Pseudounicornuate uterus

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Pseudounicornuate uterus

ORPHA:180079Morphological anomaly

Also called Incomplete unilateral Müllerian aplasia · Incomplete unilateral aplasia of the Müllerian ducts · Unicornuate uterus with rudimentary horn

What it is

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.

Key facts

Classified as
Morphological anomaly

ICD-10 codes

Q51.4filed under a broader ICD-10 category — shared with 2 other rare diseases

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Cross-references

MONDO 0015833UMLS C4749300

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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