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Start free with EleplanPosterior 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
Very common80–99%
3Sometimes5–29%
16- Abdominal pain
- Aplasia/Hypoplasia of the lungs
- Bladder diverticulum
- Dilatation of the bladder
- Fetal megacystis
- Fetal pyelectasis
- Hematuria
- Hypertension
and 8 more in this range
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
ICD-10 codes
Cross-references
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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