OESOPHAGEAL ATRESIA
Dr. Sam Chippington
Martin Churchill-Coleman
Bringing excellence to life

Atresia - is the absence of a usual opening in a
tubular structure

Embryology – the exact cause of oesophageal
atresia is not known but both the oesophagus
and trachea originate from the primitive foregut
and this is thought to be a separation defect.

Occurrence – Approximately 1:4000 live births
Bringing excellence to life
PRENATAL DIAGNOSIS

Ultrasound diagnosis is indicative rather than absolute

Obstetric Ultrasound may show unexplained
polyhydramnios

Absent stomach or small stomach

Early diagnosis allows time and place of delivery to be
planned so that early paediatric surgical correction and
neonatal care is available. This also gives time for
preparation and education of the parents.
Bringing excellence to life
Normal foetal stomach
Gastric
bubble
Spine
Umbilical
cord
Cross sectional view of fetal abdomen with vertebral column to left of the
image, the gastric bubble above and the umbilical cord to the right.
Bringing excellence to life
Absence of foetal stomach bubble
Absence of
gastric
bubble
Spine
Bringing excellence to life
Umbilical
cord
Small foetal stomach bubble
Umbilical
vein
small gastric
bubble
“collapsed”
Bringing excellence to life
Spine
FOETAL MRI

Some centres carry out 3rd
trimester MRI – using single
shot rapid acquisition T2
weighted images – the
diagnosis is considered
positive if the upper
oesophagus is dilated and the
lower oesophagus is not
demonstrated
Bringing excellence to life
CLINICAL DIAGNOSIS

After birth these babies often appear
to blow bubbles when attempting to
swallow mucous and saliva

If these signs go unnoticed the first
feed is almost always accompanied by
coughing, choking and cyanosis.

In these cases a replogle tube is
passed – typically this will reach 1012cm before reaching the blind end of
the upper pouch.
Bringing excellence to life
Bringing excellence to life
Bringing excellence to life
TYPES OF OESOPAGEAL ATRESIA,
TRACHEAL FISTULA OR
COMBINATION OF THE TWO

5 TYPES OF MALFORMATION

87% have oesophageal atresia with
distal tracheo-oesophageal fistula

8% Isolated atresia with no fistula

4% Isolated tracheo-oesophageal a
‘H’ Type fistula with no atresia

1% Atresia with upper pouch fistula

1% Atresia with upper and lower
pouch fistula
Bringing excellence to life
NEONATAL ABDOMEN CHEST
RADIOGRAPHS

CXR – will normally demonstrate a
dilated upper pouch containing a
replogle tube

ABDOMEN – a normal bowel gas
pattern will be visible where there is
connection via fistula to the lower
oesophagus

Therefore there are only two types of
malformation where the abdomen will
be gasless – the isolated atresia
without fistula and the fistula to the
upper pouch of the oesophagus
Bringing excellence to life
Demonstrating an H type
Fistula
SPINE
HEAD
Bringing excellence to life
FEET
DELAYED DIAGNOSIS

Diagnosis of four of the types is easily made
after attempting to feed a new baby

However the ‘H’ Type Fistula because there is
no atresia may not be identified for many years

Often ‘H’ Type fistula present with recurrent
chest infections or more dramatically when
diving into a swimming pool

‘H’ Type fistulae are demonstrated by
oesophagogram – taking a series of images
with the patient prone and injecting water
soluble contrast through a naso-gastric tube
which is withdrawn from the stomach to the
mouth so that the whole length of the
oesophagus is interrogated.
Bringing excellence to life
HISTORY OF TREATMENT

Oesophageal atresia is not compatible with life unless it is
surgically repaired

Before 1939 when the first successful repair took place this
diagnosis was fatal

Since that time surgical techniques and anaesthetics have
improved and the great majority of these patients survive to
lead a normal life

Most patients nowadays undergo a primary anastomosis in the
first few days of life.

Large gaps between the upper and lower pouches are
problematic and used to be bridged by colon transplants and J
tubes fashioned from the lesser curvature of the stomach.
Bringing excellence to life
ASSOCIATED DEFECTS

Associated congenital
abnormalities are discovered in
approximately one half of
patients with oesophageal
atresia

The acronym VACTERL has
been used to describe the
condition of multiple anomalies
in these infants
Bringing excellence to life
 VERTEBRAL – Hemivertebrae and scoliosis
 ANORECTAL MALFORMATION
 CARDIAC DEFECTS – VSD, Patent Ductus Arteriosus and
Tretralogy of Fallot
 TRACHEO
 ESOPHAGEAL (American esophageal)
 RENAL TRACT – Ectopic kidneys, horseshoe, duplex
systems, renal agenesis, urethral malformations and
hypospadias
 LIMB defects – radial agenesis most common
Bringing excellence to life

In addition to the vacterl
anomolies there is increased
incidence of Duodenal atresia,
malrotation, intestinal
malformations, Meckel’s
diverticulum and annular
pancreas.
Bringing excellence to life
Bringing excellence to life
Bringing excellence to life
Hemivertebra
Air filled upper
oesophagus
Replogle tube
Bringing excellence to life
 Oesophageal Atresia

First described 1703
Bringing excellence to life
 Oesophageal Atresia

First described 1703
 1936 – First surgical repair
 1939 – First successful surgical
repair

Gastrostomy inserted, delayed
surgical repair
Bringing excellence to life
 Oesophageal Atresia

First described 1703
 1936 – First surgical repair
 1939 – First successful surgical
repair

Gastrostomy inserted, delayed
surgical repair
 1941 – First primary
oesophageal anastomosis and
ligation of tracheo-oesophageal
fistula
Bringing excellence to life
 1945-1965

Focus on successful repair in
otherwise healthy neonates (and
birth weight > 2.5kg)
 By 1965

Success rate 80-90%

Refinement of the procedure
 1965- 1990
 Low birth weight
 Co-existing morbidity
Bringing excellence to life
Bringing excellence to life
Surgical
Anastomosis
Bringing excellence to life
 Newest approach
 Fewer musculoskeletal
sequelae




Winging of the scapula
Asymmetrical chest wall
Thoracic scoliosis
Breast maldevelopment
Bringing excellence to life
Bringing excellence to life
 Primary oesophageal
anastomosis

Within 48 hours of birth
 Delayed primary oesophageal
anastomosis


Unfit for surgery
Other associated anomalies
e.g.cardiac
Bringing excellence to life
 Staged repair

Long gap oesophageal atresia
 ‘Stretch’ upper pouch
 Mechanical
 Electromagnetic
 Wait for growth
 Colonic Interposition
Bringing excellence to life
Bringing excellence to life
Bringing excellence to life
 Acute

-Anastomotic leak
Pathogenesis
 Use silk sutures at the anastomosis
 Excessive anastomotic tension
 Excessive distal oesophageal

mobilisation
Colonic interposition – graft necrosis
Bringing excellence to life
Bringing excellence to life
 Late

Missed TOF

Recurrent TOF
 Present with recurrent chest infection
 Erosion through site of previous

repair
Anastomotic suture line leak
Bringing excellence to life
Bringing excellence to life
 Late

Anastomotic Stricture -40% of
repairs
 Pathogenesis
 Anastomotic leak
 Two layer anastomosis
 Anastomosis under tension
 Silk sutures
 Gap length at presentation
 Associated gastro-oesophageal reflux
Bringing excellence to life
Anastomotic stricture
Bringing excellence to life
Bringing excellence to life
Bringing excellence to life
Bringing excellence to life
Bringing excellence to life
Long Term Complications
 Oesophageal function

Disordered oesophageal motility
 Barium swallow
 Oesophageal function tests
 Manometry
 pH studies
Bringing excellence to life
Oesophageal Function
 Abnormal oesophageal motor
function



No progressive peristalsis
Lack of co-ordinated peristaltic
stripping wave
Oesophageal contractions
simultaneous
 Can involve whole oesophagus or
distal two thirds
Bringing excellence to life
Oesophageal Function
 Moderate- severe gastrooesophageal reflux
 Incompetent gastro
oesophageal sphincter
? Iatrogenic hiatus hernia


Oesophagitis
Symptoms persist into adult life
Bringing excellence to life
Oesophageal Function
 Abnormal oesophageal motor
function

No progressive peristalsis
 Moderate- severe gastrooesophageal reflux
 Reflux and disordered motility


Oesophagitis
Pulmonary symptoms
Bringing excellence to life
Long Term Complications
 Respiratory



Recurrent bronchitis
Persistent cough - ‘TOF’ cough
Pneumonia

Commonest first 8 years of life

Tracheomalacia (reported in up to
10%)
Bringing excellence to life
Respiratory Symptoms
 Recurrent inhalation of food


Consequence of dysmotility and
reflux
Fat seen in tracheal secretion
aspirates
 Association between
oesophageal stricture and
recurrent bronchitis
Bringing excellence to life
In Summary
 Survival for patients with OA
and TOF good
 Symptoms relating to
dysmotility and reflux have a
significant impact

Frequently persist into adulthood
Bringing excellence to life