Congenital esophageal stenosis: clinical and endoscopic features in adults

Congenital esophageal stenosis (CES) is an uncommon anomaly that reportedly rarely goes undiagnosed until adulthood. One variant of CES includes patients with multiple cartilaginous rings described usually in the mid-distal esophagus.

Ten patients with CES seen over the past 7 years were interviewed and their clinical and endoscopic records reviewed.

Eight patients were male and age at time of diagnosis ranged from 21 to 75 years. Meat impaction was the presenting symptom in 8 patients and 3 patients reported a relapsing history. Intermittent solid food dysphagia over extended duration (10-40 years) was reported in all patients with an estimated onset of symptoms at a mean age of 27 years (11-52 years). Endoscopically, all patients had segmental esophageal stenosis (length 2-8 cm, mean = 4.7 cm) due to 'trachea-like' multiple submucosal rings. Pseudodiverticulosis and distal reflux esophagitis were evident in 1 patient. Nine of 10 patients had no macroscopic esophagitis. Dilatation was performed by balloon insufflation (18 mm in 5 patients, 15 mm in 3 patients, 12 mm followed by 15 mm in a patient with a tight stricture) and by Savary dilators in 1 patient, without any complications. No patient had recurrent meat impaction on follow-up (1-7 years, mean = 3 years) after education about the condition.

(1) We suspect CES is an under-recognized cause for intermittent, long-standing dysphagia in adults. (2) Food impaction is a frequent initial presentation. Recognition of CES is critical for appropriate patient education and planning.