TRACHEOESOPHAGEAL FISTULA:• Tracheoesophageal fistula (TEF) is a common congenital anomaly of the

respiratory tract, with an incidence of approximately 1 in 3500 live births

TEF typically occurs with esophageal atresia (EA). Classification:Type C, which consists of a proximal esophageal pouch and a distal TEF,

accounts for 84 percent of cases. TEF occurs without EA (H-type fistula) in only 4 percent TEF and EA are caused by a defect in the lateral septation of the foregut into

the esophagus and trachea. The fistula tract is thought to derive from a branch of the embryonic lung bud

that fails to undergo branching because of defective epithelial-mesenchymal interactions

-associated anomalies in about half of the cases of TEF and EA, often as part of the VACTERL association

• Clinical features: polyhydramnios occurs in approximately two-thirds of

pregnancies [89]. Additional clinical features relate to the presence of the VACTERL association

• Infants with EA become symptomatic immediately after birth with excessive secretions that cause drooling, choking, respiratory distress, and the inability to feed.

• A fistula between the trachea and distal esophagus leads to gastric distension.

• Reflux of gastric contents through the TEF results in aspiration pneumonia and contributes to morbidity.

• Patients with H-Type TEFs may present early if the defect is large, with coughing and choking associated with feeding as the milk is aspirated through the fistula.

Dx

• The diagnosis of EA can be made by attempting to pass a catheter into the stomach.

• This finding can be confirmed with an anterior-posterior chest radiograph that demonstrates the catheter curled in the upper esophageal pouch.

• When the diagnosis is uncertain or a proximal TEF is suspected, a small amount of water-soluble contrast material placed in the esophageal pouch under fluoroscopic guidance will confirm the presence of EA.

R/x

• surgical ligation of the fistula

• isolated TEF is generally good • depends upon associated abnormalities.

• Mortality rates for EA and TEF were greater for infants with associated cardiac disease

• Complications after EA and TEF repair:anastomotic leak esophageal stricture and

recurrent fistulae, gastroesophageal reflux and aspiration.

Motility disorders and respiratory function abnormalities persist on long-term follow-up

                                                                                 

Types of tracheoesophageal fistulas

Congenital tracheal stenosis

Chest radiograph and CT scan of a tracheal diverticulum A) Chest radiograph displays a large right-sided rounded lucency at the base of the neck

above the thoracic inlet. B) The CT scan confirms the nature of the lucency. It has the

typical features of a tracheal diverticulum.

TRACHEAL LESIONS

TRACHEAL AGENESIS (aplasia or atresia)

-rare condition

-At birth, there is immediate respiratory distress, and affected infants die shortly after birth from respiratory failure.

Diagnosis: trachea cannot be intubated despite adequate visualization of the larynx.

TRACHEAL STENOSIS

• Most commonly, tracheal stenosis presents as segmental stenosis anywhere along the trachea.

• Clinical signs: retractions, dyspnea, inspiratory and expiratory stridor, hypercarbia, and hypoxemia.

• Tracheal stenosis also should be considered in the differential diagnosis of recurrent, severe, or prolonged croup.

• The diagnosis can be suspected by demonstrating a fixed intrathoracic obstruction pattern on inspiratory-expiratory flow-volume curves and confirmed by bronchoscopy, CT scan, or magnetic resonance imaging (MRI) scanning

• TRACHEOMALACIA AND BRONCHOMALACIA • instability of the trachea or bronchi resulting from abnormally soft or pliable tracheal

cartilages. • With a delay in development of the supportive structures of large airways, excessive

narrowing may occur during exhalation, • Classification of the condition is based on the anatomic area that is involved (eg,

tracheomalacia, tracheobronchomalacia, bronchomalacia).

• Clinical signs: stridor, wheezing, respiratory distress, and hyperinflation, either diffuse or localized. Cough is usually absent, and growth is not impaired

• disappear during sleep but become evident during agitation or respiratory infections. • diagnosis is established by airway fluoroscopy or flexible fiberoptic bronchoscopy. • in most patients, no specific therapy is indicated. • In the most severe instances, supplemental oxygen, continuous positive airway

pressure, tracheoplasty, or tracheostomy may be necessary. • Recently, expandable stents have been inserted with some success.