Complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) in a young woman—a rare variant of Herlyn–Werner–Wunderlich syndrome

Abstract

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is an unusual and special type of Müllerian duct anomaly. Patients usually present in adolescence, soon after menarche, with pain and pelvic mass and rarely with infertility in adulthood. Majority of the cases of OHVIRA have been reported in association with uterus didelphys and the presentation of uterus didelphys with OHVIRA is known as Herlyn–Werner–Wunderlich syndrome. A complete septate uterus with OHVIRA is exceedingly unusual. Less than 30 cases of complete septate uterus with OHVIRA have been reported to date, to the best of our knowledge. We present a rare case of incidentally detected complete septate uterus with OHVIRA in a young woman who presented with acute pain in the right iliac fossa owing to acute appendicitis.

Herlyn-Werner-Wunderlich syndrome in a young female presenting with dysmenorrhea: A case report

“…HWW syndrome is characterized by the coexistence of renal and Müllerian anomalies on the same side. The most common renal anomaly associated with the syndrome is renal agenesis and the most common Müllerian anomaly associated with the syndrome is uterine didelphys with vertical vaginal septum [1] . Other renal anomalies like megaureter, ectopic ureter, dysplastic kidney, and renal duplication may be associated with the syndrome [3] .…”

“…The development of urogenital structures occurs at 6-12 weeks of gestation [1] . Mesonephric ducts, also known as Wolffian ducts and paramesonephric ducts also known as Müllerian ducts are important for the embryogenesis of the renal collecting system, uterus, cervix, and upper vagina [2] .…”

Vaginoscopic Incision of Vaginal Septum With Preservation of the Hymen in a Child With Obstructed Hemi-Vagina Ipsilateral Renal Agenesis (OHVIRA) Syndrome

“…Recently, with the increasing number of cases that do not fit the classic definition of OHVIRA syndrome, it was suggested to incorporate a spectrum of ipsilateral renal anomalies in the definition of the syndrome including ipsilateral dysplastic, atrophic kidney and ectopic ureter [8,11,12]. On the other hand, septated uterus and single uterus have been rarely reported with the syndrome [13,14].…”

A Variant of Herlyn-Werner-Wunderlich Syndrome Presenting With Acute Abdomen: A Case Report and Review of Literature

“…The OHVIRA syndrome is included in class III of the American Fertility Society classification (Table 1) of congenital uterine anomalies and often consists of uterine duplication, though septate uterus (Class V American Fertility Society classification) has been rarely associated. 6,7 Ipsilateral renal agenesis remains the most common associated urological anomaly though multicystic dysplastic kidney, renal duplication kidneys, rectovesical bands, or crossed fused ectopia have also been reported in the literature. 8 Recently, HWW syndrome has been classified on the basis of the morphology of vagina (Table 2).…”

“…5 However, only few variants of OHVIRA are known, which includes septate uterus and ipsilateral renal anomalies. 6 A patient generally presents after menarche with nonspecific symptoms and diagnosis is often delayed till acute complications develop. 2 It is often associated with infertility, recurrent pregnancy loss, and adverse pregnancy outcomes.…”