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Congenital Esophageal Stenosis


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.