True unicornuate uterus

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True unicornuate uterus

ORPHA:180074Morphological anomaly

Also called Complete unilateral Müllerian aplasia · Complete unilateral aplasia of the Müllerian ducts · Unicornuate uterus without 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 with no rudimentary horn. 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 0015832UMLS C5848180

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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