ESOPHAGEAL ATRESIA

&

TRACHEOESOPHAGEAL

FISTULA

Abberation in developmental of esophagus

with or with out communication with

trachea.

Incidence - 1 in 3,570 to 1 in 4,500.

Successive stages in the development of the

tracheoesophageal septum during embryologic

development (A) The laryngotracheal

diverticulum forms as a ventral outpouching from the caudal part of the primitive pharynx.

(B) Longitudinal tracheoesophageal folds begin to fuse toward the midline to eventually form the tracheoesophageal septum.

(C) The tracheoesophageal septum has completely formed.

(D) If the tracheoesophageal septum deviates posteriorly, esophageal atresia with a tracheoesophageal fistula develops

• Esophageal occlusion & failure of recanalization.

Spontaneous posterior deviation of tracheoesophageal

septum.

• Mechanical factor pushing dorsal wall foregut anteriorly.

• Esophageal occlusion & failure of recanalization.

• Teratogen: doxorubicin.

• Component of cephalic neurocristopathy.

• HOX D group of genes. mutations in N-myc, Sox2, and

CHD7 genes - in syndromic EA-TEF with associated

anomalies.

• Defect in signaling pathway of the extracellular, organ

differentiation-promoting glycoprotein Shh

THEORIES OF EA & TEF

ANATOMIC VARIANTS

Are classified according to

• GROSS classification

• VOGT classification

7.8% 0.8% 85.7% 1.4% 4.2%

Anatomic Variations

85%

Most common

GROSS type C

Anatomic Variations

6%

2nd most common

Atresia alone,

no fistula

Small stomach,

gasless abdomen

Usually has a long

gap between the

esophageal ends

GROSS type A

Anatomic Variations

6%

No atresia of

the esophagus

Congenital

tracheoesophageal

fistula

"H" or "N" fistula

GROSS type E

Anatomic Variations

2%

Proximal tracheo-

esophageal fistula

No distal fistula

Small stomach,

gasless abdomen

Often has a long

gap between the

esophageal ends

GROSS type B

Anatomic Variations

l%

Proximal and

distal fistulas

("double fistula")

GROSS type D

VACTERL association: 20%

Vertebral: 17%

Anal 12%

Cardiac 20%

Renal 16%

Limb 5%

• CHARGE association: Cloboma, Heart defect, Atresia

choanae, developmental Retardation, Genital hypoplasia, Ear

deformity.

• Schisis association : Omphalocele, Neural Tube Defect,

Cleft Lip & Palate And Genital Hypoplasia.

SYNDROME ASSOCIATION:

ASSOCIATED ABNORMALITIES

Incidence of Associated Anomalies in Esophageal Atresia.

Anomaly Frequency (%)

Congenital heart disease 25

Urinary tract 22

Orthopaedic (mostly vertebral and radial) 15

Gastrointestinal (e.g., duodenal

atresia,imperforate anus) 22

Chromosomal (usually trisomy 18 or 21) 7

Total with one or more associated 58

anomalies

Associated Congenital Anomalies Reported in Patients

with Esophageal Atresia

System affected

Musculoskeletal

Gastrointestinal

Cardiac

Genitourinary.

Potential anomalies

Hemivertebrae, radial dysplasia or

amelia, polydactyly, syndactyly, rib

malformations, scoliosis, lower limb

defects

Imperforate anus, duodenal atresia,

malrotation, intestinal

malformations, Meckel's

diverticulum, annular pancreas

Ventricular septal defect, patent

ductus arteriosus, tetralogy of Fallot,

atrial septal defect, single umbilical

artery, right-sided aortic arch

Renal agenesis or dysplasia,

horseshoe kidney, polycystic kidney,

ureteral and urethral malformations,

hypospadias

Physiologic effects of distal tracheoesophageal

fistula

A-1. Hyaline membrane disease may necessitate higher ventilator pressures, which encourage air to pass through the distal fistula.

2. A distended abdomen elevates and "splints" the diaphragm.

3. Gastric distension may result in gastric rupture and pneumoperitoneum.

4. Passage of air through a distal tracheoesophageal fistula diminishes the effective tidal volume.

B- 1. Aspiration of gastric juices leads to soiling of the lungs and pneumonia

2. Gastroesophageal reflux

3. Direction of gastric fluid proximally through distal fistula.

4. Overflow of secretions or inadvertent feeding may contribute to aspiration and contamination of the airway. .

Physiologic effects of distal tracheoesophageal

fistula

DIAGNOSIS OF

ESOPHAGEAL ATRESIA

Antenatal Diagnosis:USG- (maternal

polyhydramnios, abnormal swallowing, a

distended upper esophageal pouch, a small

stomach)

Diagnostic suspicion is increased when

abnormalities known to be associated with

esophageal atresia are identified.

Predictive value- 20-40% only

A B

1.Fluid filled stomach2.Bling ending pouch of oesophagus

Dilated blind ending proximal pouch

Fetal MRI

32 week fetus -esophageal atresia , absent stomach -marked polyhydramnios

Axial T2 of thorax-no oesophagus Sagittal T2: Fluid filled

blind ending

oesophgeal pouch

Clinical Diagnosis

Prematurity

Excessive salivation in form of drooling

(copious, fine, white, frothy bubbles of

mucus in the mouth and, sometimes, the

nose).

Episodes of choking,respiratory distress

after feeds

In pure oesophageal atresia (Type A)-scaphoid abdomen

In oesophageal atresis with only proximal fistula (Type B)-scaphoid abdomen

Type E-present later in 1st year.

with repeated respiratory infections , choking,coughing

Clinical Diagnosis

(A) Diagnosis of esophageal atresia is confirmed when a 10-Fr gauge catheter cannot be passed beyond 10 cm from the gums. (B) A smaller-caliber tube –not to be used –curling can give a false impression of esophageal

continuity.

The chest radiograph

A plain radiograph will

confirm the tube has not

reached the stomach

Type E-Not diagnosed

The Gasless Abdomen

Absence of gas in the

abdomen suggests

that the patient has

either atresia without

a fistula or atresia

with a proximal

fistula only

Contrast studies

Usually not needed

Should be performed by an

experienced pediatric

radiologist

With the use of a small

amount (0.5 to 1 mL) of

water-soluble non ionic

contrast.

Management

Goals of the initial treatment:1)Attention to ventilation 2)Keep upper pouch empty3)Treated on emergency basis

So:For ↓ aspiration risk:

1)elevate neonate’s head at least 30º in infant warmer

2)Use continuous suctionIV AB and electrolyte.

Management

In infants with respiratory failure, endotracheal

intubation should be performed.

Use oscillatory ventilation with high frequency

Gastrostomy may be neededplaced on the

water seal,elevated or Intermittently be

clamped

Summary of Preoperative

Investigations

Hb,S.electrolytes,BG cross match

A plain radiograph

2d Echocardiography

Renal ultrasonography

Type of Surgery

• Thoracotomy:

Extra-pleural

Trans pleural

• VAT

Post op

Adequate analgesia-fentanyl drip

Neck flexion

?intubation and sedation

Suction

After 5-7 days oesophagogram

-to see leak,distal patency ,motility.

EA without fistula

Problem is long gap (>3cm / 3vertebral bodies)

T/t- Cervical oseophagostomy+Gastrostomy

Definite repair after 12 wks

Maneuvers to lengthen oesophagus

Nonoperative

-Bougenaige

Proximal pouch

Distal pouch

Magnetic

Operative

Using native esophagus

-upper pouch mobilization

-upper pouch myotomy

-flap lengthening of upper pouch

Using oesophageal replacement

-Colon

-Stomach-Tube

-Jejenum-Pedicle graft or free graft

-Ileum

Comparison of technique

Thoracotomy

Scoliosis

Winged scapula

Shoulder weakness

Chest wall deformity

VATSuperior visualization

Improved cosmesis

Limited working space,

Lack of articulation of

instruments,

tying suture under

tension

Complications

• Early:

Anastomotic leak

Anastomotic stricture

Recurrent TEF

• Late:

GER

Tracheomalacia

Disordered esophageal peristalsis

Anastomotic leak

Major disruption occur 3% to 5% of postoperative

leak, typically recognized early

Factors for leak:

Poor surgical technique

Ischeamia of esophageal ends

Use of myotomy

Excessive tension at anastomotic site

Reoperation: control sepsis with drainage and repair by

pleural or pericardial patch, with or with out intercostals

muscle flap buttress.

Anastomotic stricture

Spitz & Hitchcock defined stricture as the

presence of symptoms: dysphagia & recurrent

respiratory problems from aspiration or foreign

body obstruction & narrowing noted on

endoscopy.

Stricture require dilatation occur upto 80% of

patient.

Prevention-PPI +/- Dilation

Factors responsible: Poor anastomotic

technique: excessive tension, two-layered

anastomosis, silk suture, ischemia at ends,

GER, Anastomotic leak.

Traditionally treated by dilatation:

antegrade or retrograde bouginage using Savory

dilator, Gruntzig balloon dilator

53% respond to single dilatation in the first

month.

Recurrent TEF In 3% to 14 %. Due to anastomotic leak with local

inflammation & erosion through the previous site of TEF.

Risk minimized by : Pleural flap;

Vascularized pericardial flap; Azygos vein

flap.

50% of recurrent fistula missed in routine

contrast study, esophagography in the prone

position under video fluroscopy;

Bronchoscopy with cannulation of fistula is

gold standard.

Traditionally repaired by thoracotomy &

interposition; Fibrin glue, Endoscopic eradication of TEF by chemicals

or diathermy.

GERD

Occurs up to 30% to 70% .

Probably relates to shortening of intra-abdominal portion or esophageal

motor dysfunction. 9% risk of developing barrett’s esophagus.

Suspected with GIT symptoms: vomiting, dysphagea & recurrent

anastomotic stenosis; Respiratory symptoms: stridor, cyanotic spells,

recurrent pneumonia, & reactive airway disease.

Diagnosed by, upper GI dye study, 24 H pH probe data, esophageal

manomatric study.

Medical management: thickening of feeding, H2 blocker, PPI, prokinetic

agent. 45% to 75% undergo anti reflux surgery.

Risk for death

Increased risk of death & long term morbidity

include:

1. Lower birth wt (<1500 g) &

prematurity.

2. Major CHD.

3. Severe associated anomaly &

ventilator dependent

4. Long gap EA.

Risk factor & Survival

Spitz Classification:

The end