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1.  Passage of nasogastric tube through tracheo-esophageal fistula into stomach: A rare event 
Esophageal atresia with tracheo-oesophageal fistula (TEF) occurs in 1 in 3500 live births. Anorectal malformation is found to be associated with 14% of TEF. Esophageal atresia with TEF is a congenital anomaly which classically presents as excessive frothing from the mouth and respiratory distress. Rarely gastric position of the feeding tube in a case of TEF can be obtained delaying the diagnosis of TEF. We had an uncommon situation where a nasogastric tube reached the stomach through the trachea and tracheo-esophageal fistula, leading to misdiagnosis in a case of esophageal atresia with tracheoesophageal fistula. By using a stiff rubber catheter instead of a soft feeding tube for the diagnosis of esophageal atresia and TEF, such situation can be avoided.
PMCID: PMC4097162  PMID: 25032210
Esophageal atresia; Tracheoesophageal fistula; Nasogastric tube; Red rubber catheter; Misdiagnosis
2.  Early presentation of postintubation tracheoesophageal fistula: Perioperative anesthetic management 
Tracheoesophageal fistula (TEF) in adults occurs as a result of trauma, malignancy, cuff-induced tracheal necrosis from prolonged mechanical ventilation, traumatic endotracheal intubation, foreign body ingestion, prolonged presence of rigid nasogastric tube, and surgical complication. Anesthetic management for repair of TEF is a challenge. Challenges include difficulties in oxygenation or ventilation resulting from placement of endotracheal tube in or above the fistula; large fistula defect causing loss of tidal volume with subsequent gastric dilatation, atelactasis, and maintenance of one lung ventilation. The most common cause of acquired nonmalignant TEF is postintubation fistula, which develops after prolonged intubation for ventilatory support. Acquired TEF, which occurs after prolonged intubation, usually develops after 12–200 days of mechanical ventilation, with a mean of 42 days. We present a rare case of TEF that developed after 7 days of intubation. It was a difficult case to be diagnosed as patient had a history of polytrauma, followed by emergency intubation and both these conditions can contribute to tracheobronchial injury.
PMCID: PMC3275943  PMID: 22345958
Mechanical ventilation; postoperative; tracheosophageal fistula
3.  Management of patients with combined tracheoesophageal fistula, esophageal atresia, and duodenal atresia 
Patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia (DA) pose a rare management challenge.
Three patients with combined esophageal atresia (EA), tracheoesophageal fistula (TEF), and duodenal atresia safely underwent a staged approach inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week. None of the patients suffered significant pre- or post-operative complications and our follow-up data (between 12 and 24 months) suggest that all patients eventually outgrow their reflux and respiratory symptoms.
While some authors support repair of all defects in one surgery, we recommend a staged approach. A gastrostomy tube is placed first for gastric decompression before TEF ligation and EA repair can be safely undertaken. The repair of the DA can then be performed within 3–7 days under controlled circumstances.
A staged approach of inserting a gastrostomy tube and repairing the EA/TEF first followed by a duodenoduodenostomy within one week resulted in excellent outcomes.
PMCID: PMC4275966  PMID: 25460495
Esophageal atresia; Tracheo-esophageal fistula; Duodenal atresia; Surgical management
4.  Management of a congenital tracheoesophageal fistula in a young Spanish water dog 
Tracheoesophageal fistula (TEF) in dogs is a rare disease with only few reports in the literature. This report aims to contribute to the current literature on suitable diagnostic methods for TEF and to provide follow-up information after successful surgical treatment.
Case presentation
A seven-month-old intact female Spanish Water Dog was presented for further investigation of recurrent respiratory symptom. Bronchoscopy revealed a small hole-like defect in the tracheal wall at the bifurcation. The finding of the contrast material swallow study under fluoroscopy was indicative of a TEF. To further evaluate the connection between the trachea and esophagus, a computed tomography scan was performed. The TEF was surgically approached by thoracotomy through the right lateral sixth intercostal space. The fistula was identified, double ligated and divided. Histopathology confirmed the process to originate from the esophagus and to be patent. The dog was re-examined two weeks and ten months after surgery, with no evidence of recurring clinical signs.
Contrast material swallow study using fluoroscopy was the most reliable diagnostic method. Bronchoscopy may allow the fistula to be visualized, but due to a small fistular opening it can lead to a false negative result. Surgical correction by ligation and dividing of the fistula suggests a good prognosis for early diagnosed and operated TEF.
PMCID: PMC3895692  PMID: 24423070
Congenital tracheoesophageal fistula; Dog; Bronchoscopy; Fluoroscopy; Computed tomography; Histopathology
5.  Isolated congenital tracheal stenosis in a preterm newborn 
European Journal of Pediatrics  2011;170(9):1217-1221.
Severe tracheal stenosis, resulting in functional atresia of the trachea is a rare congenital malformation with an estimated occurrence of two in 100,000 newborns. If no esophagotracheal fistula is present to allow for spontaneous breathing, this condition is usually fatal. We report on a male infant born at 32 weeks of gestation. The patient presented with respiratory distress immediately after delivery due to severe congenital tracheal stenosis resulting in functional atresia of the trachea. Endotracheal intubation failed and even emergency tracheotomy did not allow ventilation of the patient lungs. The patient finally succumbed to prolonged hypoxia due to functional tracheal atresia. The etiology of tracheal atresia and tracheal stenosis is still unclear, but both conditions are frequently combined with other anomalies of the VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal/radial anomalies and limb defects) and TACRD (tracheal agenesis, cardiac, renal and duodenal malformations) association. Conclusion Successful treatment of severe congenital tracheal stenosis and tracheal atresia depends on either prenatal diagnosis or recognition of this condition immediately after birth to perform tracheotomy without delay. Nevertheless, despite any efforts, the therapeutical results of severe tracheal stenosis and tracheal atresia are still unsatisfactory.
PMCID: PMC3158335  PMID: 21590265
Congenital tracheal stenosis; Neonate; Respiratory failure; VACTERL association
6.  Endoscopic Stenting and Clipping for Anastomotic Stricture and Persistent Tracheoesophageal Fistula after Surgical Repair of Esophageal Atresia in an Infant 
Case Reports in Medicine  2014;2014:738981.
Anastomotic stricture (AS) and recurrent tracheoesophageal fistula (TEF) are two complications of surgical repair of esophageal atresia (EA). Therapeutic endoscopic modalities include stenting, tissue glue, and clipping for TEF and endoscopic balloon dilation bougienage and stenting for esophageal strictures. We report herein a two-month infant with both EA and TEF who benefited from a surgical repair for EA, at the third day of life. Two months later he experienced deglutition disorders and recurrent chest infections. The esophagogram showed an AS and a TEF confirmed with blue methylene test at bronchoscopy. A partially covered self-expanding metal type biliary was endoscopically placed. Ten weeks later the stent was removed. This allows for easy passage of the endoscope in the gastric cavity but a persistent recurrent fistula was noted. Instillation of contrast demonstrated a fully dilated stricture but with a persistent TEF. Then we proceeded to placement of several endoclips at the fistula site. The esophagogram confirmed the TEF was obliterated. At 12 months of follow-up, he was asymptomatic. Stenting was effective to alleviate the stricture but failed to treat the TEF. At our knowledge this is the second case of successful use of endoclips placement to obliterate recurrent TEF after surgical repair of EA in children.
PMCID: PMC4279268  PMID: 25580132
7.  Current knowledge on esophageal atresia 
Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the most common congenital anomaly of the esophagus. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of EA is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for EA and TEF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap EA is still controversial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastro-esophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders and deformities of the thoracic wall. Data regarding long-term outcomes and follow-ups are limited for patients following EA/TEF repair. The determination of the risk factors for the complicated evolution following EA/TEF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition. This manuscript provides a literature review of the current knowledge regarding EA.
PMCID: PMC3406418  PMID: 22851858
Esophageal atresia; Tracheoesophageal fistula; Esophageal stenosis; Long-gap; Gastro-esophageal reflux
8.  Platysma myocutaneous flap interposition in surgical management of large acquired post-traumatic tracheoesophageal fistula: A case report 
Acquired post-traumatic tracheoesophageal fistula (TEF) is an uncommon entity requiring early diagnosis. Among the many strategies in surgical management, we report a case successfully treated with a single-stage tracheal resection and esophageal repair with platysma myocutaneous interposition flap.
A 24-year-old man had a motor vehicle accident with head injury and cerebral contusion who required mechanical ventilation support. Three weeks later, he developed hypersecretion, and recurrent episodes of aspiration pneumonia. The chest computed tomography, esophagogastroduodenoscopy, and bronchoscopy revealed a large TEF diameter of 3 cm at 4.5 cm from carina. Single-stage tracheal resection with primary end-to-end anastomosis and esophageal repair with platysma myocutaneous interposition flap was performed. A contrast esophagography was done on post-operative day 7 and revealed no leakage. He was discharged on post-operative day 10. Esophagogastroduodenoscopy at 1 month revealed patient esophageal lumen. At present he is doing well without any evidence of complications such as esophageal stricture or fistula.
There are many choices of myocutaneous muscle flaps in trachea and esophageal closure or reinforcement. The platysma myocutaneous flap interposition is simple with the advantage of reduced bulkiness. Concern on the vascular supply is that flap should be elevated with the deep adipofascial tissue under the platysma to ensure that the flap survival is not threatened.
The treatment of acquired TEF with platysma myocutaneous flap is an alternative procedure for a large uncomplicated TEF as it is effective, technically ease, minimal donor site defect and yields good surgical results.
PMCID: PMC4008854  PMID: 24727740
Tracheoesophageal fistula; Tracheal reconstruction; Esophageal repair; Interposition flap; Platysma flap
9.  035. Bronchiectasis diagnosis and treatment 
Journal of Thoracic Disease  2015;7(Suppl 1):AB035.
Bronchiectasis, with was once thought to be an orphan disease, is now being recognized with increasing frequency around the world. Patients with bronchiectasis have chronic cough and sputum production, and bacterial infections develop in that result in the loss of lung function. Bronchiectasis occurs in patients across the spectrum of age and gender, but the highest prevalence is in older women. The diagnosis of bronchiectasis is made by high-resolution CT scans. Bronchiectasis, which can be focal or diffuse, may occur without antecedent disease but is often a complication of previous lung infection or injury or is due to underlying systemic illnesses. Patients with bronchiectasis may have predisposing congenital disease, immune disorders, or inflammatory disease. The treatment of bronchiectasis is multimodality, and includes therapy with antibiotics, anti-inflammatory agents, and airway clearance. Resectional surgery and lung transplantation are rarely required. The prognosis for patients with bronchiectasis is variable given the heterogeneous nature of the disease. A tailored, patient-focused approach is needed to optimally evaluate and treat in individuals with bronchiectasis. Bronchiectasis is destruction and widening of the large airways. If the condition is present at birth, it is called congenital bronchiectasis.
Bronchiectasis is often caused by recurrent inflammation or infection of the airways. Sometimes it begins in childhood after a more severe lung infection or inhaling a foreign object. Cystic fibrosis causes about a third of all bronchiectasis cases in the United States. Certain genetic conditions can also cause bronchiectasis, including primary ciliary dyskinesia and immunodeficiency syndromes. The condition can also be caused by routinely breathing in food particles while eating.
Symptoms often develop gradually, and may occur months or years after the event that causes the bronchiectasis. The may include: (I) bluish skin color; (II) breath odor; (III) chronic cough with large amounts of foul-smelling sputum; (IV) clubbing of fingers; (V) coughing up blood; (VI) fatigue; (VII) paleness; (VIII) shortness of breath that gets worse with exercise; (IX) weigh loss and (X) wheezing.
Exams and tests
When listening to the chest with a stethoscope, the doctor may hear small clicking, bubbling, wheezing, rattling, or other sounds, usually in the lower lobes of the lungs. Test may include: (I) aspergillosis precipitin test; (II) aipha-1; (III) antitrypsin blood test; (IV) chest X-ray; (V) chest CT; (VI) sputum culture; (VII) complete blood count; (VIII) genetic testing, including sweat test for cystic fibrosis; (IX) PPD skin test to check for a prior tuberculosis infection and (X) serum immunoglobulin electrophoresis.
Treatment is aimed at controlling infections and bronchial secretions, relieving airway obstruction, and preventing complications. Regular, daily drainage to remove bronchial secretions is a routing part of treatment. A respiratory therapist can show the patient coughing exercises that will help. Antibiotics, bronchodilators, and expectorants are often prescribed for infections, Surgery to resect the lung may be needed if medicine does not work or if the patient has, massive bleeding.
The outlook depends on the specific causes of the disease. With treatment, most people can lead normal lives without major disability.
Possible complications
(I) coughing up blood; (II) low oxygen levels; (III) recurrent pneumonia.
The risk may be reduced if lung infections are promptly treated. Childhood vaccinations and a yearly flu vaccine help reduce the change of some infections. Avoiding upper respiratory infections, smoking, and pollution may also reduce your risk or infection.
PMCID: PMC4332058
Bronchiectasis; spirometry; macrolides
10.  Application of MPVR and TL-VR with 64-row MDCT in neonates with congenital EA and distal TEF 
AIM: To assess the application of multiple planar volume reconstruction (MPVR) and three-dimensional (3D) transparency lung volume rendering (TL-VR) with 64-row multidetector-row computed tomography (MDCT) in neonates with congenital esophageal atresia (EA) and distal tracheoesophageal fistula (TEF).
METHODS: Twenty neonates (17 boys, 3 girls) with EA and distal TEF at a mean age of 4.6 d (range 1-16 d) were enrolled in this study. A helical scan of 64-row MDCT was performed at the 64 mm × 0.625 mm collimation. EA and TEF were reconstructed with MPVR and TL-VR, respectively. Initial diagnosis of EA was made by chest radiography showing the inserted catheter in the proximal blind-ended esophageal pouch. Manifestations of MDCT images were compared with the findings at surgery.
RESULTS: MDCT showed the proximal and distal esophageal pouches in 20 cases. No significant difference was observed in gaps between the proximal and distal esophageal pouches detected by MPVR and TL-VR. The lengths of gaps between the proximal and distal esophageal pouches detected by MPVR and TL-VR correlated well with the findings at surgery (R = 0.87, P < 0.001). The images of MPVR revealed the orifice of TEF in 13 cases, while TL-VR images showed the orifice of TEF in 4 cases.
CONCLUSION: EA and distal TEF can be reconstructed using MPVR and TL-VR of 64-row MDCT, which is a noninvasive technique to demonstrate the distal esophageal pouches and inter-pouch distance in neonates with EA and distal TEF.
PMCID: PMC3070138  PMID: 21472133
Children; Computed tomography; Congenital malformation; Esophagus; Tracheoesophageal fistula
11.  Congenital tracheoesophageal fistula and coordination of care: Expectations and realities 
Paediatrics & Child Health  2006;11(7):395-399.
Tracheoesophageal fistula (TEF) is a rare congenital anomaly with chronic morbidities. Aside from health care costs, the authors suspected that additional burden rests on the family due to hospitalizations, radiological and surgical procedures, and frequent outpatient visits. It was speculated that this complex care is poorly coordinated. The objective of the present study was to document utilization and coordination of health care services for children with TEF at the Alberta Children’s Hospital (Calgary, Alberta).
Medical records of children with TEF (primary surgical repair at Alberta Children’s Hospital between April 1994 and September 1999) were reviewed for demographics, TEF type, associated anomalies, age at diagnosis and repair, health services utilization data, and radiological and surgical procedures.
Twenty-two children were identified, of whom 18 survived beyond one year. Ten of these 18 children were male. Average gestational age and birth weight were 37.4 weeks (range 32 to 42 weeks) and 2512 g (range 780 g to 3950 g), respectively. Seventy-eight per cent of children had at least one associated anomaly. Median initial hospital and intensive care unit stays were 27.5 days and 12 days, respectively. During year 1 of life, there was a median of 31 radiological examinations, five surgical procedures, 2.5 hospital admissions and five outpatient clinic visits. Coordination of procedures and outpatient visits was poor. In year 2 of life, children had fewer procedures, admissions and outpatient visits; between years 2 and 5, procedures or admissions were rare.
Children with TEF endure multiple procedures and lengthy hospital admissions in early life. There was easy access to, but minimal coordination of, subspecialist care, procedures and admissions. A dedicated multidisciplinary clinic for children with TEF should improve care.
PMCID: PMC2528634  PMID: 19030308
Child; Chronic disease; Delivery of health care; Tracheoesophageal fistula
12.  Acquired tracheoesophageal fistula status post laryngeal neoplasm resection 
A tracheoesophageal fistula (TEF), albeit rare, can be a life-threatening condition that requires prompt identification and treatment. Pulmonary contamination and restriction of proper nutrition are common, unfortunate consequences of untreated TEFs and are often the causes of mortality in this population. In our patient, a history of laryngeal malignancy along with symptoms of chest pain and cough with ingestion of liquids, even without evidence of aspiration pneumonia, appropriately prompted investigation for potential TEF. Initial imaging through barium swallow identified the TEF, and the patient underwent treatment with endoclips by endoscopy with bronchoscopic assistance.
PMCID: PMC4387319  PMID: 25846352
TE Fistula; Endoclips; Laryngeal cancer
13.  Esophageal atresia in newborns: a wide spectrum from the isolated forms to a full VACTERL phenotype? 
VATER association was first described in 1972 by Quan and Smith as an acronym which identifies a non-random co-occurrence of Vertebral anomalies, Anal atresia, Tracheoesophageal fistula and/or Esophageal atresia, Radial dysplasia. It is even possible to find out Cardiovascular, Renal and Limb anomalies and the acronym VACTERL was adopted, also, embodying Vascular, as single umbilical artery, and external genitalia anomalies.
Data on patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) admitted in the Neonatal Intensive Care Unit (NICU) between January 2003 and January 2013 were evaluated for the contingent occurrence of typical VACTERL anomalies (VACTERL-type) and non tipical VACTERL anomalies (non-VACTERL-type). The inclusion criterion was the presence of EA with or without TEF plus two or more of the following additional malformations: vertebral defects, anal atresia, cardiovascular defects, renal anomalies and lower limb deformities, like radial dysplasia.
Among 52 patients with EA/TEF, 20 (38,4%) had isolated EA and 7 (21,8%) had a recognized etiology such a syndrome and therefore were excluded. Among 32 infants with EA and associated malformations, 15 (46,8%) had VACTERL association. The most common anomalies were congenital heart defects (73,3%), followed by vertebral anomalies (66,6%). Many patients also had additional non-VACTERL-type defects. Single umbilical artery was the most common one followed by nervous system abnormalities and anomalies of toes. Between the groups of infants with VACTERL type and non-VACTERL-type anomalies, there are several overlapping data regarding both the tipically described spectrum and the most frequently reported non-VACTERL-type malformations. Thus, it is possible to differentiate infants with a full phenotype (VACTERL full phenotype) and patients that do not meet all the criteria mentioned above, but with some homologies with the first group (VACTERL partial phenotype).
The high frequency of non-VACTERL-type anomalies encountered in full and partial phenotype patients would suggest the need for an extension of the clinical criteria for the diagnosis of VACTERL association and also for pre- and post-operative management and follow-up in the short and long term.
PMCID: PMC3726359  PMID: 23842449
Esophageal atresia; Tracheo-esophageal fistula; VATER; VACTERL; Association; Congenital malformations; Anomalies
14.  Repair of post-intubation tracheoesophageal fistulae through the left pre-sternocleidomastoid approach: a recent case series of 13 patients 
Journal of Thoracic Disease  2015;7(Suppl 1):S20-S26.
Post-intubation tracheoesophageal fistula (TEF) is a late complication of tracheotomy, while membranous trachea laceration during percutaneous dilational tracheostomy is implicated in the generation of early post-tracheotomy TEF. Surgical repair is the only viable option for these patients and the technique of repair depends on a variety of factors.
Totally 13 patients (mean age: 54.1±12.6 years; male: 8) with post-intubation TEF were managed between 2007 and 2013. The diagnosis was always made through esophagoscopy followed by endoscopic gastrostomy and bronchoscopy for repositioning of the tracheal tube just above the carina. Repair of the fistula was made in all patients through a left pre-sternocleidomastoid incision followed by dissection of the fistulous tract, suturing of esophagus and trachea and interposition of the whole pedicled left sternocleidomastoid muscle (SCMM) between the two suture lines.
Five out of the 13 procedures were performed in mechanically ventilated patients; 3 of them died from septic complications during the postoperative period while fistula recurred in 1 of those 3 patients due to extensive inflammation of the tracheal wall. The rest 8 patients underwent fistula repair after weaning from mechanical ventilation and the results of repair were excellent. The additional procedure of temporary T-tube insertion was obviated in one patient to manage extensive tracheomalacia.
The left pre-sternocleidomastoid incision is an excellent access for the repair of a post-intubation TEF without tracheal resection. The interposition of the whole left pedicled SCMM between the suture lines of trachea and esophagus avoids fistula recurrence and offers the best chance for cure.
PMCID: PMC4332095  PMID: 25774303
Tracheoesophageal fistula (TEF); post-intubation tracheo-esophageal fistula; tracheotomy; dilational tracheotomy; complications of tracheotomy; pre-sternocleidomastoid approach
15.  Complete healing of tracheoesophageal fistula in a ventilator-dependent patient by conservative treatment 
Respirology Case Reports  2013;2(1):27-29.
Acquired nonmalignant tracheoesophageal fistula (TEF) is a rare clinical condition with multiple etiologies, although post-intubation injury is the most common cause. TEFs can be fatal if left untreated due to devastating pulmonary complications caused by tracheobronchial contamination and poor nutrition. Herein, we present a case of complete healing of a post-intubation TEF under conservative treatment in a ventilator-dependent patient, and review previous studies regarding the treatment of acquired nonmalignant TEFs.
PMCID: PMC4184726  PMID: 25473556
Nonmalignant tracheoesophageal fistula; tracheostomy
16.  Expanding the BP1-BP2 15q11.2 Microdeletion Phenotype: Tracheoesophageal Fistula and Congenital Cataracts 
Case Reports in Genetics  2013;2013:801094.
The proximal q arm of chromosome 15 contains breakpoint regions BP1–BP5 with the classic deletion of BP1–BP3 best known to be associated with Prader-Willi and Angelman syndromes. The region is approximately 500 kb and microdeletions within the BP1-BP2 region have been reported in patients with developmental delay, behavioral abnormalities, and motor apraxia as well as dysmorphic features including hypertelorism, cleft or narrow palate, ear abnormalities, and recurrent upper airway infections. We report two patients with unique, never-before-reported 15q11.2 BP1-2 microdeletion syndrome findings, one with proximal esophageal atresia and distal tracheoesophageal fistula (type C) and one with congenital cataracts. Cataracts have been described in Prader-Willi syndrome but we could not find any description of cataracts in Angelman syndrome. Esophageal atresia and tracheoesophageal fistula have not been reported to our knowledge in either syndrome. A chance exists that both cases are sporadic birth defects; however, the findings of the concomitant microdeletion cannot be overlooked as a possible cause. Based on our review of the literature and the presentation of our patients, we recommend that esophageal atresia and distal tracheoesophageal fistula as well as congenital cataracts be included in the phenotypic spectrum of 15q11.2 BP1-2 microdeletion syndrome.
PMCID: PMC3707229  PMID: 23864968
17.  Esophageal atresia: Factors influencing survival - Experience at an Indian tertiary centre 
To study the clinical profile of the cases of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and various factors affecting the surgical and early postoperative management and their outcome.
Materials and Methods:
A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading.
EA with TEF was the commonest type in 117 cases (92%). Associated congenital anomalies were present in 52 (41%) patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5%) cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival (P = < 0.001). Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%). Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases). Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C.
Factors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome.
PMCID: PMC2810819  PMID: 20177477
Associated anomalies; esophageal atresia; prognosis; tracheoesophageal fistula
18.  Delayed primary reconstruction of esophageal atresia and distal tracheoesophageal fistula in a 471-g infant 
Waterston et al. have classified the risk of morbidity in infants with esophageal atresia and tracheoesophageal fistula. However, few cases of esophageal atresia with distal tracheoesophageal fistula in extremely low birth weights infants have been reported. In such infants, the selection of primary reconstruction or staged repair remains controversial. In the present report, we describe the difficulties of perioperative management of such small infants and discuss how to rescue them.
A 471-g female infant was delivered at 28 weeks’ gestation via cesarean section. Esophageal atresia with distal tracheoesophageal fistula was diagnosed. Esophageal banding and gastrostomy were performed via laparotomy on day 1. On day 74, when the infant weighed almost 1000 g, airway management was discontinued. On day 136, endotracheal intubation again became necessary because of respiratory problems, and the esophagus was reconstructed on day 141. Despite this operation, the patient died on day 276 because of continuing respiratory problems.
Esophageal banding is considered an appropriate treatment for respiratory problems in such extremely low weight infants. However, the timing of dissection of the tracheoesophageal fistula after esophageal banding is extremely important.
In the present case, ligation of the tracheoesophageal fistula and esophageal reconstruction should have been performed as soon as possible.
PMCID: PMC3540228  PMID: 23276758
Esophageal atresia; Extremely low weight infants; Esophageal banding
19.  Tracheoesophageal fistula following disc battery ingestion and foreign body impaction  
Background: Ingestion of foreign bodies may result in the formation of a tracheoesophageal fistula (TEF), which causes severe morbidity in children. We describe four cases of TEF, who underwent emergent surgery for repair.
Case presentation: In this report, we present about four patients aged between 9 months to 2.5 years, who referred due to disc battery ingestion. There were two boys and two girls. The common symptoms were cough, cyanosis, and dysphagia, choking and vomiting. The diagnosis was performed through an x-ray, barium swallow and CT Scan. All batteries were impacted in the esophagus, two in upper, one in the middle, and one in lower esophagus position. All disc batteries were removed endoscopically, but had tracheoesophageal fistula (TEF). All the patients underwent TEF repaired surgically. There was no morbidity in four patients, but one patient developed moderate esophageal stenosis, which was repaired by staged dilatation. There was no mortality in our cases.
Conclusion: Long-term impaction of foreign bodies may result in tracheoesophageal fistula. This complication may be seen earlier with alkaline disc batteries. Removal of these foreign bodies should be followed carefully for the diagnosis and treatment of these fistulas.
PMCID: PMC3895833  PMID: 24551442
Esophageal foreign body; Disc battery; tracheoesophageal fistula; Children.
20.  Late Manifestation of a Large Congenital Tracheoesophageal Fistula in an Adult 
Texas Heart Institute Journal  2006;33(1):60-62.
Congenital tracheoesophageal fistula in an adult is a rare condition. We describe the clinical presentation in a young woman after video-assisted thoracoscopic pleurodesis for spontaneous pneumothorax. She was found to have a very wide and short fistula in her neck. The tracheoesophageal defect was closed by a trap-door flap, using the posterior wall of the trachea. The patient made an uncomplicated recovery.
PMCID: PMC1413598  PMID: 16572873
Tracheoesophageal fistula/ congenital/surgery
21.  Congenital bronchoesophageal fistula in adults 
AIM: To study the clinical characteristics, diagnosis and surgical treatment of congenital bronchoesophageal fistulae in adults.
METHODS: Eleven adult cases of congenital bronchoesophageal fistula diagnosed and treated in our hospital between May 1990 and August 2010 were reviewed. Its clinical presentations, diagnostic methods, anatomic type, treatment, and follow-up were recorded.
RESULTS: Of the chief clinical presentations, nonspecific cough and sputum were found in 10 (90.9%), recurrent bouts of cough after drinking liquid food in 6 (54.6%), hemoptysis in 6 (54.6%), low fever in 4 (36.4%), and chest pain in 3 (27.3%) of the 11 cases, respectively. The duration of symptoms before diagnosis ranged 5-36.5 years. The diagnosis of congenital bronchoesophageal fistulae was established in 9 patients by barium esophagography, in 1 patient by esophagoscopy and in 1 patient by bronchoscopy, respectively. The congenital bronchoesophageal fistulae communicated with a segmental bronchus, a main bronchus, and an intermediate bronchus in 8, 2 and 1 patients, respectively. The treatment of congenital bronchoesophageal fistulae involved excision of the fistula in 10 patients or division and suturing in 1 patient. The associated lung lesion was removed in all patients. No long-term sequelae were found during the postoperative follow-up except in 1 patient with bronchial fistula who accepted reoperation before recovery.
CONCLUSION: Congenital bronchoesophageal fistula is rare in adults. Its most useful diagnostic method is esophagography. It must be treated surgically as soon as the diagnosis is established.
PMCID: PMC3068273  PMID: 21455337
Congenital bronchoesophageal fistula; Adult; Esophagography; Surgical treatment
22.  Tracheal Penetration and Tracheoesophageal Fistula Caused by an Esophageal Self-Expanding Metallic Stent 
Case Reports in Pulmonology  2014;2014:567582.
Tracheal penetration of esophageal self-expanding metallic stents (SEMS) with/without tracheoesophageal fistula (TEF) formation is a rare occurrence. We report the case of a 66-year-old female patient with advanced esophageal squamous cell carcinoma who had undergone palliative esophageal stenting on three occasions for recurrent esophageal stent obstruction. On evaluation of symptoms of breathing difficulty and aspiration following third esophageal stent placement, tracheal erosion and TEF formation due to the tracheal penetration by esophageal stent were diagnosed. The patient was successfully managed by covered tracheal SEMS placement under flexible bronchoscopy.
PMCID: PMC4167813  PMID: 25276461
23.  Early Recognition of H-Type Tracheoesophageal Fistula 
Tracheoesophageal fistula (TEF) without associated esophageal atresia (EA) is a rare congenital anomaly. Diagnosis in neonatal period is usually not made and most of the patients are treated as cases of pneumonia. A case of H-type of tracheoesophageal fistula, diagnosed within 24 hours of delivery based upon choking and cyanosis on first trial of feed, is being reported. Diagnosis was confirmed with contrast esophagram. Through cervical approach fistula was repaired and baby had uneventful post operative outcome.
PMCID: PMC3418037  PMID: 22953298
Tracheoesophageal fistula;  H-Type;  Esophagus;  Atresia
24.  The Multifactorial Origin of Respiratory Morbidity in Patients Surviving Neonatal Repair of Esophageal Atresia 
Esophageal atresia with or without tracheoesophageal fistula (EA ± TEF) occurs in 1 out of every 3000 births. Current survival approaches 95%, and research is therefore focused on morbidity and health-related quality of life issues. Up to 50% of neonates with EA ± TEF have one or more additional malformations including those of the respiratory tract that occur in a relatively high proportion of them and particularly of those with vertebral, anal, cardiac, tracheoesophageal, renal, and limb association. Additionally, a significant proportion of survivors suffer abnormal pulmonary function and chronic respiratory tract disease. The present review summarizes the current knowledge about the nature of these symptoms in patients treated for EA ± TEF, and explores the hypothesis that disturbed development and maturation of the respiratory tract could contribute to their pathogenesis.
PMCID: PMC4017156  PMID: 24829898
esophageal atresia; tracheoesophageal fistula; respiratory tract disease; lung; development; human; rodent models
25.  Esophageal atresia associated with anorectal malformation: Is the outcome better after surgery in two stages in a limited resources scenario? 
To analyze whether outcome of neonates having esophageal atresia with or without tracheoesophageal fistula (EA±TEF) associated with anorectal malformation (ARM) can be improved by doing surgery in 2 stages.
Materials and Methods:
A prospective study of neonates having both EA±TEF and ARM from 2004 to 2011. The patients with favorable parameters were operated in a single stage, whereas others underwent first-stage decompression surgery for ARM. Thereafter, once septicemia was under control and ventilator care available, second-stage surgery for EA±TEF was performed.
Total 70 neonates (single stage = 20, 2 stages = 30, expired after colostomy = 9, only EA±TEF repair needed = 11) were enrolled. The admission rate for this association was 1 per 290. Forty-one percent (24/70) neonates had VACTERL association and 8.6% (6/70) neonates had multiple gastrointestinal atresias. Sepsis screen was positive in 71.4% (50/70). The survival was 45% (9/20) in neonates operated in a single stage and 53.3% (16/30) when operated in 2 stages (P = 0.04). Data analysis of 50 patients revealed that the survived neonates had significantly better birth weight, better gestational age, negative sepsis screen, no cardiac diseases, no pneumonia, and 2-stage surgery (P value 0.002, 0.003, 0.02, 0.02, 0.04, and 0.04, respectively). The day of presentation and abdominal distension had no significant effect (P value 0.06 and 0.06, respectively). This was further supported by stepwise logistic regression analysis.
In a limited resources scenario, the survival rate of babies with this association can be improved by treating ARM first and then for EA±TEF in second stage, once mechanical ventilator care became available and sepsis was under control.
PMCID: PMC3409897  PMID: 22869975
Anorectal malformation; esophageal atresia; neonatal intensive care; tracheoesophageal fistula

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