Double uterus-hemivagina-renal agenesis syndrome

Orpha code: 3411OMIM code: 192050

Definition

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.

Disease data
Classification

Malformation syndrome

Synonyms
Double uterus and obstructed hemivagina syndrome
Niedrożna szczątkowa pochwa - anomalia nerki po tej samej stronie
Zespół Herlyn i Wernera
Zespół OHVIRA
Zespół podwójna macica - niedrożna szczątkowa pochwa
Zespół Wunderlicha
Herlyn-Werner syndrome
OHVIRA syndrome
Obstructed hemivagina and ipsilateral renal anomaly
Wunderlich syndrome
ORPHA code
3411
OMIM code
192050
ICD10 code
Q51.2
ICD11 code
LD2F.1Y

No additional description.

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