The Herlyn-Werner-Wunderlich Syndrome – A Case Report with Radiological Review

Abstract

SummaryBackgroundHWW syndrome is a very rare congenital anomaly of urogenital tract involving Mullerian ducts and mesonephric ducts. It is characterised by a triad of symptoms - uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. It can be classified based on a completely or incompletely obstructed hemivagina. It presents soon after menarche or shows delayed presentation depending on the type. It can exhibit acute pelvic pain shortly after menarche and may show non-specific and variable symptoms with resultant delay in diagnosis. The most common presentation is pain and dysmenorrhea, and pain and abdominal mass in the lower abdomen secondary to haematocolpos and/or haematometra.Case ReportPresentation of a clinical case of a 13-year-old patient with HWW syndrome presenting with regular menses, dysmenorrhea and painful lump in hypogastric region on the left side of midline. We described the role of imaging modalities in diagnosis of the Herlyn-Werner-Wunderlich syndrome with a review of literature. USG and MRI showed left renal agenesis with compensatory hypertrophy of the right kidney, uterus didelphys with haematometra and haematocervix in the left uterus with evidence of blood in a dilated retort-shaped left fallopian tube and a normal right uterus. The unique feature of our case is haematometra and haematocervix with cervical and vaginal atresia found on the left side (classification 1.2) with associated left renal agenesis.ConclusionsHWW syndrome can present early or late, depending on the type. In patients with uterine and vaginal abnormalities, a work-up for associated renal anomalies should be performed. Early intervention is needed to reduce the risk of endometriosis and infertility.

Herlyn-Werner-Wunderlich Syndrome in pregnancy

“…Herlyn-werner-wunderlich syndrome (HWWS) is a rare case, the incidence of uterus didelphys in singleton pregnancy is 1 : 3.000. [1][2][3][4][5] Pregnancy with HWWS is a high risk pregnancy, because its correlated with Recurrent Pregnancy Lost (RPL), malpresentation, Intrauterine Growth Restriction (IUGR), preterm, PPROM, preeclampsia and placenta accreta. [9][10][11][12] Its difficult to diagnosis HWWS because of the rarity of this case, high suspectious is needed.…”

“…It is most commonly diagnosed in puberty due to abdominal mass secondary to haematocolpos or haematometra, pain and dysmenorrhea. [1][2][3][4][5] The gold standart to diagnosed HWWS is with Magnetic Resonance Imaging (MRI), it can classification this sindrome also for treatment. 6 The long term complication is affected the reproductive system, but about 80% of it, can spontaneous pregnant.…”

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

“…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]. In the general population, Mullerian duct anomalies (MDA) have a prevalence of 2-3%, with 11% and 43% having uterus didelphys and renal abnormalities, respectively [5].…”

“…OHVIRA most commonly presents during menarche as pelvic pain, dysmenorrhea and an abdominal mass [5][6][7][8]. 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].…”

“…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].…”