Complex mullerian duct anomaly in a young female with primary amenorrhoea, infertility, and chronic pelvic pain
A nulliparous, 30-year-old female, presented to the infertility clinic with complaints of primary amenorrhoea and infertility, with chronic pelvic pain for more than ten years duration. The general physical examination was unremarkable with normal secondary sexual characters. The routine laboratory investigations for amenorrhoea and infertility were normal including normal hormonal profile. Pelvic examination revealed a normally developed vagina and cervix and anteverted nulliparous uterus. The patient had prior been investigated several times, including sonography of pelvis, outside our institution, with all inconclusive reports.\nThe patient underwent ultrasound in the radiology department of our institute, which demonstrated the presence of a normal sized anteverted uterus, oriented more toward the left side of pelvic cavity. The endometrial cavity was distended with echogenic fluid. The left uterine tube was dilated and filled with fluid of similar echogenecity. The cervix and left ovary were normal. A complex mass comprising of solid and cystic components was found on the right side. There was no separate visualization of right ovary. The patient was advised pelvic magnetic resonance imaging (MRI) to better characterise the ultrasound findings.\nMRI of the pelvis showed presence of two asymmetric uterine horns, smaller on the right side with thin myometrium and normal sized left with normal myometrium. Both endometrial cavities were distended with hemorrhagic contents and showed no communication to each other. There was associated hematosalpinx on either side. The cervix and vagina were normally developed, however there was no demonstrable communication between the endometrial cavity and the endocervical canal. Both the ovaries were normal. On the basis of MRI findings, a diagnosis of unicornuate uterus with cervical dysgenesis and a cavitated, noncommunicating rudimentary horn on the right side, was made [].\nThe patient was taken up for laprohysteroscopy. The cervix was well developed but the hysteroscope could not be negotiated beyond the isthmus, consistent with cervical dysgenesis. There were two uterine horns with the right side smaller than the left. Dense adhesions were found in the pelvic cavity, likely due to retrograde menstruation and Stage IV endometriosis. The omentum was adherent to the uterus and the pouch of Douglas was obliterated. The fallopian tubes on both sides were dilated with thick, oedematous walls and were found congested. Bilateral ovaries were normal.\nDue to delayed presentation of the patient, advanced endometriosis, dense pelvic adhesions and a failed attempt to recanalize/reconstruct cervix, a decision for hysterectomy was taken after obtaining patient's consent []. The uterus and both fallopian tubes were removed and adhesiolysis was done. There were no intra or postoperative complications. On a one year follow up, the patient is completely relieved of her symptoms of chronic pelvic pain.