raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:490

Omphalomesenteric cyst

Type
Morphological anomaly
Group
Disorder
Age of onset
Childhood
Status
active

A rare non-syndromic diaphragmatic or abdominal wall malformation, a remnant of omphalomesenteric duct, characterized by cuboidal or columnar epithelium with gastrointestinal differentiation. Patients may be asymptomatic or present with infraumbilical mass, umbilical lesion with secretions, abdominal pain, hernia, abscess, gastrointestinal tract bleeding, intestinal obstruction, and acute abdomen.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enOmphalomesenteric cyst
frKyste omphalo-mésentérique
deZyste, omphalomesenterische
esQuiste onfalo-mesentérico
itCisti onfalo-mesenterica
nlOmfalomesenterische cyste
ptQuísto onfalo-mesentérico
plTorbiel pępkowo-jelitowa
csOmfalomezenterická cysta
ukОмфаломезентеріальна кіста
zh卵黄管囊肿
trOmfalomesenterik kist

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q43.0NTBTattributedvalidated
ICD-11LB03.YNTBTindexvalidated
MONDO0018847Evalidated
UMLSC0266180Evalidated
GARD4081Evalidated

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

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families4 casesWorldwideCase(s)Validated24353646[PMID]
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidated24353646[PMID]_ORPHANET

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

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

7

Sources for this card