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Start free with EleplanPrune belly syndrome
ORPHA:2970Malformation syndrome
Also called Abdominal muscle deficiency syndrome · Eagle-Barret syndrome · Obrinsky syndrome · Triad syndrome
What it is
A rare lower urinary tract obstruction (LUTO) characterized by varying degrees of an enlarged urinary bladder, dilated ureters, hydronephrosis, and poorly contractile and disorganized detrusor and ureteral smooth muscle, in association with hypoplastic or absent midline abdominal skeletal musculature, and bilaterally undescended testes in males.
Key facts
- Prevalence
- 1-9 / 100 000 (at birth, Italy)
- Age of onset
- Antenatal, Neonatal
- Inheritance
- Autosomal dominant, Not applicable, X-linked recessive
- Classified as
- Malformation syndrome
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%
9Common30–79%
8Sometimes5–29%
17- Abnormality of the uterus
- Anal atresia
- Atrial septal defect
- Cognitive impairment
- Congenital hip dislocation
- Failure to thrive
- Intestinal atresia
- Intestinal malrotation
and 9 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
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
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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