Esophageal atresia is a congenital
interruption or discontinuity of the
esophagus resulting in esophageal
Tracheo- esophageal fistula is an
abnormal communication between the
esophagus and trachea .
William durston described EA in 1670
Thomes Gibson described EA with TOF in
William ladd 1939 first to operated a case of
EA with TOF in multi staged procedure &
Comeron Haight 1941 first to operated the
Patient of EA with TOF in one stage
procedure and patient survied.
The incidence of EA is about 1 in 2500-4500 birth
(2.84 per 10,000 births).
It is more common in male , male to female ratio
is 1: 1.26
There is an increased risk of EA with a first
There is a slight increasing trends in the rate of
EA with increasing maternal age.
5. It is more common in twin pregnancy and
twining increased EA about 6% cases.
Cromosomal anomaly also increased the
risk of EA, about 6.6% cases . trisomy 13
(A) EA with TOF has no specific aetiology
but some drugs which increase the risk
of this, when taken at the time of
pregnancy these are.
- OCP - Progesterone
- Estrogen - Thalidomide
7. (B) Patient who has diabetes at the time of
pregnancy increase the risk of EA with TOF.
(C) Increase the risk of this anomaly in next
offspring when the first child affected about
0.5 to 2%.
(D) When more than one child affected with
this anomaly increase about 20% risk in
8. The median pharyngeal groove develops in the
ventral aspect of foregut at day 22 of gestation.
This tissue develops into the respiratory and
Esophagus and tracheal originate from the
median ventral diverticulum of the forgut,
separated by esophagotracheal septum
9. Normally, mesenchyme proliferating between
the respiratory and digestive tubes separates
Development of an abnormal epithelial-lined
connection between the two tubes results in
the creation of a TEF .
In a full term neonate the esophagus is 9 to
10 cm in length and has a lumen of
around 3-4 mm.
The upper esophageal pouch varies in
length but usually reaches within 1 cm of the
level of the azygus vein.
The lower pouch commences from the
post. wall of the trachea and arises usually
around 0.5- 1.0cm proximal to the carina.
12. As compared to the thick, hypertrophied upper
pouch, the lower pouch is thin and has a smaller
caliber ., the lower pouch can be identified at
the thoracotomy by
1. looking for it just below the arch of azygos vein.
2.Observing it getting distended with air during
13. Arterial Supply
The cervical esophagus is supplied by the
inferior thyroid artery .
Thoracic esophagus is supplied by segmental
branches from aorta.
The distal esophagus is supplied by the ascending
branches of the left gastric artery.
15. NERVE SUPPLY
Upper half -Reccurrent laryngeal N
Lower half -plexus formed by both vagus
Sensory ,motar & secretomotor supply
Upper half- Middle cervical ganglion
Lower half-Upper 4 thoracic ganglion
1. E.C Vogt 1929 -he was classify EA in
4 type, & number 3 in three Sub type
2. Gross 1953 – he was classify EA in 7
type in alphabetic word (A-to G)
17. Vogt Gross Anomaly
1. A Absent Esophagus
2. Pure esophageal atrea 7.8%
3.a B EA with proximal fistula 0.2%
3.b C EA with distal fistula 85.8%
3c D EA with both proximal & distal F
4. E H- type fistula(4%)
F Esophageal stenosis
G Membranous Artesia
19. Associated anomalies
The early disturbance in organogenisis
which result in EA leads to other
The incidence of which varies
20. Cardio vascular anomalies – these anomalies are
the most common associated anomalies with EA
can be present in 13-30% of the cases in the
presence of skeletal anomalies
The incidence of the cardio vascular anomalies is
higher and more complex, life threatening defect
are likely VSD(19%), tetrology of fallot(5%) ,
ASD(20%) and coarctation of aorta.
21. 1. GIT Anomalies - ARM(14%), duodenal
and ileal atresia(2%), and esophageal stenosis
and malrotation .
2. Musculo skeletal anomalies- seen in
10- 15% cases and included vertebral
defect(17%), limb anomalies(15%) .
22. 1. Genito urinary anomalies- These
included hypospadiasis, undescended
testes, renal dysplasia, hydronephosis ,
vasico ureteric reflux .
2. Respiratory tract anomalies – like
choanal atresia , Tracheal webs or
3. Chromosomal anomalies- Trisomy
24. CHARGE Association– This include
H- Heart disease
A- Atresia choanae
RG- Retorded growth and development
E- Ear anomalies with deafness
Frothy white bubbles in the mouth.
Coughing or choking when feeding.
Blue color of the skin, especially when the
baby is feeding.
Very round, full abdomen
26. Fever, tachycadia, lethargy suggested
Abdominal distention in patent distal fistula.
Inability to pass a tube through the mouth and
nose in to stomach.
The history of polyhydroamonios in pregnancy
27. Diagnosis - Antenatal
(a) by USG abdomen
(1) Poly hydroamnios
(2) Failure to demonstrate the fetal stomach in
presence of normal or increased amniotic
fluid ( Normally stomach seen after 14th
week of gestation )
28. (b) High resolution real time scanning it show
fetal swallowing of amniotic fluid and
distention of upper pouch
(c) In X-Ray abdomen – The presence of
VACTERL Associated anomalies are seen.
(d) Chromosomal study.
(e) Raised amniotic fluid acetyl choline esterase
30. Frontal view of the
chest and abdomen of
demonstrates a tube
in the proximal pouch
in this patient with EA.
The presence of bowel
gas implies the
presence of a distal
32. : Contrast study with
intact upper esophageal
pouch extending to
33. H-type tracheoesophageal
fistula (TEF). Oblique
demonstrates a fistula
(arrow) arising from the
anterior esophagus and
to the trachea.
34. Conform the level of atresia ( upper pouch)
in term of thoracic vertebra.
Check the presence of abdominal gas to rule
out the pure esophageal atrisia
Asses the pulmonary shadow to rule out
pneumonia or Pneumonitis
Assess the cardiac shadow and calculate the
C.P ratio to suspect cardiac anomalies
35. (2) Echocardio graphy – for cardiac anomalies.
(3) USG Abdomen for renal anomalies.
(6) Chromosomal study.
(7) cine- esophagogram- H type
(8) preoperative base line investigation
37. Group Survival waterston Classification
A 100% Birth wt> 2.5kg & otherwise well
B 85% Birth wt 2.0 –2.5kg or higher wt with
moderate associated cardiac anomalies or
C 65% Birth Wt< 2.0 kg or higher with sever
associated cardiac anomalies or
Primary repair of TEF is urgent
Protect the lungs from aspiration pneumonia
Avoidance of feeding
Positioning of the infant slightly head up to
minimize regurgitation of gastric contents
Antibiotic therapy and physiotherapy in babies
with contaminated lung
40. The Resp.Rate and saturation are monitored
along with blood gas parameter to asses the
need for supplement oxygen and ventilation .
I V line are established to take sample and
to start fluid and antibiotic.
Inj vit K
One stage repair: optimal surgical management
Fistula is ligated, proximal and distal ends of the
esophagus are anastomosed
Right posterolateral extrapleural thoracotomy
Left thoracotomy if the p’t has right aortic arch
High risk infants unable to withstand a thoracotomy
Decompress the stomach and prevent regurgitation
via fistula into the lungs.
42. Definitive repair
24-72 hours later, when infant can
withstand both surgery and anesthesia
Method: right thoracotomy using a
posterolateral extrapleural approach
Fistula is ligated, esophageal segments
If distal esophagus is too short
Fistula is ligated
exteriorization of the upper pouch
through an esophagostomy is performed
43. Operative procedure
The infant is typically positioned for a
standard Rt posterolateral thoracotomy, with
the right arm extended above the head and
the head slightly flexed.
A curved skin incision is made around the
lower border of the scapula, extending from
the anterior axillary line posteriorly to the
44. The thorax is entered through the fourth
intercostals space by dividing the intercostals
muscles, with care taken to avoid incising the
With the extrapleural approach - In this
dissection start posterioly followed by
superior and inferiorly, Azygos vein is
exposed and divided.
45. -The lower esophagus is dissected
circumferentially at the level of fistula.
Fistula closed, mobilization of upper pouch
and lower esophagus, the tip of upper pouch
is excised .
End to end anastomosis made with single
Drain should be placed away from the
anastomosis in retro pleural space
53. POST OPERATIVE COMPLICATION
A) Early complication
(i) Anasto motic leak – 14-16%
a. Incidental :- Incidental leak are generally
radiological leak, have no symptoms
54. Minor leakage :- Saliva in chest drain but
infant clinically well.
Minor leaks are managed conservatively
with parenteral nutrition, upper pouch
suctioning, antibiotics and ensuring adequate
drainage of the extrapleural space by the in
situ chest tube)
56. Major leakage :- Mediastinitis or abscess.
Reanastomosis following a major leak is
usually always unsuccessful and in such
cases diversion in the from an
esophagostomy and gastrostomy may be life
57. (ii) Anastomotic stricture :- The factors
that have been implicated in the pathogenisis
of esophegal stricture include poor
anstomotic technique, Gastroesophageal
reflux, Anastomosis under tension and
ischemia of esophageal ends.
Balloon dilatation under fluoroscopic
guidance is the preferred treatment for
59. Recurrent tracheo esophageal fistula(3-14%) :-
Improper closure of the fistula, end to side
esophageal anastomosis with fistula ligation and
anastomotic leak with local abscess formation can
lead to a recurrent TOF.
The classic approach for management of recurrent
tracheosophageal fustula is a repeat right
thoracotomy , transpleural repair of the fistula and
interposition of an intercostals muscle or
pericardial flap after repair of the defect.
61. Gastro esophageal reflux
GERD is extremely common occurring in up
to 35 to 58% of children.
GERD appears to be due to intrinsic motor
dysfunction of the esophagus, and shortened
intraabdominal segment of the esophagus
due to anastomotic tension .
GERD may lead to esophageal strictures,
aspiration leading to pneumonia, bronchial
hyperreactivity, and permanent airway or lung
62. Tracheal inflammation, assessed by biopsy.
Esophageal biopsy specimens showed
severe inflammation in 20% of cases, and
Barrett esophagus in 6%.
65%percent of patients with EA/TEF and
GERD may respond to medical therapy, with
the remainder requiring antireflux surgery
63. The indication for surgical correction of
gastroesophageal reflux is failure of medical
evidenced by persistent reflux esophagitis or
Barrett esophagus, failure to thrive,
The usual surgical treatment is a Nissen
Timing of fundoplication within 6 months of
the initial surgery to 21 months of age.
Tracheomalacia is present clinically significant in 10
to 20% of patients.
Tracheomalacia is usually found at or just above level
of the original EA/TEF.
The site of collapse is most prominent in the distal
one-third trachea in 60% of cases, and in the middle
one third in 30%.
The soft trachea is easily compressed between the
aorta and the frequently dilated former esophageal
Bulging of the posterior wall is evident at
65. In most patients, the manifestations of
tracheomalacia are limited to a brassy
Episodes generally began after 2 to 3
months of age.
The event typically occurs during or just after
feeding, or during crying or coughing, and is
followed by cyanosis progressing rapidly to
apnea, bradycardia, and cardiac arrest.
66. Treatment for tracheomalacia is generally
reserved for patients with dying spells or
Aortopexy involves suturing the aorta to the
posterior surface of the sternum
67. Disordered peristalsis
Esophageal peristalsis, assessed by
manometry, is abnormal in 75 to 100% of
children with a history of EA.
An immotile segment or small, discoordinate
contractions is most commonly observed.
Many children with repaired EA/TEF need to
In severe cases, failure to thrive or aspiration
This may require myotomy for treatment.
68. Chest Wall Deformity
Chest wall deformities may be common in
individuals with a history of EA/TEF.
Scoliosis is more common in patients .
Progressive scoliosis requiring surgical
Inadequate Upper pouch suctioning
Oral feeds before diagnosis
Related to surgery
Anastomotic leaks – sepsis
70. Mortality -
The causes of mortality in the first year of the
- Congenital heart disease
- Recurrent pneumonia
Survival rate - 85-95%
71. long-gap oesophageal atresia
The distance between esophageal ends that
constitutes a long-gap EA lacks a strict numerical
gap intervals measuring greater than 2 cm, or greater
than two vertebral bodies as constituting along-gap
3.5 cm ‘‘ultra-long’’ gap or6 vertbral bodies.
Thus, long-gap EA can be defined as any distance
between the esophageal ends in a new born that is
too wide for the surgeon to perform primary
anastomosis of the proximal and distal esophagus
72. This is a contrast study
esophageal atresia. A dilator
is placed within the
esophageal pouch and
contrast is instilled into the
A distance of 5.5 vertebral
bodies is seen between the
esophageal remnant and the
73. Operative manoeuvres in long-gap
At the initial procedure
1. anastomosis under tension
2. tension-relieving procedures
3. flap technique
4. suture fistula
74. Delayed primary anastomosis
1. with bouginage - proximal , proximal and
2. without bouginage
3. oesophageal lenghtening techniques,flap ,
myotomy , gastric division .
1. with and without "olives"
77. O.A. with distal T.O.F.
primary anastomosis lengthening gastrostomy
under tension techniques
elective paralysis myotomy delayed primary cervical
IMV for 5 days flap oesophagostomy
78. Upper pouch bougienage
In1965,Howard described in which weighted
bougie is passed through the mouth into the
Forward pressure applied daily/twice daily for
6-12 weeks before attempting delay primary
1975,Hendren reported using ectromagnatic
field to pull togther mettallic bullets in two
79. SUTURE FISTULA
In 1971,Rehbein described the nylon thread
bridging the gap between the two end of
esophagus & attached to olives.
The olive were pushed toward each other
over time until two end esophagus pressed
togther & create a fistula.
In some instances, the two ends of the
esophagus are too far apart (>2.0 cm) for a
safe primary anastomosis.
A circular myotomy, as described by Livaditis,
can lengthen the proximal or distal end .
The myotomy is performed approximately 2.0
cm above the blind proximal atretic end to
divide the longitudinal and circular smooth
82. This increases the length of the proximal
esophagus by approximately 1.0 cm and often
facilitates a primary anastomosis without
Occasionally, a second myotomy may be
required at a more proximal level .
A proximal spiral myotomy has been described
Step ladder myotomy
85. flap technique
Gough popularized theanterior upper pouch
An inverted full thicness pouch is created &
The length of the flap should be less than
three times the breath.
It increases length about 1.5 cm.
86. flap technique
Flap technique: (1) the closed upper pouch; (2) the upper pouch is
open and its lateral wall reaches the lower segment of the oesophagus; (3) the
elongated upper pouch is anastomosed to the lower segment
87. Fokker technique
long gap esophageal atresias we used an
esophageal lengthening technique originally
described by Dr John E. Foker.
This technique consists of the placement of traction
sutures into the ends of the proximal and distal
after full mobilization and bringing these sutures
together through the thoracic wall under slight
Postoperatively, these sutures are pulled1 to 2 mm
Once the 2 ends of the esophagus are in proximity,
esophageal anastomosis is performed.
89. Scharli lesser curve elongation
Scharli describe a comman abdominal &
thoracic procedure in which adequate
vacularity of distal esophagus was insured by
dividing the left gastric artery.
Diagonal division of lesser curvature of
stomach with GIA stapler.
Mobilization of gastric cardia & fundus into
the chest to achieve anastomosis with partial
90. ESOPHAGEAL REPLACEMENT
Creteria for ideal conduit
An efficient conduit from mouth to stomach to
satisfy nutriional needs.
Conduit should grow with child
Gastric acid reflux into conduit must be
Conduit should not impair respiratory or
Surgical techanique should be simple
91. Conduit should not produce any cosmatic
Post operative complications should be
minimum & manageable
92. GASTRIC TUBE OPHAGOPLASTY
The right gastroepiploic vessels are divided
approximately 2.0 cm proximal to the pylorus, and the
greater omentum is divided up to the splenic hilum,
preserving the gastric arcade.
The reversed gastric tube is fashioned along the
greater curvature of the stomach based upon left
After the stomach is incised inferiorly, a No. 18 to 24
French chest tube is placed in the distal stomach
opening and passed superiorly along the greater
curvature of the stomach.
93. The medial portion of the stomach is then divided
from below upwards using a GIA autostapler to
fashion the tube.
An elliptical cervical incision is made around the
proximal esophagostomy site and then carefully
mobilized to avoid injury to the vagus and recurrent
Retrostrenal tunnel is created or A thoracotomy
incision is made over the sixth interspace.
The reverse gastric tube is brought up through the
hiatus into the chest behind the hilum of the lung and
then into the neck, where an end-to-end anastomosis
is performed with the proximal esophagus.
94. A reversed gastric tube
is fashioned along the
greater curvature of the
stomach, incising both
anterior and posterior
walls to conform to the
size of No. 18 to 24
95. Gastric tube reversed and anastomosed to
cervical part of
Gastric tube created from greater
curvature of stomach based
upon left gastroepiploic artery
Gastric tube reversed and
anastomosed to cervical part of
Gastric acid Reflux
Leak & stricture- cervical site
Perforation of gastric tube
Long suture line
97. gastric pull-up operation
An alternative procedure is the gastric pull-up
operation, as described by Spitz.
In which the proximal stomach is pulled up through
the hiatus to the neck level and an anastomosis to
the esophagus is performed without a formal
The gastrostomy site is closed and mobilization of the
stomach is then performed.
The greater curvature of stomach is mobilised by
division & ligation gastrocolic omentum and the short
98. The right gastric artery is preserved and the
left gastric vessels are sacrificed.
The distal esophageal stump is resected and
A pyloroplasty is performed and mobilization
of the duodenum is completed.
highest portion of the fundus of the stomach
Is identified & stay suture of different material
are inserted to avoid torsion of stomach as it
drawn through post. mediastinum.
99. The upward passage of the stomach is
accomplished through a space prepared
through the hiatus into the posterior
The cervical incision site is developed and a
plane is noted between the posterior surface
of the trachea and the prevertebral fascia .
A tunnel is created in the line of the normal
esophagus and continuity is established with
the lower abdominal hiatal dissection post. to
heart & ant. to prevertebral region
100. The fundus is brought up into the neck
incision, orientation maintained (no twist), and
an anastomosis is then completed between
the native upper esophagus and the fundus
of the stomach.
A cervical drain is left in place.
A large calibar nasogstric tube is inserted.
A fine bore feeding jejunostomy for providing
Excellent blood supply.
Incidence of leakage and strictures both
reduced to 6% of patients.
Relatively simple procedure.
Poor gastric emptying.
Bulk of the stomach is in the chest;.
104. Jejunal interposition
The jejunum was transected about 10 cm
from the ligament of Treitz .
About 20 cm of distal jejunum was mobilized,
keeping the vascular supply intact.
The first and, when needed, the second
major branch of the superior mesenteric
vessels was ligated and transected close to
the base of the mesentery.
A well-vascularized jejunal segment with
along pedicle was obtained.
105. The graft was brought up behind the pancreas and
stomach and through the posterior portion of the
The route was made sufficiently wide using Hegar
dilators in order to allow free passage of the graft and
its blood supply.
The pedicle was regularly checked to make sure that
no twisting or kinking had occurred.
Small bowel continuity was restored anterior to the
vascular pedicle of the graft by jejunojejuno
106. The thoracotomy perform and the jejunal graft
was interposed between the both esophageal
The graft lay stretched in the posterior
mediastinum after preserving the blood
Free jejunal graft interpostion with micro
vascular anstomosis may performed
Advantage-preserved peristalatic activity
107. Technique of ileocolic substernal
The first use of the colon for total esophageal
replacement in a child was described by Lundblad
Waterston performed his first successful intrathoracic
colon interposition in 1954.
Javid reported the technique of presternal ileocolic
interposition for esophageal atresia in 1954
the posterior mediastinal and substernal routes that
use the right or left colon remain the most popular
108. The patient is placed in the supine position
with the head turned to the right and the neck
The neck, chest, and abdomen are prepared
for operation and draped.
The peritoneal cavity is entered through a
midline incision extending from the xiphoid to
below the umbilicus.
The gastrostomy is taken down, the right
colon mobilized, and the appendix removed.
109. The marginal artery and vein supplying the distal 10
cm of the terminal ileum and the entire right colon
distal to the middle colic vessel is isolated by
preserving the tertiary arcades of the mesoileum and
the terminal branches of the ileocolic vessels
Intestinal continuity is restored with a single-layer
Thereafter, the ileocolic pedicle is brought behind the
lesser sac and stomach and through the
A gastrocolic anastomosis is made close to the
antrum near the greater curvature with a single layer
of interrupted sutures
110. The diaphragmatic attachments to the xiphoid are
divided and a retrosternal tunnel created by blunt
The cervical esophagostomy is taken down and the
esophagus mobilized to gain adequate length.
The retrosternal space is entered from below the
sternocleidomastoid muscle and the superior portion
of the tunnel enlarged enough to prevent
compression of the ileocolic pedicle in the
mediastinum or at the thoracic inlet.
111. The esophagoileal anastomosis is constructed with a
single layer of interrupted Vicryl sutures. The neck
and anterior mediastinum are drained with a drain
through a separate stab incision.
The sternocleidomastoid and platysma muscles are
reapproximated over the anastomosis and the skin
The gastrostomy tube is replaced proximal to the
cologastrostomy and brought out through the original
stab wound site in the abdominal wall.
Anastomotic leak- Mainly esophagocolonic
anastomossis leads to stricture
Absent peristalasis –delay ooperation until
child 18-24 month of age.
Redundancy of colon.
Necrosis of graft
Gastric outlet obsrtuction