Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases.
BACKGROUND
Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis is a rare entity, sometimes referred to as Herlyn-Werner-Wunderlich syndrome (HWW). It usually presents after menarche with progressive pelvic pain, sometimes with regular menses, and a palpable mass due to hemihaematocolpos. The diagnosis is generally made only if the suspicion of this genitourinary syndrome is raised.


OBJECTIVE
To highlight the imaging diagnostic clues in this rare condition.


METHODS
We report on 11 adolescents with this condition.


RESULTS
Sonography mostly allowed the correct diagnosis by showing uterovaginal duplication, haematocolpos or haematometrocolpos, and the absence of the ipsilateral kidney. MRI provided more detailed information regarding uterine morphology, the continuity with each vaginal channel (obstructed and nonobstructed), and the bloody nature of the contents.


CONCLUSIONS
Early and accurate diagnosis of this syndrome is important so that adequate and prompt surgical therapy (excision of the vaginal septum) can provide relief of pain and prevent further complications. It is also advisable to look for an obstructed Müllerian system whenever a multicystic dysplastic kidney or the absence of a kidney is discovered in a fetus, or girl postnatally.
Orazi
Cinzia
C
Department of Diagnostic Imaging, I.R.C.C.S. Bambino Gesù Pediatric Hospital, Rome, Italy.
Lucchetti
M Chiara
MC
Schingo
Paolo M S
PM
Marchetti
Paola
P
Ferro
Fabio
F
D000293:Adolescent; D002648:Child; D005260:Female; D006801:Humans; D007668:Kidney / Q000002:abnormalities*; D008279:Magnetic Resonance Imaging; D013577:Syndrome; D014463:Ultrasonography; D014564:Urogenital Abnormalities / Q000175:diagnosis*; D014599:Uterus / Q000002:abnormalities*; D014621:Vagina / Q000002:abnormalities*