Esophageal Atresia

Definition

  1. Congenital obstruction of the esophagus
  1. Interruption of esophageal wall continuity
  1. Presence of one or more fistulae between the malformed esophagus and trachea

Embryology

Foregut differentiation at 4th week of gestation

i. Ventral respiratory part

ii. Dorsal esophageal part

b. Laryngotracheal diverticulum invagination

Theories on Tracheoesophageal Separation

  1. Traditional theory

a. Lateral tracheoesophageal folds

b. Fusion in the midline

  1. Alternative theory

a. Cranial and caudal folds

Epidemiology

1. Most common esophageal abnormality

  1. Birth incidence of EA/TEF: 1 in 3000 - 4500 live births
  1. Slight male preponderance: 3:2 ratio

Classifications

  1. Pure esophageal atresia (7%)
  1. Esophageal atresia with a proximal fistula (1%)

3. Esophageal atresia with a distal fistula (87%)

  1. Esophageal atresia with both proximal and distal fistulae (1%)
  1. H-type fistula with no esophageal atresia (4%)

Associated Anomalies

a. Congenital heart disease (~30%)

i. VSD

ii. PDA

iii. Fallot

b. GIT anomalies (10-15%)

i. Anorectal malformation

ii. Duodenal atresia

c. GU anomalies (10-15%)

i. Hydronephrosis

ii. Multicystic dysplastic kidney

iii. Renal agenesis

d. Skeletal anomalies

i. Vertebral & rib anomalies

ii. Sacral agenesis

iii. Absent radius

f. CHARGE syndrome

i. Coloboma

ii. Heart disease

iii. Atresia

iv. Retarded growth

v. Genital hypoplasia

vi. Ear (deafness)

Diagnosis

  1. Prenatal ultrasound findings

a. Polyhydramnios

b. Small or absent stomach bubble

c. Dilated cervical esophagus (pouch sign)

  1. Postnatal

a. Excessive salivation and drooling (earliest)

b. Regurgitation, choking, and coughing during feeding

c. Manifestations of respiratory distress

i. Chemical pneumonia

ii. Abdominal distension worsens pulmonary status

iii. Aspiration of saliva exacerbates pulmonary compromise

H. Postnatal Investigation

  1. Confirmation with a large nasogastric tube and chest X-ray
  1. Differentiating between distal TEF and isolated EA

a. Air in stomach in distal TEA

  1. Chromosomal analysis if genetic defect is suspected
  1. Echocardiogram to assess associated cardiac anatomy (70%)

Preoperative Management

  1. ICU nursing
  1. Semi-sitting or head-up prone position
  1. Antibiotics, IV fluids, and vitamin K analogue
  1. Continuous low-pressure suction from the upper pouch by Riplogle catheter
  1. Intubation and ventilation in severe cases

Operative Repair

  1. Esophageal atresia with Distal Tracheoesophageal Fistula

a. Surgical goals

i. Divide fistula + end to end anastomosis

b. Standard approach: right-sided extrapleural latero-dorsal thoracotomy through the 4th interspace

c. Thoracoscopic repair as an alternative in some centers

d. Routine bronchoscopy -->identification of defect 5-7 cm above carnia / at carnia / in Rt main bronchus (long esophageal gap)

  1. Isolated Esophageal Atresia

a. Anastomosis not possible due to long distance between pouches

b. Primary procedure: placement of a gastrostomy tube

  1. Long-Gap Esophageal Atresia

a. Definition and anatomical criteria

i. Absence of fistula or prove after division of the distal fistula to have a gap of >4 cm or >2 vertebral bodies making primary repair unfeasible

b. Strategies: preservation of the patient's own esophagus or esophageal replacement

c. Surgical techniques for preserving the patient's own esophagus

i. Delayed 1ry anastomosis at 2-3 months of life (commonest)

ii. Foker external extraction technique (approximation of proximal and distal pouches outside chest)

iii. Proximal esophageal circular myotomy (to lengthen upper pouch)

iv. Creation of full thickness of anterior flap from the upper pouch

d. Esophageal replacement

i. Definition: cervical esophagotomy of the upper pouch with feeding gastrostomy

ii. When: 8-10 kg, 1 year old

iii. Options

  1. Colonic interposition
  1. Gastric tube
  1. Gastric transposition
  1. Jejunal interposition

Embryological defect

  1. Incomplete fusion between trachea and esophagus at 6-7 weeks
  1. imbalance between cranial and caudal folds

50 - 70%

most common in EA without TEF and least common in H type

Complications After Primary Repair

  1. Early complications

a. Anastomotic leak

b. Stricture

c. Recurrent fistula

  1. Late complications

a. Gastroesophageal reflux

b. Tracheomalacia

c. Disordered esophageal motility

e. VACTERL Syndrome

i. Vertebral

ii. Anorectal

iii. Cardiac

iv. Tracheo

v. Esophageal

vi. Renal

vii. Limb