This document discusses the embryology, pathology, classification, diagnosis and treatment of tracheoesophageal fistula (TEF). It begins with a description of the embryological development of the esophagus and trachea. It then covers the pathology, classifications including Gross-Vogt and Waterston systems. It discusses the clinical findings and diagnosis of TEF. It concludes with descriptions of preoperative treatment and surgical repair techniques for TEF.

1. Dr. Faheem ul Hassan
M.Ch Student
Pediatric and Neonatal Surgery
Dr. Gowhar Mufti
Asst. Professor
Pediatric and Neonatal Surgery
Tracheoesophageal Fistula

2. Dr. Faheem ul Hassan Andrabi
M.Ch
Pediatric and Neonatal Surgery
Dr. Gowhar Mufti
Asst. Professor
Pediatric and Neonatal Surgery
Tracheoesophageal Fistula

3. Historical background

4. Embryology
• no single unifying theory has been proposed
that addresses all the variations seen with
this group of anomalies.
• The pathogenesis is heterogeneous and
multifactorial and involves multiple genes

5. Embryology

6. Embryology
• Esophagus and trachea form from the
primitive foregut
• At 22 days’ gestation, the MPG
develops in the ventral aspect of the
foregut and elongates
• This tissue develops into the
respiratory and digestive tubes
• Normally, mesenchyme proliferating
between the respiratory and digestive
tubes separates the tubes

7. Embryology
• the development of an abnormal
epithelial-lined connection between
the two tubes results in the creation of
a TEF.
• The excess tissue growth may lead
to incorporation of part of the
esophagus into the posterior wall of
the trachea

8. Embryology
• Excess mesenchymal growth can
stretch and disrupt the esophagus,
creating EA

9. Blood supply

10. Pathology
• The trachea, retains a U-shaped
configuration, with a wide membranous
portion, rather than the normal C-shape.
• This commonly leads to tracheomalacia of
varying degrees of severity.
• Loss of the ciliated epithelial lining of the
trachea may also be present

11. Pathology
• The presence of EA/TEF also disrupts the
normal in utero development of the myenteric
plexus in the esophagus,
• This leads to disordered peristalsis and
impaired lower esophageal sphincter
function

12. Pathology
• Structural abnormalities are also commonly
found in the esophagus, including
disorganized muscle layers.
• This may also contribute to abnormal
esophageal motor function

13. Pathology
• The resting pressure in the whole oesophagus
is significantly higher than in normal patients
• closing pressure of the lower oesophageal
sphincter is also reduced.

14. Epidemiology
• incidence of EA, with or without TEF (EA/TEF),
is 1:3500 live-born infants
• it varies geographically from 1 in 2440 births in
Finland to 1 in 4500 births in US
• male-to-female ratios varied considerably
between types of EA-TEF defects
• However 62% of infants with EA-TEF were
male

15. Risk Factors
• First pregnancy
• Increasing maternal age
• The rate of multiple births
• In addition, EA, along with other congenital
anomalies, is significantly increased in the
offspring of in vitro fertilization patients

16. Pathology
• Structural abnormalities like disorganized
muscle layers are also commonly found in the
esophagus.
• This may also contribute to abnormal
esophageal motor function

17. Associated Anomalies
• Other congenital anomalies are frequently
associated with EA,
• associated anomaly often significantly alters
treatment and affects survival.
• The anomalies are most common in cases of
EA without TEF and are
• least common in cases of H-type TEF

18. Associated Anomalies
• 50% of patients with associated malformations
form a part of the recognizable syndromes like
– VACTERL
– CHARGE
– Fanconi anemia
– Opitz G, and
– Goldenhar.

19. Associated Anomalies
• The other 50% of patients with associated
malformations are considered as
“nonsyndromic”

20. Associated Anomalies
• 1-year survival rate of infants with congenital
heart disease (CHD) was 67% versus 95% in
those without CHD.
• The most common CV defects associated with
TEF are
– VSD (19%)
– ASD (20%)
– TOF (5%)
– PDA(13%).
– COA (1% to 4%)
– A descending aorta on the right side has been
reported in 4%

21. Gastrointestinal anomalies
• Gastrointestinal anomalies associated with EA
TEF are
– anorectal malformations(14%)
– intestinal malrotation (4%)
– duodenal atresia (2%)
– ileal atresia, annular pancreas,
– and pyloric stenosis.

22. Genitourinary defects
include
• renal agenesis or hypoplasia
• hypospadias,
• undescended testes,
• cystic renal disease,
• Hydronephrosis, VUR, PUJ & VUJ obstruction
etc
musculoskeletal malformations include limb (15%) and
vertebral (17%) anomalies

23. VACTERL
• The incidence of the VACTERL association in
the EA population is approximately 20%.
• Infants with EA-TEF frequently have VACTERL
anomalies
– Vertebral 17%
– Anal 12%
– Cardiac , 20%
– Renal , 16%
– Limb 10%)
other midline defects (cleft lip and palate, 2%; sacral
dysgenesis, 2%; urogenital anomalies, 5%).

24. VACTERL
It generally requires a minimum of three of the components to
qualify as a “VACTERL patient.”
Recently, the VACTERL-H association has been described and
includes congenital hydrocephalus
In this series by Iuchtman and colleagues, the mortality rate
was 24% in patients of EA/TEF with VACTERL.

25. Classification
• The most useful and practical classifications
are perhaps simple anatomic descriptions
Esophageal atresia without a tracheoesophageal fistula.
This malformation is almost invariably associated with a “long gap.”
Esophageal atresia without a tracheoesophageal fistula.
This malformation is almost invariably associated with a “long gap.”

26. Gross-Vogt Classification
•Atresia with a proximal tracheoesophageal fistula.
•It is an uncommon anomaly
•Abdomen is airless, and the diagnosis may be missed
•Contrast studies or endoscopy help in diagnosis
•Atresia with a proximal tracheoesophageal fistula.
•It is an uncommon anomaly
•Abdomen is airless, and the diagnosis may be missed
•Contrast studies or endoscopy help in diagnosis

27. Gross-Vogt Classification
Esophageal atresia with a distal tracheoesophageal fistula,
the most frequently encountered form of esophageal anomaly.
Esophageal atresia with a distal tracheoesophageal fistula,
the most frequently encountered form of esophageal anomaly.

28. Gross-Vogt Classification
Atresia with a double (proximal and distal) fistula.
Though rare, this form is found more
often than originally thought.
Atresia with a double (proximal and distal) fistula.
Though rare, this form is found more
often than originally thought.

29. Gross-Vogt Classification
Tracheoesophageal fistula without atresia (H-type fistula).
This anomaly may be missed in the newborn period because
swallowing is possible.
It is associated with recurrent pneumonia, and abdominal distention.
Tracheoesophageal fistula without atresia (H-type fistula).
This anomaly may be missed in the newborn period because
swallowing is possible.
It is associated with recurrent pneumonia, and abdominal distention.

30. Gross-Vogt Classification
Esophageal stenosis.

31. Waterston Classification
Group
Survival
(%)
Waterston Classification
A 100 Birth weight >2000 and healthy
B 85
Birth weight 2000-2500 and healthy
Higher birth weight but moderate presence
of pneumonia or congenital anomaly
C 65
Birth weight <2000
Higher birth weight but severe pneumonia
or complicated anomaly

32. Waterston Classification
• Group A: Good Risk: treated with immediate
operative repair
• Group B: Moderate Risk: managed by
delayed repair
• Group C: High Risk: treated by staged repair

33. Classification
• In 1989 Randolph and colleagues suggested a
refinement in Waterston’s classification on the
basis of the overall physiologic status
• All infants who demonstrated a stable cardiac
and respiratory status were chosen for
immediate repair.
• Their experience led to more and earlier
primary repairs with maintenance of excellent
survival rates in stable infants.

34. Classification
• Spitz and colleagues found that birth weight
and major cardiac disease were important
predictors of survival.
Spitz is currently the most commonly used classification

35. Diagnosis and Clinical Findings
• Prenatal EA is not commonly diagnosed.
• Prenatal detection of EA by USG relies on the
finding of a small or absent stomach bubble
and associated maternal polyhydroamnios
• predictive value of prenatal USG in the
diagnosis of EA is only 20% to 40%.

36. Diagnosis and Clinical Findings
• Most infants with EA are symptomatic in the
first few hours of life.
• The earliest clinical sign of EA is usually
– excessive salivation followed by
– regurgitation,
– choking, and
– coughing.

37. Diagnosis and Clinical Findings
Other features are
• Cyanosis,
• Respiratory distress,
• Inability to swallow, and
• Inability to pass a feeding or suction catheter
through the mouth or nose into the stomach.

38. Diagnosis and Clinical Findings
Pulmonary compromise can become significant
in TEF with distal Fistula due to
• Reflux of gastric fluid into the trachea and
lungs which leads to chemical pneumonitis
• Abdominal distension with splintage of
diaphragm

39. Diagnosis and Clinical Findings
•The diagnosis of EA can be
confirmed by passing a firm catheter
or an IFT
• A few mls of air can be Injected to
distend the upper esophageal pouch
•If necessary, 0.5 to 1.0 mL of
diluted barium can be used as a
contrast agent

40. Diagnosis and Clinical Findings
• Barium may also detect a proximal TEF
however, barium identified in the
tracheobronchial tree is more likely to represent
contrast aspirated through the larynx rather
than through a proximal TEF.
• A short upper pouch may suggest the presence
of a proximal TEF

41. Diagnosis and Clinical Findings
Absence of intestinal air suggests isolated esophageal
atresia without tracheoesophageal fistula. Also note vertebral anomalies

42. Diagnosis and Clinical Findings
• Air in the stomach confirms the presence of a
distal TEF
• Absence of air represents EA without distal
TEF but the
• presence or absence of a proximal TEF must
be confirmed by bronchoscopy.
• it is possible to have absence of intestinal air in
case of an occluded distal fistula, which is
identified at the time of operation

43. Diagnosis and Clinical Findings H-Type
The diagnosis of TEF without EA is difficult and
• requires a high index of suspicion
• The diagnosis is often delayed
• investigations are triggered by
• repeated coughing or choking during feedings,

44. Diagnosis and Clinical Findings H-Type
• XRC often shows pulmonary infiltrates suggesting
aspiration.
• The diagnosis can be made by barium esophagography
in the prone position
• Bronchoscopy with or without esophagoscopy is often
required to confirm the diagnosis

45. Diagnosis and Clinical Findings
• The clinical evidence of other anomalies should be
looked for
• Additional testing usually includes echocardiography,
renal ultrasonography, and chromosomal analysis
• it is not unusual for the finding of an anorectal
malformation to precede the clinical signs and
symptoms of a concomitant EA

46. Preoperative Treatment
• Immediate management includes measures to prevent
further aspiration and treatment of pneumonitis.
• A sump catheter should be positioned in the upper
esophageal pouch to continuously aspirate saliva
• The double-lumen Replogle type of catheter is best
for this purpose
• The infant should be positioned head-up, prone
position to minimize reflux.

47. Preoperative Treatment
• Broad-spectrum antibiotic coverage and pulmonary
physiotherapy are also initiated.
• Vitamin K analogue should also be administered before
surgery.
• Routine endotracheal intubation should be avoided
because of the (risk for gastric perforation and worsening respiratory distress)

48. EA and a large distal TEF
• severe respiratory distress may occur
• preoperative endotracheal intubation is often
necessary
• However, inspired air is diverted through the TEF into
the stomach which may worsen ventilation
• Affected infants may require emergency surgical
intervention to stabilize the pulmonary status and avoid
gastric perforation

49. EA and a large distal TEF
Intervention Complication
Gastric division
Banding of gastroesophageal Jnx
positioning the tip of the endotracheal
tube below the fistulous orifice.
Inadvertent intubation through a large
fistula may be fatal
Bronchoscopic placement of a Fogarty
balloon catheter through the fistula
technical difficulties with this approach
and problems with injury to the distal
end of the esophagus in which the
balloon is inflated
Emergency gastrostomy to
decompress the air-filled stomach
pulmonary collapse
because each breath volume bypasses
the lung

50. EA and a large distal TEF
Intervention Complication
gastrostomy
tube with an underwater seal
A small balloon catheter
passed through the gastrostomy into
the lower esophagus
can provide excellent palliation

51. Operative Repair
• Emergent operation is seldom necessary,
• A period of 24 to 48 hours permits full
assessment of the infant and treatment of
pulmonary insufficiency including atelectasis
and pneumonitis.
• open thoracotomy or thoracoscopic division of
the fistula with primary anastomosis of the
esophagus is the operative procedure of
choice.

52. Operative Repair
• If a right-sided aortic arch (2.5%) is identified on
a preoperative echocardiogram, a left-sided
thoracotomy is preferred
• A double aortic arch is not unusual and
makes a left thoracic approach difficult as well.
• The thorax is entered through the fourth
intercostal space
• Approach can be extrapleural or transpleural

53. Operative Repair
Extrapleural
• Anastomotic leak does not result in empyema
but merely causes an esophagocutaneous
fistula, which typically closes in 1 to 2 weeks.
Transpleural
• Operative time is shorter and,
• with current antibiotic options, the risk for
empyema after a leak is minimal

54. Operative Repair
• When the posterior mediastinum is exposed, the
upper pouch, distal TEF, trachea, and vagus
nerve are identified.
• every effort is made to preserve the vagal
fibers
• While ligating TEF 1-2 mm of esophagus is left
on the tracheal end of the fistula to prevent
tracheal stricture
• Leaving more esophagus may lead to
diverticulum formation

55. Operative Repair
• The extent of distal mobilization should be
minimized to avoid damaging vagal branches
and the segmental blood supply.
• However, distal mobilization should be
undertaken if it is necessary to ensure a
primary low-tension anastomosis.
• Mobilization of the upper pouch should be
sufficient to bring the upper pouch down to the
distal esophageal segment.

56. Operative Repair
• Extensive dissection of the proximal pouch
allows for identification of an undiagnosed
proximal TEF.
• Because the cervical blood supply to the
proximal pouch is excellent, extensive
dissection does not run a significant risk for
ischemic injury.

57. Operative Repair
• Care should be observed while dissecting
esophagus from trachea to avoid
inadvertently opening the trachea.
• Unfortunately, the more extensive the proximal
dissection, the greater the likelihood of
disturbance to vagal branches.

58. Operative Repair
• The effect of the type of suture on subsequent
stricture formation has been a point of debate.
• Chittmittrapap and colleagues reported a
statistically significant increased rate of
stricture with braided silk
• The drain should be placed away from the
anastomosis.

59. Operative Repair
• Another technique for handling the fistula and
uniting the esophageal ends was devised by
Sulamaa and colleagues.
• In this technique end-to-side anastomosis is
done after ligating the fistula.
• Because of concern for recurrent TEF
formation, this method has not been widely
accepted

60. Thoracoscopic repair of EA-TEF
• Three 2.5- to 5-mm transpleural access trocars, an angled
telescope are used.
benefits include
• superior visualization
• improved cosmesis,
• Avoiding scoliosis, winged scapula, chest wall asymmetry,
and maldevelopment.
However, the thoracoscopic EA-TEF repair is technically
demanding and requires advanced endosurgical skills

61. Operative Repair
• in a premature baby, the decision to operate
should be made early because the HMD will
worsen in the first 24 to 36 hours and will make
the lungs even less compliant

62. Long-gap esophageal atresia
• Although much has been said and written about
the subject, there is no precise definition of
“long-gap” EA
• Preoperative measurement of gap length can
be biased by the method used
• Some assess gap by inserting a radiopaque
tube into the upper pouch and contrast or a
flexible endoscope into the distal pouch
through a gastrostomy

63. Assessment of gap
Esophageal atresia without a TEF is almost invariably associated
with a “long gap.”
There will be gasless abdomen on x-ray

64. Gapogram

65. Assessment of gap
Preoperative assessment of gap can be roughly assessed by plain x-ray of cervical &
thoracic spine in lateral view with large bore tube in esophagus.
The distance between tube & expected carina level at T4 is measure of the gap
existing between the two ends of esophagus.

66. Assessment of gap
Tube at T1-T2 level (gap length > 2.1 cm),
(b) arrest at T3–T4 level (gap length >1 cm),
(c) arrest at T4 level (gap length = nil)
Rassiwala, M., et al., Determinants of gap length in esophageal atresia with tracheoesophageal fistula and the impact of gap
length on outcome. Journal of Indian Association of Pediatric Surgeons, 2016. 21(3): p. 126.

67. Long-gap esophageal atresia
In “long gap” atresia most pediatric surgeons
would proceed with
• a gastrostomy tube,
• a period of observation,
• and an attempted delayed primary repair.

68. Long-gap esophageal atresia
During the first several months of life, the gap
between the two ends deceases due to
• spontaneous growth
• reflux of bolus feeds

69. Techniques of Gap reduction
• The ideal surgical treatment for patients with
long-gap EA has not been determined, and the
topic is still very controversial.
• The most commonly used method is upper
pouch bougienage with forward pressure
applied daily or twice daily for 6 to 12 weeks
before attempting delayed primary repair

70. Rehbein and Schweder
• At surgery a nylon thread is passed from the
UP to LP and exteriorised through the
gastrostomy. Over this thread silver olives are
passed to elongate simultaneously both the
segments.

71. Shafer and David
• Shafer and David used a similar technique to
create a fistula between the two ends of the
esophagus by simply connecting the two ends
of the mobilized esophagus by a bridging silk
suture

72. Kimura
It is extrathoracic elongation of esophagus
UP is mobilized and brought out as an end cervical esophagostomy.

73. Kimura
Every 2 to 3 weeks the cutaneous stoma is surgically
translocated down the anterior chest wall
when enough length is achieved an end-to-end
esophageal anastomosis is done.

74. Foker
traction sutures on both the proximal
and distal esophageal pouches exit through the chest wall

75. Foker
These Traction sutures are serially pulled in opposite directions until the pouches
Approximate
Traction of 2-3 mm is applied daily and Primary repair (10 to 14 days)

76. Foker
A complication that may occur with this technique is the undesired cutting of
the esophagus by the sutures leading to leak and pneumothorax

77. Kimura Foker
• Kimura and Foker can be done in same patient
– Kimura for proximal pouch and Foker for the distal
pouch
• Bagolan advocates that significant length can
be simply obtained after 20 to 30 minutes of
traction on the esophageal ends

78. Livaditis
Involves circular myotomy of the upper part of oesophagus to gain the
extra length.
Circular myotomy reduces the tension by 50% and provide additional
length of 0.5 cm.

79. Livaditis
Kimura et al. modified the original procedure by giving spiral incision
instead of circular thus avoiding pseudodiverticulum formation and leak

80. Livaditis
• complications specific to livaditis myotomy are
– anastomotic leak,
– Peudodiverticulum formation
– oesophageal dysmotility,
– oesophageal stricture,
– dysphagia

81. Livaditis
• Several reports have suggested the use of a
distal esophageal pouch circular myotomy
in addition to proximal myotomy to achieve a
primary esophageal anastomosis.

82. Flap
Another method used to elongate the upper pouch involves the creation
of a full-thickness anterior flap as described by Gough

83. Additional techniques
• Despite the long-held opinion that the blood
supply to the distal end of the esophagus is
tenuous and may be compromised by
mobilization
• many surgeons have found that the distal
esophagus can be mobilized

84. Additional techniques
• end-to-end esophageal anastomosis is
achievable by completely mobilizing the distal
esophagus down to and even through the
esophageal hiatus of the diaphragm
• Scharli described a combined abdominal and
thoracic procedure achieved by division of the
left gastric artery, mobilization of the gastric
cardia and upper fundus.

85. Esophageal replacement
Colon replacement, anastomotic leak (30% to 50% of cases),
stricture, and intrathoracic redundant colon
with
stasis, gastric reflux, respiratory problems,
and diarrhea
reversed gastric tube similar
jejunum
Gastric transposition
A recent review reports excellent outcomes
with gastric transposition for long-gap esophageal atresia

86. EA without TEF
• Infants born with isolated EA have almost no
esophagus in their thorax.
• Ein and Shandling reported a
– Incidence of prematurity (52%) , an
– Down syndrome (11%)
– duodenal atresia (10%) ;

87. EA without TEF
• A gastrostomy tube should be placed within the
first 24 to 48 hours of life WITH delayed
primary repair
• Placement of the gastrostomy tube allows the
early institution of enteral feedings, which leads
to enlargement distal esophageal pouch

88. EA without TEF
• Over an 8- to 12-week period, the process of
esophageal elongation can be monitored by
• When lower esophagus does not reach above
the diaphragm a primary gastric transposition is
done.

89. Isolated (H-type) tracheoesophageal
fistula
• Anatomically accurately called the “N” type
• occurs in approximately 4% of esophageal anomalies
• Neonate repeatedly chokes on attempting to feed
• Patient has unexplained cyanotic spells.
• Intermittent abdominal distention can occur on
coughing or crying as air passes through the fistula into
the stomach.
• Older infants present with recurrent bouts of
pneumonia typically involving the right upper lobe.

90. Isolated (H-type) tracheoesophageal
fistula
• A reliable way to establish the diagnosis is by tube video
esophagography performed while the infant is prone.
• Bronchoscopy with esophagoscopy can typically
confirm the diagnosis
• Bronchoscopy allows for the passage of a fine catheter
or guidewire through the fistula for identification at
surgical exploration

91. Isolated (H-type) tracheoesophageal
fistula
• Most isolated TEFs can be successfully divided through
a cervical approach.
• A right thoracotomy can be used
– when the fistula is located within the thorax or
– When a recurrent fistula from a previous EA repair is being
approached

92. Isolated (H-type) tracheoesophageal
fistula
• Post-op complications are respiratory distress due to
– edema of the trachea or
– injury to the recurrent laryngeal nerves
• Esophageal leak and recurrence of isolated TEF are
rare

93. Esophageal atresia with an upper
pouch fistula
• The diagnosis can be made by a preoperative proximal
pouch contrast study, preferably performed by a
skilled pediatric surgeon
• However, a frequent point of confusion during this study
is the spillover of contrast from the pouch up into the
larynx and down into the trachea.

94. Esophageal atresia with an upper
pouch fistula
• Some surgeons rely on preoperative bronchoscopy to
confirm the diagnosis.
• A small proximal fistula can also be missed when
endoscopy is used.
• Fistula can also be identified by complete mobilization
of the upper pouch during repair of the EA.

95. Complications
• Anastomotic leak occurs in approximately
• 13% to 16% of patients
• Most leaks are clinically insignificant and can be
managed with adequate drainage and
nutritional support
• With a retropleural approach and a patent
mediastinal drain is in place, up to 95% of
anastomotic leaks close spontaneously

96. Anastomotic leak
• Anastomotic leak occurs in approximately
• 13% to 16% of patients
• Most leaks are clinically insignificant and can be
managed with adequate drainage and
nutritional support
• With a retropleural approach and a patent
mediastinal drain is in place, up to 95% of
anastomotic leaks close spontaneously

97. Anastomotic leak
• Factors that contribute to anastomotic leak
include
– poor surgical technique
– ischemia of the esophageal ends
– use of myotomy, and
– excessive tension at the anastomotic site

98. Major Anastomotic leak
• leaks which are typically recognized early 24 to 48
hours (Coran)
• involvement of more than one-fourth circumference of
the esophagus as a major leak (Chittmittrapap)
• 20% of the total nasogastric feeds draining out through
the intercostal drain

99. Major Anastomotic leak
• <20% of total nasogastric feeds come from drain with
chest complications (pneumothorax, persistent collection,
and lung collapse) (JIAPS)
• more than 4 mm dehiscence (D’Urzo)
• Leak with HDI

100. Mx of Anastomotic leak
• reoperation with adequate drainage and
repair anastomotic leak.
• A pleural or pericardial patch with or without
an intercostal muscle flap buttress, may help.
• Diversion esophagostomy and delayed
esophageal replacement may be required in
some cases.

101. Esophageal Stricture
• is a common complication of anastomosis of
the esophagus in EA
• Numerous criteria are used to define a stricture
• In a recent large series, stricture requiring
dilatation is reported to occur in up to 80% of
patients
• stricture be defined as the presence of
symptoms and narrowing noted on
endoscopy or contrast esophagography (Spitz
• and Hitchcock)

102. Esophageal Stricture
• Factors that have been implicated in the
pathogenesis of stricture include
– poor anastomotic technique (excessive tension, two-layered
anastomosis, and silk suture material),
– long gap,
– ischemia at the ends of the esophagus,
– GERD, and anastomotic leak.

103. Esophageal Stricture
• Up to 13% of children will require removal of an
esophageal foreign body after repair of EA ,
even in the absence of a stricture on contrast
studies.
• Parents have to be counseled about
– slow introduction of solids and
– keeping the food minced until the child has enough
molar teeth to grind the food adequately

104. Esophageal Stricture
• Treatment is dilation performed via
antegrade or retrograde bougienage
• Dilatation is done by
– Savary-Gilliard type prograde dilatation
over a guidewire
– Endoscopic balloon dilation

105. Esophageal Stricture
• Many strictures respond to one to three
dilations (53%) in the first months
• recalcitrant stricture resistant to repeated
dilations will require
– resection and reanastomosis or
– Esophageal replacement

106. Esophageal Stricture
• Recently, endoscopic injection of mitomycin C
and triamcinolone have been used to treat
recurrent strictures with some succes
• Many strictures do not respond to dilation
attempts if severe GERD continues to bathe
the stricture with acid

107. Esophageal Stricture
• Because all EA patients demonstrate GER
to some degree, children are routinely
prescribed histamine-2 blockers at the time of
discharge
• Strictures associated with GERD may be
treated by antireflux procedures followed by
dilatation.

108. Recurrent Tracheoesophageal Fistula
• occurs in 3% to 14% of patients
• Recurrent TEF has been attributed to anastomotic leak
with erosion trachea
• Symptoms are those seen with a congenital H-type TEF
• Techniques that have minimized recurrent TEF include
– use of a pleural flap
– vascularized pericardial flap and
– azygos vein flap

109. Gastroesophageal Reflux Disease
• all EA patients demonstrate GER to some
degree
Predisposing factors are
• trend to preserve the native esophagus
• shortening of the intra-abdominal portion of the
esophagus
• esophageal motor dysfunction

110. Gastroesophageal Reflux Disease
Symptoms
• vomiting,
• dysphagia, and
• recurrent anastomotic stenosis
• Recurrent pneumonia, and
• reactive airways disease

111. GERD
• The diagnosis is by upper gastrointestinal
contrast study.
• Twenty-four-hour pH probe data, (though not as
• standardized for children as they are for adults), typically
• Extensive esophageal manometric studies
• Multichannel esophageal impedance
combined with pH monitoring may emerge as a
superior test

112. GERD
Aggressive medical management consists of
• thickening of feedings,
• positioning of the infant in a prone or upright
posture, and
• administration of acid reduction agents such
as histamine-2 blockers, proton pump
inhibitors, and
• prokinetic agents.

113. GERD
• 45% to 75% of these infants ultimately undergo
antireflux operations
• Some surgeons prefer the anterior Thal partial-
wrap fundoplication than Nissen fundoplication
in these patients
Nissen is Debilitating dysphagia and significant complications including
wrap disruption and recurrent GERD in a third of patients

114. Tracheomalacia
• Occurs in about 75%
• the condition is problematic in
approximately 10% to 25%
patients half of whom require
surgical correction

115. Tracheomalacia
• Tracheomalacia is defined as
generalized or localized weakness
of the trachea that allows the
anterior and posterior tracheal walls
to come together during
expiration or coughing.

116. Tracheomalacia
Causes of Tracheomalacia
• Shorter cartilage than the normal
• Compression of trachea
between aorta anteriorly and
dilated esophagus posteriorly
• Intrinsic tracheal weakness
severe tracheomalacia appears less common in infants with pure EA,

117. Tracheomalacia
• clinical manifestations
• brassy or barking cough
• recurrent pneumonia
• acute, life-threatening apneic spells
• Cyanotic spells within 5 to 10 minutes of a meal
• diagnosis is established by bronchoscopy which
reveals a slitlike lumen

118. Tracheomalacia TCM
• Mild to moderate symptoms of TCM do not require
operative intervention because the symptoms tend to
improve with time
• In symptomatic patients the operative treatment Of
choice is aortopexy (ascending aorta and arch are
sutured up to the posterior surface of the sternum)

119. Tracheomalacia TCM
• Other treatment modalities are
• airway stent
• Glossopexy
• Tracheostomy

120. Thank you
drfaheemandrabi@gmail.com