 One day Term baby born to primi brought

with h/o unable to feed/ `chocking’ and

excess salivation from mouth
DIAGNOSIS?
ESOPHAGEAL ATRESIA-
TRACHEOESOPHAGEAL FISTULA
HISTORICAL PERSPECTIVE
 1670 – Durston - First reported case in conjoined
twins.
 1888 – Steele - First attempted surgical correction of
pure EA by gastrostomy.
 1899-Hoffman performed permanent gastrostomy.
 1939 – Levin and Ladd - First survivors of staged
repair of EA-TEF.
HISTORICAL PERSPECTIVE

 1941 – First successful

primary repair – Haight.

a) Left extrapleural approach.

b) Primary single layer
anastomosis.
c) Fistula ligation.
EMBRYOLOGY
• Derived from the primitive foregut.

• 4th week of gestation, respiratory diverticulum (lung bud)

appears at the ventral wall.

• Tracheoesophageal septum develops during the 4th to 5th

weeks.

• Tracheoesophageal septum gradually partitions

this diverticulum from the dorsal part of the foregut.
EMBRYOLOGY
EMBRYOLOGY
EMBRYOLOGY
EMBRYOLOGY
 Foregut divides into a ventral portion, the respiratory
primordium, and a dorsal portion, the esophagus.

 Starts caudally and ends cranially.

 Elongates with descent of heart and lungs.

 7th week reaches final relative length.

EMBRYOLOGY
 The muscular coat, formed by surrounding splanchnic
mesenchyme.

 Is striated in its upper two-thirds and innervated by

the vagus.

 In the lower third is smooth innervated by the

splanchnic plexus.
ETIOLOGY
 Esophageal occlusion.
 Spontaneous deviation of tracheoesophageal septum.
 Cozzi- cephalic neurocristopathy.
 Teratogens.
 Spitz- HOX D group.
 Gites- defect in signalling pathway of the extracellular
organ differentiation promoting glycoprotein Sonic
hedgehog.
CLASSIFICATION

 VogtIIIb,LaddIII,Gross C.
 85%
 Proximal- dilated,thickened.
Superior mediastinum.

 Distal - thin, slender.

Carina, 1 to 2cm
higher
CLASSIFICATION

 VogtII,LaddI,Gross A.

 7%.

 Upper – dilated, thick.

azygous vein.

 Distal – short,
fibrotic band.
CLASSIFICATION

 VogtIIIa,LaddII,Gross B.
 0.8%
 Fistula not at end.
 Proximal on trachea.
 Distally on dilated
esophagus.
 Diameter tiny to large.
• Small stomach,
 Gasless abdomen
CLASSIFICATION

 VogtIIIc,LaddV,Gross D.

 Less than 1%.

CLASSIFICATION

 VogtIV,Gross E.
 4%.
 "H" or "N" fistula.
 At thoracic aperature or
higher up.
 Short.
EPIDEMIOLOGY
 Total birth incidence -2.5 to 2.8 per 10,000 births.

 57.3% with atresia only, 36.4% with other

major malformations, 6.3% chromosomal anomalies.

 Non white population lower prevelance.

 Slightly higher frequency in males.

EPIDEMIOLOGY
 Higher rate low birth weight and preterm birth.

 Increased risk- first pregnancy, mother younger than

20, increasing maternal age.

 Higher rate in monozygotic twins.

EPIDEMIOLOGY

 Environmental
 Metimazole.

 Contaceptive pills.
 Progesterone and estrogen exposure.

 Maternal diabetes.

 Thalidomide exposure.

 Fetal alcohol syndrome and maternal

phenylketonuria.
GENETIC SYNDROMES

 Di George sequence.
 Polysplenia sequence.

 Holt Oram syndrome.

 Pierre Robin sequence.

 Feingold syndrome.
ASSOCIATED ANOMALIES

• 3 genes associated with EA/TEF –

MYCN haploinsufficiency – Feingold syndrome.

CHD7 – CHARGE syndrome.

SOX2 - AEG syndrome (anophthalmia- esophageal-

genital syndrome)
ASSOCIATED ANOMALIES

 Cardiovascular 35%.
Ventricular septal defect (most common),
Tetrology of Fallot 5%,
Atrial septal defect,
Patent ductus arteriosus 13%,
Coarctation of the aorta (1-1..5%)
descendinhg Aorta to right 4%.
 ASSOCIATED ANOMALIES

 Genitourinary 24%
-Hypospadias, cryptorchidism, renal agenesis.
-Renal hypoplasia, cystic renal disease.
-Hydronephrosis,vesicoureteral reflux, ureteric
duplication.
-Pelvicoureteral or vesicoureteral obstruction.
-Urachal anomalies, intersex abnormalities.
-Cloacal or bladder exstrophy, megalourethra.
-Urethral duplication, posterior urethral valves.
ASSOCIATED ANOMALIES

 Gastrointestinal 24%.

-Anorectal malformation 14%, duodenal atresia 2%,

ileal atresia.

- Malrotation 4%, annular pancreas, pyloric stenosis.

 ASSOCIATED ANOMALIES
 Skeletal 13%.
-Vertebral anomalies,
radial limb deformities.
 Choanal atresia 5.2%.
 Facial cleft 7.2%.

 Abdominal wall defect

4.3%.
 Diaphragmatic hernia
2.9%.
ASSOCIATED ANOMALIES

 Tracheobronchial anomalies 40%.

-Unilateral pulmonary agenesis, ectopic or absent
right upper lobe bronchus.
- Congenital bronchial stenosis.
- Decreased cartilaginous: membranous trachea
ratio.
-Laryngotracheoesophageal cleft.
ASSOCIATED ANOMALIES

 Neurologic 12%.

-Hydrocephalus 5.2%.
-Neural tube defects 2.3%.
-Holoprosencephaly 2.3%.
-Anophthalmia or microphthalmia 2.3%.
-Microcephaly.
ASSOCIATED ANOMALIES

 Non-random associations documented with-

VACTERL association 20%
(vertebral,anorectal,cardiac,tracheo
esophageal,renal & limb abnormalities)
CHARGE association
(coloboma,heart defects,atresia of
choanae,developmental retardation,genital
hypoplasia,ear deformities)
DIAGNOSIS

 Prenatal ultrasound
- Polyhydramnios (1 in 12).
- Small or absent stomach with distended
blind esophageal pouch (pouch sign).
 Prenatal MRI
- Non visualisation of intra-thoracic portion
of esophagus.
INVESTIGATIONS
Prenatal MRI-

- Following positive USG

findings.
- 100% sensitive
- 80% specific
- Positive if esophagus
absent in mid chest.
DIAGNOSIS

 Postnatally-
- Excessive drooling of saliva.
- Fine frothy bubbles in mouth and nose
- Choking, coughing, cyanotic
episodes,regurgitation with feeding.
- Inability to pass 10Fr OGT.
INVESTIGATIONS
Radiograph

- Confirmatory test
- OGT in esophageal pouch
- Presence or absence of gas in the abdomen
-Assess gap length
Anomalies
-Pneumonitis
- Atelectasis
- Cardiac
- Vertebral
IMAGING

Gasless A

Coiled OGT Gasless Abdomen

INVESTIGATIONS
 CONTRAST STUDIES-
Rarely needed,
Identification
of proximal fistula.
Measuring gap length.
water soluble contrast.
under fluoroscopy.
INVESTIGATIONS

 ECHO.
 Renal Scan.

 Spine Radiographs.

 Bronchoscopy – selectively.

 Esophagoscopy – selectively.
PRE OPERATIVE
 Sump catheter. Replogle
 Slow suction continous

 Mucus sucker

 Head up and prone position.

 Antibiotic coverage.
 Chest physiotherapy.
 Iv fluids.
SURGERY

 Determinants
-Type of esophageal anomaly.
-Condition of infant.
-Other congenital anomalies present.
OUTCOMES

Waterston classification (1962) and current survival

Group Description Survival (%)

A Birthweight > 5.5 lbs. and otherwise well 100

B Birthweight 4 – 5.5 lbs. and otherwise 85

healthy
Or
Birthweight > 5.5 lbs. with moderate
pneumonia
C Birthweight < 4 lbs. or higher with severe 65
pneumonia or severe cardiac anomalies
OUTCOMES

Spitz classification and survival

Group Description Percent of Survival (%)
Total (%)

I Birthweight > 1500 g without major 79 97

congenital cardiac defect

II Birthweight < 1500 g or major 19 59

congenital cardiac defect

III Birthweight < 1500 g and major 02 22

congenital cardiac defect.
EA AND TEF

 Fistula division with primary esophageal

anastomosis.
 Right thoracotomy via 4th ICS
 Fistula divided close to trachea with air-tight ligation.
 Mobilization of proximal segment with circular
myotomy if extra length required.
 Single layer closure with absorbable suture knots.
 Internal Feeding tube placed across anastomosis.
LONG GAP -ESOPHAGEAL LENGTHENING

 Non Operative
- Bougienage.
proximal, proximal and distal, magnetic.
- Spontaneous growth.
LONG GAP -ESOPHAGEAL LENGTHENING

 Operative Measures.
 Native Esophagus
Upper pouch/ lower pouch mobilization.
Myotomy.
Flap lengthening.
Multistaged extrathoracic elongation of the proximal
pouch.
Traction Sutures
Transluminal thread with olives.
Lower pouch hydrostatic distension.
Elongation of lesser curvature.
MYOTOMY
FLAPS
LONG GAP -ESOPHAGEAL LENGTHENING

 Using esophageal replacement

-Colon.
-Stomach.
Tube.
Transposition.
-Jejunum.
Pedicle graft
Free fraft.
-Iieum.
COLON
 Right, left, or transverse.

 Retrosternal or posterior mediastinal.

 Passive conduit.
COLON INTERPOSITION
 Advantages
I. Acts as a conduit antiperistaltically or
isoperistaltically.
II. Good vascular supply via marginal artery.
III. Can be placed in esophageal bed of posterior
mediastinum.
IV. Has mucous shield, which protects against reflux.
V. Responds to acid with a peristaltic rush for
clearance.
VI. Minimizes/eliminates tension on the upper and lower
esophageal segments.
COLON INTERPOSITION

 Dis- advantages.
I. Requires 3 anastomoses
II. Empties more slowly than the esophagus
III. Requires preoperative bowel preparation
IV. Long surgical procedure with extensive mobilization
V. Dilates and becomes redundant over time.
VI. Slows food transit
GASTRIC TUBE

 Reversed (antiperistaltic) tube.

-More common.
-Supplied by left gastroepiploic artery.

 Nonreversed (isoperistaltic) tube.

-Supplied by the right gastroepiploic artery.
GASTRIC TUBE
 Advantages
I. Not dilated, tortuous, or redundant.
II. Less risk of ischemia because of
robust blood supply.
III. Favorable anatomic location in the
upper abdomen.
IV. Requires fewer anastomises.
V. Has comparable diameter and
occupies less space in the thorax
and neck.
VI. Has only one suture line.
VII. No bowel preparation and is a
faster procedure.
GASTRIC TUBE

 Dis -advantages
I. Extensive
gastroesophageal reflux
II. Leaves a small gastric
reservoir
III. Creates a long suture line
IV. May result in gastric outlet
obstruction
V. Unable to reach high in
neck
VI. Difficult to place in
posterior mediastinum
GASTRIC TRANSPOSITION

Advantages

I. Readily available and easily mobilized.

II. Involves single anastomosis.
III. Adequate length available.
IV. Excellent blood supply.
V. Technically easy procedure.
VI. Low incidence of leaks and strictures.
GASTRIC TRANSPOSITION

Disadvantages
I. Large bulky -space problems intrathoracically
II. Reflux
III. Possible stricture or aspiration, lack of
gastroesophageal valve.
IV. Poor gastric emptying.
V. Affect pulmonary function.
VI. May not reach as high in neck as other methods
because of blood supply.
JEJUNUM

 Advantages

I. Caliber similar.
II. Low incidence of leaks and strictures
III. Functions as an effective gastroesophageal barrier.
IV. Does no require a bowel preparation.
JEJUNUM
 Disadvantages
I. Length of conduit limited by blood supply.
II. Infarction commonly resulting from passage through
chest.
III. Technically difficult.
IV. Requires 3 anastomosis.
V. High peptic ulcer susceptibility
VI. Blood supply lacking marginal artery
VII. High failure rate
POST OPERATIVE CARE
Ventilation
Guarded suction
Drain care
Monitoring
Routine

Specific
COMPLICATIONS
 Anastomotic leakage 14-
21%
 Fistula recurrence 3-14%
 Esophageal strictures 40%
 Gastroesophageal reflux 40-
70%
 Tracheomalacia 10-20%
 Esophageal dysmotility
PURE ATRESIA, LONG GAP TEF

 Esophagostomy
 Gastrostomy

 Delayed primary repair , innovative primary

lengthening procedures

 Esophageal replacement
THE H TYPE FISTULA

• Presentation
• Diagnosis
• Management
Thank you