Esophageal Atresia
Esophageal
atresia is a sporadic congenital disease of varied anatomic presentation
defined by atresia and/or fistulization of the esophagus to the trachea. It is
the most common congenital abnormality of the esophagus but presents only in 1:
2500 to 1: 4500 births. The type of esophageal atresia is classified on the
basis of specific anatomic patterns and the frequency of occurrence. The
etiology is unclear, with several theories proposed, generally involving abnormal
formation of a tracheal diverticulum during embryogenesis.
Genetic
abnormalities have also been identified but none that are uniformly present in
infants with this syndrome. Diagnosis can be made with prenatal ultrasound
approximately half the time, but the disorder is typically suspected in an
infant with copious secretions, suffocation and cyanosis (particularly with
breast feeding), and inability to pass an orogastric tube. Prompt recognition
is essential with diagnostic testing, including chest radiography and
bronchoscopy. The surgical procedure is dictated by the type of atresia and/or
fistulization present. Prognosis is dependent on preoperative birth weight,
respiratory status, and the rates of morbidity and mortality that may accompany
associated anomalies, particularly cardiac disorders such as ventricular septal
defects and tetralogy of Fallot. Notably, 50% of infants with esophageal
atresia have other congenital abnormalities. An acronym used clinically to
describe a subset of these infants is VACTERL (vertebral, anal, cardiac,
tracheal, esophageal, renal, and limb). The timing
of surgery depends on the type of atresia and the respiratory status of the
infant. Infants with esophagotracheal fistulization and/or aspiration need
urgent surgery, whereas infants without respiratory compromise may be followed
for several months with gastrostomy tube feeding until the esophagus matures
and grows and is more amenable to an anastomosis between the proximal and
distal ends. One of the great challenges in this surgery is having enough
remnant esophagus to accomplish this. Indeed, substitutes may be needed,
including stomach or intestinal interposition. Short and
longterm postoperative complications include anastomotic lea and/or stricture
and severe gastroesophageal reflux.
