2. Overview-
A fistula is an abnormal communication between two
epithelial-lined surfaces.
An atresia refers to complete obliteration or absence of
an orifice of a hollow organ.
A stenosis is change in diameter of a hollow organ to
become narrower. It can also be called stricture.
4. Introduction-
Esophageal atresia(EA) and tracheoesophageal fistula(TEF)
are the most common congenital anomalies of oesophagus.
It is a acquired communication between the trachea and
esophagus. Most of the patient with TEF are diagnosed
immediately following after birth.
TEF are often associated with life threatening complications
5. Definition-
Esophageal Atresia(EA)- defined as the complete absence of
continuity of oesophagus to stomach.
Tracheoesophageal Fistula (TEF)- defined as the congenital
abnormal connection between proximal or distal oesophagus
and the trachea.
A tracheoesophageal fistula, as the name implies, is an abnormal
communication between the trachea and esophagus.
7. Incidence-
EA with or without TEF is the most common oesophageal
malformation, occurring in approximately 1 in 3500 live
births. The vulnerable groups are- premature and low birth
weight (LBW) infants and mothers with polyhydramnios.
Often associated with other GI and cardiac anomalies.
8. Embryology-
The median pharyngeal groove (primitive digestive tube) develops in
the ventral aspect of foregut at day 22 of gestation, which develops
into the respiratory and digestive tubes.
These two tubes are separated by the mesenchyme.
The development of an abnormal epithelial-lined connection between
the two tubes results in the creation of the TEF. Excess mesenchymal
growth can stretch and disrupt the oesophagus, creating EA.
13. Classification- (Gross
classification)-5 types- EA with or
without TEF:-
- both distal
and proximal part of esophagus is blind and no
fistula is developed between esophagus and
trachea. Atresia/ Blind
pouch
14. Continuation.
- the proximal and distal part of esophagus is
connected to trachea by a fistula and the distal part of
esophagus is blind.
Fistula
Atresia
15. the proximal part of esophagus is
blind and distal part is connected by a
fistula to trachea.
Atresia
Fistula
16. both proximal and distal parts of esophagus are
connected to trachea by two separate fistula.
17. there is no presence of atresia in the
esophagus but is attached with trachea by a
single fistula.
18. Etiology-
The exact cause is unknown.
Some factors-
Heritable genetic factors- A 2% risk is present when one of the
sibling is affected; occasional association is with trisomies of 21, 13
and 18 chromosomes.
Maternal alcohol and smoking
Exogenous sex hormones
Exposure to methimazole
Prolonged mechanical ventilation via endoctracheal tube
Maternal polyhydamnios
Diabetes Mellitus during the first trimester of pregnancy.
19. Pathophysiology-
Upper part of esophagus is developed
from retropharyngeal segment and the
lower part of the first part of primitive gut.
At 4-5 weeks of gestation the laryngeal-
tracheal groove is formed.
Two longitudinal furrows develop and
separate the respiratory tract permordium
from oesophagus.
Deviation cellular growth of the septum
results in formation of fistula between
esophagus and trachea
22. Intermittent unexplained cyanosis and laryngospasm.
Poor feeding
Seal- bark cough
Violent response after first or second swallow of feeding
such as infant cough and chokes fluid through nose and
mouth.
23. Diagnosis-
Ultrasound scanning- prenatal diagnosis may reveal the size of
gastric bubble , polyhydramnios and other associated anomalies
Plain X-ray of abdomen
Chest X-ray using air contrast in the proximal pouch. If distal TEF is
present, air in the stomach will be seen on X-ray.
Passing of radiopaque catheter through oesophagus- the catheter
does not reach to the desired length.
Tracheo-brochoscopy- to examine the tracheobronchiolar tree. It helps in
detection of associated tracheobronchial anomalies.
ECG- for infants with clinical signs of cardiovascular disease.
27. Management-
Immediate management-
-Infant is managed with head elevated at a position of 30degree to
reduce the risk of gastric secretion entering into lungs.
-Nasogastric aspiration of secretions for every 5-10mins. Blind
pouch should be washed with normal saline to prevent blocking of
tube with thick mucus.
-IV fluid administration.
-Airway clearance with oxygen therapy.
-Nothing per mouth
28. Supportive management-
-Maintenance of nutritional requirements and warmth.
-Infection prevention
-Antibiotic therapy
-Respiratory support
-Chest physiotherapy and postural drainage
29. Medical management-
In healthy infants without pulmonary complications, primary repair is
done along with parenteral nutrition and upper pouch suction.
Pre-0peratively, a cuffed endotracheal tube is place distal to the fistula
site in order to prevent the reflux of gastric contents to the lungs.
30. Surgical management-
Done by end-to-end anastomosis with excision of the fistula by right
postolateral thoracotomy with internal chest drainage.
It is done with infants more than 2kg body weight with no pneumonia,
thereby emphasising clinically stable conditions.
32. •Intially gastrostomy is performed, followed by esophageal
anastomosis or colonic transplant after one year. This is done mainly
for premature and very sick infants with congenital anomalies.
•Circular oesophagotomy- performed on proximal pouch to gain
length and allow for primary anastomosis at initial surgery.
•Cervical oesophagotomy/ Esophagocoloplasty- When
oesophagus ends are too widely separated oesophageal replacement
with the segments of intestine (colonic reposition).
It is done at the age of 18 to 24 months.
• After surgical methods, IV fluid therapy is given for the first 48 hours
followed by gastrostomy feeding.
33. Nursing management-
A mother who is carrying the foetus may have polyhydramnios, which occurs
in approximately in 33% cases and virtually 100% of mothers with foetuses with
oesophageal atresia without fistula.
VACTERL (vertebral defects, anorectal malformations, cardiovascular
defects, tracheoesophageal defects, renal anomalies, and limb deformities) refers
a set of associated anomalies that should be readily apparent upon physical
examination.
The presence of the others must be assessed.
34. a. Preoperative-
related to abnormal communication between
trachea and oesophagus as evidenced by excessive secretions, respiratory
distress.
elated to feeding
difficulties as evidenced by regurgitation of feed.
related to obstruction secondary to anatomical defect as
evidenced by drooling of saliva.
elated to abnormal communication between oesophagus
and trachea.
related to inadequate oral intake.
elated to neonatal surgical emergency.
related to physical defect and hospitalization.
35. b.Postoperative-
related to surgical interventions as evidenced by
assessment.
related to inadequate oral intake
as evidenced assessment and observation.
related to surgical interventions as evidenced by facial grimancing and cry
of the infant.
related to surgical wound as evidenced by assessment of
the surgical site incision.
related to the hospital procedures.
related to complex surgery.
related to prolonged illness.
related to home- based long term care.
36. a. Preoperative-
•Preventions of aspirations by positioning, suctioning and NPO.
•Assessment of respiratory status.
•Administration of oxygen.
•Prevention of dehydration by IV fluid, intake and output chart, monitoring of vital
signs and general health.
•Perform oropharyngeal suctioning to remove accumulations of secretions.
•Placement of suction catheter in proximal pouch for every 10-15mins.
•Watch for any choking and cyanosis during feeding.
•Prevention of infections by infection control measures.
•Teach family about gastrostomy feeding techniques.
•Reducing parental anxiety by explaining the outcomes of the impending surgery
and by emotional support.
37. b.Postoperative-
•Maintaining of clear airway.
•Providing adequate feeding by IV fluid and gastrostomy feeding.
•Pain management-
•By tactile stimulations
•Administering of analgesics (if necessary)
•Provision of comfortable position with comfort devices.
•Periodic inspection and assessment of the surgical site.
•Maintaining of chest tube drainage with necessary precautions.
•Monitoring of child’s condition and detection of problems early.
•Prevention of infections by careful handling of the baby.
•Stimulating parent-child bondage by parental participation of child care.
•Improving knowledge by necessary health education, encouraging questions
and explaining answers.
38. Complications-
a. Recurrent pneumonia
b. Acute lung injury
c. Acute respiratory distress syndrome, lung abscess
d. Poor nutrition
e. Bronchiectasis from recurrent aspiration
f. Respiratory failure
g. Hiatal hernia
h. GERD
i. Anastomotic leak
j. Tracheomalacia
k. The major postoperative complications are-
tracheal stenosis and recurrent fistula.
