raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:141121

Congenital subglottic stenosis

Type
Malformation syndrome
Group
Disorder
Age of onset
Neonatal
Status
active

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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enCongenital subglottic stenosis
frSténose sous-glottique congénitale
deSubglottisstenose, kongenitale
esEstenosis subglótica congénita
itStenosi subglottica congenita
nlCongenitale subglottisstenoseCongenitale subglottische stenose
ptEstenose subglótica, congénita
plWrodzone zwężenie podgłośniowe
csVrozená subglotická stenóza
ukВроджений субгортанний стеноз
zh先天性声门下狭窄
trDoğumsal subglottik stenoz

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q31.1Especificvalidated
ICD-11LA71.3Especificvalidated
MONDO0015395Evalidated
UMLSC0396051Evalidated
GARD19952Evalidated
MedDRA10087149Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

0 records

No prevalence record in this release.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card