Esophageal atresia & tracheo-esophageal fistula

2. DR. TAREQ MORSHED
RESIDENT, PHASE- A
CARDIOVASCULAR & THORACIC SURGERY
BSMMU.
Oesophageal Atresia &
Tracheoesophageal
Fistula

3. Objectives
 Definition.
 Epidemiology.
 Embryology .
 Types.
 Associated anomalies.
 Pathophysiology.
 Diagnosis & Investigations
 Management
 Complications

4. Esophageal Atresia
Congenital discontinuity of esophageal
lumen is known as esophageal atresia.
Tracheoesophageal Fistula
Abnormal communication between trachea
and esophagus.

6. Epidemiology
 1 per 2500 – 3000 live births.
 50% have 1 or more associated anomalies:
Cardiac, Anorectal , Genitourinary,
vertebral/skeletal & others.
 2% risk of recurrence is present when sibling
is affected.
 Increased in advanced maternal age.

7. Embryology
Both the esophagus and trachea
originate from the primitive foregut
and this is thought to be a separation
defect.

8. Stages in the development
(A) The laryngotracheal diverticulum forms as a
ventral outpouching from the caudal part of the
primitive pharynx.
(B) Longitudinal tracheoesophageal folds begin to
fuse toward the midline to eventually form the
tracheoesophageal septum.
(C) The tracheoesophageal septum completely forms.

12.  At week 4,the tracheoesophageal
diverticulum developed to
tracheoesophageal septum. There is a
failure to separation in the septum
leading to fistula.
 During week 8,the primitive gut
failure of recanalization leads to
atresia.

14. Classification
According to the system formulated by Gross:-
 Type A - Esophageal atresia without fistula or pure
esophageal atresia (10%)
 Type B - Esophageal atresia with proximal TEF (<1%)
 Type C - Esophageal atresia with distal TEF (85%)
 Type D - Esophageal atresia with proximal & distal TEFs
(<1%)
 Type E - TEF without esophageal atresia or H-type fistula
(4%)
 Type F - Congenital esophageal stenosis (<1%)

16. Associated Anomalies
 Vertebral defects.
 Anorectal malformations.
 Cardiovascular defects.
 Renal anomalies.
 Limb deformities.
25% of all patients with esophageal atresia

17. Associated Congenital Anomalies Reported in
Patients with Esophageal Atresia

18. Pathophysiology
 A fetus with esophageal atresia cannot effectively
swallow amniotic fluid.
Polyhydramnios Premature labour
 Aspiration of saliva or milk, if the baby is allowed
to suckle, can lead to an aspiration pneumonitis.

20.  In a baby with esophageal atresia and a distal TEF,
the lungs may be exposed to gastric secretions.
 Also, air from the trachea can pass down the distal
fistula when the baby cries, strains, or receives
ventilation. This condition can lead to over distension
of abdomen which ultimately may cause acute gastric
perforation, which is often lethal.

21. DIAGNOSIS
 Antenatal Diagnosis of esophageal atresia is done
during routine maternal Ultrasonography

 Polyhydramnios
 A small stomach
 A distended upper esophageal pouch

22. Clinical Diagnosis
 Prematurity
 Excessive drooling (copious, fine, white, frothy
bubbles of mucus in the mouth and
sometimes, the nose)
 If these signs go unnoticed the first feed is almost
always accompanied by coughing, choking and
cyanosis.
 After birth these babies often appear to blow bubbles
when attempting to swallow mucous and saliva.
 Sign & Symptoms of respiratory distress due to
aspiration pneumonia.

23. (A) Diagnosis of esophageal atresia is confirmed when
a 10-gauge (French) catheter cannot be passed beyond
10 cm from the gums.
(B) A smaller-caliber tube is not used because it may
curl up in the upper esophageal segment, giving a false
impression of esophageal continuity.

25. Investigations
1st line Investigations:-
 Plain radiograph
 Esophagogram
 Endoscopy
Others:-
 Renal Ultrasonography
 Echocardiography
 Bronchoscopy
 Laboratory investigations.

26. A plain radiograph
Fig (A) Orogastric catheter
in the blind esophageal
pouch & presence of air in
the stomach.
Fig (B)The radiopaque tube
in the blind esophageal
pouch & absence of air in the
stomach

27. esophagogram (Contrast X-Ray)
Plain x-ray with contrast in the upper esophagus above the atresia.

28. Renal ultrasonography
To evaluate associated kidney anomalies, ureteral
anomalies, or both.

29. Echocardiography
Who have clinical signs of Cardiovascular disease.

30. Management
Aims of management:-
 Prevention of Aspiration Pneumonia & its
consequences.
 Establishment of communication between
proximal & distal end of esophagus.
 Separation of esophagus from trachea.

31. Preoperative management
 The oropharynx should be cleared.
 The infant's head should be elevated.
 IV fluids & Nutrition.
 Oxygen therapy.
 In infants with respiratory failure,
endotracheal intubation should be
performed.
 Broad Spectrum Antibiotics.

32. Timing of surgical correction
(A) Immediate Primary Repair
1. No pulmonary complications, viz. severe
pneumonia.
2. An arterial PaO2> 60mm Hg in room air.
3. Not associated with major congenital
malformations which are compatible with
life.
4. Gap between proximal and distal esophageal
pouch less than 2.5 cm.

33. (B) Delayed surgical intervention
1. Associated with pneumonia, sepsis or
cardiac malformation.
2. Severe prematurity
3. The gap between proximal and distal
pouch is more than 2.5cm.

34. (C) Staged operation:-
The indications are:
1. If primary repair can not be done due to
reasons mentioned in delayed surgical
repair
2. Failed primary repair.
3. Long gap between two esophageal pouches
viz.in type 1 esophageal atresia, where gap
is usually more than 3-4 cm.

35. Stages of surgical correction
 Left cervical esophagostomy and feeding
gastrostomy immediately after birth.
 Thoracotomy & Ligation of tracheoesophageal
fistula.
 Replacement of the gap between proximal and
distal esophagus by isolated vascularized
segment of colon or by gastric tube.

36. Complications
(A) Early :-
 Pneumothorax
 Anastomotic leakage
 Recurrence of fistula
 Pulmonary atelectasis or infection
(B) Late:-
 Anastomatic Stricture
 Gastroesophageal reflux(GER)
 Tracheomalacia
 Disordered esophageal Peristalsis(Dysphagia)

37. Prognosis

38. Take Home Message
 Early diagnosis allows time and place of delivery to
be planned so that early pediatrics surgical
correction and neonatal care is available.
 This also gives time for preparation and education of
the parents.

39. Thank You!