raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:180079

Pseudounicornuate uterus

Synonyms: Incomplete unilateral Müllerian aplasia · Incomplete unilateral aplasia of the Müllerian ducts · Unicornuate uterus with rudimentary horn

Type
Morphological anomaly
Group
Disorder
Status
active

A rare, non-syndromic uterovaginal malformation characterized by a crescent-shaped, small-sized uterus containing a single horn and fallopian tube associated with a rudimentary second horn (which can be solid or contain a cavity with functioning endometrium and be communicating or non-communicating). Urinary tract anomalies are frequently associated.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enPseudounicornuate uterusIncomplete unilateral Müllerian aplasia · Incomplete unilateral aplasia of the Müllerian ducts · Unicornuate uterus with rudimentary horn
frUtérus pseudo-unicorneAplasie müllerienne unilatérale incomplète · Aplasie unilatérale incomplète des canaux de Müller · Utérus unicorne avec utérus rudimentaire controlatéral
dePseudo-Uterus unicornisAplasie des Müller-Ganges, inkomplette unilaterale · Uterus unicornis mit rudimentärem Horn
esÚtero pseudo-unicorneAplasia mulleriana unilateral incompleta · Aplasia unilateral incompleta de los conductos de Müller · Útero unicorne con cuerno rudimentario
itUtero pseudo-unicorneAplasia mülleriana unilaterale incompleta · Aplasia unilaterale incompleta del dotto mülleriano · Utero unicorne con corno rudimentale
nlPseudo-eenhoornige uterusEenhoornige baarmoeder met rudimentaire hoorn · Onvolledige unilaterale Mülleriaanse aplasie · Onvolledige unilaterale aplasie van gang van Müller · Pseudo-eenhoornige baarmoeder
ptÚtero pseudo-unicórneo
plRzekoma macica jednorożnaMacica jednorożna z rogiem szczątkowym · Niecałkowita jednostronna aplazja przewodu Müllera
csNekompletní jednostranná aplázie Müllerových vývodůNeúplná jednostranná Müllerova aplázie · Uterus unicornis cum cornu rudimentario · Uterus pseudounicornis
ukПсевдооднорога маткаНеповна одностороння аплазія мюллерових проток · Неповна одностороння аплазія Мюллера · Однорога матка з рудиментарним рогом
zh假性单角子宫单角子宫伴残角
trPsödounikollis uterusEksik unilateral Müllerian aplazisi · İlkel boynuz ile seyreden unikollis uterus · Müllerian kanallarının eksik unilateral aplazisi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q51.4NTBTattributedvalidated
ICD-11LB44.2NTBTindexvalidated
MONDO0015833Evalidated
UMLSC4749300Evalidated

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