Posterior urethral valve

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Posterior urethral valve

ORPHA:93110Morphological anomaly

Also called PUV

What it is

A rare, congenital, fetal lower urinary tract obstruction (LUTO) anomaly characterized by an abnormal congenital obstructing membrane or leaflets that are located within the posterior urethra associated with significant obstruction of the male bladder restricting normal bladder emptying.

Key facts

Prevalence
1-9 / 100 000 (Europe)
Age of onset
All ages
Inheritance
Autosomal recessive, Not applicable, X-linked recessive
Classified as
Morphological anomaly

Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Gene

BNC2Disease-causing germline mutation(s)

ICD-10 codes

Q64.2filed under a broader ICD-10 category

Cross-references

GARD 7439MEDDRA 10036369MONDO 0019640OMIM 618612UMLS C0238506

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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