Double uterus - hemivagina - renal agenesis

General Information (adopted from Orphanet):

Synonyms, Signs: Double uterus and obstructed hemivagina syndrome
Obstructed hemivagina and ipsilateral renal anomaly
Herlyn-Werner syndrome
Wunderlich syndrome
OHVIRA syndrome
Number of Symptoms 15
OrphanetNr: 3411
OMIM Id: 192050
ICD-10:
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: < 60 cases [Orphanet]
Inheritance: Unknown
[Orphanet]
Age of onset: Neonatal
Infancy
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Syndromic renal or urinary tract malformation
 -Rare developmental defect during embryogenesis
 -Rare genetic disease
 -Rare renal disease
Syndromic urogenital tract malformation
 -Rare developmental defect during embryogenesis
 -Rare genetic disease
 -Rare urogenital disease
Syndromic uterovaginal malformation
 -Rare gynecologic or obstetric disease

Symptom Information: Sort by abundance 

1
 (HPO:0003762) Uterus didelphys 8 / 7739
2
 (HPO:0008678) Renal hypoplasia/aplasia Very frequent [Orphanet] 127 / 7739
3
 (HPO:0000130) Abnormality of the uterus Very frequent [Orphanet] 86 / 7739
4
 (HPO:0000104) Renal agenesis 68 / 7739
5
 (HPO:0000137) Abnormality of the ovary Occasional [Orphanet] 41 / 7739
6
 (HPO:0000142) Abnormality of the vagina Very frequent [Orphanet] 24 / 7739
7
 (HPO:0000858) Menstrual irregularities Very frequent [Orphanet] 42 / 7739
8
 (HPO:0008670) Partial vaginal septum 1 / 7739
9
 (HPO:0000795) Abnormality of the urethra Frequent [Orphanet] 38 / 7739
10
 (HPO:0000107) Renal cyst Frequent [Orphanet] 126 / 7739
11
 (HPO:0002566) Intestinal malrotation Occasional [Orphanet] 89 / 7739
12
 (HPO:0100026) Arteriovenous malformation Occasional [Orphanet] 38 / 7739
13
 (OMIM) Two cervices 1 / 7739
14
 (OMIM) Unilateral hematocolpos 1 / 7739
15
 (HPO:0000006) Autosomal dominant inheritance 2518 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: