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double uterus-hemivagina-renal agenesis syndrome - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

double uterus-hemivagina-renal agenesis syndrome

MONDO:0008636

Also known as:Double uterus and obstructed hemivagina syndromeHerlyn-Werner syndromeOHVIRA syndrome

Overview

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.

Diagnosis

Biomarker and diagnostic research for double uterus-hemivagina-renal agenesis syndrome has been reported in the published literature.

Prognosis

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

No clinical trials have been registered for double uterus-hemivagina-renal agenesis syndrome.

209 publications have been identified in PubMed for double uterus-hemivagina-renal agenesis syndrome. Kisho has analyzed 94 by research type. Research spans Case Report / Case Series (26%), Basic Science / Preclinical (21%), and Clinical Trial Publication (20%).

Research Type	Count	% of Total
Patient case studies	24	26%

Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 9:49 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:192050

Orphanet

European rare disease database

Orphanet:3411

GARD

Genetic and Rare Diseases Info Center

GARD:1910

Questions for Your Care Team

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double uterus-hemivagina-renal agenesis syndrome

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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