pediatric surgery seminar on esophageal atresia

1. Esophageal atresia &
Tracheoesophageal Fistula
By;DR.Workneh Tesfaye(GSR3)

2. OUTLINE
• Definition and history
• Embryology
• Pathogenesis and risk factors
• Epidemiology
• Clinical manifestation and diagnosis
• Management
• Complications
• summary

3. Definitionandhistory
• Developmental anatomical discontinuity of esophagus with or with out
communication with trachea
• 1670 William Durston first published
EA in a conjoint twin
• Isolated EA-TEF published by
Thomas Hill in 1840
• The first operative attempt was
undertaken in 1888 by Charles Steel
• Robert Shaw did the first fistula ligation
and 1o esophageal anastomosis for EA-TEF
• In 1941 Cameron Haight at the University
of Michigan performed the first successful
primary repair of EA

4. Embryology
• Normal foregut embryology isstillnot clear
• Thedivision of foregut isthe result of fusion of invaginating lateral
longitudinal ridges
• This creates aseptum dividing the foregut into adorsaldigestive tract &
ventral respiratory system
• formation of TEseptumisbelievedto begin caudally & end cranially

5. Pathogenesis
• The pathogenesis is unknown
• Occurs sporadically
• It is unlikely that simple, inheritable genetic mechanism is
responsible
• Most likely heterogeneous & multifactorial and involves
multiple genes & complex gene-environment interactions

6. Cont’d…
Theories
 Esophageal occlusion & failure of recanalization
 Spontaneous deviation of tracheoesophageal septum
 Abnormal migration of putative tracheoesophageal septum
 Mechanical obstruction
 Teratogen: doxorubicin
 Component of cephalic neurocristopathy
 Defect in signaling pathway

7. Cont’d…
Risk factors
 Adriamycin exposure
 Mother with prolonged exposure to contraceptive pills
 Exposed to progesterone & estrogen during pregnancy
 Infant of Hyperthyroid, diabetic mother and
 Intrauterine exposure to thalidomide

8. Epidemiology
• Finland 1 in 2440; US & Australia 1 in 4500 live birth.
• 62% male, (2.29 for TEF alone, 1.44 for EA-TEF & 1.33 for EA )
• Non white have lower prevalence than white population
• Increased risk with first pregnancy, mother <20 yrs, and increasing
age
• Multiple birth: (TEF-3.7%; EA-TEF 4.9% EA 8.8% )
• Chromosomal anomalies 6%-10% (trisomy 18)
• 0.5%-2% recurrent risk in if one affected child; 20% if more
than one sibling affected, 3%-4% in an affected child born to
affected parents

9. Association
• Overall incidence 50-70%
 Anatomical:
 Cardiovascular 35%
 Genitourinary 24%
 Gastrointestinal 24%
 Neurologic 12%
 Musculoskeletal 20%

10. Cont’d…
Syndromic
• VACTREL association: 20%
 Vertebral 17%
 Anal 12%
 Cardiac 20%
 Renal 16%
 Limb 5%
• CHARGE association: ( Coloboma, Heart defect, Atresia choanae,
developmental Retardation, Genital hypoplasia, Ear deformity)
• Schisis association: (omphalocele, neural tube defect, cleft lip &
palate and genital hypoplasia)

11. Cont’d…
Other less common anomaly
 Choanal atresia 5.2%
 Facial cleft 7.2%
 Abdominal wall defect 4.3%
 Diaphragmatic hernia 2.9%
 47% incidence of tracheobronchial abnormalities:
 Abnormalities of Vagal nerve innervations that are not related to
surgical intervention is also reported
 Down syndrome
 DiGeorge sequence …

12. Classifications

13. Diagnosis
Prenatal detection
 It is not commonly diagnosed
 USG relies on finding of ;
 anechoic area in the middle of the neck,
 small or absent stomach bubble and
 associated maternal polyhydroamnios;
predictive value 20-40%,
 Fetal MRI may be a useful
adjunct for diagnosis

14. Cont’d…
Postnatal
• Most are symptomatic in the 1st few hrs
• The earliest sign is excessive salivation,
• First feeding is followed by
regurgitation, choking and coughing
• Cyanosis with or with out feeding,
• Respiratory distress
• If distal fistula present the abdomen
distends
• inability to passafeeding tube in thestomach

15. Cont’d…
• Diagnosis of EA can be confirmed by injecting air through the tube and taking trunk film
• 0.5 to 1.0 mL of diluted barium can be used

16. Cont’d…
• Absence of air in the abdomen represents isolated EA
• proximal TEF must be
confirmed by bronchoscopy

17. Management
 Aims of management:-
o Prevention of Aspiration Pneumonia & its consequences.
o Establishment of communication between proximal & distal
end of esophagus.
o Separation of esophagus from trachea

18. Cont’d….
Preoperative treatment;
• NPO & IVF 10% D & hypotonic saline
• Start broad spectrum antibiotic & chest physiotherapy.
• A sump catheter/double lumen Replogle Catheter for continuous low
pressure negative suction
• Upright sitting/ head-up prone position
• Oxygen therapy
• In infants with RF do ETI

19. Cont’d…
• Vit K analog should be given
• Keep infant warm
• If premature, may need surfactant
• Avoid bag ventilation
• Renal ultrasonography
• Echocardiograph
• Chromosomal analysis

20. Cont’d…
Anesthetic technique
 Classic approach is GA without muscle paralysis
 minimal usage of premed,
 careful positioning of ET tube,
 manual control of adequate ventilation with minimal gas flow
through fistula until it ligated
Surgery
• operative treatment of an EA/TEF is not an emergency procedure
• Type of Surgery
Thoracotomy: Extra-pleural/ Trans pleural
 VA

21. “To anastomose the ends of an infant’s esophagus the Surgeon must be as delicate
and precise as a skilled watchmaker. No operation offers a greater opportunity for
pure technical artistry.” Willis Pott, 1950
• Positioning: positioned
for a standard right
posterolateral thoracotomy,
• If a right sided aortic
arch (2.5%) is identified
a left sided thoracotomy
my performed
• Incision; a muscle-sparing
approach

22. Cont’d…
• An extrapleural approach is though to protect the pleural space in
case of an anastomotic leak

23. Cont’d…
• If the distal fistula starts from the trachea under azygos vein, transect it
• The fistula should
be dissected and
mobilized close to the
trachea
• Fistula should be transected
Leaving 1 to 2 mm of
esophagus on the
tracheal end of the fistula

24. Cont’d…
• hole in the trachea is
closed with interrupted
5-0 absorbable sutures
• If proximal pouch is strongly
adherent, dissected sharply
• The distal end of the proximal
pouch is opened
• An E-E-A is performed with
5-0 absorbable sutures

25. Cont’d…
• A chest drain is not routinely utilized
• A small nasogastric tube is inserted until the very end of the
closure of the chest, enabling the removal of residual air
• When a trans-rib approach is used, the periosteum
should be closed
• When intercostal approach is used, the adjacent two ribs should be
approximated with one 3-0 absorbable suture
• Oral feeds (preferably breast milk) commenced at 2–3 days if the
child is stable

26. Cont’d…
Wide Gap Esophageal Atresia with Distal Fistula
Usually anastomosis can be made
upper pouch bougienage
Spontaneous growth
Electromagnetic field to pull together
Nylon thread bridging
Multistage, extra thoracic elongation technique
 external traction technique
circular myotomy of the upper part of the esophagus
 mobilization of the gastric cardia and upper fundus
postoperative head flexion, paralysis, and MV support
Esophageal replacement

27. Cont’d…
Esophageal Atresia without Distal Fistula
 incidence of a proximal fistula is high
 Initial treatment is a gastrostomy with laryngotracheobronchoscopy
 The residual gap to be bridged should be periodically measured
Esophageal reconstruction will be done around 12 weeks
If persisting respiratory problems intervene at earlier time
An alternative is cervical esophagostomy
If a delayed primary anastomosis is not feasible, gastric pull-up or a jejunal, ileal,
or colonic interposition can be used
Mechanical ventilation with muscle relaxation for 5 days ?
A chest drain when there is doubt regarding the quality of the anastomosis

28. Cont’d…
ISOLATED TRACHEOESOPHAGEAL FISTULA (4%)
The fistula runs from the trachea downward to the esophagus
 Respiratory symptoms, especially choking, usually occur immediately after birth
Abdominal distension and flatulence may be present
Older children present with recurrent pneumonia
Suspect if evidence of aspiration pneumonitis with gastric distention on x-ray
Bronchoscopy with esophagoscopy can confirm

29. Cont’d…
• Most can be divided
through a cervical approach
• inferior thyroid artery and
middle thyroid vein may be
divided
• The fistula is divided close
to the esophagus
• May need interposing muscle
tissue
• A right thoracotomy when
the fistula is the thorax

30. Prognosis
• Recently 85% to 95% overall survival achieved
• Poor prognosis if
• (1)lower birth wt (<1500 g)/prematurity
• (2) major CHD
• (3) severe associated anomalies and ventilator dependency
• (4) a long gap EA

31. Complications
• 49% had complications in the first year and 54% after 1 year
Early:
 Anastomotic leak
 Anastomotic stricture
 Recurrent TEF
• Late:
 GER
 Tracheomalacia
 Disordered esophageal peristalsis

32. Anastomotic leak
• Occurs in 14% to 16%,
• Major disruption in 3% to 5% , recognized early
• Factors for leak:
 Poor surgical technique
 Ischemia of esophageal ends
 Use of myotomy
 Excessive tension at anastomotic site
• managed with adequate drainage & nutritional support,
• 95% close spontaneously.
• Reoperation: control sepsis with drainage and repair by pleural or
pericardial patch, with or with out intercostal muscle flap buttress

33. Anastomotic stricture
Defined as the presence of dysphagia & recurrent aspiration or
narrowing noted on endoscopy
• Stricture require dilatation occur 80% of patients
• Factors responsible:
 Poor anastomotic technique
 excessive tension,
 two-layered anastomosis,
 silk suture,
 Ischemia at ends,
 GER,
 Anastomotic leak
• Treated by dilatation, 53% respond to single dilatation in the first month

34. Recurrent TEF
• Occur in 3% to 14 %
• It is due to local inflammation & erosion through the previous
site of TEF
• Can be minimized by using Pleural flap, Vascularized
pericardial flap, Azygos vein flap
• Confirmed by esophagography in prone position under
video fluoroscopy
• Bronchoscopy with cannulation of fistula is gold standard
• Endoscopic eradication by chemicals or diathermy
• Repaired by thoracotomy & interposition

35. GERD
• Occurs in 30% to 70%
• Related to shortening of intra-abdominal portion or esophageal
motor dysfunction
• Presents with vomiting, dysphagia, recurrent anastomotic
stenosis or respiratory symptoms
• 9% risk of developing barrett’s esophagus
• Diagnosed by, upper GI dye study, 24 H pH probe data,
esophageal manometric study.
• Medical management: thickening of feeding, H2 blocker, PPI,
• Anti reflux surgery

36. SUMMARY
• EA-TEF commonly associated with other anomaly
• Proper & specific pre-operative management
• Complete plan of management including expected post-
operative complication
• Primary anastomosis is usually possible
• Survival rate is improving
• Half of the patients may have some complications

37. REFERENCES
• ARNOLD G. CORAN, N. SCOTT ADZICK, ANTHONY A. CALDAMONE, et al.
Congenital Anomalies of the Esophagus. pediatric surgery 7th ed.
• GEORGE W. HOLCOMB III, J. PATRICK MURPHY. esophageal atresia and
tracheoesophageal malformations. Ashcraft’s Pediatric Surgery 6th ed.
• DAKSHESH H. PARIKH, DAVID C.G. CRABBE, ALEXANDER W. AULDIST, STEVEN S.
ROTHENBERG. Congenital Malformations. Pediatric Thoracic Surgery 1st ed.
•

38. THANK YOU