raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:180074

True unicornuate uterus

Synonyms: Complete unilateral Müllerian aplasia · Complete unilateral aplasia of the Müllerian ducts · Unicornuate uterus without 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 with no rudimentary horn. Urinary tract anomalies are frequently associated.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enTrue unicornuate uterusComplete unilateral Müllerian aplasia · Complete unilateral aplasia of the Müllerian ducts · Unicornuate uterus without rudimentary horn
frUterus unicorne vraiAplasie müllerienne unilatérale complète · Aplasie unilatérale complète des canaux de Müller · Uterus unicorne sans utérus rudimentaire
deEchter Uterus unicornisAplasie des Müller-Ganges, komplette unilaterale · Uterus unicornis ohne rudimentäres Horn
esÚtero unicorne verdaderoAplasia mulleriana unilateral completa · Aplasia unilateral completa de los conductos de Müller · Útero unicorne sin cuerno rudimentario
itUtero unicorne veroAplasia mülleriana unilaterale completa · Aplasia unilaterale completa del dotto mülleriano · Utero unicorne senza corno rudimentale
nlEchte eenhoornige baarmoederEenhoornige baarmoeder zonder rudimentaire hoorn · Volledige unilaterale Mülleriaanse aplasie · Volledige unilaterale aplasie van gang van Müller
ptÚtero unicórneo verdadeiro
plPrawdziwa macica jednorożnaCałkowita jednostronna aplazja przewodu Müllera · Macica jednorożna bez rogu szczątkowego
csUterus unicornisÚplná jednostranná Müllerova aplázie · Kompletní jednostranná aplázie Müllerových vývodů
ukСправжня однорога маткаПовна одностороння аплазія мюллерових проток · Повна одностороння мюллерова аплазія · Однорога матка без рудиментарного рогу
zh真性单角子宫单角子宫不伴残角
trGerçek unikollis uterusTam unilateral Müllerian aplazisi · İlkel boynuz ile seyretmeyen unikollis uterus · Müllerian kanallarının tam unilateral aplazisi

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10Q51.4NTBTattributedvalidated
ICD-11LB44.2NTBTindexvalidated
MONDO0015832Evalidated
UMLSC5848180Evalidated

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