2. 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
3. EA/TEF
▪ 1 per 2500 – 3500 live births
▪ Sporadic, non-syndromal
▪ Dysmotile distal esophagus
▪ Deficiency of tracheal cartilage
▪ 50% have 1 or more associated anomalies:
cardiac, anorectal, GU, vertebral/skeletal,
others
5. 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. .
6. 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
7. ASSOCIATED CONGENITAL ANOMALIES REPORTED IN
PATIENTS WITH ESOPHAGEAL ATRESIA
System affected
Musculoskeletal
Gastrointestinal
Cardiac
Genitourinary.
Potential anomalies
Hemivertebrae, radial dysplasia or
amelia, polydactyly, syndactyly, rib
malformations, scoliosis, lower limb
defects
Imperforate anus, duodenal atresia,
malrotation, intestinal
malformations, Meckel's
diverticulum, annular pancreas
Ventricular septal defect, patent
ductus arteriosus, tetralogy of
Fallot, atrial septal defect, single
umbilical artery, right-sided aortic
arch
Renal agenesis or dysplasia,
horseshoe kidney, polycystic kidney,
ureteral and urethral malformations,
hypospadias
8. 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.
9. FETAL MRI
▪ This 32 week
fetus had
esophageal
atresia and an
absent stomach,
resulting in
marked
polyhydramnios
10. 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.
11. CLINICAL DIAGNOSIS
▪ Prematurity
▪ Any excessively drooling (copious, fine, white, frothy bubbles of
mucus in the mouth and, sometimes, the nose).
12. 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
13. 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.
14. 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 .
15. 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.
16.
17.
18. ANATOMIC VARIATIONS-GROSS
TYPE A
6%
Atresia alone,
▪ no fistula
▪ Small stomach,
gasless abdomen
▪ Usually has a long
gap between the
esophagealends
Sx-
esophagastomy+gastrosto
my followed by gastric
19. ANATOMIC VARIATIONS-GROSS
TYPE B
▪ 2%
▪ Proximal tracheo-
esophageal fistula
▪ No distal fistula
Small stomach,
▪ gasless abdomen
▪ Often has a long
gap between the
esophagealends
20. 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).
22. THE CHEST RADIOGRAPH
▪ A plain radiograph will
confirm the tube has
not reached the
stomach
23. OPERATIVE REPAIR OF
ESOPHAGEAL ATRESIA
▪ Surgical repair is delayed (1-2days) in infants with low birth
weight, pneumonia or other major anomalies.
26. ANATOMIC VARIATIONS- GROSS
TYPE D
▪ l%
▪ Proximal and
distal fistulas
("double fistula")
▪ Primary closure of
fistula and end to end
anastomosis
27. ANATOMIC VARIATIONS
▪ 6%
▪ No atresia of
the esophagus
▪ Congenital
tracheoesophageal
fistula
"H" or "N" fistula
▪ GROSS type E
28. ▪ 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).
