Esophgeal atresia, Tracheoesophageal fistula

1. Esophageal atresia
and tracheosophageal
fistula
S.Gomathi
This Photo by Unknown Author is licensed under CC BY-SA-NC

2. Esophageal
atresia
failure of the esophagus to form a
continuous passage from the pharynx
to the stomach during embryonic
development.
EA can occur with tracheoesophageal
fistula (TEF), which is an abnormal
connection between the trachea and
esophagus.

3. incidence
• EA/TEF occurs in approximately 1 in 3,500
births

4. Associations:
• a. Down syndrome.
• b. VACTERL syndrome (vertebral defects,
anal atresia, cardiac anomaly, TEF fistula
with EA, renal defects, and radial limb
dysplasia).

5. Etiology
• 1. Cause is unknown in most cases and
likely multifactorial.
• a. inheritable genetic factor
• b. Six to 10% due to chromosomal
(structural) abnormalities, including
trisomies (12, 18, 21), and partial
deletions such as 13q13-qter, 22q11.2.
• c. Teratogenic stimuli, such as the
anticancer drug adriamycin and
diethylstilbestrol (DES).

7. pathophysiology
Failure of proper separation
of the embryonic channel
into the esophagus and
trachea
during the 4th and 5th weeks
of gestation.

8. classification
• Esophageal atresia can be classified as
• a. Type I (Type A): proximal and distal segments
of esophagus are blind; there is no connection
to trachea; accounts for approximately 7% of
cases; second most common.
• b. Type II (Type B): proximal segment of
esophagus opens into trachea by a fistula; distal
segment is blind; rare, 0.8% of cases.

9. • c. Type III (Type C): proximal segment of esophagus has
blind end; distal segment of esophagus connects into
trachea by a fistula; most common, with 86% of cases.
(Discussion is limited to this type.)
• d. Type IV (Type D): esophageal atresia with fistula
between proximal and distal ends of trachea and
esophagus (rare, 0.7% of cases).
• e. Type V (Type E): proximal and distal segments of
esophagus open into trachea by a fistula; no
esophageal atresia but sometimes referred to as an H-
type fistula; occurs in 4.2% of cases; not usually
diagnosed at birth.

10. Clinical
Manifestations
These appear soon after birth.
1. Excessive secretions.
a. Constant drooling.
b. Large amount of secretions from nose.
c. Saliva or formula accumulates in upper
esophageal pouch and is aspirated into airway.

11. 2. Intermittent,
unexplained
cyanosis and
laryngospasm
• a. Caused by aspiration of accumulated
saliva in blind pouch.
• b. Gastric acid is regurgitated through distal
fistula.

12. 3. Abdominal
distention.
• a. Occurs as a result of air entering the lower
esophagus through the fistula and passing
into the stomach, especially when the child
is crying.

13. 4. Violent
response after
first or second
swallow of
feeding
• a. Infant coughs and chokes.
• b. Fluid returns through nose and mouth.
• c. Cyanosis occurs.
• d. Infant struggles.

14. • 5. Poor feeding.
• 6. Inability to pass catheter through nose or
mouth into stomach; tip of catheter stops at
blind pouch, or atresia.

15. Diagnostic Evaluation
• 1. Ultrasound scanning techniques enable TEF to be
identified in utero for some infants.
• 2. Failure to pass a 10F catheter (smaller catheters may
coil) into the stomach through nose or mouth. Catheter is
left in situ while an x-ray confirms the diagnosis.
• 3. pH of tracheal secretions is acidic.
• 4. Flat-plate x-ray of abdomen and chest may reveal
presence of gas in stomach and catheter coiled in the
blind pouch. Barium x-ray may be used in some cases.
• 5. Electrocardiogram and echocardiogram are performed
because there is a high association with cardiac
anomalies.

16. Management
Immediate Treatment
1. Propping infant at 30-degree angle, supine or side-
lying, to prevent reflux of gastric contents.
2. Nasogastric (NG) tube remains in the esophagus
and is aspirated frequently to prevent aspiration until
continuous low suction is applied.
3. Pouch is washed out with normal saline to prevent
thick mucus from blocking the tube.
4. Gastrostomy to decompress stomach and prevent
aspiration; later used for feedings.

17. • 5. Nothing by mouth (NPO); IV fluids.
• 6. Comorbidities, such as pneumonitis and
heart failure, are treated.
• 7. Supportive therapy includes meeting
nutritional requirements, IV fluids,
antibiotics, respiratory support, and
maintaining thermally neutral environment.

18. Surgery
• 1. Prompt primary repair:
fistula found by
bronchoscopy is divided,
followed by esophageal
anastomosis of proximal and
distal segments if infant
weight permits and is
without pneumonia.

19. 2. Short-term
delay:
• subsequent primary repair is used to
stabilize infant and prevent deterioration
when the patient’s condition contraindicates
immediate surgery.

20. 3. Staging:
• initially, fistula division and
gastrostomy are performed with
later secondary esophageal
anastomosis or colonic
transposition performed
approximately 1 year later to
effect total repair.
• Approach may be used with a
very small, premature infant or a
very sick neonate or when
severe congenital anomalies
exist.

21. 4. Circular
esophagomyotomy
performed on proximal pouch to gain length and
allow for primary anastomosis at initial surgery.

22. • 5. Cervical esophagostomy: when ends of
esophagus are too widely separated,
esophageal replacement with segment of
intestine (colonic transposition) is done at
ages 18 to 24 months.
• 6. Fiberoptic tracheoscopy-assisted repair of
tracheoesophageal fistula can expedite and
facilitate surgery on ventilated patients.
• 7. Staged repair resulted in least amount of
GI dysmotility postoperatively

23. Nursing
Diagnoses
Preoperative
• Risk for Aspiration related to structural
abnormality.
• Risk for Deficient Fluid Volume related to
inability to take oral fluids.
• Anxiety of parents related to critical
situation of neonate.

24. Postoperative
• Ineffective Airway Clearance related to
surgical intervention.
• Ineffective Infant Feeding Pattern related to
defect.
• Acute Pain related to surgical procedure.
• Impaired Tissue Integrity related to
postoperative drainage.
• Risk for Injury related to complex surgery.
• Risk for Impaired Attachment related to
prolonged hospitalization.

25. Nursing
Interventions
• Preventing Aspiration 1. Position the infant supine with head
and chest elevated 20 to 30 degrees to prevent or decrease
reflux of gastric juices into the tracheobronchial tree. a. This
position may also ease respiratory effort by dropping the
distended intestines away from the diaphragm. b. Side-lying
position can be used if the risk of aspiration from being supine
is greater than the risk of sudden infant death syndrome
(SIDS); supine positioning is preferred to reduce the risk of
SIDS. c. Turn frequently to prevent atelectasis and pneumonia.

26. • 2. Perform intermittent nasopharyngeal suctioning or maintain indwelling
Sump tube with constant suction to remove secretions from esophageal
blind pouch.
• a. Tip of tube is placed in the blind pouch.
• b. Sump tube allows air to be drawn in through a second lumen and
prevents tube obstruction by mucous membrane of pouch.
• c. Maintain indwelling tube patency by irrigating with 1 mL normal saline
solution frequently

27. • 3. Place the infant in an Isolette or under a radiant warmer with high humidity to
aid in liquefying secretions and thick mucus. Maintain the infant’s temperature in
thermoneutral zone and ensure environmental isolation to prevent infection by
using Isolette.
• 4. Administer oxygen, as needed.
• 5. Suction mouth to keep it clear of secretions and prevent aspiration. Provide
mouth care.
• 6. Be alert for indications of respiratory distress. a. Retractions. b. Circumoral
cyanosis. c. Restlessness. d. Nasal flaring. e. Increased respiration and heart rate.

28. • 7. Maintain NPO status.
• 8. Administer antibiotics, as ordered, to prevent or treat associated
pneumonitis.

29. Observe
Observe infant
carefully for any
change in condition;
report changes
immediately.
Check
Check vital signs, color
and amount of
secretions, abdominal
distention, and
respiratory distress.
Evaluate
Evaluate for
complications that can
occur in any neonate or
premature infant.

30. 10. Be available and recognize need for emergency care or resuscitation.
a. Have resuscitation equipment on hand.
b. Accompany the infant to other departments and the operating room in Isolette with portable oxygen
and suction equipment.
11. Monitor for signs or symptoms that may indicate additional congenital anomalies or complications.
12. Gastrostomy tube (GT) may be placed before definitive surgery to aid in gastric decompression,
prevention of reflux, or nutrition.

31. Preventing Dehydration
• 1. Administer parenteral fluids and electrolytes as prescribed.
• 2. Monitor vital signs frequently for changes in blood pressure (BP) and
pulse, which may indicate dehydration or fluid volume overload. 3. Record
intake and output, including gastric drainage (if GT for decompression is
present) and weight of diapers.

32. Reducing Parental Anxiety
Explain procedures and necessary events to parents as soon as possible.
Explain
Orient parents to hospital and intensive care nursery environment. 3. Allow family to hold and
assist in caring for infant.
Orient
Offer reassurance and encouragement to family frequently.
Offer
Provide for additional support by social worker, clergy, and counselor, as needed.
Provide

33. Concept map on EA& TEF

34. Thank you