Congenital esophageal stenosis in adults

Congenital esophageal stenosis is thought to be a rare disease confined to infancy and childhood with only a few case reports in adults described.

We report five patients between the ages of 19 and 46 yr who presented with this disorder over a 2-yr period.

Patients had been labeled with reflux strictures, webs, or as idiopathic in the past. All patients had chronic solid food dysphagia, some since early childhood. The location of the stricture varied, occurring in the mid or proximal esophagus in four, but throughout the esophagus in one. Radiographic and endoscopic appearance was a smooth concentric stricture or multiple rings, sometimes tracheal in appearance. Endosonography was performed in two patients, both of whom had focal circumferential hypoechoic wall thickening with disruption of the normal layer pattern corresponding to the areas of luminal narrowing. All patients dilated had good symptomatic response, with resolution of symptoms up to 6 months in follow-up.

We suggest that congenital esophageal stenosis does occur in adults and may be underrecognized. Its endosonographic appearance is described.