Background: Congenital esophageal stenosis (CES) is an uncommon anomaly tha
t reportedly rarely goes undiagnosed until adulthood. One variant of CES in
cludes patients with multiple cartilaginous rings described usually in the
mid-distal esophagus. Methods:Ten patients with CES seen over the past 7 ye
ars were interviewed and their clinical and endoscopic records reviewed. Re
sults: 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 o
ver extended duration (10-40 years) was reported in all patients with an es
timated onset of symptoms at a mean age of 27 years (11-52 years). Endoscop
ically, all patients had segmental esophageal stenosis (length 2-8 cm, mean
= 4.7 cm) due to 'trachea-like' multiple submucosal rings. Pseudodiverticu
losis and distal reflux esophagitis were evident in 1 patient. Nine of 10 p
atients 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 pati
ent, 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 intermitten
t, long-standing dysphagia in adults. (2) Food impaction is a frequent init
ial presentation. Recognition of CES is critical for appropriate patient ed
ucation and planning.