Pediatric Surgery Clinic

Esophageal Atresia and

Tracheoesophageal Fistula
 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
 Esophageal atresia is a congenital
abnormality in which the midportion of
the esophagus is absent.

Incidence is between 1 in 3,570 and

1 in 4,500.
Anatomic Variations

85%

Most common

VOGTtype3(b)

GROSS type C
Anatomic Variations

6%
Atresia alone,
no fistula

Small stomach,

gasless abdomen
Usually has a long

gap between the

esophagealends
VOGT types 1 and 2

GROSS type A
Anatomic Variations
2%
Proximal tracheo-

esophageal fistula
No distal fistula

Small stomach,
gasless abdomen

Often has a long

gap between the

esophagealends
VOGT type 3(a)

GROSS type B
Anatomic Variations

l%
Proximal and
distal fistulas
("double fistula")
VOGT type 3(c)
GROSS type D
Anatomic Variations
6%
No atresia of
the esophagus
Congenital
tracheoesophageal
fistula
"H" or "N" fistula
GROSS type E
Physiologic effects of distal tracheoesophageal
fistula
1. Hyaline membrane disease may
necessitate higher ventilator
pressures, which encourage air to
pass through the distal fistula.
2. A distended abdomen elevates
and "splints" the diaphragm.
3. Gastric distension may result in
gastric rupture and
pneumoperitoneum.
4. Passage of air through a distal
tracheoesophageal fistula diminishes
the effective tidal volume.
. (B) 1. Aspiration of gastric juices
leads to soiling of the lungs and
pneumonia
2. Gastroesophageal reflux
3. Direction of gastric fluid
proximally through distal fistula.
4. Overflow of secretions or
inadvertent feeding may contribute
to aspiration and contamination of
the airway. .
 Associated Abnormalities

Incidence of Associated Anomalies in Esophageal Atresia.

Anomaly Frequency (%)
Congenital heart disease 25
Urinary tract 22
Orthopaedic (mostly vertebral and radial) 15
Gastrointestinal (e.g., duodenal
atresia,imperforate anus) 22

Chromosomal (usually trisomy 18 or 21) 7

Total with one or more associated 58
anomalies
 Associated Congenital Anomalies Reported in Patients with
Esophageal Atresia
System affected Potential anomalies
Musculoskeletal Hemivertebrae, radial dysplasia or
amelia, polydactyly, syndactyly, rib
malformations, scoliosis, lower limb
defects

Imperforate anus, duodenal atresia,

Gastrointestinal malrotation, intestinal
malformations, Meckel's
diverticulum, annular pancreas

Cardiac Ventricular septal defect, patent

ductus arteriosus, tetralogy of Fallot,
atrial septal defect, single umbilical
artery, right-sided aortic arch
Renal agenesis or dysplasia,
Genitourinary. horseshoe kidney, polycystic kidney,
ureteral and urethral malformations,
hypospadias
 DIAGNOSIS OF
ESOPHAGEAL ATRESIA

Antenatal Diagnosis (maternal

polyhydramnios, a small stomach, a
distended upper esophageal pouch, or
abnormal swallowing)
Diagnostic suspicion is increased when
abnormalities known to be associated with
esophageal atresia are identified.
Fetal MRI

This 32 week
fetus had
esophageal
atresia and an
absent stomach,
resulting in
marked
polyhydramnios
 Clinical Diagnosis

Prematurity
Any excessively drooling (copious, fine,
white, frothy bubbles of mucus in the
mouth and, sometimes, the nose).
Clinical Diagnosis

. (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.
The chest radiograph
A plain radiograph will
confirm the tube has
not reached the
stomach
The Gasless Abdomen
Absence of gas in the
abdomen suggests
that the patient has
either atresia without
a fistula or atresia
with a proximal fistula
only
Contrast studies
should be performed by
an experienced pediatric
radiologist, or after
transfer to the tertiary
institution, and with the
use of a small amount
(0.5 to 1 mL) of water-
soluble contrast. Care
must be taken to avoid
aspiration.
 Management
Measures should be taken to reduce the risk of
aspiration(continuous suctioning of the upper
pouch, the infant's head should be elevated).
In infants with respiratory failure, endotracheal
intubation should be performed.
Transfer to a major tertiary pediatric institution
is best not delayed .
 Summary of Preoperative
Investigations
A plain radiograph
Renal ultrasonography and echocardiography
are routine preoperative investigations
Endoscopy or a careful midesophageal contrast
study performed in a tertiary center. In some
centers, bronchoscopy is performed routinely in
all infants with esophageal atresia.
Operative Repair of Esophageal
Atresia
Surgical repair is delayed (1-2days) in
infants with low birth weight, pneumonia
or other major anomalies.
Operative Repair of Esophageal
Atresia
Operative Repair of Esophageal
Atresia
Tracheoesophageal fistula
without atresia (type E).

Respiratory difficulty
after feedings in a 3-day-
old boy. Barium
esophagogram clearly
shows an H-shaped
fistula between the
trachea and the middle
segment of the
esophagus (arrowhead).
Barium is filling the
bronchi of the right lower
lobe (arrows).
Tracheoesophageal fistula without
atresia (type E).
Esophagogram shows
a fistula (arrow)
arising from the
anterior portion of the
esophagus (e) and
passing cephalad to
the posterior portion
of the trachea (t).
Tracheoesophageal fistula without
atresia (type E).

Endoscopic diagnosis
 Congenital
tracheoesophageal
fistula
The end