double uterus-hemivagina-renal agenesis syndrome
Findings
No curated finding names double uterus-hemivagina-renal agenesis syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Double uterus, hemivagina and renal agenesis is a rare congenital urogenital anomaly characterized by the presence of double uterus (didelphys, bicornuate or septum-complete or partial), unilateral cervico-vaginal obstruction (obstructed hemivagina-communicant, not communicant or septate and unilateral cervical atresia) and ipsilateral renal anomalies (renal agenesis and/or other urinary tract anomalies). Patients are usually diagnosed at puberty after menarche due to recurrent severe dysmenorrhea, chronic pelvic pain, excessive foul smelling mucopurulent discharge, spotting and intermenstrual bleeding (depending on the existence of uterine or vaginal communications). fever, dyspareunia, and a palpable abdominal, pelvic or vaginal mass (mucocolpos or pyocolpos) may also be present.
Definition from the Mondo Disease Ontology (MONDO:0008636), read 2026-09-29. CC BY 4.0.
Features
12 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal uterine cervix morphologyHPOHP:0012888
- Very frequent (80% to 99% of cases)
- Breech presentationHPOHP:0001623
- Very frequent (80% to 99% of cases)
- Chronic painHPOHP:0012532
- Very frequent (80% to 99% of cases)
- DysmenorrheaHPOHP:0100607
- Very frequent (80% to 99% of cases)
- DyspareuniaHPOHP:0030016
- Very frequent (80% to 99% of cases)
- Fever
Where it sits
Other names
5 names
Resolves to: double uterus-hemivagina-renal agenesis syndrome
- Also called
- Double uterus and obstructed hemivagina syndromeHerlyn-Werner syndromeobstructed hemivagina and ipsilateral renal anomalyOHVIRA syndromeWunderlich syndrome