Herlyn-Werner-Wunderlich syndrome: a case report

Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital disorder of the Müllerian ducts in which there is uterus didelphys, obstructed hemivagina and unilateral renal agenesis. The most common presentation is an abdominal mass secondary to hematocolpos, pain and dysmenorrhea. However, in some cases, such as the one we present here, menses are normal due to an obstructed hemivagina, and diagnosis can be delayed. We describe evaluation and surgical management of a 13-year-old girl with this condition who was diagnosed by computed tomography (CT) scan and confirmed by pelvic ultrasound and surgical exploration, as well as a review of the literature.

Three Cases of Didelphys Uterus, Including Uterus Didelphys with Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA Syndrome) AKA Herlyn-Werner-Wunderlich Syndrome), with a Systematic Review

“…If ultrasound suggests MDA, MRI can confirm it, which is the gold standard for diagnosis [1,18]. Three dimensional ultrasounds have also shown promising results but, are not available in many clinical settings [13,[19][20][21].…”

“…Herlyn-Werner-Wunderlich (HWW) syndrome also known as obstructed Hemivagina Ipsilateral renal agenesis (OHVIRA) is characterized by the presence of a didelphys uterus, obstructed Hemivagina and Ipsilateral renal agenesis [1][2][3]. It is a very rare genitourinary tract anomaly involving the Mullerian and Mesonephric duct development, most often occurring during the sixth to ninth week of embryologic development [4].…”

“…Depending on the level of embryologic malformation, management commonly involves surgical intervention in the form of excision of the vaginal septum or hemihysterectomy [1][2][3][4][5]. In addition to relieving the pain and discomfort associated with the collection of debris and blood, surgical intervention may reduce the incidence of pelvic endometriosis, infection, adhesions and infertility [5][6][7][8][9].…”

Herlyn-Werner-Wunderlich and Prader-Willi syndromes: more than a coincidence?

“…Incidences of obstructive Müllerian anomalies are believed to be between 0.1% and 3.8% and HWWS accounts for approximately 5% of these 1 6. Studies of uterine organogenesis have implicated the Hox and Wnt genes as regulators of uterine morphology.…”

A Case Report on Secondary Dysmenorrhea with Herlyn-Werner-Wunderlich Syndrome

“…Herlyn-Werner-Wunderlich Syndrome (이하 HWWS)은 중복자궁(uterine didelphys), 한쪽 질의 폐쇄(obstructed hemivagina), 동 측 신장의 무발생(ipsilateral renal agenesis) 을 특징으로 하는 매우 드문 뮐러관 기형 중 하나로, 추정 발생률은 0.1~3.8%이다 [1][2][3] . 전형적인 증상으로는 폐쇄된 쪽에 월 경혈이 고이게 되므로 초경 이후 월경통 과 하복부 또는 질의 팽윤 및 종괴의 형 성이 나타나며, 폐쇄된 자궁이나 질 부 위에 월경혈의 방출이 차단되어 역류되 면 질혈종이나 자궁내혈종, 난관혈종 또 는 자궁내농양 같은 합병증을 초래할 수 있으며, 불완전 폐쇄의 경우에는 부정출 혈이나 화농성 질분비물이 나타난다 4,5) .…”