MALFORMACIONES MÜLLERIANAS: A PROPÓSITO DEL SÍNDROME DE OHVIRA. REPORTE DE CASOS

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome with a single septate uterus: first case report

Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) – A Fetal Autopsy Case

Obstructed hemivagina and ipsilateral renal anomaly syndrome: a case series of 4 patients with insights into diagnosis and treatment strategies

Uterus didelphys with obstructed hemivagina and ipsilateral renal anomaly (OHVIRA syndrome): A case report

Proposal of the 3O (Obstruction, Ureteric Orifice, and Outcome) Subclassification System Associated with Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA).

The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is characterized by the triad uterus didelphys, obstructed hemivagina, and ipsilateral renal dysplasia. To make a radiological diagnosis, knowledge of this syndrome is of paramount importance. Early recognition may prevent complications such as unnecessary surgical procedures, endometriosis, and infections, which could adversely affect fertility. A 1-day-old female newborn in whom a right-sided cystic kidney abnormality was seen on antenatal ultrasound was admitted with anuria and intralabial mass. Besides the multicystic dysplastic right kidney, ultrasound revealed a uterus didelphys with right-sided uterus dysplasia, an obstructed right hemivagina, and an ectopic ureteric insertion. The diagnosis of obstructed hemivagina and ipsilateral renal anomaly syndrome with hydrocolpos was made and the hymen was incised. Later, ultrasound helped in diagnosing a pyelonephritis in the afunctional right kidney that was not draining into the bladder (hence no culture could be obtained), requiring intravenous antibiotics and a nephrectomy. Obstructed hemivagina and ipsilateral renal anomaly syndrome is an anomaly of the Müllerian and Wolffian ducts of unknown cause. Patients typically present after menarche with (progressive) abdominal pain, dysmenorrhea, or urogenital malformations. In contrast, prepubertal patients can present with urinary incontinence or an (external) vaginal mass. The diagnosis is confirmed by an ultrasound or magnetic resonance imaging. Follow-up includes repeated ultrasounds and monitoring of kidney function. Treatment consists of drainage of the hydrocolpos/hematocolpos; in some cases, further surgery is indicated. Consider obstructed hemivagina and ipsilateral renal anomaly syndrome in girls with genitourinary abnormalities: early recognition prevents complications later in life.

Obstructed Hemivagina and Renal Anomalies in Patients with and without Anorectal Malformations

The acronym for obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) was created to describe patients with an obstructed hemivagina and ipsilateral renal anomaly and enables inclusion of other uterine anomalies except uterus didelphys. The main goal of this article is to present a rare case of OHVIRA syndrome with intrapartal rupture of obstructed hemivagina. We present an unusual case of OHVIRA syndrome with single uterus, uterine septum (previously resected by hysteroscopy) and renal agenesis, unrecognized before labor and followed by an intrapartal rupture of obstructed hemivagina. Various symptoms, included in OHVIRA acronym, are a result of different morphologic variants included in this syndrome. Most likely, in our case, the absence of communication between the cervical canal/patent vagina and the obstructed hemivagina prevented formation of mucocolpos or hematocolpos. This rare clinical variant made our MRI diagnosis of obstructed hemivagina, as well of the entire OHVIRA syndrome, ineffective. We did not find OHVIRA cases in the literature with a single septate uterus and single cervix, associated with obstructed hemivagina diagnosed during and after labor and presented with intrapartal rupture of hemivagina. Because of lack of guidelines, the final decision about management of the labor in such rare cases of OHVIRA syndrome is difficult.

Multiple variants of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome – one clinical center case series and the systematic review of 734 cases

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome presents with complex diagnostic and therapeutic challenges and is characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. A 14-year-old female with a history of anorectal malformation and urogenital sinus anomaly presented with menstrual blood in her urine, abdominal pain, and distension. Investigations revealed a bicornuate uterus, vesicovaginal fistula, and right ovarian cyst, leading to the diagnosis of OHVIRA syndrome. A multidisciplinary approach resulted in salpingo-oophorectomy and subtotal hysterectomy. This case highlights the diagnostic challenges and emphasizes the role of advanced imaging and a multidisciplinary team in managing such complex conditions. It stresses the importance of patient-centered surgical planning tailored to the individual's anatomy and reproductive goals. Early recognition and a tailored, multidisciplinary approach are crucial in managing OHVIRA syndrome and improving outcomes for patients with rare congenital anomalies.

BACKGROUND: Hemivaginal obstruction syndrome and ipsilateral renal anomalies are known as Herlyn-Werner-Wunderlich syndrome (HWWs) or better known by the acronym obstructed hemivagina and ipsilateral renal anomaly (OHVIRA). It occurs as a result of complete failure of the Müllerian ducts to fuse and accounts for about 5% of Müllerian duct anomalies. Initial manifestations usually appear as a result of secretions accumulating within the hemivaginal obstruction. Patients with HWWs can come with complaints of lower abdominal pain, severe dysmenorrhea, pelvic or vaginal mass, abnormal vaginal discharge, acute urinary retention, fever, or vomiting. Therefore, careful diagnosis and appropriate management of this condition are desirable. The objective of the study was to report on the management of the obstruction due to HWWs. Patient was suspected hematocolpos with uterus didelphys from ultrasound and suggested right renal agenesis from BNO IVP. Diagnosis then confirmed by laparoscopy diagnostic. CASE REPORT: A 12-year-old patient was admitted to the gynecology ward of Dr. M. Djamil Central General Hospital from polyclinic with abdominal pain since 4 months before being admitted to the hospital. The pain gets worse every day before menstruation and decreases when given painkillers. CONCLUSION: OHVIRA syndrome is a triad characterized by hemivaginal obstruction and agenesis of the ipsilateral kidney (OHVIRA) and uterus in the delphys. Clinical symptoms in general are cyclic dysmenorrhea, palpable mass due to accumulation of menstrual flow, and severe pelvic pain which can later develop into a persistent form as a result of prolonged retention of menstrual secretions and obstructed hemivagina. Management options are surgical resection of the obstructed vaginal septum and drainage.

Case Report: Neonate Presenting with Acute Kidney Injury Secondary to Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome: Long-Term Follow-Up

Hydronephrosis: A Rare Presentation of Uterine Didelphys with Obstructed Hemivagina and Ipsilateral Renal Anomaly

Background: Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome (HWWS), is a rare Müllerian malformation. Gynaecologic malignancies reported in association with OHVIRA syndrome/HWWS are exceptional and scattered across isolated case reports and small case series, leading to significant challenges for screening, early diagnosis, and optimal management. The primary aim of this study was to comprehensively review and synthesize the clinicopathologic features, treatment approaches, and reported outcomes of patients with OHVIRA-associated gynaecologic neoplasms. Methods: A systematic review of published cases of gynecologic malignancies in OHVIRA/HWWS was conducted using PubMed/MEDLINE and Scopus from database inception through January 2026. To expand the current evidence, an illustrative novel institutional case is also presented and integrated into the analysis. Results: A total of 21 cases were identified and analysed; reported tumours predominantly involved the lower genital tract (vagina/cervix), with a recurrent representation of adenocarcinoma, particularly clear cell histology, and a frequent origin from the obstructed or non-visible compartment when side was described. Endometrial and ovarian involvement was uncommon. Conclusions: The anatomical complexity of OHVIRA syndrome creates a diagnostic blind spot that warrants heightened clinical suspicion, rigorous MRI-based anatomic delineation, and side-specific evaluation in symptomatic patients. By synthesizing the available literature, this review underscores the necessity for tailored, multidisciplinary management and proactive surveillance strategies in this rare population.

Surgical Management of OHVIRA and Outcomes