Herlyn–Werner–Wunderlich syndrome presenting with infertility: Role of MRI in diagnosis
A 22-year-old female, cohabiting with her husband for last 3 years, was evaluated for primary infertility in a private hospital. She attained menarche at the age of 13 years and had a normal menstrual history with no complaints of excessive dysmenorrhoea, dyspareunia, or vaginal discharge. Routine hematological laboratory tests were normal. An abdominal USG and computed tomography (CT) were performed, which revealed absent left kidney, a bicornuate uterus, bilateral cystic adnexal lesions, and left hydrosalpinx. She was taken up for laparotomy and underwent left ovarian cystectomy and adhesiolysis. Histopathology from the left adnexal lesion showed endometriotic cysts and aspirated pelvic fluid showed hemosiderin-laden macrophages. Postoperatively, she was kept on medical management for endometriosis.\nShe did not resume her menses for 3 months post-surgery and urine pregnancy test was negative, following which she was referred to our institute for further evaluation. MRI examination of the pelvis was performed to assess the current status of endometriosis. MRI revealed two separate uterine cavities, cervices, and vaginas, suggestive of uterus didelphys [Figures -]. The right uterine cavity, cervix, and vagina were normal. The left uterine cavity and cervical canal were dilated and filled with fluid which was hyperintense on both T1W and T2W MRI [Figures and ] with few T2-hypointense areas [] suggesting blood products. Left hemivagina was dilated [Figures , , and ] with blood products within, implicating the presence of an obstructing left vaginal septum. Multiloculated left adnexal cystic lesions with blood products were seen suggestive of endometriotic cysts []. In addition, a tubular structure was noted in left adnexal location extending laterally from left uterine cornu with hemorrhagic fluid within, indicative of left hematosalpinx. Right ovary was normal. In addition, there was absent left kidney [] with loculated hemorrhagic fluid collections in bilateral paracolic gutters, suggestive of peritoneal endometriosis [].\nHence, a diagnosis of uterus didelphys with left hematometra, hematosalpinx, hematocolpos, and left hemivaginal obstruction with adnexal and peritoneal endometriosis was made. Considering that she also had absent left kidney, a final diagnosis of HWWS or obstructed hemivagina ipsilateral renal anomaly (OHVIRA) syndrome was made. Since the patient had already undergone recent laparotomy, no further surgery was immediately performed. She has been kept on short-term conservative management with oral contraceptives to suppress menstruation and laparoscopic vaginal septal excision has been planned after 3 months.