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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.

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.

BACKGROUND

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).

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

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.

Objective:

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.

Results:

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.

Conclusion

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.

Acquired tracheoesophageal fistula through esophageal diverticulum is infrequent. We report tracheoesophageal fistula through esophageal diverticulum in a 55-year-old male who had a prolonged tracheostomy tube during 6 months, and a NG tube during 18 months. He suffered from recurrent pneumonia. He complained of a cough associated with eating, and production of sputum mixed with food. To help evaluate the aspiration to the lung and the cause of aspiration, he was tested using gastrointestinal scintigraphy (gastric emptying study), a chest CT scan (pre & post contrast), and esophagoduodenoscopy. The chest CT scan revealed an acquired tracheoesophageal fistula through esophageal diverticulum, and esophagoduodenoscopy revealed a 3 mm sized fistula that was located -33 cm from the upper incisor. We treated the tracheoesophageal fistula by clipping under esophagoduodenoscopy. The symptoms of fever, cough, and aspiration were no long observed after the clipping was completed.

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.

A case of acquired tracheoesophageal fistula (TEF) is presented in a 44-year-old female who presented with acute respiratory failure due to bilateral aspiration pneumonia. The patient had persistent air leak while on mechanical ventilation and underwent bronchoscopy which revealed the above etiology. Histopathology showed Barrett’s esophagitis. The patient underwent primary closure followed by a short course of proton pump inhibitors. There are only two prior reported cases of acquired TEF associated with Barrett’s esophagitis. This condition should be taken under consideration when investigating of an explained persistent air leak in a mechanically ventilated patient.

Malignant tracheoesophageal fistula occurs infrequently in patients with irradiated esophageal and lung cancer, uncommonly in patients with untreated mediastinal malignancies, infection, or trauma, and rarely in primary adenocarcinoma of the lung. The natural progression of this entity leads to rapid deterioration and death from overwhelming pulmonary infection.

Definitive treatment depends on the demonstration and localization of the fistula. Computerized tomography (CT) chest scanning with dilute oral contrast is the ideal means of identification of the fistula. CT scanning without contrast may outline sufficiently the pathology to avoid the usage and side effects of contrast media in the lungs.

A review of one case of tracheoesophageal fistula due to pulmonary adenocarcinoma is presented where emergency CT scanning without contrast resulted in rapid and accurate diagnosis, leading to prompt and appropriate management.

Images

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.

Esophageal duplication cyst is a rare congenital anomaly. They can be associated with other congenital anomalies, such as spinal abnormalities, and tracheoesophageal fistulas. In adults, almost of the patients with esophageal duplication cyst is asymptomatic and accidentally diagnosed by chest X-ray or computed tomography. However, cysts may become symptomatic owing to complications such as esophageal stenosis, respiratory system compression, rupture, infarction, or malignancy. Complete surgical resection is the standard treatment even in patients with asymptmatic cysts. Traditional approach for resection is via thoracotomy. But, the thoracoscopic approach makes more indicate for mediastinal diseases, because of minimally invasive for patients. We describe a case with esophageal duplication cyst, which contained the ectopic pancreatic tissue in the solid portion, resected under the thoracoscopic approach in adult.

Background

The non-malignant, acquired tracheoesophageal fistulas (TEF), resulting from tracheal intubation are usually iatrogenic lesions. Tracheal lesions resulting from intubation may occur and pneumomediastinum, pneumothorax or subcutaneous emphysema may develop due to the stream of air.

Case Presentation

We present a-39-year old, Caucasian patient, developing severe hypoxia fallowing cesarean section under general anesthesia. The findings of the patient were diffuse subcutaneous emphysema; together with pneumothorax and pneumomediastinum TEF was diagnosed in the patient by bronchoscopy and eusophagoscopy performed due to cough and difficulty in swallowing developing after extubation.

Conclusion

It is important to the clinicians to be aware of the TEF can be accompanied to the traumatic intubation and urgent endoscopy or water-soluble contrast radiography may be prudent.

A 45-year-old man complaining of cough, dyspnea, and difficulty in swallowing was referred to our hospital. Chest CT scan showed a mediastinal mass compressing the trachea. He was diagnosed with poorly differentiated lung carcinoma by percutaneous needle biopsy. Bronchoscopy and upper gastrointestinal endoscopy revealed a tracheoesophageal fistula (TEF). Long-lasting febrile neutropenia made it impossible to continue chemotherapy, but a course of radiotherapy (total 61 Gy) was completed. The next endoscopy revealed closure of the TEF. Chemoradiotherapy (CRT) has been reported to close TEF in esophageal cancer, but the risk of a CRT-induced worsening of the fistula has dissuaded physicians from using CRT to treat TEF in lung cancer patients. CRT may serve as a palliative treatment for TEF in lung cancer as well as esophageal cancer.

INTRODUCTION

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.

PRESENTATION OF CASE

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.

DISCUSSION

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.

CONCLUSION

In the present case, ligation of the tracheoesophageal fistula and esophageal reconstruction should have been performed as soon as possible.

Scoliosis as a late complication of thoracotomy has been described previously, but reports are rare. We present the case of a 22 year-old female referred for symptomatic scoliosis. Radiographs demonstrated a severe, structural, upper-thoracic scoliosis with associated right-sided rib fusions. Her medical history was noteworthy for a right posterolateral thoracotomy for repair of a tracheoesophageal fistula during infancy. Radiographs from her general surgical treatments during infancy demonstrated no congenital rib or vertebral anomalies. This report reviews her case in detail as well as the scarce literature available regarding scoliosis secondary to thoracotomy at a young age.

Tracheoesophageal fistula (TEF) after prolonged intubation could present as chronic aspiration and could be mistaken as unilateral or bilateral vocal fold palsy, especially when there was combined posterior glottic synechia. We present a case of post-intubation TEF which was successfully treated with tracheal resection and anastomosis with primary esophageal closure. The accompanying posterior glottic web was treated by endoscopic technique of web lysis, with topical application of mitomycin C solution.

Tracheal agenesis (TA) is a severe congenital disorder with often an unexpected emergency presentation. There is complete or partial absence of the trachea below the larynx, with presence or absence of a tracheoesophageal fistula (TOF). A neonate with TA is described, and another 48 cases found in literature are reviewed. Due to absence of a TOF, five cases were diagnosed prenatally because of congenital high airway obstruction syndrome (CHAOS). When a TOF is present, polyhydramnion and several other congenital malformations seen on the ultrasound examination should alert clinicians of potential tracheal problems. Prenatal magnetic resonance imaging (MRI) may provide a definitive diagnosis. Postnatal diagnosis is based on recognition of specific clinical signs in the newborn with TA: respiratory distress with breathing movement without appropriate air entry, no audible cry, and failed endotracheal intubation. Despite progress in surgical interventions, mortality remains high. Prenatal diagnosis of TA is possible, but only if a TOF is absent resulting in CHAOS. Prenatal diagnosis of polyhydramnion and other congenital malformation should alert clinicians of potential tracheal problems. Prenatal MRI may provide a definitive diagnosis.

Background

Gastro-bronchial fistula (GBF) is a rare and devastating complication following esophagectomy. Making the correct diagnosis is difficult and there is no agreement on the treatment for this rare condition.

Case presentation

We report the case of a 56-year-old man who presented with features of repeated aspiration and chest infections six years following an esophagectomy for Barrett's esophagus. Despite extensive investigations the cause of symptoms was difficult to determine. The correct diagnosis of fistula from stomach to right main stem bronchus was made at bronchoscopy under general anesthesia. After ruling out local recurrence of cancer, a successful primary repair was carried out by resection of fistula and direct repair of gastric conduit and bronchus. He is well after 6 months of treatment.

Conclusion

Late development of gastro-bronchial fistula is a rare complication of esophageal resection that may be difficult to diagnose.

Surgical resection and direct closure is the treatment of choice, although the method of treatment should be tailored according to the anatomy of the fistula and the patient's condition.

The diagnostic feature of esophageal atresia (EA) is the inability to pass a catheter into the stomach. EA can be ruled out if the feeding tube can be passed into the stomach. In EA, when a tracheo-esophageal fistula (TEF) is present, theoretically the feeding tube can find its way into the stomach via tracheal route and through the TEF. We report such a rare occurrence. In this situation, the diagnosis and further management of EA and TEF was delayed.

Esophageal atresia and tracheoesophageal fistula (EA/TEF) are major congenital malformations affecting 1:3500 live births. Current research efforts are focused on understanding the etiology of these defects. We describe well-known animal models, human syndromes, and associations involving EA/TEF, indicating its etiologically heterogeneous nature. Recent advances in genotyping technology and in knowledge of human genetic variation will improve clinical counseling on etiologic factors. This review provides a clinical summary of environmental and genetic factors involved in EA/TEF.

Aims:

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.

Results:

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.

Conclusions:

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.

A 63-year-old man with esophageal achalasia for more than 20 years complained of respiratory distress. He was admitted as an emergency to the referral hospital three months previously. Computed tomography revealed tracheobronchial stenosis due to advanced esophageal cancer with tracheal invasion. He underwent tracheobronchial stenting and chemoradiotherapy. A large tracheoesophageal fistula (TEF) developed after irradiation (18 Gy) and chemotherapy, and he was unable to eat. Thereafter, he was referred to our hospital, where we performed esophageal bypass surgery using a gastric conduit. A percutaneous cardiopulmonary support system was prepared due to the risk of airway obstruction during anesthesia. A small-diameter tracheal tube inserted into the stent achieved ordinary respiratory management. No anesthesia-related problems were encountered. Oral intake commenced on postoperative day 9. He was discharged on postoperative day 23 and was able to take in sustenance orally right up to the last moment of his life. Esophageal bypass under general anesthesia can be performed in patients with large TEF with sufficient preparation for anesthetic management.

A newborn female infant born to a woman on immunosuppressive medications including mycophenolate mofetil (MMF) for a renal graft secondary to lupus nephritis presented with congenital diaphragmatic hernia (CDH) and additional findings of microtia, esophageal atresia with tracheoesophageal fistula, cleft palate, congenital heart defect, digital anomalies, and dysmorphic facial features. Pulmonary hypoplasia resulted in death at day two of life. She was presumed to have Fryns syndrome based on diagnostic criteria established for this recessive disorder with prominent features including CDH, facial anomalies, and nail hypoplasia. In retrospect, this infant's findings are more likely the result of teratogenic exposure to MMF, as more recent data have emerged linking aural atresia, digital anomalies, and dysmorphic features to this drug. To date, this is the only human report of CDH in an infant with prenatal exposure to MMF, although the manufacturer's package insert alludes to animal studies with a broad spectrum of malformations, including CDH. Thus, a teratogenic exposure can mimic a known Mendelian genetic syndrome, and caution is urged in presuming a genetic etiology for infants with potential teratogenic exposure to relatively new drugs with limited published animal data.

The case of a 46-year-old man with congenital broncho-oesophageal fistula is presented. The patient had had recurrent chest infections since childhood and a recent history of heartburn and flatulence. A barium-swallow examination showed a small sliding hiatal hernia and an oesophageal diverticulum communicating via a fistula with the apical and posterior segmental bronchi of the left lower lobe. Bronchography showed bronchiectasis in this lobe. At thoracotomy resection of the broncho-oesophageal fistula and left lower lobectomy were performed. The patient's recovery was uneventful. Twenty published cases are reviewed. The classification of congenital broncho-oesophageal fistulae should take account not only of the type of fistula but also of the type and size of the bronchus with which the fistula communicates, the age of the patient at the first appearance of symptoms, and the duration of symptoms.

Images

A coronary-bronchial artery fistula is a very rare congenital anomaly of the coronary artery whose etiology and pathogenesis have not yet been clarified. Most patients with coronary-bronchial fistulas are asymptomatic; however, some patients present with congestive heart failure, infective endocarditis, myocardial ischemia induced by a coronary steal phenomenon, or rupture of an aneurysmal fistula. Furthermore, patients with a coronary-bronchial artery fistula rarely manifest life-threatening hemoptysis due to the associated bronchiectasis. We report herein the case of a patient with a coronary-bronchial artery fistula who had bronchiectasis and a history of massive hemoptysis and myocardial ischemia.