ESOPHAGEAL ATRESIA AND TRACHEOSOPHAGEAL FISTULA

1. WELCOME

2. OESOPHAGEALATRESIA &
TRACHEOESOPHAGEAL
FISTULA
Dr. Raghu Nath Karmaker
Phase B, Resident
Paediatric Surgery
MMCH

3. Objectives:-
• Definition.
• Epidemiology.
• Embryology of Esophagus & Trachea.
• Types & Classifications.
• Associated anomalies.
• Pathophysiology.
• Diagnosis & Investigations
• Management
• Complications

4. Esophageal Atresia
Congenital discontinuity of esophageal lumen
is known as esophageal atresia.

5. Incidence
 1 per 2500- 4500 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.

6. Embryology
The exact cause of esophageal atresia is
not known but both the esophagus and
trachea originate from the primitive
foregut and this is thought to be a
separation defect.

8. Successive stages in the development of
the Tracheoesophageal septum during
embryologic 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 has completely
formed.
(D) If the tracheoesophageal
septum deviates posteriorly,
esophageal atresia with a
tracheoesophageal fistula
develops.

9. Classification
According to the system formulated by Gross, the types of
esophageal atresia and their approximate incidence in all
infants born with esophageal anomalies are as follows:-
• Type A - Esophageal atresia without fistula or so-called
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 so-called H-
type fistula (4%)
• Type F - Congenital esophageal stenosis (<1%)

11. Associated Anomalies: Approximately 10-15%
babies with oesophageal atresia may have a group of
anomalies known as VACTREL.
(VACTERL)
• Vertebral defects.
• Anorectal malformations.
• Cardiovascular defects.
• Tracheo-Esophageal defect.
• Renal anomalies.
• Limb deformities.
Co-existing anomalies are highest in the group of
oesophageal atresia without fistula (58%) and least
common in H type fistula (27%)

12. Associated Congenital Anomalies Reported in
Patients with Esophageal Atresia

13. Pathophysiology
• A fetus with esophageal atresia cannot effectively swallow
amniotic fluid.
• A fetus with esophageal atresia and a distal TEF, fetus is not
swallowing amniotic fluid Polyhydramnios Premature
labour
• Aspiration of saliva or milk, if the baby is allowed to suckle, can
lead to an aspiration pneumonitis.
• 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.

14. DIAGNOSIS OF ESOPHAGEAL
ATRESIA
• Antenatal Diagnosis of esophageal atresia
is done during routine maternal
Ultrasonography
• Polyhydramnios
• A small stomach
• A distended upper esophageal
pouch

16. At Birth:
(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.

17. 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

18. Clinical Diagnosis:
• Prematurity
• Any excessively 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.

19. Investigations
1st line Investigations:-
• A plain radiograph
• Esophagogram
• Endoscopy
Others:-
• Renal Ultrasonography
• Echocardiography
• Bronchoscopy
• Laboratory investigations.

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

21. Renal ultrasonography
is used to evaluate associated kidney anomalies,
ureteral anomalies, or both.

22. Echocardiography
Who have clinical signs of Cardiovascular disease.

23. Laboratory Studies:
• CBC count
• Electrolyte levels
• Venous gas concentrations
• BUN & Serum Creatinine levels
• Blood glucose level
• Serum calcium level
• Blood grouping & Rhesus typing

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

25. 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.

26. Timing of surgical correction
(A) Immediate Primary Repair
Following criteria are useful in making the decision for
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 oesophageal
pouch less than 2.5 cm

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

28. (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 oesophageal
pouchs viz.in type 1 oesophageal
atresia,where gap is usually more than 3-4
cm.

29. 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.

30. Operation Classic Repair
Curved incision is made 1cm below
the inferior angle of the scapula
Division of the S/C Tissue and muscles is carried
out with diathermy to minimize blood loss

31. The azygos vein and the posterior
mediastinum should be exposed
Mobilization of lower esophagus
and division of fistula

32. Fistula tract is identified and cut
and tracheal site is closed by 5/0
polypropylene sutures
Identification of upper pouch

33. Anastomosis of upper pouch with lower
pouch under tension

34. Wound closure

35. Complications
(A) Early :-
• Pneumothorax
• Anastomotic leakage
• Recurrence of fistula
• Pulmonary atelectasis or infection
(B) Late:-
• Anastomotic Stricture
• Gastro esophageal reflux (GER)
• Tracheomalacia
• Disordered Oesophageal Peristalsis(Dysphagia)

36. Prognosis

37. 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.

38. Thank You