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.

UNASSIGNED: OHVIRA syndrome can be misdiagnosed due to its rarity, resulting in the need for more invasive interventions than vaginoscopy. Also, delayed diagnosis of OHVIRA syndrome can affect patient\'s quality of life by leading to chronic gynecological diseases such as endometriosis and pelvic inflammatory disease.
UNASSIGNED: Obstructive hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is one of the infrequent congenital Mullerian duct anomalies characterized by obstructed hemivagina and ipsilateral renal agenesis. This study presents a 20-year-old virgin female who was diagnosed with OHVIRA syndrome and treated by vaginoscopy using the hymen preservation technique. Also, she was misdiagnosed with non-communicating rudimentary uterine horn 4 years ago. Late or misdiagnosis of OHVIRA syndrome can affect fertility and pregnancy outcomes. Therefore, early diagnosis and management are crucial. OHVIRA syndrome\'s misdiagnosis is possible with other Mullerian duct anomalies, such as a rudimentary uterine horn. Also, patients with misdiagnosis undergo unnecessary interventions.

Congenital malformations of the female genital organs are rare anomalies and their incidence is estimated to be up to 7% in the general population. Müllerian ducts abnormalities are one of the causes of infertility and occur in approximately 16% of women with recurrent miscarriages. Sex development disorders are diagnosed at different stages of the patient\'s life depending on the accompanying ailments. Alarming signs of genital malformations include primary amenorrhea or dysmenorrhea, dyspareunia, and periodic abdominal pain.

Obstructed hemivagina and ipsilateral renal agenesis/anomaly (OHVIRA) syndrome is a very rare condition affecting girls. The time of diagnosis varies, from cases of prenatal diagnosis up to adulthood, including pregnancy or delivery. Most commonly, it is recognised during puberty and usually as an acute condition. We present a case report of an adolescent girl who underwent the treatment because of acute abdominal pain. The case is interesting due to a previous diagnosis of one-side renal agenesis. It appears to be useful to perform a diagnostic pelvic imaging at the time of diagnosis of renal agenesis or to plan to perform it at the beginning of puberty, to prevent the need for urgent surgery. This management may allow the planning of proper follow-up, minimising the risk of possible complications.

We present here a case of complex uterine anomaly-obstructed hemivagina with ipsilateral renal agenesis (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome in a 13-year-old girl with a history of recurrent urinary tract infections (rUTI). In the emergency room, a trans-abdominal sonography revealed an ovarian cyst and renal agenesis, without any suspicion of vaginal obstruction. This led to a delay in the diagnosis of this uncommon anomaly. Finally, MRI findings confirmed the presence of OHVIRA syndrome. As the congenital anomalies of the kidney and urinary tract (CAKUT) are present in almost one third of cases associated with genital malformations, urologists should carefully screen patients with rUTI. The patient underwent simultaneous laparoscopy and vaginoscopy, which was in our opinion the most appropriate therapeutic decision. In this article, we are also going to discuss the role of laparoscopy in the management of OHVIRA syndrome, as well as other surgical techniques described in the literature.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a complex Mullerian and Wolffian duct anomaly, which is difficult to diagnose before puberty. We present a rare case of a congenital syndrome known as OHVIRA in a 21-year-old female who came with complaints of intermittent type of lower abdominal pain, dysmenorrhea, and oligomenorrhea with frequent visits to different hospitals without any radiological investigations done. Early magnetic resonance imaging (MRI) investigations helped her in diagnosing and managing this syndrome.

Uterus didelphys is a rare Müllerian anomaly, often diagnosed during menarche or in women with a personal history of infertility and/or recurrent pregnancy loss. Its association with other genitourinary anomalies is frequent and may determine the existence of established syndromes. This case report refers to a 13-year-old female patient diagnosed with OHVIRA syndrome (Obstructed Hemivagina with Ipsilateral Renal Agenesis), a condition wherein the presence of a didelphic uterus is associated with hemivagina obstruction and ipsilateral renal agenesis. The patient presented with cyclic pelvic pain, related to the presence of hematocolpos and hematometra, which persisted despite several surgical approaches, including vaginal septum excision and correction of cervical stenosis. The recurrence of the condition indicated exploratory laparotomy, revealing two hemi-uteri and two uterine cervixes, with hematometra on the right. A subtotal hemihysterectomy was performed on the right. Post-procedure, the patient developed with regular menstrual cycles and improvement of pelvic pain complaints. Given the limited prevalence and low index of suspicion, the potential requirement for surgical intervention and its potential impact on reproductive future, diagnosing and treating OHVIRA syndrome and other Müllerian anomalies poses notable challenges in clinical practice. Hence, sharing different therapeutic approaches of a rare diagnosis with the scientific community is of paramount importance to aid in early diagnosis and effective management of similar clinical cases.

A patient in her early 20s presented with constant and progressive lower abdominal and back pain, mainly on the right side of the abdomen, purulent vaginal discharge and pyrexia. A radiological assessment revealed a possible tubo-ovarian abscess and the incidental diagnosis of ipsilateral renal agenesis. The patient was treated for pelvic inflammatory disease (PID); however, after antibiotic administration and since the symptoms did not resolve, an abdominal MRI was requested, which revealed uterus didelphys with two cervices, an obstructed haemivagina and evidence of haematocolpos. The diagnosis of Obstructed Hemi-Vagina with Ipsilateral Renal Agenesis (OHVIRA) syndrome was confirmed, and the patient underwent the excision of the vaginal septum, the drainage of the haematopyocolpos and the laparoscopic drainage of the tubo-ovarian abscess. She achieved a good recovery.

Obstructed hemivagina and ipsilateral renal agenesis syndrome is a rare complex of structural abnormalities of female urogenital tract. Moreover, delayed treatment of this condition can reportedly lead to pelvic infection, endometriosis, and infertility. We present a rare case of obstructed hemivagina and ipsilateral renal agenesis syndrome wherein appropriate treatment was initiated early due to the identification of left renal agenesis. A 14-year-old girl visited the gynecology department due to worsening left lower abdominal pain after menarche. She was diagnosed with renal dysplasia in the pediatric department at the ages of 3 and 9 years, and it was recommended that she visits the gynecology department right after menarche. She experienced her first menarche at the age of 14 years. Imaging studies revealed uterine didelphys with occlusion of the left vagina and formation of hematocolpos. Additionally, hematomas were observed in the left uterine cavity and the left fallopian tube; left renal agenesis was confirmed. Based on these findings, we diagnosed this patient with obstructed hemivagina and ipsilateral renal agenesis syndrome and performed a surgical incision and excision of the closed left vaginal wall, resulting in the disappearance of the hematocolpos. Approximately 2 years postoperatively, the patient has remained recurrence-free. Early diagnosis and treatment are crucial for preserving fertility in patients with obstructed hemivagina and ipsilateral renal agenesis syndrome, and early referral to the obstetrics and gynecology department is recommended if renal dysplasia is detected during childhood.

Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) syndrome is a rare congenital malformation of the Mullerian duct. We report on a 34-year-old female who presented to the emergency department for cramping lower abdominal pain and pelvic pressure with vaginal spotting. Physical exam showed marked swelling in the right adnexa, and laboratory exams were within normal limits except for positive Coronavirus disease 2019 (COVID-19) status. Transvaginal ultrasound revealed 3 well circumscribed, round hypoechoic complex cystic lesions with arterial doppler detected within the peripheral walls. Magnetic resonance imaging of the abdomen and pelvis showed a right hemivagina, right hematosalpinx, right hematometra and right renal agenesis, compatible with OHVIRA syndrome. The patient was informed of elective surgical procedure but was unable to undergo surgery at this time secondary to COVID status. The patient was therefore recommended oral contraceptive therapy for suppression of menses and protection of endometrial lining.