TRACHEOESOPHAGEAL FISTULA:

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• 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

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• 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.

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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.

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R/x

• surgical ligation of the fistula

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• 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

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Types of tracheoesophageal fistulas

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Congenital tracheal stenosis
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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.
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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.

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• 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

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• 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.

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