Congenital esophageal stenosis in adults

OBJECTIVE: Congenital esophageal stenosis is thought to be a rare disease c onfined to infancy and childhood with only a few case reports in adults des cribed. METHODS: We report five patients between the ages of 19 and 46 yr who prese nted with this disorder over a 2-yr period. RESULTS: Patients had been labeled with reflux strictures, webs, or as idio pathic in the past. All patients had chronic solid food dysphagia, some sin ce early childhood. The location of the structure varied, occurring in the mid or proximal esophagus in four, but throughout the esophagus in one. Rad iographic and endoscopic appearance was a smooth concentric structure or mu ltiple rings, some times tracheal in appearance. Endosonography was perform ed 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 resp onse, with resolution of symptoms up to 6 months in follow-up. CONCLUSIONS: We suggest that congenital esophageal stenosis does occur in a dults and may be underrecognized. Its endosonographic appearance is describ ed.