Esophagus Atresia

Dwi Rezki Amalia

Medical Faculty of Lambung
Mangkurat University
 Embriology
The esophagus and trachea derive from the primitive foregut. During the
fourth and fifth weeks of embryologic development, the trachea forms as
a ventral diverticulum from the primitive pharynx (caudal part of the
foregut). A tracheoesophageal septum develops at the site where the
longitudinal tracheoesophageal folds fuse together. This septum divides
the foregut into a ventral portion, the laryngotracheal tube and a dorsal
portion (the esophagus). Esophageal atresia results if the
tracheoesophageal septum is deviated posteriorly. This deviation causes
incomplete separation of the esophagus from the laryngotracheal tube
and results in a concurrent tracheoesophageal fistula.
 Embriology
Esophageal atresia as an isolated congenital anomaly
may occur, rarely. In these cases, the atresia is
attributable to failure of the recanalization of the
esophagus during the eighth week of development and
is not associated with tracheoesophageal fistula. A
recent experimental animal model, wherein prenatal
exposure to adriamycin leads to esophageal atresia
and tracheoesophageal fistula, may increase our
understanding of the embryogenesis of these
malformations.
 Definition
Esophageal atresia refers to a congenitally
interrupted esophagus.
One or more fistulae may be present between the
malformed esophagus and the trachea.
 Etiology
The exact cause of esophageal atresia is still unknown, but it appears to
have some genetic components. Up to half of all babies born with
esophageal atresia have one or more other birth defects, such as:
trisomy 13, 18 or 21
other digestive tract problems, such as diaphragmatic hernia, intestinal
atresia or imperforate anus
heart problems, such as ventricular septal defect, tetralogy of Fallot or
patent ductus arteriosus
kidney and urinary tract problems, such as horseshoe or polycystic
kidney, absent kidney or hypospadias
muscular or skeletal problems
Esophageal atresia and tracheoesophageal fistula are also often found
in babies born with VACTERL syndrome. This is a non-random collection
of abnormalities that may also involve the spine, heart, lower digestive
tract, kidneys and limbs. Not all babies born with VACTERL syndrome
have abnormalities in all of these areas.
 There are also molecular and
morphogenetic factors related to EA, such
as apoptosis, the Sox2, Shh, Gli-2, Gli-3,
Pcsk5 and FOX genes and the transcription
factors Nkx2.1 and Tbx4. A failure in the
expression of these genes or in the apoptotic
programs that they regulate is responsible for
EA. However, a whole understanding of these
processes remains incomplete. In addition,
environmental factors have been suggested to
increase the risk for the development of
tracheoesophageal anomalies. Further studies
are required for a universally accepted
explanation for the pathophysiology of EA/TEF.
 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 and distal TEFs (<1%)
Type E - TEF without esophageal
atresia or so-called H-type fistula
(4%)
Type F - Congenital esophageal
stenosis (<1%) (not discussed in
this article)
Classification
 Epidemiology
The incidence of esophageal atresia is 1 case in
3000-4500 births. This frequency may be
decreasing for unknown reasons. Internationally,
the highest incidence of this disorder is reported
in Finland, where it is 1 case in 2500 births.
Pre Operative Classification
There are three primary
classifications of preoperative risks
regarding EA: the Waterston,
Montreal and Spitz classifications
 Waterston Classification
According to Waterston, the risk factors to be considered
are birth weight (BW), the presence or absence of
pneumonia and complications from associated congenital
anomalies.

In this classification scheme, patients are categorized into :

group A (BW > 2500 g, with no other complications)
group B (BW between 1800 g and 2500 g with no other
complications or BW > 2500 g with moderate
pneumonia/congenital anomaly)
group C (BW <1800 g, with no other complications or BW >
2500 g with severe pneumonia/severe congenital anomaly)
 Montreal Classification
In the Montreal classification scheme for EA, factors
such as dependence on mechanical ventilation
(MV) and associated congenital anomalies are
considered to be of high prognostic significance.
Patients are classified into :
group (isolated major anomaly, isolated
dependence on Mechanical Ventilation or the
presence of non-significant anomalies)
group (presence of severe congenital anomalies
or dependence on Mechanical Ventilation
associated with one major anomaly).
 Spitz Classification
Spitz drafted the most recent classification
method by associating BW and cardiac
anomalies (CA) as risk factors for EA.
In this classification scheme, patients are
divided into :
group (BW > 1500 g, without CA)
group (BW < 1500 g or the presence of
CA)
group (BW < 1500 g with CA)
 Sign and Symptom
Mother : polyhydramnion

Baby : The first signs of esophageal atresia are usually

clearly seen very soon after birth. The most common
are:
frothy white bubbles in your babys mouth
Coughing or choking when feeding
vomiting
blue color of the skin, especially when your baby is
feeding
difficulty breathing
very round, full abdomen
 Diagnosis
Before birth, a mother's ultrasound may show too much amniotic
fluid. This can be a sign of EA or other blockage of the baby's
digestive tract.
The disorder is usually detected shortly after birth when the
infant tries to feed and then coughs, chokes, and turns blue.
If EA is suspected, the health care provider will try to pass a small
feeding tube through the infants mouth or nose into the stomach. If
the feeding tube cant pass all the way to the stomach, the infant
will likely be diagnosed with EA.

An x-ray is then done and will show any of the following:

An air-filled pouch in the esophagus.
Air in the stomach and intestine.
If a feeding tube has been inserted before the x-ray, it will appear
coiled in the upper esophagus.
Usg : poly-hydramnion on
mother
 Management
Management plans for a delayed repair of the esophageal atresia
may include placing a 10-French Replogle double-lumen
tube through the mouth or nose well into the upper pouch to
provide continuous suction of pooled secretions from the proximal
portion of the atretic esophagus. The baby may be positioned
in the 45 sitting position. Prophylactic broad-spectrum
antibiotics (eg, ampicillin and gentamicin) may be used.
General supportive care and total parenteral nutrition are
needed.
 Atresia Esophagus Repair
Foker tecnique for long gap esophagus proximal
and distal. (difficult to replicate by other surgeon)
 Atresia Esophagus Repair

Other option for long gap esophagus proximal and

distal gastrostomy followed by gastric pull-up,
colonic transposition and jejunum transposition.

gastrostomy followed by gastric Colonic

pull-up transposition
Atresia Esophagus Repair

Jejunal transposition
 Non surgical treatment
Magnetic compression method ???
 Maintenance After Esophageal
Repair
Using G-tube for feeding
Make a hole in the neck to drain the
mucous and saliva
 Complication
Any attempt at feeding could cause
aspiration pneumonia as the milk
collects in the blind pouch and
overflows into the trachea and lungs.
Furthermore, a fistula between the
lower esophagus and trachea may
allow stomach acid to flow into the
lungs and cause damage. Because of
these dangers, the condition must be
treated as soon as possible after
Post Operative Complication
Post operative complications sometimes
arise, including a leak at the site of closure
of the esophagus. Sometimes a stricture, or
tight spot, will develop in the esophagus,
making it difficult to swallow. This can
usually be dilated using medical instruments.
In later life, most children with this disorder
will have some trouble with either swallowing
or heartburn or both. Esophageal dismotility
occurs in 75-100% of patients.
 Prognosis
Statistics regarding mortality in esophageal atresia are constantly changing and
improving. One must consider the classification system used in reporting such
statistics.

Mortality relative to the Montreal classification is as follows:

Class I - Mortality of 7.3%
Class II - Mortality of 69.2%

Mortality relative to the Spitz grouping is as follows [17] :

Group I - Mortality of 3%
Group II - Mortality of 41%
Group III - Mortality of 78%

Mortality relative to the Waterston categorization is as follows [18] :

Category A - Mortality of 0%
Category B - Mortality of 4%
Category C - Mortality of 11%

Fetuses with prenatal diagnoses of esophageal atresia seem to have a worse

prognosis. The cohort of babies in whom esophageal atresia is detected prenatally
has a 75% mortality, whereas the cohort of babies in whom esophageal atresia is
not detected prenatally has a 21% mortality. Babies who survive have varied
morbidities related to any of the associated anomalies and complications. However,