Clinical History
22-year-old unmarried woman presented to the outpatient department complaining of irregular menstrual cycle and lower abdominal pain for 3 months. She attained menarche at the age of 12 years. Physical examination showed well-developed breasts and normal axillary and pubic hair distribution. Baseline laboratory analysis and hormonal profile were normal.
Imaging Findings
MRI examination of the pelvis was done. Multiplanar and multisequential imaging including T1WI, T2WI, T2-FATSAT sequences were acquired.
The examination showed two uteri and cervices separated by a longitudinal vaginal septum. (Fig. 1) The right uterine cavity and cervix were distended and obliterated by fluid with blood MR signal returning heterogenously high signals on T1 and T2 weighted image. (Fig. 1a, 2, 3)
Right kidney and ureter were not visualized. Left kidney was seen at its normal anatomical location.
Discussion
OHVIRA (obstructed hemi-vagina and ipsilateral renal anomaly)/Herlyn-Werner-Wunderlich (HWW) syndrome is a rare complex congenital anomaly of the female genital system. It is a triad of obstructed hemi-vagina, uterine didelphys and ipsilateral renal anomaly. Incidence of Mullerian duct anomalies ranges from 0.8% to 4% and the incidence of the OHVIRA syndrome is estimated to be between 0.1-3.5% of all Mullerian anomalies. [1] The aetiology of the syndrome is unknown. However, it is multifactorial and associated with Mullerian ducts fusion anomalies in utero.
The classical presentation is of a young girl presenting with severe dysmenorrhea, few months to years after attaining menarche. Pelvic pain is the most common presenting symptom (90%) followed by an abdominal mass (40%) and pressure symptoms. Patients can also present at a later age with foul-smelling vaginal discharge due to pyocolpos. [2] Because of regular menstruation in these patients (at menarche), diagnosis is often delayed. Patients can present with complications like endometriosis, hydrosalpinx, secondary infection and pelvic adhesions causing chronic pelvic pain. Thus if not diagnosed and treated in time, it can lead to long-term problems like infertility.
The diagnostic modalities commonly used are ultrasonography and MRI. Ultrasound can diagnose the collection inside the uterus or vagina (secondary to obstruction, and may raise the differential of adenexal masses i.e. endometriomas and cystadenoma), but cannot identify the type of Mullerian anomaly, while MRI due to its multiplanar and tissue characterization abilities can give precise information about uterine morphology and about the continuity with each vaginal (obstructed and non-obstructed) lumen. MRI has more sensitivity in detecting the uterine contour, the shape of the cavity, the presence of a septum, as well as the presence of the associated pathology such as endometriosis, pelvic inflammation, and adhesions. Thus MRI is the gold standard method for accurate diagnosis and allows the most appropriate treatment option to be used. [3]
The standard management of these patients is excision of vaginal septum and drainage of haematometrocolpos. A simple excision of the vaginal septum can relieve the patient of her symptoms and avoid infertility. [4] Altchek and Paciuc have reported pregnancy to occur twice in a previously-obstructed didelphys uterus after surgical correction. [5] The surgeon must, therefore, make every effort to preserve the obstructed uterus.
In conclusion, greater awareness of rare entity and early diagnosis with timely intervention can prevent patient future complications. MRI has proved to be of great help in correct diagnosis of this rare anomaly, avoiding laparoscopy.
Differential Diagnosis List
Herlyn-Werner-Wunderlich syndrome / OHVIRA syndrome
Ovarian cystadenoma
Endometrioma
Mullerian duct anomaly (Bicornuate uterus)
Final Diagnosis
Herlyn-Werner-Wunderlich syndrome / OHVIRA syndrome
