Congenital esophageal stenosis: a rare malformation of the foregut

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.

Fig. 1

Development of the esophagus in the primitive digestive tube (light grey – foregut; dark grey – midgut; black – hindgut) 1) Trachea extends from the common foregut tube as the lung buds grow 2) Both the trachea and the esophagus elongate from the common foregut tube and are separated by a mesenchymal septum; 3) Separation of the trachea and the esophagus starts from the level where the lung grows out and moves rostrally.

Fig. 2

The most important facts about congenital esophageal stenosis (CES) – from symptoms to treatment, with flow chart of CES treatment TBR: tracheobronchial remnants, FMS: fibromuscular thickening or fibromuscular stenosis, EM: membranous webbing or esophageal membrane.