Imaging findings are diagnostic of obstructed hemi-vagina with ipsilateral renal agenesis (OHVIRA). This is a rare condition consisting of a triad of uterine didelphys (a type III Müllerian duct anomaly), hematocolpos, and ipsilateral renal agenesis. If any of these three features are absent, the diagnosis of OHVIRA is excluded. Clinical presentation is typically 2-12 months post menarche. Though rare pre-pubertal onset has been documented secondary to maternal hormonal influence, the mean age of presentation is 17 years old. Symptoms are typically non-specific including dysmenorrhea and a palpable pelvic mass. Complications: Acute complications of OHVIRA include primarily infection of retained blood products resulting in pyohematocolpos or pyosalpinx. Rarely pelviperitonitis can occur if hematocolpos progresses to a hematosalpinx which ruptures. Long term complications include endometriosis, thought secondary to retrograde flow of blood products, which can lead to development of pelvic adhesions. Infertility issues are thought largely to be secondary to endometriosis and/or infection more so than uterine didelphys. Additional obstetric complications are greater in patients without surgical correction and most patients who carry a pregnancy to term have no increased difficulty. Treatment: Surgical correction with vaginal septectomy or division is the first line treatment for OHVIRA. If surgical correction is achieved early, the risk of complications including infertility are greatly diminished.
SPR 2022 Annual Meeting & Postgraduate Course