chapter 6-1 Esophageal atresia and TEF.ppt

Esophageal atresia and TEF

1. Eyayalem M.
Esophageal Atresia and
Tracheoesophageal Fistula

2. INTRODUCTION
•Tracheoesophageal fistula(TEF) is a congenital or
acquired communication between the trachea
and esophagus.
•Esophageal atresia is a congenital abnormality in
which the midportion of the esophagus is absent.
•Incidence is between 1 in 3,570 -1 in 4,500
•Esophageal atresia(EA) occurs in association with
TEF in about 90% of instances.
•TEF most likely leads to fatal pulmonary
complications.
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3. Introduction
•Most congenital TEFs are diagnosed
immediately following birth or in infancy.
•They are commonly associated with other
congenital anomalies.
•Acquired TEF occur secondary to malignant
disease, infection,, trauma, etc.
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4. Introduction
•Most congenital TEFs are diagnosed
immediately following birth or in infancy.
•They are commonly associated with other
congenital anomalies.
•Acquired TEF occur secondary to malignant
disease, infection,, trauma, etc.
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5. Etiology
•Exact aetiology of TEF is unclear.
•Embryologically believed to be due to
incomplete closure of laryngotracheal groove.
•Observed in mothers that used imidazole
containing decongestants in the first trimester
of pregnancy.
•Association with trisomies 18, 21, 13.
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6. Etiology
•Associated risk factors
•Advanced maternal age
•European ethnicity
•Obesity
•Low socioeconomic status
•Tobacco smoking
•Infants < 1500g ( highest mortality).
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7. Etiology
•Approximately 50% affected infants have
associated anomalies.
•Syndromes include VATER/VACTERL( vertebral,
anorectal, cardiac, tracheal, esophageal, renal,
radial).
•Some genetic factors have been implicated
with discrete mutations in syndromic cases.
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8. EMBRYOLOGY
•The esophagus and trachea both develop from
the primitive foregut.
•During 4 –6 weeks of life the caudal part of
the foregut forms the ventral diverticular
which gives rise to the trachea.
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9. Embryology
•The longitudinal tracheoesophageal fold fuses
to form a septum, dividing the foregut into a
ventral laryngotracheal tube and a dorsal
esophagus.
•Posterior deviation of the septum causes
incomplete separation of the esophagus from
the laryngo tracheal tube resulting in TEF.
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10. Embryology
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11. Embryology
•Isolated esophageal atresia can occur when
the esophagus fails to recannalize by the 8
th
week.
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12. Successive stages in the development of the
tracheoesophageal septum during embryologic development.
•(A)The laryngotracheal diverticulum forms
as a ventral outpouching from the caudal
part of the primitive pharynx.
•(B)Longitudinal tracheoesophageal folds
begin to fuse toward the midline to
eventually form the tracheoesophageal
septum.
•(C)The tracheoesophageal septum has
completely formed.
•(D)If the tracheoesophageal septum
deviates posteriorly, esophageal atresia
with a tracheoesophageal fistula develops

13. Introduction

14. Classification of
Tracheoesophageal fistula
Anatomic characteristics Percentageof cases
Esophageal atresia with distal TEF87%
Isolated esophageal atresia without
TEF
8%
Isolated TEF (H type). 4%
Esophageal atresiawith proximal TEF1%
Esophageal atresia withproximal and
distal TEF
1%
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15. Classification –2
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16. The most common types of EA &TEF

17. Anatomic Variations
•85%
•Most common
•VOGTtype3(b)
•GROSS type C

18. Anatomic Variations
6%
Atresia alone,
•no fistula
•Small stomach,
gasless abdomen
•Usually has a long
gap between the
esophagealends
•VOGT types 1 and 2
•GROSS type A

19. Anatomic Variations
• 2%
•Proximal tracheo-
esophageal fistula
•No distal fistula
Small stomach,
•gasless abdomen
•Often has a long
gap between the
esophagealends
•VOGT type 3(a)
•GROSS type B

20. Anatomic Variations
• l%
•Proximal and
distal fistulas
("double fistula")
•VOGT type 3(c)
•GROSS type D

21. Anatomic Variations
•6%
•No atresia of
the esophagus
•Congenital
tracheoesophageal
fistula
"H" or "N" fistula
•GROSS type E

22. Physiologic effects of distal tracheoesophageal fistula
•1. Hyaline membrane disease may
necessitate higher ventilator pressures,
which encourage air to pass through
the distal fistula.
•2. A distended abdomen elevates and
"splints" the diaphragm.
•3. Gastric distension may result in
gastric rupture and
pneumoperitoneum.
•4. Passage of air through a distal
tracheoesophageal fistula diminishes
the effective tidal volume.
•. (B) 1. Aspiration of gastric juices leads
to soiling of the lungs and pneumonia
•2. Gastroesophageal reflux
•3. Direction of gastric fluid proximally
through distal fistula.
•4. Overflow of secretions or
inadvertent feeding may contribute to
aspiration and contamination of the
airway. .

23. Associated Abnormalities
Incidence of Associated Anomalies in Esophageal Atresia.
Anomaly Frequency (%)
•Congenital heart disease 25
•Urinary tract 22
•Orthopaedic (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

24. Associated Congenital Anomalies Reported in Patients with Esophageal
Atresia
System affected
Musculoskeletal
Gastrointestinal
Cardiac
Genitourinary.
Potential anomalies
Hemivertebrae, radial dysplasia or
amelia, polydactyly, syndactyly, rib
malformations, scoliosis, lower limb
defects
Imperforate anus, duodenal atresia,
malrotation, intestinal
malformations, Meckel's
diverticulum, annular pancreas
Ventricular septal defect, patent
ductus arteriosus, tetralogy of Fallot,
atrial septal defect, single umbilical
artery, right-sided aortic arch
Renal agenesis or dysplasia,
horseshoe kidney, polycystic kidney,
ureteral and urethral malformations,
hypospadias

25. DIAGNOSIS OF ESOPHAGEAL
ATRESIA
•Antenatal Diagnosis(maternal
polyhydramnios, a small stomach, a distended
upper esophageal pouch, or abnormal
swallowing)
•Diagnostic suspicion is increased when
abnormalities known to be associated with
esophageal atresia are identified.

26. Fetal MRI
•This 32 week
fetus had
esophageal
atresia and an
absent stomach,
resulting in
marked
polyhydramnios

27. Clinical Diagnosis
•Prematurity
•Any excessively drooling (copious, fine, white,
frothy bubbles of mucus in the mouth and,
sometimes, the nose).

28. Clinical Diagnosis
Diagnosis of esophageal
atresia is confirmed when a
10-gauge (French) catheter
cannot be passed beyond 10
cm from the gums
A smaller-caliber tube is not used
because it may curl up in the upper
esophageal segment, giving a false
impression of esophageal continuity.

29. The chest radiograph
•A plain radiograph will
confirm the tube has
not reached the
stomach

30. The Gasless Abdomen
•Absence of gas in the
abdomen suggests that
the patient has either
atresia without a fistula
or atresia with a
proximal fistula only

31. Contrast studies
•should be performed by an
experienced pediatric
radiologist, or after transfer
to the tertiary institution,
and with the use of a small
amount (0.5 to 1 mL) of
water-soluble contrast. Care
must be taken to avoid
aspiration.

32. Management
•Measures should be taken to reduce the risk of
aspiration(continuous suctioning of the upper pouch,
the infant's head should be elevated).
•In infants with respiratory failure, endotracheal
intubation should be performed.
•Transfer to a major tertiary pediatric institution is
best not delayed.

34. 34
treatment
Goals of the initial treatment:
1)Attention to ventilation
2)↓ upper pouch pressure
3)Determine appropriate time for surgery
So:
For ↓ aspiration risk:
1)elevate neonate’s head at least 30º in infant warmer
2)Use “sump” catheter on34continiouse suction
IV AB and electrolyte.