Congenital Esophageal Stenosis: Clinical And Endoscopic Features In Adults

Background: 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. Methods:Ten patients with CES seen over the past 7 years were interviewed and their clinical and endoscopic records reviewed. Results: Eight patients were male and age at time of diagnosis ranged from 27 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. Conclusion: (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.