Congenital esophageal stenosis: a rare malformation of the foregut

Nagoya J Med Sci. 2019 Nov;81(4):535-547. doi: 10.18999/nagjms.81.4.535.

Abstract

Congenital esophageal stenosis (CES) is a type of esophageal stenosis, and three histological subtypes (tracheobronchial remnants, fibromuscular thickening or fibromuscular stenosis, and membranous webbing or esophageal membrane) are described. Symptoms of CES usually appears with the introduction of the semisolid alimentation. Dysphagia is the most common symptom, but esophageal food impaction, respiratory distress or failure to thrive can be clinical manifestations of CES. Wide spectrum of differential diagnoses leads to delayed definitive diagnosis and appropriate treatment. Depends on hystological subtype of CES, some treatment procedures (dilation or segmental esophageal resection) are recommended, but individually approach is still important in terms of frequency and type of dilation procedures or type of the surgical treatment. Dysphagia can persist after the treatment and a long follow-up period is recommended. In 33% of patients with CES, a different malformations in the digestive system, but also in the other systems, are described.

Keywords: congenital esophageal stenosis; dysphagia; esophageal dilatation; esophageal stricture; infant.

Publication types

  • Review

MeSH terms

  • Animals
  • Congenital Abnormalities / metabolism
  • Congenital Abnormalities / pathology
  • Deglutition Disorders / metabolism*
  • Deglutition Disorders / pathology*
  • Esophageal Atresia / metabolism
  • Esophageal Atresia / pathology
  • Esophageal Stenosis / metabolism*
  • Esophageal Stenosis / pathology*
  • Humans
  • Models, Biological