Congenital
Esophageal Stenosis
Congenital esophageal stenosis (CES) is a rare clinical condition found
in 1 in 25,000 to 50,000 live births, although the true incidence remains
unknown. CES is characterized by an intrinsic circumferential narrowing of the
esophageal lumen that is present at birth, although not necessarily symptomatic
in the neonatal period.
Its etiology remains unknown, but an
embryo logic origin has been suggested. There are three histologic types of
CES: ectopic tracheobronchial remnants in the esophageal wall, segmental
fibromuscular hypertrophy of the muscle and submucosal layers, and a membranous
diaphragm or stenosis. CES is frequently associated with esophageal atresia.
CES is found equally in male and female children and is typically diagnosed
before the age of 14, most commonly by 2 years.
The most common presenting
symptoms are dysphagia, food impaction, and respiratory symptoms. The
stenosis may also be found incidentally. The diagnosis is typically made by
barium swallow and/or endoscopy with the visualization of cartilaginous
remnants. The ringlike appearance may cause confusion with eosinophilic
esophagitis. Some patients may be effectively treated with esophageal dilation,
though surgical therapy with resection is commonly required, particularly if
the stenosis occurs in association with esophageal atresia.
