It could be desirable to achieve to an early diagnosis and treatment of HWW syndrome, in order to relieve acute symptoms, preserve normal fertility and prevent several medical complications. The correct diagnosis can be difficult due to the absence of specific findings upon physical examination and to non specific symptoms which delay the diagnosis.
Ultrasonography (US) is a very helpful tool in the diagnosis of Müllerian duct anomalies, infact the detection of haematocolpos, which appears as a fluid collection with low-level echoes, can make the diagnosis of genitor-urinary tract anomaly (didelphic uterus) easier, although it cannot identify the type of Müllerian ducts anomaly (MDA), whereas MRI, in cases when the differentiation of the uterine anomaly is not easily achievable by means of previous examination (physical examination, US, hysterosalpingography), is a suitable technique for the non-invasive evaluation of female pelvic anatomy because it provides more detailed information regarding uterine morphology, the continuity with each vaginal (obstructed and non-obstructed) lumen, and fluid content nature [12
When a high suspicion of MDA exists, US should be performed initially to delineate any abnormalities in the genital tract, however, in order to obtain an accurate MDA classification, magnetic resonance imaging must be performed.
Although US, CT scan, and hysterosalpingography are commonly used for diagnosis because of their low cost, MRI is the most accurate method for diagnosis and it could help patients in maintaining fertility by allowing the most appropriate treatment option to be used [13
In fact, in comparison to the other modalities, MRI is more sensitive in detecting the uterine contour, the shape of the intrauterine cavity, the character of the septum, as well as associated aspects such as endometriosis, pelvic inflammation and adhesions.
However, laparoscopy should now be considered the gold standard for the evaluation of female reproductive tract anomaly, although it could be reserved when the diagnosis is not clear after imaging or when MRI is not available and not performed as a routine procedure [7
The decision to perform a laparoscopy is based on the following:
- the interval between menarche and diagnosis;
- the severity of the symptoms;
- the presence of a hematometra or pyometra.
Laparoscopy can be also therapeutic in some selected cases such as: drainage of hematocolpos/hematometrocolpos, septectomy, or marsupialization of the blind hemivagina. It is considered very helpful in order to reduce pelvic pain and lower the risk of infection and of further hematometra [1
The outcome of pregnancy in these patients reveals that 87% go on to have a successful pregnancy, while abortions occur in 23% of the patients, 15% have preterm births, and 62% have full-term pregnancies and uncomplicated deliveries.
If surgery is not an immediate option for patients with HWW syndrome, menstrual suppression with combined oral contraceptive pills is advised to prevent further accumulation of hematocolpos and further hematometra [14
The consequences that potentially occur due to the failure of treatment include urinary retention, hematosalpinx (blood in the fallopian tube), endometriosis and rupture of a tubo-ovarian abscess [16
With each diagnosis of didelphys uterus in association with obstructed hemivagina on the lower abdomen MRI, HWW syndrome should be excluded and the exam should be extended to the upper abdomen to check for the presence of ipsilateral kidney.
Renal tract anomalies are associated with MDA in up to 30% of cases due to the close embryologic relationship between the paramesonephric and mesonephric ducts.
The most common renal tract anomaly associated with MDA is renal agenesis with right side prevalence [1
]. Therefore, in all patients who present an MDA, it is imperative to investigate the urinary tract.
If renal agenesia is diagnosed, patients should have continuous follow-up to check for renal functioning as the risk of renal failure is very high [19
]. Another very common condition in HWW syndrome patients is associated endometriosis.
The underlying pathophysiological mechanism is potentially the increased risk of retrograde menstruation: because of the obstructed vaginal outflow, the risk of endometrial tissue reflux through the fallopian tube, which can result in retrograde endometriosis, is higher, confirming the theory postulated by Sampson [20
Several reports have confirmed that an association with endometriosis is present in obstructive Müllerian anomalies such as HWW syndrome, but it has not been confirmed to date in non-obstructive malformations. Therefore, it is imperative that MRI technology should be used to evaluate the presence of endometriosis.
Additionally, the presence of associated hematocolpos and hematometra, as well as the presence of an irregularly shaped or sized adnexal structure, or potentially expanding masses inside, need to be detected.
Another kind of obstructive Müllerian anomalies, different from the HWW syndrome, is OHVIRA syndrome, characterized by a variety of associated disorders. Infact, while the classic presentation includes obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), some authors reported other renal anomalies, such as duplicated kidneys, dysplastic kidneys or retrovescical bands [21
]. There have been also few reports of varied uterine anatomy; infact, a series of 42 cases of OHVIRA syndrome described, in 22% of cases, a septate uteri [15
], but the majorority of cases are duplicated or didelphys uterus, although it has been described a case with two cervices, two uterine cavities and a single uterus [22