal atresia with double tracheoesophageal fistula--a case report and review of the literature.

Esophageal atresia with double tracheoesophageal fistula (TEF) is a very rare anomaly, and the accurate preoperative diagnosis of proximal TEF is very difficult. This paper describes a baby girl who presented with esophageal atresia with double, proximal, and distal TEF. The distal TEF was diagnosed before operation, whereas the proximal TEF was found intraoperatively. Overlooking the presence of proximal TEF can lead to increased morbidity and mortality due to severe respiratory infection and the necessity of a second operation. Great care must therefore be taken to not overlook the presence of proximal TEF in patients with this anomaly.     

Recurrent tracheoesophageal fistula after foreign body impaction

We describe a case of recurrent tracheoesophageal fistula in a child previously operated for esophageal atresia and tracheoesophageal fistula following impaction of a foreign body. After extracting the foreign body, the diagnosis of recurrence was confirmed by esophagogram and bronchoscopy. The fistula was approached through transpleural thoracotomy and repair effected without significant difficulty. The possibility of unrecognized foreign body impactions as the etiological factor for late recurrence of tracheoesophageal fistula is discussed.     

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.     

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     

Congenital esophageal stenosis associated with esophageal atresia/tracheoesophageal fistula: clinical and radiologic features

Background  

Congenital esophageal stenosis (CES) can be associated with esophageal atresia/tracheoesophageal fistula (EA/TEF). Because there are a variety of degrees of obstruction and symptoms of CES, it is frequently difficult to make a pre- and post-operative diagnosis of the distal CES associated with EA/TEF.     

Bone morphogenetic protein expression patterns in human esophageal atresia with tracheoesophageal fistula

The organogenesis of esophageal atresia with tracheoesophageal fistula (EA/TEF) remains unknown. The fistula tract appears to develop from a non-branching trifurcation of the embryonic lung bud. The non-branching growth of the fistula differs from the other lung buds and suggests a deficiency in bone morphogenetic protein (BMP) signaling, since BMPs are critical to proper lung development and branching. With IRB approval, portions of newborn human proximal esophageal pouch and distal fistula samples were recovered at the time of surgical repair of EA/TEF. The tissues were processed for immunohistochemistry. Commercially available fetal tissues were used as controls. In control tissues, BMP ligands (BMP 2, 4, and 7) were all present in the esophagus but absent in the trachea. BMPRIA was absent in both tissues. BMPRIB was detected in trachea but not in esophagus and BMPRII was detected in esophagus but not in trachea. In the EA/TEF specimens, all BMP ligands were present in the proximal esophageal pouch but absent in the fistula tract. BMPRIA and BMPRIB were not detected in either tissue. However, BMPRII was found in both fistula tract and proximal pouch. The submucosa of the fistula appears to maintain a mixed (identical neither to lung, esophagus, or trachea) BMP signaling pattern, providing one mechanism which could potentially explain the esophageal dismotility and lack of lung branching seen in the fistula/distal esophagus.     

Combined free autologous auricular cartilage and fascia lata graft repair for a recurrent tracheoesophageal fistula

Pediatric Surgery International - Repair of recurrent tracheoesophageal fistula (TEF) after repair of congenital esophageal atresia continues to be a difficult problem. The most common re-operation...     

An uncommon association of H-type tracheoesophageal fistula with infantile hypertrophic pyloric stenosis

Although infantile hypertrophic pyloric stenosis following esophageal atresia repair is known, infantile hypertrophic pyloric stenosis following H-type tracheoesophageal fistula has not been encountered previously. A case of H-type tracheoesophageal fistula and infantile hypertrophic pyloric stenosis is presented. The patient, operated on for H-type fistula, a rare congenital anomaly of the esophagus, on the tenth day of life was readmitted 19 days later because of continuous vomiting after every feeding. The clinical findings and physical and radiological examinations revealed infantile hypertrophic pyloric stenosis which required surgical treatment. It is suggested that the association of H-type tracheoesophageal fistula with infantile hypertrophic pyloric stenosis is coincidental, given the estimated incidence of one in every 84,375,000 males and 337,500,000 females.     

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.     

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.     

Tracheal atresia, proximal esophageal atresia, and distal tracheoesophageal fistula: report of two cases and review of literature

Two cases with unusual primitive foregut anomalies are described. Both children, born within 24 hours of each other, had complete tracheal atresia, proximal esophageal atresia, and distal tracheoesophageal fistula along with some minor anomalies; both needed immediate tracheostomy for adequate ventilation. At 15 months of age, both patients are thriving and developing normally with the exception of their speech. A review of the literature revealed no other survivors with this condition. Diagnosis of tracheal atresia should be kept in mind when a newborn infant exhibits respiratory distress without an audible cry associated with failure to advance the endotracheal tube beyond the vocal cords. An immediate tracheostomy can be lifesaving in these children.     

Choanal atresia associated with tracheoesophageal fistula: the spectrum of carbimazole embryopathy

This article focuses on the case of a newborn infant boy with bilateral choanal atresia, tracheoesophageal fistula, and bilateral fifth-finger clinodactyly. This infant had been exposed to carbimazole in utero during the treatment of maternal Graves disease. Teratogenic defects caused by carbimazole were recently recognized, and their phenotypes have been defined. Choanal atresia, esophageal atresia, athelia or hypothelia, developmental delay, hearing loss, and dysmorphic facial features have all been reported. To our knowledge, this is the first documented case of tracheoesophageal fistula without esophageal atresia (H type). Knowledge of the teratogenic potential of carbimazole is important when managing Graves disease in women of childbearing age.     

Cardiovascular malformations associated with tracheoesophageal fistula and esophageal atresia.

Cardiovascular malformations were present in 48 of 326 (14.7%) patients with tracheoesophageal fistula. The most common cardiac lesion was ventricular septal defect. When tracheoesophageal fistula or esophageal atresia was associated with other gastrointestinal anomalies, tetralogy of Fallot and atrial septal defect were very frequent; when skeletal anomalies were present, the cardiac malformations were usually complex. The hospital mortality in infants with tracheoesophageal fistula and cardiovascular malformations is 79% in contrast to 23% for infants without cardiac abnormalities.     

Recurrent tracheoesophageal fistula after thoracoscopic repair: vanishing clips as a potential sign

We present a case of a neonate who underwent surgery for esophageal atresia (EA) with tracheoesophageal fistula (TEF) with an unusual finding on postoperative chest radiographs. In retrospect, this was a clue to a recurrent TEF: disappearance of the surgical clips from the site of surgical repair. Knowledge of this radiographic finding could aid in the diagnosis of a recurrent fistula in patients with previous repair of EA.     

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 and acquired tracheoesophageal fistulas in children

Tracheoesophageal fistulas (TEF) are an anomalous communication between airway and esophagus. There are several types of TEF. Congenital are mainly associated to an esophageal atresia. The type III or C, in which the upper segment of the esophagus ends in a blind pouch and there is distal tracheoesophageal fistula above the carina, accounts for 85% of esophageal atresias. The other are extremely infrequent. H-type or N-type TEF, classified as type 5 or E, is an uncommon variant and accounts for less than 4%.Recurrent TEF is a serious complication after first surgery of esophageal atresia and TEF. The rate of recurrence of TEF is estimated between 3-15%. The treatment is a challenge with a high rate of recurrence after surgery.Classical symptoms of RTEF include coughing especially after drinking, abdominal distension, repeated cyanosis, and respiratory infections. In the case of H-type fistula the symptoms are similar but appear during the first month of life.In this chapter we presented the management and alternative treatments of the congenital and acquired TEF.     

先天性食管闭锁及气管食管瘘11例

目的探讨先天性食管闭锁及气管食管瘘的早期诊断和治疗方法。方法先天性食管闭锁患儿11例均在入院24h内行食管泛影葡胺或碘化油造影确诊,并经胸膜外入路行食管吻合术,分析总结其诊断和治疗。结果治愈9例(81.8%),死亡2例(18.2%)。1例术后有食管吻合口狭窄,行食管扩张术后治愈;1例术后吻合口瘘,经抗感染、营养支持、充分引流后瘘口愈合。结论早期诊断、及时手术、术后护理及并发症的防治对提高先天性食管闭锁患儿的存活率有重要作用。     

Role of preoperative 3D CT reconstruction for evaluation of patients with esophageal atresia and tracheoesophageal fistula

Objective

The role of preoperative contrast-enhanced computerized tomography (CT) of chest with three-dimensional (3D) reconstructions was evaluated in neonates with esophageal atresia and tracheoesophageal fistula.

Methods

This was a prospective study which investigated 30 cases of esophageal atresia with tracheoesophageal fistula. All patients were evaluated preoperatively with contrast-enhanced spiral CT using a low-dose CT protocol. 3D CT reconstruction images were evaluated for the type of esophageal atresia, the distance between the upper and lower esophageal pouches, origin, level and position of the fistula, and the presence or absence of any other cardiac, pulmonary or mediastinal lesions and the findings were correlated with the findings at surgery. The radiation dose for each patient was calculated using the formula—Effective dose (E)?=?DLP?×?(E/DLP)age.

Results

All the 30 cases had type-C esophageal atresia with tracheoesophageal fistula as per Gross classification. The exact site of the fistula could be identified only in 26 (80?%) cases. The mean gap between the upper pouch and lower fistula was 0.95?±?0.57?cm (range 0.2–2.8?cm) on CT scan and 1.38?±?0.61?cm (range 0.5–3.2?cm) at surgery. On statistical analysis, the correlation was found to be significant (p?Conclusion Though preoperative CT scan of chest has many advantages, it involves significant exposure to ionizing radiation and risk of radiation-induced cancer in the future. Additionally in 20?% of cases, the fistula could not be located on CT scan. The most common variety of esophageal atresia and tracheoesophageal fistula is Gross type C (86?%) that has low to intermediate gap (97?%) and can be anastomosed primarily. Thus, CT scan can provide good anatomical delineation, but may not help in surgical decision making. Hence, performing CT in these cases would unnecessarily expose the neonates to ionizing radiation. Therefore, there is no role for CT scan in the routine preoperative assessment of EA with distal TEF.