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Congenital esophageal stenosis

Case contributed by Angelica Maria Ruiz Gaviria
Diagnosis certain

Presentation

Two month history of episodic regurgitation, increasing in frequency after starting semi-solid foods. Treated for gastroesophageal reflux without improvement.

Patient Data

Age: 7 months
Gender: Female

Barium esophagogram

Fluoroscopy

There is a smooth concentric narrowing of the middle third of the esophagus, associated with proximal esophageal dilation and accumulation of contrast medium.

At the level of the left main bronchus, there is concentric narrowing of the esophageal lumen of up to 3 mm in the middle third of the esophagus.

Endoscopy

Photo

Upper digestive tract endoscopy at 17 cm from the dental arch, demonstrates stenosis of 80% of the oesophagal lumen, through which the endoscope could not cross.

Case Discussion

Congenital esophageal stenosis is a malformation of the esophageal wall architecture, whose incidence is 1 in 25,000 to 50,000 live births1; it is classified into three types: tracheobronchial remnant, fibromuscular stenosis, and membranous diaphragm, with a frequency of 29.9%, 53.8%, and 16.2% of cases, respectively2.

Newborns may present with respiratory distress and failure to pass a nasogastric tube 1. Alternatively, symptoms begin after weaning, presenting with salivation, bronchial aspiration, food regurgitation, recurrent respiratory infections, vomiting, stridor, dysphagia, and weight loss 3.

An esophagogram may demonstrate concentric stenoses with well-defined borders and variable length in any esophageal segment. These findings can be associated with proximal esophageal dilation, tracheoesophageal fistula, and gastro-esophageal reflux. Computed tomography makes it possible to exclude extraesophageal causes such as congenital tracheal, bronchopulmonary, and vascular abnormalities 4. Upper digestive tract endoscopy corroborates the diagnosis, ruling out esophagitis or retained foreign body. Endoscopic treatment is appropriate for selected cases 3,4.

Our patient underwent surgical treatment via a thoracoscopic approach with segmental resection of the esophageal stenosis area plus end-to-end anastomosis. The histopathological report showed esophageal stenosis of the fibromuscular variety.

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