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Purpose: This study aims to evaluate different surgical approaches to long-gap esophageal atresia (LGEA) with or without tracheoesophageal fistula (TEF) is unclear. Methods: A systematic literature review was done comparing gastric transposition versus esophageal lengthening with delayed primary anastomosis in infants with LGEA+/-TEF. The primary outcome was time to full oral feeds. Secondary outcomes were time to full enteric feeds, need for further surgery, growth, mortality, and postoperative adverse events. Results: No comparative studies were found. However, the literature was re-interrogated for non-comparative studies. Four hundred thirty-eight articles were identified and screened, and 18 met the inclusion criteria. All were case series. Forty-three infants underwent gastric transposition, and 106 had esophageal lengthening with delayed primary anastomosis. One study on gastric transposition reported time to full oral feeds, and one study in each group reported growth. Time to full enteric feeds was reported in one study in each group. 30% of infants had further surgery following gastric transposition, including hiatus hernia repair (5/43, 12%) and esophageal dilation (7/43, 16%). Following esophageal lengthening, 62/106 (58%) had anti-reflux surgery, 58/106 (55%) esophageal dilatation and 11/106 (10%) esophageal stricture resection. Anastomotic complications occurred in 13/43 (30%), gastrointestinal in 16/43 (37%), respiratory in 17/43 (40%), and nerve injury in 2/43 (5%) of the gastric transposition group. In the esophageal lengthening group, anastomotic complications occurred in 68/106 (64%), gastrointestinal in 62/106 (58%), respiratory in 6/106 (6%), and none sustained nerve injury. Each group had one death due to a cause not directly related to the surgical procedure. Conclusions: This systematic review highlights the morbidity associated with both surgical procedures and the variety in reporting outcomes. [ABSTRACT FROM AUTHOR]

The value of laryngotracheobronchoscopy (LTB) immediately prior to repair of esophageal atresia with or without tracheo-esophageal fistula is contentious. Currently, there is a wide range of opinion on the utility of this investigation which is reflected by huge variation in clinical practice. This review is a critical analysis of the arguments for and against performing routine LTB prior to esophageal atresia repair. Reserving LTB for selected cases only is potentially disadvantageous since it limits the surgeon's and anesthetist's familiarity with the procedure, equipment, and range of potential findings. There is sufficient evidence to suggest that routine preoperative LTB becomes the standard of care. [ABSTRACT FROM AUTHOR]

The first thoracoscopic esophageal atresia with tracheo-esophageal fistula (EATEF) repair was performed in March of 2000. This report evaluates the results and evolution of the technique over the last decade. Thoracoscopic esophageal atresia repair has proven to be an effective and safe technique. Initial experience resulted in a higher stricture rate but this improved with experience and changes in technique over the last decade. The outcomes are similar to or superior to that of an open thoracotomy and avoid the musculoskeletal morbidity associated with that technique. [ABSTRACT FROM AUTHOR]

Purpose Esophageal atresia and tracheo-esophageal fistula (EA-TEF) result from abnormal division of the foregut into esophagus and trachea thus, it may influence airway branching and lung development. The present study examined lung morphogenesis in fetuses with EA-TEF focusing in the expression of FGF10 and its receptor FGFR2 IIIb. Methods Pregnant rats received either 1.75 mg/kg i.p. adriamycin or vehicle on E7, E8 and E9. Embryos were recovered at E15, E18 and E21 and lungs processed for immunohistochemistry and RT-PCR. Three groups were studied: control, adriamycin-exposed with EA-TEF, and adriamycin-exposed without EA-TEF. Comparisons were performed with Mann–Whitney or t tests (significance level, 5 %). Results Lung weight at E15 and E18 were significantly lower in adriaEA fetuses in which the relative mRNA levels of FGF10 were significantly higher. These differences disappeared near term. The receptor FGFR2 IIIb messenger was only significantly increased in adria noEA fetuses at E15. Immunohistochemical study was consistent with these findings. Conclusions Abnormal expression of FGF10 during earlier stages of development, when the lungs are smaller than controls, suggests a compensatory response aimed at ‘‘catching up’’ delayed tracheobronchial branching. Whether similar changes take place in the human condition and influence respiratory physiology remain to be determined. [ABSTRACT FROM AUTHOR]

Congenital esophageal stenosis (CES) is a rare congenital abnormality that is difficult to diagnose and often masquerades as other types of structural esophageal disease. We report three cases of CES with different presenting symptoms. We advocate for balloon dilation as the preferred first approach to therapeutic intervention. CES is an important clinical entity in the evaluation of pediatric esophageal disorders and should be suspected in young infants with dysphagia. [ABSTRACT FROM AUTHOR]

CHARGE association and DiGeorge syndrome (DGS) rarely occur together and only eight cases have been reported in the English literature. Two were associated with esophageal atresia (EA) and severe congenital heart anomalies. We report a third case of EA with tracheoesophageal fistula (EA-TEF) associated with coarctation of the aorta (CoA), CHARGE association, and DGS. The challenge for management in this complicated case is the background DGS which influences surgical outcome because of Ca++ imbalance and immune deficiency that can be life-threatening and require bone marrow transplantation. [ABSTRACT FROM AUTHOR]

Introduction: Initial management of wide-gap esophageal atresia and tracheo-esophageal fistula, especially in developing countries, often involves ligation of the fistula, proximal esophagostomy and a gastrostomy. The conventional gastrostomy requires an abdominal incision and has several complications. The authors present an alternative technique of 'gastrostomy' that does not require an abdominal incision.Method: Through a standard right thoracotomy, the fistula is divided and tracheal end closed. If primary anastomosis is not possible due to wide gap, the lower esophageal pouch is mobilised and brought out as a stoma on the left posterior chest wall, lateral to the paraspinal muscles in the 7th or 8th intercostal space. A feeding tube is passed through the stoma into the stomach. The thorax is closed with a drain. Proximal esophagostomy is performed in the neck. The thoracic stoma is intermittently intubated for feeding until the child is ready for gastric transposition.Results: The technique was successfully used in 5 patients. Feeding through the stoma could be established in 4. One patient had stomal retraction and died of resultant sepsis. One patient has subsequently undergone gastric transposition and one more is waiting for it. Two patients died of unrelated causes before esophageal replacement could be carried out.Discussion: This is an alternative technique for feeding 'gastrostomy'. We have named the procedure as "Thoracic Gastrostomy" because the stoma is located on the chest but functions as a gastrostomy. The advantages include avoidance of a laparotomy and its complications, easy feeding by intermittent intubation, and availability of a virgin stomach for future gastric transposition. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Subclavian artery puncture is an infrequent complication of subclavian vein cannulation. In spite of the limited ability to apply direct pressure to the bleeding vessel, significant hematoma or exsanguinating bleeding rarely occurs. This study was a retrospective chart review of a neonate who underwent extrapleural repair of a tracheo-esophageal fistula and sustained significant blood loss following an accidental ipsilateral subclavian artery puncture. This case highlights the importance of an intact apical pleura in controlling bleeding from the subclavian vessels. Thus, ipsilateral percutaneous subclavian venipuncture should be avoided in patients in whom the apical pleura is not intact. [ABSTRACT FROM AUTHOR]