A Complex Case Report of OHVIRA syndrome: Uterine didelphys, obstructed hemivagina, and renal agenesis

We report the case of a 26-year-old woman, gravida 2 para 1, who presented to our emergency department with chronic pelvic pain and persistent white leukorrhea. She had a history of surgical drainage for hematocolpos at the age of 16, although no specific diagnosis was made at the time. Her medical history was otherwise unremarkable, with no chronic conditions, allergies, or history of sexually transmitted infections. She had no known family history of congenital genitourinary anomalies or renal malformations. On physical examination, the abdomen was soft, depressible, and non-tender. Speculum examination revealed white, non-foul-smelling leukorrhea and a macroscopically normal cervix. Bimanual pelvic examination identified a firm, palpable mass bulging into the left vaginal wall. Initial laboratory tests were within normal limits: white blood cell count 6,700/mm³ (normal range: 4000-10,000/mm³), hemoglobin 12.9 g/dL (12-16 g/dL), platelet count 235,000/mm³ (150,000-400,000/mm³), C-reactive protein <5 mg/L, and serum creatinine 0.78 mg/dL (0.6-1.2 mg/dL). Pelvic ultrasound showed a bicornuate uterus with a vesical “V” sign and an intercornual distance greater than 4 cm, as well as a left lateral uterine mass measuring 4 × 4 cm, heterogeneous and non-vascularized on Doppler imaging. Pelvic MRI confirmed the presence of a didelphic uterus consistent with the ESHRE/ESGE classification U3b C2 V2 ( Figs. 1 and 2 ), indicating complete uterine duplication (U3b), 2 separate cervices (C2), and a longitudinal vaginal septum (V2). The longitudinal septum divided the vagina into 2 hemivaginas: the right hemivagina was patent, while the left hemivagina was obstructed and markedly distended with fluid consistent with hematocolpos ( Figs. 3 and 4 ). A narrow communicating tract was visible between the 2 hemivaginal cavities. T1-weighted images showed mild hyperintensity, and T2-weighted sequences showed marked hyperintensity of the content, consistent with subacute hemorrhagic fluid. There was no evidence of communication between the collection and the uterine cavity or cervix. Both uterine horns and cervices appeared normal in size and morphology. The right hemivagina was collapsed, and both ovaries were normal; a corpus luteum was noted in the left ovary. Coronal T2-weighted images revealed absence of the left kidney, with compensatory hypertrophy of the right kidney ( Fig. 5 ). Fig. 1 Coronal T2-weighted MRI demonstrating uterus didelphys (black arrow) classified as ESHRE/ESGE U3b C2 V2. Fig 1 – Fig. 2 Axial contrast-enhanced abdominal and pelvic CT images showing didelphys uterus(black arrow). Fig 2 – Fig. 3 Coronal T2-weighted MRI showing obstructed left hemivagina (white arrow) Due to Transverse Vaginal Septum. Fig 3 – Fig. 4 MRI features of obstructed left hemivagina with mildly hyperintense hemorrhagic content (*) on T1-weighted image. Fig 4 – Fig. 5 Coronal contrast-enhanced abdominal and pelvic CT image showing distension of the left hemivagina (Arrow) consistent with hematocolpos and left renal agenesis (*) on the Same Side as the vaginal obstruction. Fig 5 – Coronal T2-weighted MRI demonstrating uterus didelphys (black arrow) classified as ESHRE/ESGE U3b C2 V2. Axial contrast-enhanced abdominal and pelvic CT images showing didelphys uterus(black arrow). Coronal T2-weighted MRI showing obstructed left hemivagina (white arrow) Due to Transverse Vaginal Septum. MRI features of obstructed left hemivagina with mildly hyperintense hemorrhagic content (*) on T1-weighted image. Coronal contrast-enhanced abdominal and pelvic CT image showing distension of the left hemivagina (Arrow) consistent with hematocolpos and left renal agenesis (*) on the Same Side as the vaginal obstruction. These findings confirmed the diagnosis of OHVIRA syndrome. The patient underwent complete resection of the obstructing vaginal septum under direct visualization, extending from the external cervical os to the lateral vaginal wall. The procedure allowed effective drainage of the hematocolpos. The postoperative course was uneventful, and at follow-up, the patient reported complete resolution of pelvic pain and leukorrhea. She was counseled on the nature of the syndrome and the importance of ongoing gynecological follow-up, especially in view of future reproductive planning.

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This case highlights a rare presentation of OHVIRA syndrome, characterized by uterine didelphys, an obstructed hemivagina, and ipsilateral renal agenesis. The clinical manifestation of this condition largely depends on whether communication exists between the obstructed Müllerian structures and the contralateral functional tract. MRI remains the gold standard for diagnosing Müllerian anomalies, particularly in evaluating the presence or absence of such communication. Surgical management should be tailored to the specific anatomical findings: while a simple obstructing vaginal septum may be effectively treated with septum resection, more complex cases involving cervical agenesis often require hemi hysterectomy of the affected uterus.

Congenital uterine anomalies affect approximately 5.5% of the general female population, with didelphic uterus accounting for a significant proportion [ 1 ]. The exact etiology of OHVIRA syndrome remains incompletely understood. A prevailing theory suggests that abnormal development of the mesonephric (Wolffian) duct impairs both renal formation and fusion of the Müllerian (paramesonephric) ducts. The mesonephric duct normally serves as a scaffold for caudal migration and fusion of the Müllerian ducts. Its developmental failure may result in ipsilateral renal agenesis, uterine didelphys, and obstructed hemivagina [ 2 , 6 ]. The syndrome is typically diagnosed in adolescence, although delayed diagnosis is not uncommon due to the presence of a functional contralateral hemivagina that allows apparently normal menstruation [ 5 ]. Common presenting features include cyclic pelvic or abdominal pain, hematocolpos, and vaginal discharge. In some cases, diagnosis is delayed until complications such as pelvic infections or endometriosis arise [ 5 ]. A retrospective study by Zhu et al. proposed a classification system for OHVIRA syndrome based on whether there is communication between the obstructed hemivagina and the functional contralateral genital system [ 6 ]. The classification includes: • Class 1: Complete Obstruction—characterized by a blind hemivagina or cervical atresia, leading to significant accumulation of menstrual blood. The average age of diagnosis for this type is 12.3 ± 1.8 years, with patients often presenting with abdominal distension at menarche. • Class 2: Incomplete Obstruction—characterized by a fine communication between the duplicated structures (cervices or vaginas). The average age of diagnosis is around 21 years, and clinical presentation includes irregular menstrual cycles, dysmenorrhea, and complications such as mucopurulent discharge. Class 1: Complete Obstruction—characterized by a blind hemivagina or cervical atresia, leading to significant accumulation of menstrual blood. The average age of diagnosis for this type is 12.3 ± 1.8 years, with patients often presenting with abdominal distension at menarche. Class 2: Incomplete Obstruction—characterized by a fine communication between the duplicated structures (cervices or vaginas). The average age of diagnosis is around 21 years, and clinical presentation includes irregular menstrual cycles, dysmenorrhea, and complications such as mucopurulent discharge. Ultrasound and MRI are crucial in diagnosing OHVIRA syndrome, with MRI offering the highest sensitivity due to its ability to visualize the extent of Müllerian duct anomalies and determine the presence of communication between the 2 Müllerian systems [ 4 ]. Ultrasound, however, remains the first-line diagnostic tool and has been found to have a diagnostic accuracy of 90.9% [ 5 ]. Surgical management is tailored according to the nature of the obstruction. In cases of simple hemivaginal septum obstruction, septum resection is the treatment of choice, while more complex cases, such as those involving cervical agenesis, may require hemi hysterectomy for proper resolution of the obstruction and preservation of fertility [ 7 , 8 ]. Ugurlucan et al. [ 7 ] reported the surgical approach for 28 patients with OHVIRA syndrome, performing vaginal septum resection in 28 cases and hemi hysterectomy in 4. A more recent review by Kudela et al. [ 8 ] reviewed 734 cases and found that 86.5% of patients underwent vaginal septum resection, while 2.2% required hemi hysterectomy, typically in more complex cases.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare and complex congenital anomaly involving the development of both the genitourinary and renal systems. First described in 1922, this condition results from abnormal embryological development of the Müllerian (paramesonephric) and Wolffian (mesonephric) ducts [ 1 , 2 ]. It is classically defined by a triad of anomalies: uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis [ 3 ]. Although OHVIRA syndrome is most frequently diagnosed during adolescence—typically after menarche—advances in imaging modalities have facilitated earlier detection, including during the neonatal and prepubertal periods [ 4 ]. Common presenting symptoms include cyclic pelvic or abdominal pain, dysmenorrhea, and menstrual irregularities. In cases of delayed diagnosis, severe complications such as endometriosis, pelvic infections, or infertility may develop [ 5 ]. Early recognition and accurate diagnosis are critical for preventing these complications and for preserving reproductive potential. This article reviews the embryological basis, clinical presentation, and diagnostic and therapeutic strategies involved in the management of OHVIRA syndrome.