Congenital subglottic stenosis

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Congenital subglottic stenosis

ORPHA:141121Malformation syndrome

What it is

A rare larynx anomaly characterized by a partial or complete narrowing of the upper airway extending from just below the vocal folds to the lower border of the cricoid cartilage. Clinical presentation is variable and includes recurrent, croup-like, upper respiratory infections, stridor, dyspnea, barking cough, and in most severe cases acute airway compromise at delivery. It may be an isolated finding, or associated with other congenital anomalies and syndromes.

Key facts

Age of onset
Neonatal
Classified as
Malformation syndrome

ICD-10 codes

Q31.1ICD-10 names this disease exactly

Cross-references

GARD 19952MEDDRA 10087149MONDO 0015395UMLS C0396051

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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