raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:141219

Nasal dorsum fistula

Type
Morphological anomaly
Group
Disorder
Status
active

A rare otorhinolaryngological malformation characterized by the presence of a dermoid cyst, located on the dorsum of the nose, which presents a fistula, often extending to the intracranial region. Patients present a firm, slow-growing mass, which contains skin and dermal elements (including hair follicles and sebaceous glands), that do not transilluminate or compress, and may be associated with intermittent or chronic discharge of sebaceous material, soft tissue and skeletal deformity, and local infection. Meningitis, convulsions and cerebral abscess may be observed if intracranial extension exists.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enNasal dorsum fistula
frFistule du dos du nez
deNasenrückenfistel
esFístula dorso nasal
itFistola del dorso del naso
nlNeusdorsumfistel
ptFístula do dorso nasal
plPrzetoka/torbiel grzbietu nosa
csPíštěl/cysta nosního hřbetu
ukНориця спинки носа
zh鼻背侧瘘管
trBurun dorsum fistülü

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q18.8NTBTattributedvalidated
ICD-11LA5YNTBTindexvalidated
MONDO0015410Evalidated
MONDO15410Evalidated
UMLSC0339853Evalidated
GARD19963Evalidated

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