Overview
Definition:
Type C Tracheoesophageal Fistula (TEF) refers to a congenital anomaly where the esophagus ends in a blind pouch (esophageal atresia) and is connected to the trachea by a fistula originating from the distal segment of the esophagus
This is the most common type of TEF, accounting for approximately 80% of cases
It is often associated with other congenital anomalies, forming part of the VATER or EA/TEF syndrome.
Epidemiology:
The incidence of EA/TEF is estimated to be around 1 in 2500 to 4500 live births
Type C is the most frequent subtype
There is no significant sex predilection
Associated anomalies occur in about 50-80% of patients, including vertebral defects, anal atresia, cardiac anomalies, renal anomalies, limb malformations, and tracheoesophageal anomalies.
Clinical Significance:
TEF is a life-threatening condition requiring prompt diagnosis and surgical intervention
Failure to repair can lead to aspiration pneumonia, recurrent respiratory infections, malnutrition, and failure to thrive
Accurate understanding of surgical repair techniques and potential complications is crucial for optimal patient outcomes and success in surgical postgraduate examinations.
Clinical Presentation
Symptoms:
Excessive drooling of saliva from the mouth
Coughing and choking spells during feeding attempts
Cyanosis during feeding
Abdominal distension due to air entering the stomach from the fistula
Recurrent pneumonia
Failure to thrive.
Signs:
Difficulty passing an orogastric (OG) or nasogastric (NG) tube, which may coil in the upper esophageal pouch
Auscultation may reveal bubbling sounds over the lungs, indicative of aspiration
Palpable air in the stomach on abdominal examination suggests a distal TEF.
Diagnostic Criteria:
The diagnosis is primarily clinical, based on the presence of symptoms suggestive of TEF in a neonate
Confirmation is achieved through imaging studies, most importantly by attempting to pass a radiopaque catheter into the esophagus to identify the blind pouch and visualize the fistula with contrast
Chest X-ray may show a gastric bubble (indicating a distal TEF) and aspiration pneumonia
A contrast esophagram is definitive.
Diagnostic Approach
History Taking:
Detailed antenatal history for polyhydramnios or suspected fetal anomalies
Immediate postnatal history focusing on feeding difficulties, cyanosis, and respiratory distress
History of recurrent chest infections or failure to gain weight.
Physical Examination:
Thorough examination of the airway for signs of distress
Careful observation of feeding attempts
Auscultation for abnormal breath sounds
Complete physical examination to identify associated anomalies (e.g., imperforate anus, vertebral abnormalities, cardiac murmurs, limb deformities).
Investigations:
Plain chest X-ray: May show aspiration pneumonia, a dilated proximal esophageal pouch, and a gastric bubble (if distal TEF)
Attempted passage of a feeding tube: If the tube cannot be advanced into the stomach, it suggests esophageal atresia
a coiled tube in the upper pouch is highly suggestive
Contrast esophagram: The gold standard for diagnosis, visualizing the blind pouch and the fistula tract with injected contrast medium
Bronchoscopy: May be performed to assess the tracheal and bronchial anatomy and the fistula orifice, especially if there are concerns about tracheal integrity or stenosis
Echocardiography: To rule out associated cardiac anomalies
Renal ultrasound: To assess for renal anomalies
Skeletal survey: To identify vertebral anomalies.
Differential Diagnosis:
Other causes of neonatal respiratory distress and feeding difficulties, such as choanal atresia, laryngomalacia, tracheomalacia, diaphragmatic hernia, and congenital heart disease.
Management
Initial Management:
Immediate stabilization: Maintain airway and oxygenation
Suction secretions from the pharynx and blind pouch to prevent aspiration
Elevate the head of the infant to 30-45 degrees to minimize gastroesophageal reflux into the tracheobronchial tree
Insert a large-bore (e.g., 10 Fr) Foley catheter into the proximal esophageal pouch, inflate the balloon, and use it for continuous suction to drain pooled secretions
Administer antibiotics to treat or prevent aspiration pneumonia
Nutritional support: Initially, parenteral nutrition should be initiated
Gastrostomy tube insertion for feeding may be considered in stable infants prior to definitive repair.
Surgical Management:
The definitive treatment is surgical repair, typically performed via a thoracotomy (usually right-sided for distal TEF)
The goals are to ligate the fistula and re-establish esophageal continuity
The procedure involves division of the fistula and an end-to-end or end-to-side anastomosis of the esophagus
The timing of repair is debated
historically, repair was delayed, but current practice favors early repair, often within the first few days of life, especially in stable infants
Transpleural approach (Right Thoracotomy) is common for Type C TEF
Esophageal ligation of the fistula and division
Esophageal anastomosis (end-to-end or end-to-side).
Supportive Care:
Close monitoring of vital signs, oxygen saturation, and fluid balance
Aggressive pulmonary toilet with suctioning as needed
Pain management
Gradual introduction of oral feeds postoperatively once the anastomosis is confirmed to be intact and the infant is tolerating oral intake
Long-term nutritional support if needed.
Complications
Early Complications:
Anastomotic leak: Leakage of saliva or food from the esophageal anastomosis, leading to mediastinitis or pleural effusion
Recurrent TEF: The fistula can reform, often due to dehiscence of the anastomosis
Pneumothorax or chylothorax: Due to surgical manipulation of the pleural space
Vocal cord paralysis: Injury to the recurrent laryngeal nerve, affecting voice and swallowing
Gastrostomy tube site infection.
Late Complications:
Esophageal stricture: Narrowing of the esophageal anastomosis, leading to dysphagia
Tracheomalacia and broncho-malacia: Weakness of the tracheal or bronchial walls, causing stridor, recurrent wheezing, and cough
Gastroesophageal reflux disease (GERD): Common sequela, requiring medical management
Long-term respiratory problems: Chronic cough, recurrent pneumonia, and reactive airway disease
Dysphagia: Difficulty swallowing, persistent in some cases.
Prevention Strategies:
Meticulous surgical technique to minimize nerve injury and ensure secure anastomosis
Prompt diagnosis and treatment of aspiration pneumonia
Careful handling of the esophagus and trachea during surgery
Postoperative vigilant monitoring for signs of complications
Aggressive management of GERD to protect the anastomosis and prevent esophagitis.
Prognosis
Factors Affecting Prognosis:
Gestational age and birth weight of the infant
Presence and severity of associated anomalies (especially cardiac and pulmonary)
Timeliness and success of surgical repair
Development of complications such as pneumonia or anastomotic leak
Presence of significant tracheomalacia.
Outcomes:
With modern surgical management, the survival rate for isolated EA/TEF is high (over 95%)
However, infants with multiple congenital anomalies or significant prematurity have a poorer prognosis
Long-term morbidity related to respiratory issues and esophageal dysfunction can affect quality of life.
Follow Up:
Regular follow-up with a pediatric surgeon, gastroenterologist, and pulmonologist is essential
This includes monitoring for growth and development, assessment of feeding, management of GERD, evaluation of respiratory symptoms, and screening for esophageal strictures or dysphagia
Pulmonary function tests may be indicated
Speech and swallowing assessments are important.
Key Points
Exam Focus:
Type C TEF is the most common subtype
It requires prompt surgical repair
Key complications include anastomotic leak, recurrent TEF, and tracheomalacia
Early and aggressive management of aspiration pneumonia is vital
The F\o ley catheter technique for the proximal pouch is a critical initial step.
Clinical Pearls:
Always consider TEF in any neonate with unexplained respiratory distress or feeding difficulties
The inability to pass a feeding tube into the stomach is a red flag
A distal TEF is often associated with a gas bubble in the stomach on plain X-ray
Right thoracotomy is the preferred approach for most Type C TEFs.
Common Mistakes:
Delaying surgical intervention due to prematurity or associated anomalies without adequate stabilization
Inadequate suctioning of the proximal pouch, leading to continued aspiration
Misinterpreting X-rays, missing the gastric bubble or overlooking signs of aspiration pneumonia
Insufficient management of tracheomalacia or GERD postoperatively.