Double uterus-hemivagina-renal agenesis syndrome
Synonyms: Double uterus and obstructed hemivagina syndrome · HWW syndrome · Herlyn-Werner-Wunderlich syndrome · OHVIRA syndrome · Obstructed hemivagina and ipsilateral renal anomaly
- Type
- Malformation syndrome
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Antenatal, Infancy, Neonatal
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Double uterus-hemivagina-renal agenesis syndrome | Double uterus and obstructed hemivagina syndrome · HWW syndrome · Herlyn-Werner-Wunderlich syndrome · OHVIRA syndrome · Obstructed hemivagina and ipsilateral renal anomaly |
| fr | Syndrome d'utérus double-hémivagin-agénésie rénale | Hémivagin obstrué et anomalie rénale ipsilatérale · Syndrome HWW · Syndrome OHVIRA · Syndrome de Herlyn-Werner-Wunderlich · Syndrome de double utérus et d'hémivagin obstrué |
| de | Uterusverdoppelung-Hemivagina-Nierenagenesie-Syndrom | HWW-Syndrom · Herlyn-Werner-Wunderlich-Syndrom · OHVIRA-Syndrom · Obstruierte Hemivagina mit ipsilateraler Nierenagenesie · Syndrom der Uterusduplikatur mit obstruierter Hemivagina |
| es | Síndrome de útero doble-hemivagina-agenesia renal | Anomalía renal ipsilateral y hemivagina obstruida · Síndrome OHVIRA · Síndrome de Herlyn-Werner-Wunderlich · Síndrome de doble útero y hemivagina obstruida |
| it | Sindrome utero doppio-emivagina-agenesia renale | Sindrome HWW · Sindrome OHVIRA · Sindrome dell'utero doppio e dell'emivagina ostruita · Sindrome Herlyn-Werner-Wunderlich · Emivagina ostruita e anomalia renale ipsilaterale |
| nl | Dubbele uterus - hemivagina - renale agenesie-syndroom | Dubbele uterus en geobstrueerde hemivagina-syndroom · Geobstrueerde hemivagina en ipsilaterale nieranomalie · HWW-syndroom · OHVIRA-syndroom · Syndroom van Herlyn-Werner-Wunderlich |
| pt | Síndrome de duplo útero-hemivagina-agenesia renal | HWW · Hemivagina obstruída e anomalia renal ipsilateral · Síndrome Herlyn-Werner-Wunderlich · Síndrome OHVIRA · Síndrome de duplo útero e vagina obstruída |
| pl | Podwójna macica - niedrożna szczątkowa pochwa - agnezja nerki | Niedrożna szczątkowa pochwa - anomalia nerki po tej samej stronie · Zespół Herlyn i Wernera · Zespół OHVIRA · Zespół podwójna macica - niedrożna szczątkowa pochwa · Zespół Wunderlicha |
| cs | Syndrom zahrnující zdvojení dělohy, hemivaginu a agenezi ledvin | OHVIRA syndrom · Syndrom zahrnující zdvojení dělohy a obstrukční hemivaginu · Herlynův-Wernerův syndrom · Obstrukční hemivagina a ipsilaterální anomálie ledviny · Wunderlichův syndrom · Herlyn-Wernerův syndrom |
| uk | Синдром подвійної матки-гемівагини-ниркової агенезії | Синдром подвійної матки та непрохідної гемівагіни · Синдром Герліна-Вернера · Непрохідна гемівагіна та іпсилатеральна аномалія нирок · Синдром OHVIRA · Синдром Вундерліха |
| zh | 双子宫-半阴道-肾发育不全综合征 | 梗阻性半阴道-同侧肾发育不全综合征 |
| tr | Çift uterus-hemivajina-renal agenez sendromu | OHVIRA sendromu · Wunderlich sendromu · Herlyn-Werner sendromu · Çift uterus ve tıkalı hemivajina sendromu · Tıkalı hemivajina ve ipsilateral böbrek anomalisi |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 192050 | E | validated | |
| ICD-10 | Q51.2 | NTBT | attributed | validated |
| ICD-11 | LD2F.1Y | NTBT | index | validated |
| MONDO | 0008636 | E | validated | |
| UMLS | C4302552 | E | validated | |
| GARD | 1910 | E | validated | |
| MedDRA | 10083351 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (3):
- ORPHA:93547Syndromic renal or urinary tract malformation
rare developmental anomalies during embryogenesis; rare renal diseases; rare transplant-related disorders - ORPHA:165707Syndromic urogenital tract malformation
rare developmental anomalies during embryogenesis; rare genetic diseases; rare urogenital diseases - ORPHA:180148Syndromic uterovaginal malformation
rare gynecological and obstetric diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 60 cases | Worldwide | Case(s) | Validated | ORPHANET | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
12 annotations · validated 2017-08-21| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0012888 | Abnormality of the uterine cervix | |
| HP:0001623 | Breech presentation | |
| HP:0012532 | Chronic pain | |
| HP:0100607 | Dysmenorrhea | |
| HP:0030016 | Dyspareunia | |
| HP:0001945 | Fever | |
| HP:0030711 | Hydrocolpos | |
| HP:0100608 | Metrorrhagia | |
| HP:0008670 | Partial vaginal septum | |
| HP:0001622 | Premature birth | |
| HP:0000104 | Renal agenesis | |
| HP:0003762 | Uterus didelphys | |
Run the differential on the 12 obligate / very frequent terms
Annotation source: 934555[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/3411
- This record as JSON: /data/d/3411.json