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OESOPHAGEAL ATRESIA

Dr. Sam Chippington


Martin Churchill-Coleman

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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

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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.

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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.

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Absence of foetal stomach bubble

Absence of
gastric
bubble

Umbilical
Spine cord

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Small foetal stomach bubble

Umbilical
vein

small gastric
bubble
collapsed
Spine

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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

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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 10-
12cm before reaching the blind end of
the upper pouch.

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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

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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

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Demonstrating an H type
Fistula

SPINE

HEAD FEET

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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.

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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.

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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

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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


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In addition to the vacterl
anomolies there is increased
incidence of Duodenal atresia,
malrotation, intestinal
malformations, Meckels
diverticulum and annular
pancreas.

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Air filled upper
Hemivertebra oesophagus

Replogle tube

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Oesophageal Atresia
First described 1703

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Oesophageal Atresia
First described 1703

1936 First surgical repair


1939 First successful surgical
repair
Gastrostomy inserted, delayed
surgical repair

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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
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1945-1965
Focus on successful repair in
otherwise healthy neonates (and
birth weight > 2.5kg)
By 1965
Success rate 80-90%
1965- 1990
Refinement of the procedure
Low birth weight
Co-existing morbidity

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Surgical
Anastomosis

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Newest approach

Fewer musculoskeletal
sequelae
Winging of the scapula
Asymmetrical chest wall
Thoracic scoliosis
Breast maldevelopment

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Primary oesophageal
anastomosis
Within 48 hours of birth

Delayed primary oesophageal


anastomosis
Unfit for surgery
Other associated anomalies
e.g.cardiac

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Staged repair
Long gap oesophageal atresia
Stretch upper pouch
Mechanical
Electromagnetic
Wait for growth
Colonic Interposition

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Acute -Anastomotic leak

Pathogenesis
Use silk sutures at the anastomosis
Excessive anastomotic tension
Excessive distal oesophageal
mobilisation
Colonic interposition graft necrosis

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Late
Missed TOF
Present with recurrent chest infection
Recurrent TOF
Erosion through site of previous
repair
Anastomotic suture line leak

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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

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Anastomotic stricture

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Long Term Complications

Oesophageal function
Disordered oesophageal motility
Barium swallow
Oesophageal function tests
Manometry
pH studies

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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

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Oesophageal Function

Moderate- severe gastro-


oesophageal reflux

Incompetent gastro-
oesophageal sphincter
? Iatrogenic hiatus hernia

Oesophagitis
Symptoms persist into adult life

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Oesophageal Function

Abnormal oesophageal motor


function
No progressive peristalsis

Moderate- severe gastro-


oesophageal reflux

Reflux and disordered motility


Oesophagitis
Pulmonary symptoms

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Long Term Complications

Respiratory
Recurrent bronchitis
Persistent cough - TOF cough
Pneumonia

Commonest first 8 years of life

Tracheomalacia (reported in up to
10%)

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Respiratory Symptoms

Recurrent inhalation of food


Consequence of dysmotility and
reflux
Fat seen in tracheal secretion
aspirates

Association between
oesophageal stricture and
recurrent bronchitis

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In Summary

Survival for patients with OA


and TOF good

Symptoms relating to
dysmotility and reflux have a
significant impact
Frequently persist into adulthood

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