oesophageal atresia

ESOPHAGEAL ATRESIA

& TRACHEO-

ESOPHAFEAL FISTULA

By Fahad H. Al Hulaibi

208004222

Kingdom of Saudi Arabia

Ministry of Higher Education

King Faisal University

College of Medicine

OBJECTIVES: Definition. Epidemiology. Embryology of esophagus & Trachea. Types & classifications. Associated anomalies. Pathophysiology. Diagnosis & Treatment.

DEFINITION ESOPHAGEAL ATRESIA: is a disorder of the digestive system in

which the esophagus does not develop properly.

؟!!

Atrasia: Congenital absence or closure of a

normal body opening.

Normal Atrasia

Fisula: Is a permanent abnormal passageway

between two organs in the body or between an organ and the exterior of the body.

Normal Fistual

EPIDEMIOLOGY

1 case in 3000-4500 births.

the highest incidence of this disorder is in Finland, where it is 1 case in 2500 births.

Series1; 1

2500

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

Increase in advanced maternal age.

EMBRYOLOGY OF ESOPHAGUS & TRACHEA

At week 4 , the Tracheabroncheal diverticulum developed to Tracheabroncheal sputum. There is a failure to separation in the sputum

leading to fistula.

During week 8, the primitive gut failure to recanalization.

That lead to atrasia.

TYPES & CLASSIFICATIONS

Type A - Esophageal atresia without fistula or so-called 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 and distal TEFs (< 1%)

Type E - TEF without esophageal atresia or so-called H-type fistula (4%)

Type F - Congenital esophageal stenosis (< 1%) (This is not discussed in this article)

ETIOLOGY Currently, most authorities believe that the

development of esophageal atresia has a nongenetic basis.

In a 1987 Kluth eschews has the concept that esophageal vascular events, ischemic events, or both may be causes in cases of esophageal atresia without fistula.

In 2003, Spilde et al reported esophageal atresia-TEF formations  Adriamycin induced teratogenesis.

ASSOCIATED ANOMALIES (VACTERL) Vertebral defects. Anorectal malformations. Cardiovascular defects. TrachoEsophageal deffect. Renal anomalies. Limb deformities.

25% of all patients with esophageal atresia

CHARGE Coloboma. Heart defects. Atresia choanae. Developmental retardation. Genital hypoplasia. Ear deformities .

Neurologic defects -Neural tube defects, hydrocephalus, tethered cord, holoprosencephaly

GI defects -Duodenal atresia, ileal atresia, hypertrophic pyloric stenosis, omphalocele, malrotation, Meckel diverticulum

Pulmonary defects - Unilateral pulmonary agenesis, diaphragmatic hernia

Genitalia defects - Undescended testicles, ambiguous genitalia, hypospadias

PATHOPHYSIOLOGY A fetus with EA cannot effectively

swallow amniotic fluid.

A fetus with esophageal atresia and a distal TEF, amniotic fluid presumably flows through the trachea and down the fistula to the gut Polyhydramnios  premature labor

The neonate with EA cannot swallow and there is copious amounts of saliva.

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 an acute gastric perforation, which is often lethal.

DIAGNOSIS

Prenatal:

1. polyhydramnios

2. PrenatalUltrasonography

sensitivity  40%

Abscenc of stomach bubbles, with fliud filled loops of bowels

Post-natal:

1. White, frothy bubbles of mucus in the mouth and, sometimes, the nose.

2. Episodes of coughing, choking and cyanosis.

3. These episodes may be exaggerated during feeding.

DIAGNOSIS

Laboratory Studies:

CBC countElectrolyte levelsVenous gas concentrationsBUN and serum creatinine levelsBlood glucose levelSerum calcium levelABG concentrations, as necessary

DIAGNOSIS

Genetic testing:

chromosome analysismicroarray genomic hybridization (array

GH)

Chest radiography

NG tube arrested

Air in stomach 

AP view

Lateral view

CT scan

 Renal UltraSonographyis used to evaluate associated kidney

anomalies, ureteral anomalies, or both.

Echocardiography. who have clinical signs of cardiovascular

disease. 

Limb radiography   if the limbs appear abnormal. 

TREATMENT Preoperative management:

1. The oral pharynx should be cleared.2.  The infant's head should be elevated.3. IV fluids (10% dextrose in water).4. Oxygen therapy is used.5. In infants with respiratory failure,

endotracheal intubation should be performed.

6. broad-spectrum antibiotics(such as ampicillin plus gentamicin)

Surgical techniques vary according to surgeons' preferences and variations in pathologic anatomy.

The best esophagus is the patient's esophagus.

Infants born with esophageal atresia without fistula early gastrostomy

Kimura, Livaditis, Scharli, or Foker procedures

Infants born with esophageal atresia with fistula early gastrostomy

REFERANCES Roberta A Pagon, Editor-in-chief, Thomas D Bird, Cynthia R Dolan,

Karen Stephens, and Margaret P Adam, GeneReviews™, Seattle (WA): University of Washington, Seattle; 1993.

Amulya K Saxena, Esophageal Atresia With or Without Tracheoesophageal Fistula, Medscape. eMedicine, Updated: Apr 25, 2012, link: (http://emedicine.medscape.com/article/935858-overview#showall )

DWAYNE C. CLARK, Esophageal Atresia and Tracheoesophageal Fistula, 1999 Feb 15;59(4):910-916.

Kronemer KA, Snyder-Warwick A. Esophageal atresia/tracheoesophageal fistula. 2008. Available online at eMedicine. Accessed 4-1-11.