Esophageal atresia (EA) is the congenital malformation that represent the failure of the esophagus to develop a continuous passage upto the stomach Tracheo esophageal fistula (TEF) is the congenital malformation where the trachea and esophagus fails to separate into distinct structures and a passage is created between the two.

1. ESOPHAGEAL ATRESIA AND TRACHEO-
ESOPHAGEAL FISTULA
(EA &TEF)
PRESENTER:
Mrs. Pooja Saharan
M.Sc. (N)
Child Health Nursing

2. CONTENT
• Definition EA with TEF
• Incidence and etiology of EA with TEF
• Pathophysiology of EA with TEF
• Types of EA with TEF
• Clinical features of EA with TEF
• Diagnosis of EA with TEF
• Management of EA with TEF
– Medical management
– Surgical management
– Nursing management

3. INTRODUCTION
• Esophagus: tube that connects the mouth
to the stomach
• Trachea: "windpipe"
• Atresia: absence of a normal opening
• Congenital: found at birth
• Fistula: abnormal passage from a body
organ to the body surface or between two
internal body organs.

4. Anatomy and Physiology of oesophagus
Anatomy: Esophagus is a hollow
muscular tube extending from
the lower end of pharynx to the
cardiac orifice of stomach .
 Length of the esophagus is 8-
10cm at birth ,and doubles in
the Ist 2-3 years of life reaching
25cm in an adult.
 The abdominal portion of the
esophagus is as large as
stomach in an 8 week old fetus
but gradually shortens to a few
mm at birth , attaining a final
length of 3cm by a few years of
age.

5. PHYSIOLOGY
• Esophagus can be divided into 3 areas : UES(upper
esophageal sphincter),Esophageal body and
LES(lower esophageal sphincter).
• Swallowing is initiated by elevation of tongue ,
propelling the bolus into the pharynx
• The LES act as barrier against gastro esophageal
reflux ,relaxes , as swallowing is initiated ,at nearly
same time as the UES relaxation .

6. DEFINITION
• Esophageal atresia (EA) is the
congenital malformation that
represent the failure of the esophagus
to develop a continuous passage upto
the stomach
• Tracheo esophageal fistula (TEF) is
the congenital malformation where
the
trachea and esophagus fails to
separate

7. INCIDENCE & EPIDEMIOLOGY
• Occurs in 1:3000 to 1:4500 live births.
• Equal gender incidence
• EA associated with prematurity.
• A history of maternal polyhydroamnios is present in
approx. 50% of infants with the defects.
• Often present with VATER/VACTERL syndromes.
(VATER/VACTERL is acronyms that describe the
associated anomalies of vertebral, ano-rectal,
cardiovascular, tracheo-esophageal, and renal and limb

8. CONTD…
• Possible influences:
– Inherent genetic factor , teratogens
– Environment factor
• Prematurity & low birth weight.

9. INCIDENCE & EPIDEMIOLOGY
Incidence of Associated Anomalies in EA & TEF
Anomaly Frequency (%)
• Congenital heart disease 25
• Urinary tract 22
• Orthopedic (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

10. PATHOPHYSIOLOGY • Upper part of esophagus is
developed from retropharyngeal
segment and the lower part from
pregastric segment of the first
part of primitive gut.
• At 4- 5weeks of gestation the
laryngo-tracheal groove is
formed.
• Two longitudinal furrows
develop and separate the

11. Deviation or altered cellular growth of the
septum results in formation of fistula
between esophagus and trachea.

12. PATHOPHYSIOLOGY
Successive stages in the development of the tracheoesophageal septum during
embryologic development.

13. TYPES OF TEF

14. TYPE A: Esophageal Atresia without
fistula (8%):
• It is the second most common type.
• There is no connection of esophagus to
trachea.
• The upper (proximal) segment and lower
( distal) segment of esophagus is blind.

15. TYPE II: Esophageal atresia with
Tracheoesophageal fistula (upper)
• It is rare and found in
less than 1% of all
cases. Upper segment
of esophagus open into
trachea by a fistula.
• The distal or lower
segment is blind.

16. TYPE III – Esophageal Atresia with
Tracheoesophageal fistula (lower)
(80-90%)
• It is the most common
type. In this condition,
proximal or upper
segment of the
esophagus has blind
end.
• The distal lower
segment of esophagus
connects into trachea
by a fistula.

17. TYPE IV – Esophageal Atresia with
Tracheoesophageal fistula both upper
and lower segment
• It is also rare (less
than 1%).
• There is a EA with
fistula between
both proximal and
distal ends of
trachea and
esophagus.

18. TYPE V – H- type TEF
• It is found in about 4 % of
all cases and not usually
diagnosed at birth.
• Both proximal/ upper
and distal/ Lower
segments of esophagus
open into trachea by a
fistula.
• No esophageal atresia is
present.

19. CLINICAL MANIFESTATIONS
The disorder is usually detected soon after birth when
feeding is attempted on the basis of following :
1. Violent response occurs on feeding
 Infant coughs and chokes
 Fluid returns through nose and mouth.
 Cyanosis occur
 The infant struggles

20. Contd.…
2. Excessive secretions coming out of nose and constant drooling of
saliva.
3. Saliva is frothy.
4. Abdominal distension occurs in presence of type III, IV and V
fistula.
5. Intermittent unexplained cyanosis and laryngospasm, caused by
aspiration of accumulated saliva in blind oesophageal pouch.
6. Pneumonia may occur due to overflow of milk and saliva from
oesophagus through fistula into the lungs.

21. DIAGNOSTIC EVLUATION
The EA/TEF may be suspected prenatally if:
 Ultrasound examination reveals polyhydramnios,
absence of a fluid-filled stomach, a small abdomen,
lower-than-expected fetal weight, and a distended
esophageal pouch.
 Fetal MRI may be used to confirm the presence of
EA/TEF

22. TEF may be detected postnatally by
 X-ray taken with radiopaque catheter placed in esophagus to check
for obstruction; standard chest X- ray shows a dilated air-filled
upper esophageal pouch and can demonstrate pneumonia.
 Inability to pass a NG tube into stomach because it meets
resistance
 Bronchoscopy visualizes fistula between trachea and esophagus;
 Abdominal ultrasound and echocardiogram to check for cardiac
abnormalities.

24. The management can be divided into:
• Immediate management
• Medical management
• Surgical management
• Nursing management

25. IMMEDIATE MANAGEMENT
• Immediately after diagnosis, the infant should be
managed with propped up position (300 angle) to
prevent reflux of gastric secretion, and nothing
per month, airway clearance.
• The blind pouch to be washed with normal saline
to prevent blocking of the tube with thick mucus.
• Gastrotomy is done to decompress the stomach
and to prevent aspiration and afterwards to feed
the infant.

26. CONTD..
• Supportive care should include maintenance
of nutritional requirements and warmth,
prevention of infections, antibiotic therapy,
respiratory support, detection and treatment
of complications, continuous monitoring of
patient’s condition, chest physiotherapy and
postural drainage.

27. Medical Management
• The infant is immediately deprived of oral intake
(NPO)
• Start IV fluids.
• Place infant in the position least likely to cause
aspiration of either mouth or stomach secretions
i.e. supine with head end raised to 45° or head
turned to one side.
• Removal of secretions from the mouth and upper
pouch requires frequent or continuous suction
with Replogle’s catheter every 5 min gently with
pressure of 50 cm of H₂O.
• Broad spectrum antibiotic therapy is often
instituted.

28. II. SURGICAL MANAGEMENT
Immediate primary repair: indications are-
no pulmonary complications
weight of the child >2 to 2.5kgs
no major congenital malformations
healthy baby
gap between distal and proximal esophagus <
2cm
Delayed surgical intervention: indications are-
pneumonia
sepsis
congenital malformations
severe prematurity

29. SURGICAL MANAGEMENT
STAGES OF OPERATION:
-left cervical esophagostomy (to allow drainage
of saliva through stoma of neck) and gastrostomy
-thoracotomy and ligation of TEF
-Replacement of the gap between proximal and
distal esophagus at 6-8 months of
age by isolated segment
of colon or by gastric tube.

30. PROCEDURE
Right posterolateral thoracotomy through 4th
intercoastal space
Proximal esophageal pouch is mobilised after ligating
vena azygous
Distal esopahgus is not mobilized due to its fragile
blood supply. Fistula with trachea is identified and
tracheal end of fistula is closed.
End to end anastomosis of distal and proximal
esophagus is done by single layer of 4/0 synthetic sutures
Sterile feeding tube of size 6-8 fr is left in stomach,
passed through nose.
Chest is closed with chest tube drain

32. PREOPERATIVE CARE
• Detection of this malformation immediately after
birth.
• Diagnosis should be made before the initial
feeding; customary for nurse to give first feeding
of plain water of to be present when a parent
feeds the child to observe infant's response.
• Infant placed in incubator/radiant warmer, and
oxygen administered to help relieve respiratory
distress.
• When a new born suspected of having TEF, the
most desirable position is supine with the head
elevated on an inclined plain of at least 45
degrees.

33. Contd.......
• Frequent suctioning of pharynx and esophagus
and also continuous drainage of secretion are
necessary
• Catheter needs attention because it has tendency
to become clogged with mucus.
• In staged repair, a gastrostomy tube is inserted
and left open so that air entering stomach
through the fistula can escape, thus minimizing
the danger of regurgitation.
• Feeding through the gastrostomy tube and
irrigations with fluid are contraindicated before
surgery in the infant with a distal TEF.
• Give IVF to prevent the electrical imbalance

34. NURSING ASSESSMENT
• Assess the infant’s respiratory status (early recognition
helps to prevent aspiration).
• Assess for excessive amounts of mucous with drooling.
• Assess for coughing, chocking and cyanosis when fed.
• Check for expelled feeds through nose immediately
following feeding (coughing, chocking, struggling and
resultant cyanosis).
• Check for abdominal distension caused inspired air.

35. NURSING DIAGNOSIS
• Impaired gaseous exchange related to abnormal opening
between esophagus and trachea as evidenced by cyanosis.
• Risk for injury related to surgical procedures.
• Anxiety related to difficulty swallowing, discomfort due to
surgery as evidenced by parents frequently asked
questions.
• Altered family processes related to children with physical
defects.

36. NURSING MANAGEMENT
• Other interventions include:
Respiratory assessment
Airway management
Thermoregulations
Fluid and electrolyte management
Potential nutritional support.

37. POSTOPERATIVE CARE
• Same as the care of any high-risk newborn.
• Ventilatory support required.
• The infant is returned to the radiant heater/ incubator.
• Gastrostomy tube is connected to gravity drainage until the
infant can tolerate feedings.
• Tube is elevated and secured at a point above the level of
stomach. This allows gastric secretion to pass to the
duodenum, and swallowed air can escape through the open
tube.
• Tracheal suction should only be done using a pre-measured
catheter and with extreme caution to avoid injury to the
suture line.
• Broad spectrum antibiotics given as prescribed.

38. Suctioning
• Pressure cycle ventilator is used.
2 saline bottles are required(one for oral and one for ET
tube)
Keep pulse oxymeter attached
Disconnect the ET tube and do suctioning of ET tube
first followed by oral suctioning
Keep an eye on pulse oxymeterIf saturation is less, then
stop suctioning and connect the ET tube with ventilator
If secretions are thick, then pour sodium bicarbonate or
distilled water 1-2 drops in tube
After that do oral suctioning
Repeat the procedure as required

39. Contd..........
• If tolerated, gastrostomy feedings may be started
and continued until the esophageal anastomosis is
healed.
• Before oral feeding is initiated and the chest tube is
removed, a contrast study or esophagram is
performed to verify the integrity of the esophageal
anastomosis.
• The initial attempt at oral feeding must be carefully
observed to make sure that infant can swallow
without choking.
• Oral feedings are begun with sterile water, followed
by frequent small feedings of formula.

40. Contd........
• Until the infant can take a sufficient amount by
mouth, gastrostomy feedings or parental nutrition
may supplement oral intake.
• Infants are usually not discharged until they are
taking oral fluids well and the gastrostomy tube is
removed.
• However, the infant who has a palliative surgery will
be discharged with the gastrostomy tube in place.
• The nurse is responsible for making that the
caregiver is educated and has practiced the care of
the gastrostomy.

41. NURSING MANAGEMENT
-SPECIAL PROBLEMS
1. Upper respiratory complications-
• Pneumonia, respiratory distress (atelectasis, pneumothroax,
and laryngeal edema).
• Persistent respiratory difficulty after removal of secretions
reported to surgeon immediately (stricture).
• Infant monitored for anastomotic leaks (purulent chest tube
drainage, increased WBC count, and temperature instability).
2. Skin care-
• Esophagostomy difficult to care because the skin is irritated
by moisture from continuous discharge of saliva.
• Frequent removal of drainage
• Application of protective ointment
• Enterostomal therapist consulted.

42. 3. Non nutritive sucking-
• Provided by pacifier (not recommended now a
days).
• Small amounts of water or formula are given orally
(sham feeding),
• Although liquid drains, this allows the infants to
develop mature sucking patterns.
• Who remain NPO or who have not received oral
stimulation has difficulty with eating by mouth after
corrective surgery and may develop oral
hypersensitivity and food aversion.
• Firm guidance to learn how to take food into mouth
and swallow after repair.

43. 4. Parental guidance-
• Parents need to adjust.
• Immediate transfer of the sick newborn to the ICU
and the length of hospitalization.
• Encouraging parents to visit the infant participate in
care when appropriate, and express their feelings
facilitate the attachment process.
• Parents kept fully informed of the child’s condition.
•

44. COMPLICATIONS
• Tracheomalacia (weakness of tracheal wall)
• Anastomotic leak (tension)
• Strictures (narrowing, esophageal dilation)
• Dysphagia (esophageal motility disorder)
• Respiratory distress
• Gastroesophageal reflux (positioning,
semisolids, fundoplication).

45. RESEARCH ABSTRACT
• Zani A. Wolinska J Outcome of
esophageal atresia/tracheoesophageal fistula in
extremely low birth weight neonates
(<1000 grams). Pediatr Surg Int. 2016 Jan;
32(1):83-8.
• PURPOSE:
To review the outcomes of extremely low birth weight
(ELBW, <1000 g) infants with
esophageal atresia/tracheoesophageal fistula (EA/TEF).

46. • METHODS:
Health records of ELBW EA/TEF infants treated
from 2000 to 2014 were reviewed
(REB1000046653). Demographics, operative
approach and postoperative complications were
analyzed. Data are reported as median (range).
• RESULTS:
Of 268 EA/TEF infants, 8 (3 %, five females) were
ELBW (930 g, 540-995). Gestational age was
28 weeks (23-32). Seven had type-C EA/TEF and one
type B.

47. OUTCOMES
• One trisomy 18 infant received no treatment and died; one
initially diagnosed as type A had primary repair at 126 days of
life (DOL); six underwent TEF ligation (three trans-pleural)
with primary repair in one and delayed anastomosis in two
(DOL 120 and 178). The remaining three died (gastrostomy
dehiscence and peritonitis, liver hemorrhage during peritoneal
drain insertion, severe chronic lung disease and brain
hemorrhages). At a median follow-up of 3 years (range
15 months-5 years), all survivors are thriving.

48. SUMMARY
• Definition EA with TEF
• Incidence and etiology of EA with TEF
• Pathophysiology of EA with TEF
• Types of EA with TEF
• Clinical features of EA with TEF
• Diagnosis of EA with TEF
• Management of EA with TEF
– Medical management
– Surgical management
– Nursing management

49. BIBLIOGRAPHY
• Ghai OP, Paul K Paul, Bagga Arvind. Essential
Pediatrics. CBS publishers. 17th edition. Pp 151
• Meharban singh. Care of newborn. 6th edition.
Sagar publications. Pp 138
• Marilyn J. Hockenberry, Wilson, Essentials of
Pediatric Nursing, Elsevier, 8th edition. 465-473.
• www. Wikipedia. Com/tracheoesophageal atresia/