Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: Common presentation of an unusual variation

Abstract

Mullerian duct abnormalities are congenital malformations that are easily missed and can lead to incorrect diagnosis and unnecessary operative procedures. In this case, a young female presented with cyclic pelvic pain that continued after previous surgical resection of an ovarian cyst. Further investigation with clinical examinations and multimodality imaging demonstrated ipsilateral renal agenesis and a Class III Mullerian duct anomaly (MDA) requiring a second operative procedure. It is believed that this case is a variant of the described obstructed hemi-vagina with ipsilateral renal agenesis (OVIRA) anomaly as pathologically there was ipsilateral renal agenesis and complete vaginal agenesis in our case. It is imperative to have a high clinical suspicion of mullerian duct abnormalities when encountering a patient with other urogenital anomalies. This will decrease the amount of misdiagnoses, guide appropriate surgical intervention, and decrease the risk of future reproductive complications.

Rare urogenital anomaly variant with a separated didelphic uterus and ipsilateral renal agenesis without hemivagina: A case report

“…(f)). A similar case was reported by Adair et al (). From the viewpoint of embryology, damage to the right Wolffian duct might affect right urethral buds and fusion of the Müllerian ducts.…”

“…OHVIRA syndrome is considered a developmental anomaly of the Müllerian and Wolffian ducts. Although uterine anomalies occur in 2–3% of women, OHVIRA syndrome is rare and accounts for only 0.16–10% of uterine anomalies (Lin et al ; Adair et al ). The typical symptoms are dysmenorrhea after menarche.…”

A Rare Case: Ohvira Syndrome (Obstructed Hemivagina and Ipsilateral Renal Anomaly) With Unicornuate Uterus

“…Magnetic resonance imaging (MRI) is the modality of choice for the diagnosis of HWWS and also for other such anomalies because of better anatomic delineation of pelvic structures and higher sensitivity of the MRI for blood products. Since the vaginal septum can be treated by septal excision, and the delay in the diagnosis may worsen the associated endometriosis, 8 hence early diagnosis and treatment is beneficial.…”

Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report

“…Instead the caesarean sections rate is 84% and it reflects the high incidence of the breech presentation (51%) [29, 32]. In the presentation of this case we drew attention to its rarity because it involves the left part of the body, while usually is the right side affected [28, 33, 34] and the association with a malformation of the skeleton of the lower limbs, ectrodactyly of the right foot, was not ever been reported in literature; we found only a case report which described the combination of HWWS and lumbar scoliosis [35] and we are not sure if the skeletal involvement could be only an incidental finding. However, not all authors agree that it is a rarity the involvement of the left side: Yavuz et al presented a case series of 13 women affected by HWWS and 6 of them had anomalies on the left side of the uro-genital tract [36].…”