raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:431344

Urachal sinus

Type
Morphological anomaly
Group
Disorder
Inheritance
Not applicable
Age of onset
Infancy, Neonatal
Status
active

Urachal sinus is a type of congenital urachal anomaly resulting from the failure of the umbilical end of the urachus to close, without continuity to the bladder, and that is usually asymptomatic but can present with continuous cloudy umbilical discharge, tender midline infraumbilical mass and fever when infected.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enUrachal sinus
frSinus de l'ouraqueSinus ouraquien
deUrachussinus
esSeno uracal
itSeno uracale
nlUrachussinus
ptSeio uracal
plZatoka moczownika
csUrachální sinus
ukПазуха урахуса
zh脐尿窦
trUrakial sinüs

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q64.4NTBTattributedvalidated
ICD-11LB03.0NTBTindexvalidated
MONDO0018552Evalidated
UMLSC3472657Evalidated

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

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

4 annotations · validated 2025-10-08
HPOTerm (as annotated by Orphanet)Diagnostic
Occasional (29-5%)
HP:0002027Abdominal pain
HP:6000171Abdominal wall mass
Very rare (<4-1%)
HP:0004321Bladder fistula
HP:0002664Neoplasm

Annotation source: 36895469[PMID]_34277280[PMID]

7

Sources for this card