Abstract Obstructed hemivagina and ipsilateral renal anomaly OHVIRA , also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since Only a handful of these cases have been associated with pyocolpos. Symptoms usually present shortly after menarche when hematocolpos develops during menstruation resulting in dysmenorrhea and a pelvic mass. The pelvic mass is the collection of blood products within the obstructed hemivagina.
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Abstract The Herlyn—Werner—Wunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain during menses secondary to haematocolpos.
Awareness is necessary in order to diagnose and treat this disorder properly before complications occur. MRI is the preferred modality for the delineation of uterine malformation. When renal anomalies are encountered, a screening should also be made for congenital abnormalities of the reproductive tract and vice versa.
The authors report a case of a girl with this condition who had a prenatal diagnose of right renal agenesis and presented at 13 years old with pelvic pain caused by haematocolpos. Background The Herlyn—Werner—Wunderlich HWW syndrome is a rare variant of mullerian duct anomalies characterised by the combination of uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis.
This syndrome was described for the first time in and was suspected in a young woman with regular menstruation and gradually increasing pelvic pain and a pelvic mass after menarche. Case presentation A year-old girl, presented to the Emergency Department with a 4-day history of increasing right lower quadrant RLQ and hypogastric pain, nausea and sporadic vomiting without fever, diarrhoea or urinary symptoms.
She was on the fourth day of menstrual bleeding. Her medical history revealed a right-sided renal agenesis diagnosed prenatally. The menarche had occurred 5 months before, her cycles were regular and the menstrual bleeding usually lasted about 4—5 days with severe dysmenorrhoea. She had not been sexually active yet. The main physical examination finding was tenderness on RLQ and hypogastric palpation, and doubtful Blumberg sign. An abdominal ultrasonography complemented by pelvic CT was performed on the day of admission, confirmed the absence of the right kidney and showed an image suggestive of haematocolpos on the right side and two uterine bodies.
The patient was then referred to the gynaecological department where she was medicated with a continually administered oral contraceptive to suppress menses and relieve symptoms until a MRI was obtained and a corrective surgery planned. The pelvic MRI showed two separate uteri with two separate cervices uterus didelphys bicollis figure 1A and two proximal vaginas.
The right vagina was distended and filled by a slightly hypointense material on T2-weighted images figure 1B,C and hyperintense material on T1-weighted images figure 1D that did not suppress on fat-suppressed T1-weighted sequences figure 1E , suggesting a blood collection haematocolpos.
Only the distal portion of the left vagina was identified. Although a longitudinal vaginal septum was not directly visualised, its presence was admitted.
Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos
Abstract The Herlyn—Werner—Wunderlich syndrome is a rare congenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain during menses secondary to haematocolpos. Awareness is necessary in order to diagnose and treat this disorder properly before complications occur. MRI is the preferred modality for the delineation of uterine malformation.
Herlyn–Werner–Wunderlich syndrome: a rare cause of pelvic pain in adolescent girls
Gartner duct cysts Treatment and prognosis Treatment is surgical, with resection of the septum dividing the two hemivaginas in order to relieve the obstruction. Subsequent successful pregnancy in the obstructed uterus has been reported 5. Differential diagnosis When uterovaginal obstruction results in significant dilatation, the imaging differential may include a large adnexal mass such as a cystadenoma or endometrioma. References 1.