Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos

Abstract

Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the urogenital tract, which is characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents at puberty with pelvic pain, dysmenorrhea, and a vaginal or pelvic mass. Although rare, it may present with purulent vaginal discharge due to secondary infection of the obstructed hemivagina, making diagnosis difficult. A careful pelvic examination to identify the cervix and vagina is the key to the diagnosis of Müllerian duct anomalies and magnetic resonance imaging can provide additional useful information. The optimal treatment is full excision and marsupialization of the obstructing vaginal septum so that both uteri can drain through the patent vagina. The authors report a case of a 22-year-old female with an unusual presentation of Herlyn-Werner-Wunderlich syndrome complicated by pyocolpos, which was successfully managed by vaginal septum resection and drainage of pus.

Unusual Cause of Sepsis in a 10-Year-Old Female

“…4 OHVIRA syndrome is rare, with an estimated incidence of 1:20 000. 1,2 In OHVIRA syndrome, the vaginal septum results in one vagina being unobstructed and one obstructed, which occurs in only 6% of cases with uterine duplication. 3 Renal agenesis is present in 92% to 100% of patients with obstructed hemivagina, where it is only present in 63% to 81% of patients with uterine duplication alone.…”

“…3 Renal agenesis is present in 92% to 100% of patients with obstructed hemivagina, where it is only present in 63% to 81% of patients with uterine duplication alone. 2,3 OHVIRA typically presents in an adolescent female with progressive dysmenorrhea or menorrhagia, with the abnormal anatomy discovered on pelvic examination or imaging studies. The obstructed hemivagina is usually sterile and filled with blood from menstrual cycles.…”

“…3 Another complication of microperforations is the possibility of ascending infection to the hematocolpos, leading to pyocolpos and abscess formation that can cause clinical findings of pelvic inflammatory disease. 2,3 Pyocolpos has been associated with leukocytosis and elevated inflammatory markers. 2 In some patients, the hematocolpos results in a dilation of the uterine cavity that has the potential to extend into the fallopian tube, a finding called hematosalpinx.…”

“…2,3 Pyocolpos has been associated with leukocytosis and elevated inflammatory markers. 2 In some patients, the hematocolpos results in a dilation of the uterine cavity that has the potential to extend into the fallopian tube, a finding called hematosalpinx. 3 If this larger hematocolops becomes infected it can result in TOA formation, 2,3 as in our patient.…”

“…2 In some patients, the hematocolpos results in a dilation of the uterine cavity that has the potential to extend into the fallopian tube, a finding called hematosalpinx. 3 If this larger hematocolops becomes infected it can result in TOA formation, 2,3 as in our patient. Due to the obstruction, there may also be retrograde menstruation on the obstructed side resulting in bleeding into the peritoneal space, which is more likely if the vaginal septum is thick, thus limiting the distension of the hemivagina distally.…”