Herlyn-Werner-Wunderlich syndrome presenting with dysmenorrhea: a case report

Abstract

Background: Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hematocolpos. We present the first case report on Herlyn-Werner-Wunderlich syndrome from Bangladesh.

Case presentation: A 15-year-old Asian girl presented with lower abdominal pain of 3 months' duration. She had had menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. Abdominal examination found a tender mass on the right iliac fossa. Further evaluation with ultrasound revealed distended endometrial cavity filled with complex fluid and nonvisualization of the right kidney. Pelvic magnetic resonance imaging showed absent right kidney and two separate endometrial stripes surrounded by endometrium and a muscular layer. The right endometrial cavity and cervix were distended with blood. This magnetic resonance imaging finding is consistent with Herlyn-Werner-Wunderlich syndrome with uterine didelphyis, right-sided hematometra resulting from obstructed hemivagina, and ipsilateral agenesis of the right kidney. The vaginal septum was resected for vaginoplasty. She was discharged 5 days after surgery and came for follow-up after 7 days. Vaginal examination revealed a healthy wound with no adhesion of the vaginal wall. She also informed us that she had started regular menstruation without any pain 30 days after the operation.

Conclusion: An unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to complications such as endometriosis and infertility. Awareness is required; otherwise, misdiagnosis clearly can occur.

Keywords: Herlyn-Werner-Wunderlich syndrome; Müllerian anomaly; Obstructed hemivagina; Vaginoplasty.

Fig. 1

Ultrasonogram of lower abdomen showing endometrial cavity (arrow) filled with large, thick collection that extends up to the cervix

Fig. 2

Coronal short tau inversion recovery image showing absence of right kidney (dashed arrow) and two separate endometrial stripes (uterine didelphys) surrounded by separate muscle coat (arrows)

Fig. 3

Sagittal T2-weighted image showing dilation of cervical canal (short arrow) with abrupt termination at vaginal fornix (long arrow), resulting in obstructed hemivagina. Endometrial cavity was mildly dilated and contained hemorrhagic collection

Fig. 4

Axial T2-weighted image showing two separate uterine horns (short arrows) and distention of right endometrial cavity (long arrow)

Fig. 5

Vaginal septum (arrow) was identified perioperatively for resection

Fig. 6

Drainage of tarry inspissated blood

Fig. 7

Reconstructed vaginal canal after resection of vaginal septum

Fig. 8

Herlyn-Werner-Wunderlich syndrome based on vaginal morphology [9]