Esophageal atresia

• Most frequent congenital anomaly of the esophagus, affecting 1 /4,000 neonates

• an abnormality in which the middle portion of the esophagus is absent. The esophagus may end blindly into a pouch or may connect to the trachea

• >90% have an associated tracheoesophageal fistula (TEF)

Type Name Description

A ‘”Long Gap", “Pure” or “Isolated” Esophageal Atresia

Esophageal Atresia without fistula.Type A

B Esophageal Atresia with proximal TEF

(tracheoesophageal fistula)

upper esophageal pouch connects abnormally to the trachea.

C Esophageal Atresia with distal TEF (tracheoesophageal

fistula)

lower esophageal pouch makes an abnormal connection with the trachea.The upper esophageal pouch ends blindly.

D Esophageal Atresia with both proximal and distal TEFs (two tracheoesophageal fistulas)

upper and lower esophageal pouches make and abnormal connection with the trachea in two

separate, isolated places.E TEF (tracheoesophageal

fistula) ONLY with no Esophageal Atresia

the esophagus fully intact and capable of its normal functions, however, there is an abnormal

connection between the esophagus and the trachea

F Esophageal Stenosis Also known as an Esophageal Stricture.esophagus fully intact gradually narrows,

Presentation-Frothing or bubbling of the mouth and nose after birth - Drooling- Choking-Episodes of coughing, cyanosis and respiratory distress

Diagnosis• Prenatal ultrasound- maternal polyhydramnios

• inability to pass a nasogastric or orogastric tube in newborn

• Plain radiography- shows radiolucent, blind-end dilated pouch of upper esophagus

• On lateral view– Anterior displacement of trachea– Rarely, air-fluid level in pouch

Preoperative Management-maintain a patent airway-prevent aspiration of stomach contents or saliva into the lungs -prone position to prevent stomach secretions from refluxing into lungs -Intravenous antibiotics to help prevent the development of pneumonia -The "VACTERL" workup is completed prior to surgical repair

Features• V - Vertebral anomalies• A - Anal atresia• C - Cardiovascular anomalies• T - Tracheoesophageal fistula• E - Esophageal atresia• R - Renal (Kidney) and/or radial anomalies• L - Limb anomalies (in front of or above the

central axis of the limb)

Surgical management

-Primary anastamosis of proximal and distal esophagus usually when infant is a few months old

- gastrostomy, gastric pull-up, colonic transposition and jejunum transposition

-

Postoperative care• Postoperatively patients may be extubated as tolerated.• Any attempt at reintubation should be performed carefully

in order to avoid accidental and catastrophic intubation of the esophagus.

• The head is flexed slightly forward in order to decrease tension on the esophagoesophagostomy.

• Oral suctioning performed only to the level of the pharynx. • Endotracheal tube suctioning must be performed carefully

in order to avoid trauma or perforation at the site of the TEF closure.

Outcome

• Current overall survival rates= 85% - 90% • Mortality is usually secondary to associated anomalies. • Immediate postoperative complications include:

• anastomotic leak in 15% of cases.• stricture formation in approximately 15% of cases • recurrent tracheoesophageal fistula in 5% of cases and requires

reoperation with division and ligation of the fistula

• The most common long term problems :• Gastroesophageal reflux in up to 70% • Tracheomalacia in approximately 25%