Infected products of conception in obstructed hemivagina after medical management of early pregnancy loss: A case report.

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

ObjectivesObstructed Hemi-Vagina Ipsilateral Renal Agenesis (OHVIRA) syndrome is a rare congenital malformation. Incidence of unilateral renal agenesis affects 1:1000 live births and the association between renal and Mullerian anomalies is...

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

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.

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

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

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), also called Herlyn-Werner-Wunderlich syndrome, is an extremely rare Müllerian duct anomaly accompanied by Wolffian duct anomalies. A 10-year-old intact female Yorkshire Terrier weighing 3.35 kg was presented with anorexia, depression, vomiting, and abdominal pain. Radiography, ultrasonography, and computed tomography revealed uterine didelphys, obstructed hemivagina, a cystic structure around the right uterus, and right renal agenesis, leading to the diagnosis of OHVIRA syndrome. An ovariohysterectomy and decompression of the obstructed right hemivagina were performed, and the cystic structure near the right uterine horn was removed by en bloc resection, along with the right uterus and ovary. After the surgical intervention, the patient's symptoms including abdominal pain, anorexia, and depression were immediately resolved. The patient was followed up for 1 month postoperatively with ultrasonography at 2-week intervals, which revealed the progression of mild fluid retention in the right hemivagina. However, no additional urogenital findings were identified, and the patient continued to exhibit no overt clinical symptoms. This case report describes the diagnosis and surgical treatment of the first documented case of OHVIRA syndrome in an animal. Unlike in human medicine, where vaginal septectomy is performed to prevent dilatation of the obstructed hemivagina and thereby resolve clinical symptoms, performing ovariohysterectomy combined with fluid aspiration from the obstructed hemivagina showed a favorable postoperative prognosis in the dog.

We read with interest the report by Zivkovic et al. [1] entitled ‘‘Unusual case of OHVIRA syndrome with complication’’. Mullerian duct anomalies are rare congenital female genital tract anomalies that are divided into seven classifications and subtypes. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is characterized mainly by uterus didelphys, septate uterus, an obstructed hemivagina, and renal agenesis. The most common complications associated with Mullerian anomalies include endometriosis, dysmenorrhea, cervix hematoma, abortion, and premature birth. The case reported by Zivkovic et al. [1] is a very rare complication of intrapartal rupture during delivery. We have also experienced very rare case of OHVIRA syndrome. A 35-year-old gravida 0 female was transferred to our institution for uterine myoma and uterine anomalies. Pelvic examination revealed a large uterus, double cervix, and hard palpable mass in the left vagina wall. CT revealed a suspicious underlying hemivaginal obstruction, hematoma with calcification, left renal agenesis with uterus didelphys, and subserosal myomas (Fig. 1a). MRI revealed uterus didelphys with a left hemivaginal obstruction, a large adenomyotic cyst in the left cervix, and left renal agenesis (Fig. 1b). The calcified mass in our patient had formed secondary to old menstrual blood. Our patient was unaware of her calcified vaginal masses before CT or MRI examination. Classification of Mullerian duct anomalies depends mainly on fusion of the Mullerian ducts. Therefore, unclassified Mullerian duct anomalies or uncommon complications are found incidentally. Addition of our case of OHVIRA syndrome with vaginal calcified masses such as stones to cases of vaginal rupture during delivery will provide clinically useful information to obstetricians and other readers.

Obstructed hemivagina with an ipsilateral renal anomaly (OHVIRA) syndrome is a congenital malformation that presents as a uterine didelphys with an obstructed hemivagina and an associated ipsilateral renal aberration. The clinical symptoms usually manifest after menarche. Unlike the typical presentation in adolescence, this case report features a neonatal presentation of OHVIRA syndrome with an unusual renal association. A female twin delivered at 35 weeks of gestation was transferred to our institution after birth from an outside hospital due to respiratory distress and for evaluation of the left multicystic dysplastic kidney identified on prenatal ultrasound. Physical examination and lab results, including a complete blood count, and a basic metabolic panel, including blood urea and serum creatinine, were within the normal range for age. Abdominal and pelvic ultrasound showed multicystic dysplastic left pelvic kidney, congenital hepatic cyst measuring 6 mm, uterine didelphys with duplication of the vaginal canal, and obstructed left hemivagina corresponding to the OHVIRA syndrome. Further testing revealed a normal chromosomal microarray, small patent foramen ovale on the echocardiogram, no vertebral or rib anomalies on the spinal x-ray, normal hearing test, and mild optic cupping on the ophthalmological evaluation. The pediatric surgeon and urologist recommended an outpatient follow-up and elective surgery in the future. This is a unique case presenting in the neonatal period with an unusual association. Timely intervention can help prevent obstetric complications.

Herlyn-Werner-Wunderlich syndrome is an unusual congenital anomaly of the female genitourinary system, which is described as uterine didelphys with Obstructed Hemi-vagina and Ipsilateral Renal Anomaly (OHIRA), also known as OHVIRA syndrome. Mullerian duct anomalies have an incidence of 2–3%. While OHVIRA constitutes 0.16–10% of these Mullerian duct anomalies. Symptoms usually present shortly after menarche when hematocolpos develops during menstruation resulting in dysmenorrhea and a pelvic mass. The pelvic mass is the collection of blood products within the obstructed hemivagina. The first study in the diagnostic work-up is usually ultrasonography, which typically demonstrates a pelvic fluid collection which can simulate other disease processes thus confounding the diagnosis. MRI findings of the pelvis reveal a didelphis uterus. Imaging of the abdomen reveals agenesis of the ipsilateral kidney. MRI is beneficial in characterizing the didelphys uterus and vaginal septum for pre-operative planning.

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

Uterine Didelphys With Obstructed Hemivagina and Ipsilateral Renal Agenesis Presenting With Hydronephrosis

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome should be redefined as ipsilateral renal anomalies: Cases of symptomatic atrophic and dysplastic kidney with ectopic ureter to obstructed hemivagina

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

History of present illness: A 14-year-old female presented with rectal pain, pelvic pressure, urinary hesitancy and difficulty defecating despite daily laxative use. She had a history of irregular periods and was currently menstruating. Her vital signs were normal. Her abdominal exam was unremarkable and the external genitourinary exam showed a visible vaginal introitus and no masses. Significant findings: Due to pain out of proportion to her exam, an ultrasound of her pelvis was obtained and showed a blood-filled distended uterus, or hematometrocolpos (white arrow), with a 4.9 cm right ovarian cyst (blue arrow). A pelvic magnetic resonance imaging (MRI) then revealed an obstructed right hemi-vagina, normal left uterus and vagina and ipsilateral renal agenesis (red arrow) with normal left kidney (double arrow) consistent with obstructed hemivagina, ipsilateral renal agenesis (OHVIRA) syndrome. The patient underwent surgical repair with complete resolution of symptoms.Discussion: OHVIRA is rare syndrome that occurs due to a failure of lateral fusion of the Mullerian ducts.1 It affects an estimated 0.1%-3.8% of the population.2 The majority of these cases are associated with ipsilateral renal anomalies, but up to half can have contralateral anomalies.1,2 The most common presenting symptom is pelvic pain.1 On physical exam, patients may have a bulge in the vaginal wall which represents hematometrocolpos due to obstruction of outflow of menstrual blood. Transabdominal pelvic ultrasound or MRI are helpful modalities for diagnosis in adolescents.1,3 In one pediatric case series of OHVIRA, all 8 patients had a history of normal menses and presented with acute or chronic pelvic pain that began after menarche.2 All patients in this case series were initially misdiagnosed, due to the rarity of the disorder and the fact that most patients have a history of regular menses despite progressive pelvic pain. Definitive treatment requires surgical excision of the vaginal septum. Post-surgical prognosis is typically good;4-6 potential complications include endometriosis and increased risk for preterm labor or malpresentation.7 Topics: Pelvic pain, obstructed hemivagina, renal agenesis, rectal pain, hematometrocolpos, hematocolpos.