This document discusses the anatomy, embryology, classification, pathophysiology, clinical features, diagnosis, and management of esophageal atresia with tracheo-esophageal fistula (TEF). It notes that TEF occurs due to incomplete separation of the foregut during embryological development. The classification system describes the different types of atresia and fistula presentations. Surgical repair aims to divide the fistula and perform an end-to-end anastomosis of the esophageal pouches. Long-term complications can include tracheomalacia, gastroesophageal reflux, and swallowing and motility issues.

1. Esophageal Atresia withEsophageal Atresia with
Tracheo-EsophagealTracheo-Esophageal
FistulaFistula

2. AnatomyAnatomy
In full term neonate esophagus is 9 – 10 cmIn full term neonate esophagus is 9 – 10 cm
in length, lumen 3 – 4 mmin length, lumen 3 – 4 mm
Arterial supply:Arterial supply:
 Upper 1/3 – Inferior thyroid artery –Upper 1/3 – Inferior thyroid artery –
vertically orientedvertically oriented
 Middle 1/3 – Bronchial arteries, directMiddle 1/3 – Bronchial arteries, direct
branches from aorta – transversely oriented.branches from aorta – transversely oriented.
 Lower 1/3 – Left Gastric & Phrenic arteriesLower 1/3 – Left Gastric & Phrenic arteries..

3. Embryology:Embryology:
Normal development begins at 4rth wk.Normal development begins at 4rth wk.
Median laryngotracheal grooveMedian laryngotracheal groove
As foregut elongates ridges appear on theAs foregut elongates ridges appear on the
lateral wall which fuse, starting caudally, tolateral wall which fuse, starting caudally, to
separate laryngotracheal tube fromseparate laryngotracheal tube from
oesophagus.oesophagus.
Separation is not complete till 5th wk whenSeparation is not complete till 5th wk when
bifurcation of trachea lies at T4 level.bifurcation of trachea lies at T4 level.

4. Incomplete separation of foregut fromIncomplete separation of foregut from
laryngotracheal groove along withlaryngotracheal groove along with
unbalanced distribution of foregut cellunbalanced distribution of foregut cell
material results in TEF.material results in TEF.
The lung buds develop at the caudal endThe lung buds develop at the caudal end
of primordial trachea.of primordial trachea.
Stomach appears as dilatation of foregutStomach appears as dilatation of foregut
at 5th wk.at 5th wk.

6. Pathological anatomyPathological anatomy
Two pouches: Upper & lower.Two pouches: Upper & lower.
 Upper pouch varies in length but usuallyUpper pouch varies in length but usually
reaches the arch of the Azygous vein. It isreaches the arch of the Azygous vein. It is
thick & hypertrophied.thick & hypertrophied.
Distance between the two pouches couldDistance between the two pouches could
vary from 0.5 – 6 cms , average 1 cm.vary from 0.5 – 6 cms , average 1 cm.

7. ClassificationClassification
Vogt & Gross classification:Vogt & Gross classification:
A.A. EA without fistula 6 – 8%EA without fistula 6 – 8% BB.. Upper pouch fistula with lowerUpper pouch fistula with lower
pouch atresia 1%pouch atresia 1%
CC.Upper pouch atresia with lower pouch fistula 85%..Upper pouch atresia with lower pouch fistula 85%.
The lower pouch commences from the posteriorThe lower pouch commences from the posterior
Wall of the trachea, 0.5 – 1 cm from the carina.Wall of the trachea, 0.5 – 1 cm from the carina.

8. DD.. Double esophageal fistulaeDouble esophageal fistulae E.E. H type esophageal fistulaH type esophageal fistula
2 -32 -3%% 3 -5%3 -5%
FF. Esophageal stenosis. Esophageal stenosis
GG.. Membranous atresiaMembranous atresia

10. PathophysiologyPathophysiology
Pooling of saliva in the blind upper pouch resultsPooling of saliva in the blind upper pouch results
in aspiration pneumonia.in aspiration pneumonia.
Escape of air down the fistula into the stomachEscape of air down the fistula into the stomach
causes distension & splinting of the diaphragm.causes distension & splinting of the diaphragm.
Gastro esophageal reflux occurs in more thanGastro esophageal reflux occurs in more than
70% of TEF. Results in acid reflux into lungs70% of TEF. Results in acid reflux into lungs
through fistula & chemical pneumonitis.through fistula & chemical pneumonitis.

11. Membranous part of trachea is widerMembranous part of trachea is wider
than normal, hence c shaped cartilagethan normal, hence c shaped cartilage
which keeps trachea open duringwhich keeps trachea open during
expiration is less in circumference. Thisexpiration is less in circumference. This
results in partial collapse of trachealresults in partial collapse of tracheal
lumen during expiration –lumen during expiration –
TracheomalaciaTracheomalacia

12. Associated anomaliesAssociated anomalies
VACTERL / VATER associationVACTERL / VATER association : Vertebral: Vertebral
AnalAnal
CardiacCardiac
Tracheo –EsophagealTracheo –Esophageal
RenalRenal
LimbLimb
CHARGECHARGE : Coloboma: Coloboma
Heart diseaseHeart disease
Atresia choanaeAtresia choanae
Retarded growthRetarded growth
Genital hypoplasiaGenital hypoplasia
Ear (deafness)Ear (deafness)

13. Waterston’s PrognosticWaterston’s Prognostic
classification:classification:
GroupGroup BirthBirth
weightweight
PulmonaryPulmonary
statusstatus
CongenitalCongenital
anomaliesanomalies
SurvivalSurvival
AA > 2500g> 2500g NoNo
pneumoniapneumonia
NoNo
anomaliesanomalies
100%100%
BB 1800 –1800 –
2500g2500g
ModerateModerate
pneumoniapneumonia
ModerateModerate
anomaliesanomalies
85%85%
CC < 1800g< 1800g SevereSevere
pneumoniapneumonia
SevereSevere
CardiacCardiac
anomaliesanomalies
65%65%

14. Clinical featuresClinical features
Incidence: 1 per 3000-3500 live birthsIncidence: 1 per 3000-3500 live births
Antenatal USG: Maternal PolyhydramniosAntenatal USG: Maternal Polyhydramnios
Classically newborn with frothing at the mouth inspiteClassically newborn with frothing at the mouth inspite
of oropharyngeal suction, drooling of saliva,of oropharyngeal suction, drooling of saliva,
choking, coughing, dyspnea, cyanosis especially ifchoking, coughing, dyspnea, cyanosis especially if
baby has been fed.baby has been fed.
Firm red rubber tube passed into oropharynx getsFirm red rubber tube passed into oropharynx gets
arrested about 10cms from the alveolar ridgearrested about 10cms from the alveolar ridge

15. DiagnosisDiagnosis
X ray with stiff red rubber tube in the esophagus – AP &X ray with stiff red rubber tube in the esophagus – AP &
Lateral views.Lateral views.
Soft tubes like NG tubes will be found to coil in the upperSoft tubes like NG tubes will be found to coil in the upper
pouch.pouch.
 Confirm diagnosisConfirm diagnosis
 Presence of air in the abdomen s/o lower pouch fistula.Presence of air in the abdomen s/o lower pouch fistula.
Absence s/o pure EA.Absence s/o pure EA.
 Level of upper pouch in terms of thoracic vertebrae.Level of upper pouch in terms of thoracic vertebrae.
 Assess pulmonary & cardiac status.Assess pulmonary & cardiac status.
 Vertebral anomalies.Vertebral anomalies.

18. ManagementManagement
Routine investigationsRoutine investigations
ECHO: To look for cardiac anomalies &ECHO: To look for cardiac anomalies &
side of arch of aortaside of arch of aorta
USG abdomenUSG abdomen
SurgerySurgery

19. Pre operative managementPre operative management
Surgical repair is not an emergency. Necessary toSurgical repair is not an emergency. Necessary to
stabilise & evaluate completely.stabilise & evaluate completely.
Continuous or frequent upper pouch suction withContinuous or frequent upper pouch suction with
low pressure.low pressure.
Prone or lateral head up positionProne or lateral head up position
Warmer careWarmer care
Supplemental O2Supplemental O2
IV antibiotics.IV antibiotics.

20. Operative managementOperative management
Aim: To achieve complete primary repairAim: To achieve complete primary repair
Anaesthesia: ETGA. Positioning of ET distal toAnaesthesia: ETGA. Positioning of ET distal to
the fistula is desirable but is often not possible.the fistula is desirable but is often not possible.
Position: Left lateral positionPosition: Left lateral position
Incision: Transverse incision just below theIncision: Transverse incision just below the
angle of the scapula. Divide the Latissimusangle of the scapula. Divide the Latissimus
dorsi & Serratus anterior. Fourth intercostaldorsi & Serratus anterior. Fourth intercostal
space is identified & opened.space is identified & opened.

22. Procedure: Extrapleural dissection.Procedure: Extrapleural dissection.
Azygous vein identified & divided.Azygous vein identified & divided.
Lower pouch identified. Fistula dividedLower pouch identified. Fistula divided
flush with trachea & tracheal end closed.flush with trachea & tracheal end closed.
Another ET is passes into the oral cavity &Another ET is passes into the oral cavity &
oropharynx to identify upper pouch.oropharynx to identify upper pouch.
Single layer end-end anastamosis afterSingle layer end-end anastamosis after
passing NG tube into the stomach acrosspassing NG tube into the stomach across
the anastamosis.the anastamosis.

23. Procedure: Extrapleural dissection.

27. Post-opPost-op
Electively paralyse & ventilate the child forElectively paralyse & ventilate the child for
24 – 48 hours in slightly flexed position.24 – 48 hours in slightly flexed position.
Tube feeds started after 72 hrs.Tube feeds started after 72 hrs.
Dye study done on day 5 to look for leak.Dye study done on day 5 to look for leak.

28. ComplicationsComplications
Early:Early:
 Anastamotic leakAnastamotic leak
 Anastamotic strictureAnastamotic stricture
 Recurrent TEFRecurrent TEF
 Swallowing incoordinationSwallowing incoordination
 AspirationAspiration

29. Late:Late:
 TracheomalaciaTracheomalacia
 Gastro esophageal reflux, Barrett's esophagusGastro esophageal reflux, Barrett's esophagus
 Motility disorders, dysphagia, bolus impactionMotility disorders, dysphagia, bolus impaction
 Asthma, bronchitisAsthma, bronchitis
 Scoliosis, chest wall deformitiesScoliosis, chest wall deformities..

30. Long gap TEFLong gap TEF
If the distance between the two pouches isIf the distance between the two pouches is
greater than 2 vertebral bodies.greater than 2 vertebral bodies.
At thoracotomy, it is difficult to bring theAt thoracotomy, it is difficult to bring the
pouches together after fistula ligationpouches together after fistula ligation
inspite of extensive mobilisation.inspite of extensive mobilisation.
Options:Options:
Primary anastamosis under tensionPrimary anastamosis under tension
Flap / myotomyFlap / myotomy

31. Cervical esophagostomy & tubeCervical esophagostomy & tube
gastrostomy followed by esophagealgastrostomy followed by esophageal
replacement.replacement.
Esophageal replacement at 1 year of ageEsophageal replacement at 1 year of age::
Colonic interpositionColonic interposition
Gastric tubeGastric tube
Gastric pull upGastric pull up
Jejunal interpositionJejunal interposition

32. Pure esophgeal atresiaPure esophgeal atresia
All pure atresia are long gap.All pure atresia are long gap.
No need of thoracotomy as there is noNo need of thoracotomy as there is no
fistulafistula
Proceed with cervical esophagostomy &Proceed with cervical esophagostomy &
tube gastrostomy followed by esophagealtube gastrostomy followed by esophageal
replacement at 1yr of age.replacement at 1yr of age.

33. H type fistulaH type fistula
Esophageal continuity is maintained, there isEsophageal continuity is maintained, there is
no atresia. Hence may present in infancy orno atresia. Hence may present in infancy or
later.later.
70% of fistulae occur at T2 level.70% of fistulae occur at T2 level.
Classical triad of symptoms:Classical triad of symptoms:
Coughing/choking/cyanosis after feeds.Coughing/choking/cyanosis after feeds.
Recurrent lower respiratory tract infectionsRecurrent lower respiratory tract infections
Gaseous abdominal distensionGaseous abdominal distension

34. DiagnosisDiagnosis
Plain X ray chest : Air esophagogramPlain X ray chest : Air esophagogram
Catheter test to look for bubbling of air.Catheter test to look for bubbling of air.
Cine-esophagogram: Infant in prone position.Cine-esophagogram: Infant in prone position.
NG tube placed in the distal esophagus.NG tube placed in the distal esophagus.
Water soluble contrast injected as catheter isWater soluble contrast injected as catheter is
withdrawn slowly under fluoroscopy.withdrawn slowly under fluoroscopy.
Dye will be seen to leak into trachea at the site ofDye will be seen to leak into trachea at the site of
fistula.fistula.

35. BronchoscopyBronchoscopy
Management:Management:
Excision of fistula with repair of trachea &Excision of fistula with repair of trachea &
esophagus through an oblique rightesophagus through an oblique right
cervical incision just above the clavicle.cervical incision just above the clavicle.