ESOPHAGEAL ATRESIA AND

TRACHEO-ESOPHAGEAL
FISTULA
(EA &TEF)
Group 1
Introduction
Esophageal atresia (EA) is the congenital malformation that
represent the failure of the esophagus to develop a continuous passage
upto the stomach
Tracheo esophageal fistula (TEF) is the congenital malformation where
the trachea and esophagus fails to separate into distinct structures and a
passage is created between the two.
Epidemiology
Occurs in 1:3000 to 1:4500 live births.
Equal gender incidence
EA associated with prematurity.
A history of maternal polyhydroamnios is present in approx. 50% of
infants with the defects.
Often present with VATER/VACTERL syndromes.
Possible influences
Inherent genetic factor , teratogens , Environment factor
Prematurity & low birth weight.
Pathophysiology
Upper part of esophagus is developed from retropharyngeal segment and the lower part from pregastric
segment of the first part of primitive gut.
At 4- 5weeks of gestation the laryngo-tracheal groove is formed.
Two longitudinal furrows develop and separate the respiratory premordium from esophagus.
Deviation or altered cellular growth of the septum results in formation of fistula between esophagus and
trachea.
Classification
Type A: It consists of blind upper and blind lower
esophageal segment without any tracheo-
esophageal fistula (3.7 to 7%).
Type B: It involves fistula from trachea to upper
oesophageal segment (0.8%).
Type C:It type in which proximal esophageal segment
terminates in a blind pouch and the distal esophageal
segment is connected to trachea or primary bronchi
by a short fistula at or near tracheal bifurcation (86%).
Type D : It involves fistula from trachea to both upper
and lower esophageal segments (0.7 to 6%).
Type E : It refers to H type TEF which are having
otherwise normal trachea and oesophagus that are
connected by a fistula (4.4- 7%)
RFs
Mother with prolong exposure to contraceptive pills.
Exposed to progesterone & estrogen during pregnancy.
Infant of Hypothyroid, diabetic mother and after intrauterine
exposure to thalidomide.

Association
Overall incidence50-70%
Anatomical:
Cardiovascular35%
Genitourinary24%
Gastrointestinal24%
Neurologic12%
Musculoskeletal20%
Structural Association:
VECTRAL association: 20%
Vertebral: 17% , Anal12% , Cardiac20% , Renal16% ,Limb5%
CHARGE association:( Cloboma, Heart defect, Atresia choanae, developmental Retardation, Genital hypoplasia, Ear
deformity).
Schisis association:(omphalocele, neural tube defect, cleft lip & palate and genital hypoplasia).
Other less common anomaly:
Choanal atresia 5.2%,
Facial cleft 7.2%,
Abdominal wall defect4.3%,
Diaphragmatic hernia 2.9%

Diagnosis
Prenatal detection:
USG relies on finding of an anechoic area in the middle of the
neck, small or absent stomach bubble and associated
maternal polyhydroamnios; predictive value 20-40%,
A baby with single umbilical artery may also be a suspect.
Fetal MRI may be a useful adjunct for diagnosis
Postnatal:
Most infants with EA are symptomatic in the first few hours.
earliest sign is excessive salivation, typically first feeding is
followed by regurgitation, choking and coughing other
features are cyanosis with or with out feeding, respiratory
distress, inability to swallow and inability to pass a feeding
tube in the stomach.
If distal fistula present the abdomen distends.

Cont.
A sterile 6-8 fr. red rubber catheter is passed orally. If it gets arrested at
10cm from the gum margin, the diagnosis of EA is established.(type A) If a
polyurethane catheter is used, it may get coiled up in upper part of
esophagus and accumulated secretions may be mistaken for stomach
contents. But the later should have acidic pH.
If the catheter lies in the upper blind pouch and there is a gas bubble in the
abdomen, diagnosis of EA & TEF is Confirmed (type c, d & e).
If the catheter fails to pass, an upright plain X-ray chest and abdomen
(combined) is taken with 5-10ml air injected through catheter to give better
contrast.
Cont.
If the catheter lies in esophageal pouch and there is no
gas bubble in the abdomen, diagnosis of pure EA is
confirmed (type a & b).
Lungs may show atelectasis and aspiration pneumonia
H- type fistula is seldom recognized early in life and
recurrent attacks of pneumonia may be a presenting
feature. Confirmation of diagnosis is difficult and
necessitates esophagoscopy and bronchoscopy.
Treatment
Medically :
The infant is immediately deprived of oral intake (NPO)
Start IV fluids.
Place infant in the position least likely to cause aspiration of either mouth or
stomach secretions i.e. supine with head end raised to 45 or head turned to
one side.
Removal of secretions from the mouth and upper pouch requires frequent or
continuous suction with Replogles catheter every 5 min gently with pressure of
50 cm of HO.
Broad spectrum antibiotic therapy is often instituted.
Cont.
SURGICAL MANAGEMENT
Immediate primary repair: indications are-
no pulmonary complications
weight of the child >2 to 2.5kgs
no major congenital malformations
healthy baby
gap between distal and proximal esophagus < 2cm
Delayed surgical intervention: indications are-
pneumonia
sepsis
congenital malformations
severe prematurity
gap > 2.5cm.
Cont.
STAGES OF OPERATION:
left cervical esophagostomy (to allow drainage of saliva through stoma of neck) and
gastrostomy
thoracotomy and ligation of TEF
Replacement of the gap between proximal and distal esophagus at 6-8 months of
age by isolated segment of colon or by gastric tube.
Procedure :
Right posterolateral thoracotomy through 4
th
intercoastal space
Proximal esophageal pouch is mobilised after ligating vena azygous
Distal esopahgus is not mobilized due to its fragile blood supply. Fistula with trachea
is identified and tracheal end of fistula is closed.
End to end anastomosis of distal and proximal esophagus is done by single layer of
4/0 synthetic sutures
Sterile feeding tube of size 6-8 fr is left in stomach, passed through nose.
Chest is closed with chest tube drain
Outcome
Between 1939 to 1969 Dr. Haight did 284 infants with EA and report
as 52% overall survival rate.
85-95% overall survival.
H-type fistula have less association.
VECTRAL association have high mortality .
Increased risk of death & long term morbidity include:
1.Lower birth wt (<1500 g) & prematurity.
2.Major CHD.
3.Sever associated anomaly & ventilator dependent
4.Long gap EA.


Complications
Early:
Anastomotic leak
Anastomotic stricture
Recurrent TEF
Late:
GER
Tracheomalacia
Disordered esophageal peristalsis

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