Herlyn-werner-wunderlich syndrome: MRI findings, radiological guide (two cases and literature review), and differential diagnosis

Abstract

BackgroundHerlyn-Werner-Wunderlich (HWW) syndrome is a very rare congenital anomaly of the urogenital tract involving Müllerian ducts and Wolffian structures, and it is characterized by the triad of didelphys uterus, obstructed hemivagina and ipsilateral renal agenesis. It generally occurs at puberty and exhibits non-specific and variable symptoms with acute or pelvic pain shortly following menarche, causing a delay in the diagnosis. Moreover, the diagnosis is complicated by the infrequency of this syndrome, because Müllerian duct anomalies (MDA) are infrequently encountered in a routine clinical setting.Cases presentationtwo cases of HWW syndrome in adolescents and a differential diagnosis for one case of a different MDA, and the impact of magnetic resonance (MR) imaging technology to achieve the correct diagnosis.ConclusionsMR imaging is a very suitable diagnostic tool in order to perform the correct diagnosis of HWW syndrome.

Sonographic diagnosis of Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: a report of two cases

“…OHVIRA syndrome is usually discovered at puberty, shortly after menarche because of increasing pelvic pain, dysmenorrhoea and pelvic mass secondary to haematometrocolpos from retained blood in the obstructed hemivagina 4 . It may remain unrecognised initially as regular menstrual flow from the patent unobstructed hemivagina gives the appearance of normal menses, and dysmenorrhoea, if present, is a common complaint in this age group 7 .…”

“…Symptom relief of dysmenorrhoea with non‐steroidal anti‐inflammatory and oral contraceptive medication may also lead to a delay in diagnosis 9 . The clinical presentation may be variable and non‐specific features such as acute abdominal, 4 , 10 pelvic 9 , 11 or vaginal 12 pain, dysuria, 13 urinary retention, 7 vaginal discharge 14 , 15 and infertility 16 have been reported. The two cases presented were both known to have an absent kidney and this should prompt investigation for Mullerian duct anomalies and vice versa.…”

“…The acronym OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) syndrome was proposed in 2007 to allow inclusion of other uterine and renal anomalies 3 . The incidence of uterine didelphys related to this syndrome is approximately 1/2,000 to 1/28,000, and it is accompanied by unilateral renal agenesis in 43% of cases 4 . It has been estimated that there would be only four to five cases of uterine didelphys and obstructed hemivagina per year in Australia 5 .…”

Spectrum of Congenital Renal Anomalies

“…Usually there is compensatory hypertrophy of solitary kidney which was observed in our study (Fig 6). Renal agenesis is usually associated with trisomy 21 and 22, Mullerian anomalies, congenital heart disease, club foot, and VACTREL (15) . In our study, only Mullerian anomaly (Herlyn Werner Wunderlich syndrome) was associated with renal agenesis.…”

Herlyn-Werner-Wunderlich Syndrome: Case Report

“…Tanıyı kesinleştirmek ve uterovajinal anatomiyi detaylandırmak için pelvis MRG %100'e yakın doğruluk göstermektedir. 3,11 MRG, uterus konturlarının, uterin kavitenin ve vajinal septumun şeklinin, sıvı koleksiyonunun natü-rünün belirlenmesinde, eşlik eden endometrioz, adezyon, inflamasyon gibi komplikasyonların saptanmasında ayrıntılı bilgiler sağlamaktadır. Bu çalış-mada kesin tanısını MRG ile koyduğumuz HWW sendromu olgusu sunulmuştur.…”