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

Abstract

BackgroundHerlyn-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 presentationA 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.ConclusionAn 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.

Incidental diagnosis of Herlyn-Werner-Wunderlich Syndrome in a nulliparous woman: a case report in Bhutan

“…Absence of one kidney by ultrasound in small hospitals should alert clinicians about this syndrome. Radiological review by Khaladkar et.al 12 showed an absence of left kidney and a study by Nishu et.al 13 found non-visualization of ipsilateral kidney by ultrasound similar to the ultrasound findings in our patient. Similar finding of an absent right kidney and possible uterine anomaly on ultrasound which was later confirmed by MRI was described by Del Vescovo et.al 11 .…”

Herlyn-Werner-Wunderlich Syndrome: A Case Report on a Congenital Uterine Anomaly With Literature Review

“…Different clinical presentations exist for those patients with HWWS. 1,3,9–11 Zhu et al 12 developed a classification system for these clinical presentations and ease of reference. Class I consists of patients with complete hemi-vaginal obstruction with or without cervical atresia.…”

Herlyn–Werner–Wunderlich syndrome in a multiparous female

“…A typical presentation of this syndrome is dysmenorrhea in adolescent age group, a few months to years after attaining menarche. 9 Patients can also present with pelvic pain, mass, abnormal vaginal discharge, infertility, endometriosis, complicated pregnancy, and labor. 10 The initial clinical diagnosis is incorrect in the majority of the cases because of its rare incidence and misleading presenting signs and symptoms.…”