Review of surgical and anesthetic management for esophageal atresia with tracheoesophageal fistula,unilateral pulmonary agenesis and dextrocardia

Association of unilateral severe pulmonary atresia or agenesis and esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) and dextrocardia is a rare and highly lethal combination. We report a case of a full-term female infant who had EA with TEF, right lung agenesis, and dextrocardia. Repair of the fistula took place on day of life 3. We describe anesthetic and surgical concerns of this patient’s case which are keys to a good operative outcome as well as follow-up for the patient.     

Preoperative intubation of the distal tracheoesophageal fistula for gastric decompression in patients with esophageal atresia

In patients with esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) Gross type C and D, escape of ventilatory gases through the TEF exposes the stomach to gaseous distension with the risk of respiratory distress and gastric perforation. In seven patients with EA, Gross type C, primary esophagoesophagostomy was planned within the first 2 days of life. After induction of anesthesia by mask, a rigid ventilation bronchoscope was inserted into the trachea and the TEF was cannulated with a catheter that was pushed down to the stomach. After removal of the endoscope, the patient was intubated beside or over the inserted catheter. Until division and closure of the TEF, continuous suction over the catheter prevented gaseous gastric distention. Intubation of a distal TEF in patients with Gross type C and D EA can easily be combined with routine preoperative bronchoscopy, and is a safe and effective method to decompress a distended stomach and prevent respiratory distress and gastric perforation during controlled ventilation.     

Long-term survival in a patient with congenital laryngeal atresia and multiple malformations

Laryngeal atresia (LA) is an uncommon congenital anomaly, and only a few cases with long-term survival are reported in the literature. The authors describe a 2-year-old boy with LA and esophageal (EA), intestinal, and urethral atresia (UA). Immediately after birth, severe respiratory distress and mild abdominal distension became evident. Endotracheal intubation was unsuccessful and emergency tracheal puncture was performed, after which a tracheostomy was constructed. Direct laryngoscopy revealed LA at the vocal cord level. A cystostomy and gastrostomy were constructed immediately because of UA and EA. A cystocutaneostomy, ileocolic anastomosis, and resection of a tracheoesophageal fistula (TEF) were simultaneously performed 1 day after birth. The EA was proximal with a distal TEF. The gap between the ends of the upper and lower esophagus was 4 cm in length. It was thought impossible to perform a primary anastomosis, and therefore, a gastrostomy and resection of the TEF using multiple-stage surgery was undertaken. Intestinal resection and anastomosis were performed due to intestinal stenosis from necrotizing enterocolitis at the age of 3 months. Hypoxic encephalopathy developed due to accidental obstruction of the tracheostomy tube at the age of 10 months, and physical therapy was begun. He required a cutaneous nephrostomy due to a right hydromegaureter with vesicoureteric reflux and a left non-functioning kidney at the age of 23 months. He has been hospitalized for partial ventilatory assistance for 2 years at our institution. The course of this patient seems noteworthy in relation to the genesis of the multiple malformations. Accepted: 1 May 1997     

Treatment Strategy and Long-Term Prognosis for Patients With Esophageal Atresia and Congenital Heart Diseases

A review examined six consecutive cases of patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) who underwent cardiac surgery at the authors’ institution between 2001 and 2011 for associated complex congenital heart diseases. All the patients had a normal karyotype and showed EA with distal TEF. In all cases, gastrostomy was the initial surgical intervention. Cardiac surgery was performed concurrently with gastrostomy for one patient who had a total anomalous pulmonary venous connection with pulmonary venous obstruction. For two patients with duct-dependent pulmonary circulation, EA/TEF was corrected in the neonatal period, and an aortopulmonary shunt operation was electively performed after the first month of life. For two patients with duct-dependent systemic circulation, repair of EA/TEF was performed concurrently with gastrostomy, followed by palliative cardiac surgery during the neonatal period. For another patient without duct-dependent circulation, repair of EA/TEF was performed in the neonatal period. No mortality occurred during a median follow-up period of 6.2 years. However, respiratory complications including severe tracheomalacia for two patients, recurrent episodes of respiratory infection for three patients, and severe gastroesophageal reflux for five patients caused considerable long-term morbidity.     

Is ligation of azygos vein necessary in primary repair of tracheoesophageal fistula with esophageal atresia?

INTRODUCTION: Congenital tracheoesophageal fistula with esophageal atresia (TEF with EA) is not an uncommon disease of newborns. Classical approach for primary repair of TEF with EA is right thoracotomy with extrapleural approach, ligation of the azygos vein, identification and ligation of tracheoesophageal fistula, identification of upper esophageal pouch and end-to-end anastomosis. This study was conducted to evaluate if the ligation of the azygos vein is a must during primary repair of TEF with EA. METHOD: We studied 50 randomly selected cases in the last two years (Jan 2003 - Jan 2005). In 25 babies (group A), primary repair was done with preservation of the azygous vein while 25 babies (group B) were operated with a classical approach with ligation and division of the azygos vein. The postoperative result was comparative in both cases. RESULT: Postoperative pneumonitis was higher in the babies operated with the classical approach (56 % in group B and 12 % in group A) and was found to be statistically significant. In postoperative complication, anastomotic leak was slightly higher in group B but was not statistically significant. Mortality rate was similar in both groups. Babies in group A were hemodynamically more stable in comparison to group B. CONCLUSION: Preservation of the azygos vein maintains the normal venous drainage of mediastinum and hence decreases the postoperative chest congestion and pneumonitis in the postoperative period in cases of congenital esophageal atresia with tracheoesophageal fistula, so it should be preserved whenever possible.     

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.     

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.     

The right-sided aortic arch in children with esophageal atresia and tracheo-esophageal fistula: a repair through the right thoracotomy

Purpose  The management of the esophageal atresia and tracheo-esophageal fistula (EA/TEF) with right-sided aortic arch (RAA) is controversial. The preoperative diagnostic techniques may fail to show RAA associated with EA/TEF. Surgeon may need to make a decision to change the side of thoracotomy. The aim of the current study was to evaluate the possibility of preoperative diagnosis of RAA and the primary anastomosis through right chest. Methods  A retrospective review was performed in EA/TEF patients between February 2001 and 2008. A total of 79 patients (35 female, 44 male) with EA/TEF were reviewed. Eleven (13%) patients (5 female, 6 male) had an RAA. Echocardiography was performed in 10 of 11 patients with RAA. The chest was accessed through the right side in all patients. Results  The incidence of RAA was found to be higher in our study than previous studies (13%). Right thoracotomy was performed successfully in all patients. Three patients died due to multiple congenital anomalies and 1 patient due to bleeding postoperatively. Five of 10 had normal echocardiography findings. Only one patient with RAA has been successfully diagnosed by preoperative echocardiographic examination. Seven patients had no complication after operation. Their follow-up was uneventful. Conclusion  Preoperative recognition of RAA with echocardiography is unlikely in patients with EA/TEF but the presence of RAA does not decrease the success rate of EA/TEF repair through the right thoracotomy.     

Esophageal atresia with distal tracheoesophageal fistula associated with situs inversus

The presence of situs inversus (SI) causes difficulty in the repair of esophageal atresia with tracheoesophageal fistula (EA/TEF). We report two cases: the first infant had situs SI abdominis with a right-sided aortic arch and the second had situs inversus totalis. To our knowledge, these are the first reported cases of repair of EA/TEF in a patient with SI. Accepted: 27 June 2000     

Modified method of primary esophageal anastomosis with improved outcome in cases of esophageal atresia with tracheoesophageal fistula

Survival rates for infants who have esophageal atresia (EA) with or without fistula (TEF) have improved dramatically in the past 50 years. Despite excellent long-term survival for patients with esophageal atresia with tracheoesophageal fistula (EA-TEF), many significant complications can occur. Anastomotic leak at the esophagoesophagostomy site is one such problem resulting in considerable morbidity and mortality in these patients. The methods of esophageal anastomosis for long period has remained the simple end to end anastomosis of esophageal ends with various modifications described from time to time. The present study aims to study the effect on the early postoperative complications, following horizontal mattress suture technique on the primary esophageal anastomosis in cases of EA-TEF. A total of 32 patients with EA-TEF, were operated by our technique during a period of 1 year (2007–2008). The results were compared with the patients (n = 66), who were operated by the traditional simple technique during the same period. Among those patients in whom the esophageal anastomosis was done by horizontal mattress suture, only one had major anastomotic leak, while two had minor anastomotic leaks, as compared to six and nine cases correspondingly in other patients in whom anastomosis was done by simple technique. There was single mortality. We propose that, the utilization of our technique of horizontal mattress suture in primary anastomosis of esophagus in cases of EA-TEF significantly reduces the risk of anastomotic leaks and subsequent morbidity and mortality.     

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     

Cineradiographic evaluation of postoperative patients with esophageal atresia and tracheoesophageal fistula

 Esophageal dysmotility is a common occurrence after successful repair of esophageal atresia (EA) and tracheoesophageal fistula (TEF). The etiology of this motility disorder, however, remains controversial. Cine-barium esophagograms were performed in 25 survivors of EA/TEF at a mean age of 30.5 months. A clinical evaluation was done at the same time and the results were classified as “excellent”, “good”, and “fair”. The study revealed that 88% of patients had dysmotile esophageal segments and segmental contractions. Normal peristalsis was seen in only 16% of patients and 64% showed to-and-fro movements. The bolus transit time was delayed in 60%. The clinical status of these patients correlated well with the cine-radiographic findings. Thus, abnormal motility of the esophagus, which may be inherent, exists in postoperative survivors of EA/TEF and may be responsible for the clinical status of these patients. Accepted: 8 November 1999     

先天性食管闭锁伴气管食管瘘大鼠模型研制及其病理组化研究

目的：通过对怀孕大鼠腹腔注射阿霉素，制成食管闭锁及气管食管瘘胚胎大鼠模型，并就其适用性及解剖学特点进行了探讨。方法：在Wister大鼠怀孕第8，9两天给予腹腔注射阿霉素1．75mg/kg，对照组在相应孕期内注射相同剂量生理盐水，纪录孕期大鼠体重波动，第21d剖腹取出胎鼠，体式显微镜下显微解剖确定是否存在食管闭锁及气管食管瘘，并检视其他系统脏器的发育情况，测量腹段食管长度，取出气管叉水平下段食管作组织病理检查。结果：显微解剖发现其腹段食管较短，提示EA－TEF可能存在抗反流屏障的缺陷；HE及VG染色显示其远端食管有气管化倾向，粘膜呈假复层纤毛柱状上皮，肌层稀疏，排列紊乱，胶原显微增生，透射电镜显示近膈肌端食管环肌纤维中线粒体数量减少，对肌纤维的伸缩支持功能下降，提示EA－TEF患儿食管壁结构可能存在先天性的异常，从而影响了食管正常的动力学功能；一氧化氮合成酶组成化学染色提示病变段食管壁中NOS分布密度增强，可能影响了正常的平滑肌舒张及食管蠕动功能，神经细胞粘附分子（NCAM）免疫组织化学染色显示食管壁中NCAM呈高表达状态，提示EA－TEF食管壁内神经肌肉接头呈未成熟状态，影响了正常的神经－肌肉联系，导致食管动力学功能异常结论：EA－TEF胎鼠的食管壁发育不成熟，且近膈肌端食管壁环肌纤维中线粒体数目减少并远离中心，可能是引起食管动力学功能异常的根本原因。     

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.     

Evaluation of neonates with esophageal atresia using chest CT scan

Recent literature stresses various factors that influence outcome in neonates with esophageal atresia (EA), including associated congenital anomalies, ventilator dependency, and gap length. Although the existing modalities can fairly accurately assess the first two factors, a comparable modality for assessing the interpouch gap in a natural unstretched position preoperatively is lacking. A technique of direct sagittal computed tomography (CT) scanning of the chest, initially employed by Tam et al. in 1987, was found to provide fairly accurate information on the level of origin of the fistula and the interpouch distance, with pouches in natural, unstretched positions, in two neonates with EA and distal tracheoesophageal fistula (TEF). However, no further studies were reported using this technique. We studied 10 neonates with EA and distal TEF, the most common variant, using this technique. The addition of axial cuts was done in the event of a short upper pouch or suboptimally-clear anatomical details in sagittal chest CT films. The preoperative information in our study subjects was found to corroborate well with operative findings except in the one patient with laryngotracheoesophageal cleft (LTEC). Prior information of a left bronchoesophageal fistula in two of the neonates with dextrocardia greatly helped us in saving operative time and difficulties and postoperative hassles. The chest CT scan proved to be a noninvasive and quick means for investigating neonates with the most common variant of EA. CT scanning could be extended for routine preoperative evaluation of neonates with EA to prepare for intraoperative problems, besides helping prognostication.     

A chest tube may not be needed after surgical repair of esophageal atresia and tracheoesophageal fistula

Background

After definitive repair of esophageal atresia (EA) and tracheoesophageal fistula (TEF), a chest tube (CT) may be left in place to diagnose and conservatively treat a postoperative anastomotic leak, although its necessity is controversial. The purpose of this study was to determine if the use of a CT decreases rates of early postoperative complications after EA and TEF repair.

Methods

A retrospective chart review was performed to identify all patients with EA and TEF who underwent repair between 1985 and 2012. Univariate analysis was performed to compare patients who did or did not have a CT in terms of preoperative characteristics and postoperative outcomes.

Results

One hundred twenty neonates were included; 69 had a CT inserted intraoperatively, while 51 did not. The two groups were similar in terms of patient characteristics. There was no statistically significant difference between the groups in terms of length of hospital stay (31?±?12 vs. 36?±?16 days, p?=?0.5), or complication rates (13% vs. 12%, p?=?0.9) for those with or without CT, respectively.

Conclusions

The use of a CT does not alter early postoperative complications after EA/TEF repair.

     

Search for the azygos: a lesson learnt from a case with left superior vena cava, esophageal atresia and tracheo-esophageal fistula

The azygos vein is of superior importance during the operation for esophageal atresia with tracheo-esophageal fistula (EA; TEF). Its location helps the surgeon to locate the fistula. Newborns with persistent left superior vena cava (LSVC) may have alterations in the location of the azygos, and since LSVC is not uncommon in EAs with fistulae, it is important to locate the azygos during a pre-operative echocardiogram. Foreknowledge of a possible absent azygos may avoid morbidity while trying to locate the fistula. We present a case of a newborn with EA, TEF, and LSVC in which the azygos vein was absent.     

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.     

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.     

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.