Congenital esophageal stenosis

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Congenital esophageal stenosis

ORPHA:645749Morphological anomaly

Also called CES · Congenital oesophageal stenosis

What it is

A rare non-syndromic esophageal malformation characterized by intrinsic narrowing of the esophagus, caused by congenital malformation of esophageal wall architecture present at birth. Patients manifest dysphagia and progressive vomiting. Esophageal food impaction, failure to thrive or respiratory distress can be present. Symptoms are often attributed to colic or reflux, thus diagnosis is often difficult.

Key facts

Prevalence
1-9 / 100 000 (at birth, United States)
Age of onset
Adolescent, Childhood, Infancy, Neonatal
Inheritance
Not applicable
Classified as
Morphological anomaly

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

ICD-10 codes

Q39.3ICD-10 names this disease exactly

Cross-references

MEDDRA 10010564MONDO 0957459UMLS C1963580

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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