Teaching Case

A 12-year-old girl presented with dysmenorrhea 6 months after the menarche. The menstrual cycles were irregular.

As protocol for a single kidney, routine ultrasounds (US) were performed.
Pre-pubertal pelvic US didn't show abnormal findings.
Due to severe dysmenorrhoea, a pelvic US during the menstruation was performed, showing two hemiuteri and two hemivaginas, with left haematometrocolpos.
Magnetic resonance imaging (MRI) was acquired with T1-weighted images (T1WI) and T2-weighted images (T2WI) in three planes.
It showed two hemiuteri, two hemicervix and two hemivaginas. The left obstructed hemivagina wasn't significantly distended and haemorrhagic content wasn't identified. It was assumed that the hemivagina was incompletely obstructed, with complete drainage of the menstrual residue previously seen on US. A dilated left ureteral stump was identified.
These findings suggested a diagnosis of Herlyn-Werner-Wunderlich syndrome with an ectopic ureter.
Before the surgical procedure oral contraceptives were stopped, anticipating the expansion of the obstructed hemivagina.
Despite this, haematometrocolpos didn't develop, leading to unsuccessful drainage attempt.
A sequential US, 3 months later, showed haematometrocolpos.
A second drainage was effective.
The patient remained asymptomatic since then.

Background
Herlyn-Werner-Wunderlich syndrome (HWWS) is characterised by uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis.
HWWS may be caused by failure of vertical and lateral fusion of Mullerian ducts, around 9 weeks of gestation [1, 2].
The incidence is estimated between 0.1-3.8% [1, 3, 4].
There is a predilection for the right side [2-5].

Clinical Perspective
Symptoms generally occur 2-12 months after menarche [3]. Onset under 5 years of age has been described [6]. Rarely it may present as an acute abdomen [7].
Zhu et al. suggested a new classification of HWWS in 1 – completely obstructed hemivagina – and 2 – incompletely obstructed hemivagina. Classification 1 presents with haematometrocolpos, haematosalpinx and haemoperitoneum. Classification 2 presents with purulent or bloody vaginal discharge and ascending genital system infection years after menarche [1].

Imaging Perspective
Transabdominal and transvaginal US (if sexually active) may show two hemiuteri and dilatation of the proximal hemivagina. The haemorrhagic content can range from anechogenic to inhomogeneous with mixed echogenicity [3].
MRI should be obtained in three different planes (one parallel to the long axis of the uterus) and both T1WI and T2WI should be acquired. The obstructed hemivagina may show high signal intensity in T1WI and T2-shading sign (loss of signal intensity from T1WI to T2WI due to high concentration of protein and iron) [3].
Ipsilateral renal agenesis should be documented.
Occasionally a ureteral stump can be identified [3].
There is a potentially increased risk of retrograde menstruation due to the obstructed vaginal outflow with subsequent reflux of endometrial tissue through the fallopian tube, which can result in retrograde endometriosis, as such MRI should assess the presence of endometriosis [5].

Outcome
The treatment of choice is drainage of haematometrocolpos and surgical excision or marsupialisation of the longitudinal vaginal septum. Septectomy is ideally made when a large haematocolpos develops, as the septal tissue becomes thinner and easier to excise [1, 3]. If surgery is postponed, menstrual suppression with gonadotropin-releasing hormone analogues is advised to prevent accumulation of haematometrocolpos, especially if cervical atresia coexist [1, 3]. When septectomy is not feasible unilateral hysterectomy may be considered [4, 7]
Acute complications of HWWS include pyohaematocolpos, pyosalpix and pelviperitonitis [1, 5].
Long-term complications are endometriosis (17.1%), pelvic adhesions, increased risk of abortion (74%), premature delivery (22%), caesarean section (80%), infertility and tumours of the obstructed portions [3-5].

Take home messages
A gynaecological investigation should be done in neonates with renal agenesis [2, 3, 6].
A renal investigation is advisable whenever a didelphys uterus and obstructed hemivagina is diagnosed [5].