esophageal atresia & tracheo-esophageal fistula

DR. TAREQ MORSHEDRESIDENT, PHASE- A

CARDIOVASCULAR & THORACIC SURGERY BSMMU.

Oesophageal Atresia & Tracheoesophageal

Fistula

Objectives

Definition.Epidemiology.Embryology .Types.Associated anomalies.Pathophysiology.Diagnosis & Investigations ManagementComplications

Esophageal AtresiaCongenital discontinuity of esophageal lumen is known as esophageal atresia.

Tracheoesophageal FistulaAbnormal communication between trachea and esophagus.

Epidemiology

1 per 2500 – 3000 live births.50% have 1 or more associated

anomalies: Cardiac, Anorectal , Genitourinary, vertebral/skeletal & others.

2% risk of recurrence is present when sibling is affected.

Increased in advanced maternal age.

Embryology

Both the esophagus and trachea originate from the primitive foregut and this is thought to be a separation defect.

Stages in the 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 completely forms.

At week 4,the tracheoesophageal diverticulum developed to tracheoesophageal septum. There is a failure to separation in the septum leading to fistula.

During week 8,the primitive gut failure of recanalization leads to atresia.

Classification

According to the system formulated by Gross:-Type A - Esophageal atresia without fistula or pure

esophageal atresia (10%)Type B - Esophageal atresia with proximal TEF (<1%)Type C - Esophageal atresia with distal TEF (85%)Type D - Esophageal atresia with proximal & distal

TEFs (<1%)Type E - TEF without esophageal atresia or H-type

fistula (4%)Type F - Congenital esophageal stenosis (<1%)

Associated Anomalies

Vertebral defects.Anorectal malformations.Cardiovascular defects.Renal anomalies.Limb deformities.

25% of all patients with esophageal atresia

Associated Congenital Anomalies Reported in Patients with Esophageal Atresia

Pathophysiology

A fetus with esophageal atresia cannot effectively swallow amniotic fluid.

Polyhydramnios  Premature labour

Aspiration of saliva or milk, if the baby is allowed to suckle, can lead to an aspiration pneumonitis.

In a baby with esophageal atresia and a distal TEF, the lungs may be exposed to gastric secretions.

Also, air from the trachea can pass down the distal fistula when the baby cries, strains, or receives ventilation. This condition can lead to over distension of abdomen which ultimately may cause acute gastric perforation, which is often lethal.

DIAGNOSIS

Antenatal Diagnosis of esophageal atresia is done during routine maternal Ultrasonography

Polyhydramnios A small stomach

A distended upper esophageal pouch

Clinical Diagnosis

PrematurityExcessive drooling (copious, fine, white,

frothy bubbles of mucus in the mouth and sometimes, the nose)

If these signs go unnoticed the first feed is almost always accompanied by coughing, choking and cyanosis.

After birth these babies often appear to blow bubbles when attempting to swallow mucous and saliva.

Sign & Symptoms of respiratory distress due to aspiration pneumonia.

(A) Diagnosis of esophageal atresia is confirmed when a 10-gauge (French) catheter cannot be passed beyond 10 cm from the gums.(B) A smaller-caliber tube is not used because it may curl up in the upper esophageal segment, giving a false impression of esophageal continuity.

Investigations

1st line Investigations:-Plain radiographEsophagogramEndoscopyOthers:-Renal UltrasonographyEchocardiographyBronchoscopyLaboratory investigations.

A plain radiograph

Fig (A) Orogastric catheter in the blind esophageal pouch & presence of air in the stomach.

Fig (B)The radiopaque tube in the blind esophageal pouch & absence of air in the stomach

esophagogram (Contrast X-Ray)

Plain x-ray with contrast in the upper esophagus above the atresia.

Renal ultrasonography

To evaluate associated kidney anomalies, ureteral anomalies, or both.

Echocardiography

Who have clinical signs of Cardiovascular disease.

Management

Aims of management:-Prevention of Aspiration Pneumonia & its

consequences.Establishment of communication between

proximal & distal end of esophagus.Separation of esophagus from trachea.

Preoperative management

The oropharynx should be cleared.The infant's head should be elevated.IV fluids & Nutrition.Oxygen therapy.In infants with respiratory failure,

endotracheal intubation should be performed.

Broad Spectrum Antibiotics.

Timing of surgical correction

(A) Immediate Primary Repair 1. No pulmonary complications, viz. severe

pneumonia.2. An arterial PaO2> 60mm Hg in room air.3. Not associated with major congenital

malformations which are compatible with life.

4. Gap between proximal and distal esophageal pouch less than 2.5 cm.

(B) Delayed surgical intervention1. Associated with pneumonia, sepsis or

cardiac malformation.2. Severe prematurity3. The gap between proximal and distal

pouch is more than 2.5cm.

(C) Staged operation:- The indications are:1. If primary repair can not be done due to

reasons mentioned in delayed surgical repair

2. Failed primary repair.3. Long gap between two esophageal

pouches viz.in type 1 esophageal atresia, where gap is usually more than 3-4 cm.

Stages of surgical correction

Left cervical esophagostomy and feeding gastrostomy immediately after birth.

Thoracotomy & Ligation of tracheoesophageal fistula.

Replacement of the gap between proximal and distal esophagus by isolated vascularized segment of colon or by gastric tube.

Complications

(A) Early :- PneumothoraxAnastomotic leakage Recurrence of fistulaPulmonary atelectasis or infection (B) Late:-Anastomatic StrictureGastroesophageal reflux(GER)TracheomalaciaDisordered esophageal Peristalsis(Dysphagia)

Prognosis

Take Home Message

Early diagnosis allows time and place of delivery to be planned so that early pediatrics surgical correction and neonatal care is available.

This also gives time for preparation and education of the parents.

Thank You!