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introduction

 anomalies present the pediatric surgeon with a unique and

complex congenital disease

 In 1959, Dr. Willis Potts wrote, “To anastomose the ends of an

infant’s esophagus, the surgeon must be as delicate and precise
as a skilled watchmaker.

 improvements in anesthetic and neonatal intensive care have

made repair of these anomalies
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Embryology

 During the fourth week of gestation the foregut starts to

differentiate into a ventral respratory part and a dorsal
esophageal part

 At 6–7 weeks of gestation, the separation between trachea and

esophagus is complete

 The traditional theory postulates that the ventral respiratory

system separates from the esophagus by the formation of lateral
tracheoesophageal folds that fuse in the midline and create the
tracheoesoph- ageal septum
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Embryology

THIS THEORY OF LONGITUDINAL

TRACHEOESOPHAGEAL FOLDS MERGING TO FORM A
SEPTUM HAS BEEN CHALLENGED
 A/TEF would then be due to an imbalance in the growth of these folds

 Furthermore, rat studies suggest that EA/TEF results from

disturbances in either epithelial proliferation or apoptosis

 More recent studies show that ectopic expression of sonic hedgehog

occurs in the tissues between the notochord and the gut.
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Epidemiology

 The birth incidence of EA/TEF is 1 in 2500–3000 live births

 There is a slight male preponderance of 1.26:1

 Environmental factors that have been implicated include the use

of methimazole in early pregnancy, prolonged use of
contraceptive pills, progesterone and estrogen exposure,
maternal diabetes, and thalidomide exposure

 Chromosomal anomalies are found in 6–10% of the patients. The

total number of trisomy 18 cases exceeds the total number of
trisomy 21 cases
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Associated Anomalies

 Cardiac (13–34%)

 Vertebral (6–21%)

 Limb (5–19%)

 Anorectal (10–16%)

 Renal (5–14%)
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Classification
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Diagnosis

 ANTENATAL DIAGNOSIS
 The prenatal diagnosis of EA/TEF relies, in principle, on two
nonspecific signs: polyhydramnios and an absent or small stomach
bubble
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Diagnosis
POSTNATAL DIAGNOSIS

 If the pregnancy was complicated by polyhydramnios and the

child presents with anomalies that fit the VACTERL association
(e.g., radial aplasia) , passage of a tube or catheter into the
stomach should be performed to assess esophageal
patency (10 Fr NGT)

 saliva accumulates in the proximal esophagus and mouth

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Diagnosis

 A chest film is then obtained with

downward pressure on the tube. With EA,
the tip of the tube is found to be slightly
curled in the blind upper pouch around T2–
T4 (Fig. 27.2)

 the two best imaging modalities are

computed tomography (CT) and MRI

 Although MRI would certainly be preferable

due to its absence of radiation exposure, it
requires general anesthesia
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Diagnosis

 Bronchoscopy can also be performed prior to exploration, not only to

assess the site of the distal fistula, but to also look for an upper pouch
fistula
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Management

PREOPERATIVE

 transferred to a Level I pediatric surgical center

 A 10 French Replogle tube is placed in the upper esophagus and set to

continuous suction

 positioned head-up and on his or her side

 If the child is in respiratory distress, endotracheal intu- bation and ventilation

may be needed (Gentle lowpressure ventilation)

 emergency ligation of the fistula may be needed as a lifesaving maneuver

(not postponing the second/ definitiveOperative Repair operation longer than
7–14 days )
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Operative Repair
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Operative Repair
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Postoperative Management

 Mechanical ventilation with muscle relaxation for 5 days has

been advocated after an anastomosis is performed under
considerable tension
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Complications From Operative Repair

 ANASTOMOTIC LEAKS

 ANASTOMOTIC STRICTURE

 RECURRENT TRACHEOESOPHAGEAL FISTULA

 VOCAL CORD DYSFUNCTION

 RESPIRATORY MORBIDITY

 THORACOTOMY-RELATED MORBIDITY
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 THANK YOU