FRCA Notes

Tracheo-Oesophageal Fistula

This topic is explicitly mentioned by the Intermediate curriculum, but is yet to form the basis of a CRQ/SAQ.

Resources

  • Tracheo-oesophageal fistulae and oesophageal atresia are inter-related conditions with an incidence of 1 in ∽3,500
  • The most common patterns are:
    • Oesophageal atresia with distal (lower) tracheo-oesophageal fistula (86%)
    • Isolated oesophageal atresia (8%)
    • Isolated tracheo-oesophageal fistula (4%)
    • Oesophageal atresia with proximal (upper) tracheo-oesophageal fistula (2%)

Gross (anatomical) classification

  • Describes six anatomic variations of TOF ± oesophageal atresia
  • The most common lesion is oesophageal atresia with a distal tracheo-oesophageal fistula

Spitz classification

Group Features Survival rate
I Birth wt. >1.5kg and no major cardiac anomaly 98.5%
II Birth wt. <1.5kg or major cardiac anomaly 82%
III Birth wt. <1.5kg and major cardiac anomaly 50%


  • The aetiology is poorly understood
  • Almost certainly some genetic component:
    • Twin concordance is 2.5%
    • Specific chromosomal anomalies predispose e.g. trisomy 18, trisomy 21

Associations

  • ∽50% of infants with oesophageal atresia/tracheo-oesophageal fistula have associated congenital anomalies

  • Associated anomalies include:
    • Congenital cardiac disease (35%); particularly anomalies of the aortic arch
    • VACTERL (25%)
    • Vertebral anomalies (23%)
    • Renal agenesis (10%)
    • DiGeorge syndrome
    • Pierre-Robin sequence
    • Down's syndrome
    • Holt-Oram syndrome

Antenatal

  • Not easily diagnosed antenatally; only 37% are diagnosed antenatally
  • Ultrasound examination may demonstrate a small or absent stomch bubble, or polyhydramnios from oesophageal obstruction, though this is a non-specific finding
  • If detected antenatally, delivery should take place in a specialist centre with neonatal surgical facilities

Postnatal

  • More commonly presents postnatally, with clinical features of:
    • Excessive salivation
    • Repeated episodes of coughing/choking
    • Choking and cyanosis with feeding
    • Aspiration pneumonia ± sepsis

  • Diagnosis is confirmed by:
    • Inability to pass NG tube/catheter more than 10cm into the oesophagus
    • Chest radiograph demonstrating catheter in the blind upper pouch

  • Referral to tertiary centre for ongoing neonatal management
  • There is a high risk of aspiration through the fistula, so management should be to:
    • Stop all oral feeding; may need empirical placement of a PEG to allow feeding
    • NG tube i.e. double-lumen Replogle tube to allow both regular irrigation and suction of the oesophageal pouch
      • The tube is often placed on continuous suction (-2 to -3kPa) and flushed every 15mins
    • H2RA
    • Semi-upright positioning
    • IV fluid maintenance

Surgical repair

  • Surgical exploration and repair should ideally occur within 24hrs, although staged repair is sometimes used
  • Right posterolateral thoracotomy or VATS, but no lung deflation required as extra-pleural technique used and surgeons just retract/compress tissue
  • Surgical retraction or compression of the mediastinum can cause:
    • Desaturations, which may require manual ventilation and/or recruitment manoeuvres
    • Reduced cardiac output
  • The oesophagus is detached from the trachea and anastamosed to the other blind end
  • The trachea is repaired at the point of oesophageal insertion
  • Surgeons site a trans-anastomotic NG tube

  • If oesophageal anastomosis is not possible e.g. due to a large gap between the upper and lower oesophageal pouches, then:
    • Gastrostomy i.e. for PEG feeding
    • ± Oesophagostomy to allow secretion drainage without the need for Replogle tube

Perioperative management of the child tracheo-oesophageal fistula repair


History and examination

Investigations

  • Bloods inc. FBC, U&E, Group and save
  • CXR to check for aspiration
  • TTE to check for cardiac abnormalities
  • Renal ultrasound to screen for renal abnormalities
  • If lumbar/sacral dimple then image back to ensure normal anatomy prior to administering neuraxial analgesia

Optimisation

  • Correction of fluid deficiency
  • Correction of hypoglycaemia and electrolyte imbalance
  • Antibiotics e.g. for aspiration
  • Operative management should take place in a neonatal specialist surgical centre, ideally within 24hrs or in the first few days of life
  • Delays risk aspiration pneumonitis due to aspiration of saliva or stomach acid

  • Requires close communication with surgeons owing to shared airway

Monitoring and access

  • AAGBI
  • Adequate IV access
  • Arterial line as risk of haemodynamic instability e.g. due to mediastinal shift during surgical retraction

Anaesthetic induction & bronchoscopy

  • A gas induction, spontaneously breathing induction technique is preferred
  • One should avoid bag-mask ventilation or CPAP, to prevent inflating the stomach via the fistula

  • Bronchoscopy may be performed to confirm diagnosis, assess size and position of the fistula
  • Spontaneous ventilation via the ventilating side port of the bronchoscope is possible

  • Following bronchoscopy, an 3.0 - 3.5mm ETT is placed
  • Endobronchial intubation is intentionally performed
  • The tube is then withdrawn until bilateral breath sounds are heard
  • This ensure the tip of the ETT is distal to the fistula site
  • Bevel placed posteriorly i.e. to occlude the fistula while allowing ventilation of both longs

  • Ongoing ventilation on NICU, although early extubation if repair appears satisfactory and no other indication for artificial ventilation
  • Maintain hydration with IV fluid, although aim for trans-anastomotic NG feeding by 48hrs post-operatively
  • Regular suctioning of the trans-anastamotic NG tube
  • Ongoing antibiotics for 48hrs
  • Monitor for complications (see below)

  • Gastro-oesophageal reflux (35 - 74%)
    • The most common long-term problem, especially if there was delayed primary repair
    • Probably reflects abnormalities of mesenteric plexus development and impaired LOS tone
    • Standard management: H2RA, PPI, prokinetics
    • Up to 45% of patients will require fundoplication

  • Anastamotic leak (10-20%)
    • Manifests as early pneumothorax and saliva draining from chest drain
    • 50% will develop an oesophageal stricture

  • Tracheomalacia (13%)
  • Repeated aspiration (23%)
  • Recurrent pneumonia (19%)
  • Recurrent tracheo-oesophageal fistula (9 - 13%)
  • Anastamotic strictures (10%)