Pelvic pain and a missing kidney: Unveiling OHVIRA syndrome with a tubo-ovarian abscess

A 46-year-old woman with no significant personal or family history, she was born from a non-consanguineous marriage and had her menarche at the age of 17. Her menstrual cycles were regular with no particular incidents with an average of 28 to 30 days with bleeding lasting an average duration of 7 days. The patient is currently divorced after a marriage who last 10 years, but she had never been pregnant. Her past medical history included cholecystectomy a decade prior. She reported secondary amenorrhea for 3 months, she did not give it importance as she confused it with cycle irregularities related to menopause, and did not consider pregnancy due to her marital status. She presented with acute hypogastric pain radiating to the back but denied urinary symptoms or vaginal discharge. Clinical examination revealed a high fever, a palpable hypogastric mass, and tenderness without signs of inflammation Laboratory tests showed hyperleukocytosis with a white blood cell count of 20,000 cells/µL and an elevated CRP level of 145 mg/L, the beta-HCG test was negative, while renal function tests and other blood work were within normal limits. She was admitted with a preliminary diagnosis of left renal obstruction following severe pain and imaging findings suggesting hydronephrosis of ectopic kidney. However, further evaluation with ultrasound and CT revealed that the left kidney was absent ( Fig. 1 ), indicating renal agenesis rather than obstruction. Additionally, a complex multilocular pelvic lesion with a solid component measuring 9 × 8 × 11cm, and significant mesenteric fat stranding was observed ( Fig. 2 ). Given these findings, an MRI was performed to further delineate the anatomy, which revealed a uterus didelphys ( Fig. 3 ) with an obstructed hemivagina filled with blood, consistent with OHVIRA syndrome. A tubo-ovarian abscess was also identified on the same side as the obstructed hemivagina ( Fig. 4 ). Fig. 1 Enhanced CT scan showing in coranal plan (A): Red dotted circle: right kidney VS Yellow dotted circle: showing the absence of the left kidney. Enhanced CT scan in the delayed phase with maximum intensity projection (MIP) reconstruction coronal view: Blue arrows: right ureter. Green arrow: empty bladder with the water filled balloon of the urinary catheter. Black arrow: urinary catheter. Fig 1 – dummy alt text Fig. 2 Enhanced CT scan showing in sagittal plan (A) coronal plan (B) and axial plan (C). Green dotted circle: left Tubo-ovarian abscess. Yellow dotted circle : Hematocolpos. Red doted line and red arrows: left and right uterus bodies. Fig 2 – dummy alt text Fig. 3 Coronal T2 weighted image (A) Axial T2 weighted image (B) Axial T1 weighted image (C) and Axial T2* (D) weighted image showing showing: Red dotted circle: didelph uterus. Blue arrow: hematocolpos. Fig 3 – dummy alt text Fig. 4 Axial enhanced T1 weighted image with fat saturation (A) and non-enhanced axial T1 FSE with fat saturation (B) Axial Diffusion weighted image (C) with ADC map (D) showing: Green star : Tubo-ovarian abscess. Yellow arrow : fat stranding near to the wall of the abscess. Fig 4 – dummy alt text Enhanced CT scan showing in coranal plan (A): Red dotted circle: right kidney VS Yellow dotted circle: showing the absence of the left kidney. Enhanced CT scan in the delayed phase with maximum intensity projection (MIP) reconstruction coronal view: Blue arrows: right ureter. Green arrow: empty bladder with the water filled balloon of the urinary catheter. Black arrow: urinary catheter. Enhanced CT scan showing in sagittal plan (A) coronal plan (B) and axial plan (C). Green dotted circle: left Tubo-ovarian abscess. Yellow dotted circle : Hematocolpos. Red doted line and red arrows: left and right uterus bodies. Coronal T2 weighted image (A) Axial T2 weighted image (B) Axial T1 weighted image (C) and Axial T2* (D) weighted image showing showing: Red dotted circle: didelph uterus. Blue arrow: hematocolpos. Axial enhanced T1 weighted image with fat saturation (A) and non-enhanced axial T1 FSE with fat saturation (B) Axial Diffusion weighted image (C) with ADC map (D) showing: Green star : Tubo-ovarian abscess. Yellow arrow : fat stranding near to the wall of the abscess. Initial management included broad-spectrum antibiotics to control the infection. The patient subsequently underwent surgical intervention via a median laparotomy, which confirmed the MRI findings and revealed a giant left tubo-ovarian abscess with tubo-ovarian adhesions, associated with left hemivagina distally closed by a septum, featuring a small drainage tract complicated by hematocolpos. These findings classify the syndrome as type U3bC2V1. The patient recovered well and was followed postoperatively for fertility preservation counselling. Secondary amenorrhea in this case likely indicates progression of the obstructed hemivagina, leading to complications such as tubo-ovarian abscess. Its recognition is crucial, as it prompts timely imaging and may necessitate surgical intervention to relieve the obstruction and prevent further adverse outcomes.

Written informed consent was obtained from the patient for the writing and publication of this article and accompanying images.

OHVIRA syndrome remains a diagnostic challenge, especially in atypical presentations beyond adolescence [ [3] , [11] ]. With the growing accessibility of imaging, OHVIRA syndrome is increasingly being diagnosed in women prior to conception [ 12 ]. This case highlights the indispensable role of imaging, particularly MRI as the gold standard in accurately identifying Müllerian anomalies [ 5 ]. Early diagnosis is essential to enable timely surgical management, which involves complete resection of the vaginal septum to ensure effective menstrual drainage and prevent complications such as fibrosis and vaginal stenosis [ [7] , [11] ]. A clear understanding of the reproductive outcomes linked to this condition is essential for healthcare providers to effectively counsel and manage affected patients [ [9] , [12] ]. Radiologists, urologists, and gynecologists should maintain a high index of suspicion when evaluating complex pelvic pathologies and collaborate closely in a multidisciplinary approach [ 11 ]. It is essential that healthcare providers prioritize early imaging in patients with unexplained pelvic pain and ipsilateral renal anomalies [ [2] , [10] ], facilitate prompt surgical referral, and engage in long-term follow-up to monitor reproductive and renal outcomes, thereby optimizing patient care and preventing serious sequelae [ [11] , [12] ].

OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal Anomaly) is a rare congenital Müllerian duct anomaly with an estimated prevalence ranging from 0.16% to 10% [ [1] , [11] ]. It arises from developmental defects involving both the Wolffian (mesonephric) and Müllerian (paramesonephric) ducts [ [6] , [8] ]. The Wolffian ducts, which form the kidneys, also serve as inductive structures necessary for the proper fusion of the Müllerian ducts [ 8 ]. A developmental anomaly affecting the caudal segment of 1 Wolffian duct may result in unilateral renal agenesis alongside an obstructed hemivagina [ 5 ]. Renal agenesis is the most frequent non-genital anomaly and consistently occurs ipsilateral to the vaginal obstruction [ [4] , [7] ]. Other associated renal anomalies may include renal dysplasia and ectopic ureters [ 5 ]. Most cases are diagnosed in adolescence due to cyclic pain and hematocolpos [ [5] , [11] ], but delayed recognition in adulthood can lead to severe complications such as chronic pelvic pain, endometriosis, and infections [ [3] , [12] ]. In this case, the patient’s pain was initially attributed to renal obstruction, a common misdiagnosis when renal anomalies are present. The absence of the left kidney was an early clue that led to further imaging, which ultimately revealed the Müllerian anomaly [ [2] , [10] ]. This highlights the importance of considering congenital reproductive tract anomalies when evaluating pelvic pain in patients with renal malformations. Imaging plays a pivotal role in diagnosis. Ultrasound is typically the first-line modality but may be limited in delineating complex anatomy [ 5 ]. Computed tomography (CT) aids in assessing renal anomalies and pelvic masses but lacks specificity for Müllerian anomalies. MRI remains the gold standard, providing superior soft tissue resolution to confirm uterine duplication, localize vaginal obstruction, and identify complications such as hematocolpos, tubo-ovarian abscess, endometriosis, and infertility [ [3] , [5] ]. Differential diagnoses for this patient included pelvic inflammatory disease, complicated ovarian cyst [ 3 ]. However, the combination of renal agenesis, a didelphys uterus, and a fluid-filled hemivagina on MRI confirmed OHVIRA syndrome [ 5 ]. This case illustrates how delayed diagnosis can lead to infectious complications, requiring both medical and surgical management [ [1] , [11] ]. The standard treatment involves surgical excision of the obstructing vaginal septum to restore vaginal continuity, relieve symptoms, and prevent sequelae such as endometriosis caused by retrograde menstrual flow [ [7] , [9] ]. Surgery is generally performed around puberty, after the development of secondary sexual characteristics, but earlier intervention may be necessary in cases complicated by pelvic inflammatory disease, abscess, or significant hematocolpos [ 11 ]. Surgical approaches include single-stage vaginoplasty, which combines drainage and septum resection in 1 procedure, and a 2-stage method, where hematocolpos is drained first, followed by definitive resection [ [7] , [11] ]. While single-stage surgery is generally preferred due to its simplicity and favorable outcomes, the 2-stage method may be indicated in cases of active infection, anatomical distortion, or incomplete prior interventions. Less invasive techniques, such as hysteroscopic resection under transabdominal ultrasound guidance, have also been described, particularly when hymenal preservation is desired. A retrospective study by Smith and Laufer of 27 patients reported 26 vaginal reconstructions, with 6 patients requiring 2-stage procedures due to infection or restenosis. Although complications are uncommon, reported adverse events include vesicovaginal fistula, bladder injury, and recurrent stenosis, which may necessitate additional interventions such as vaginal or cervical dilatation or repeat vaginoplasty. These findings highlight the importance of individualized treatment plans considering anatomy, clinical presentation, and reproductive goals. Following successful surgery, most patients achieve normal sexual function, and many conceive and carry pregnancies to term [ [9] , [12] ]. Hemihysterectomy, formerly considered as a treatment option, is now discouraged as studies demonstrate comparable pregnancy potential in both uterine horns [ 12 ]. Early recognition of OHVIRA syndrome is also crucial to avoid long-term sequelae including infertility, spontaneous abortion, and tubo-ovarian abscesses [ 12 ]. Tiago et al. reported a prenatal diagnosis of OHVIRA syndrome at 36 weeks’ gestation based on findings of right renal agenesis and hydrocolpos, underscoring the value of thorough prenatal ultrasound examination. The detection of a solitary kidney and/or pelvic mass during prenatal imaging should prompt consideration of Herlyn-Werner-Wunderlich syndrome to facilitate timely postnatal management and improved outcomes [ 6 ]. Despite its rarity, OHVIRA syndrome has significant multidimensional impacts on patients’ quality of life if untreated or diagnosed late. Chronic pelvic pain, recurrent genital infections, infertility, and psychological distress are common and impose considerable physical and emotional burdens. Delayed diagnosis increases the risk of retrograde menstruation leading to endometriosis and further fertility impairment. Gabriele et al, in a retrospective cohort of 28 patients, identified cases of endometriosis and renal dysfunction during a median follow-up of 3.5 years, despite most patients remaining asymptomatic postoperatively [ 11 ]. Other cohort studies report that up to 4% of patients experience long-term renal impairment associated with their solitary functional kidney, emphasizing the necessity of ongoing nephrological surveillance. Furthermore, Gholoum et al. stressed that early detection and prompt surgical management are key to reducing complications and optimizing reproductive and overall health outcomes [ 7 ]. Given the complex nature of OHVIRA syndrome, a multidisciplinary, long-term care approach is warranted, encompassing not only surgical correction but also renal monitoring, pain management, fertility counseling, and psychosocial support to ensure holistic well-being throughout the patient’s life course. Early diagnosis of OHVIRA syndrome through thorough clinical and radiological evaluation is essential to prevent serious complications such as infertility, chronic pelvic pain, and infections. Gynecologists, urologists, and radiologists should maintain a high index of suspicion and promote multidisciplinary management, tailoring surgical treatment to each patient’s timing, anatomy, and reproductive goals to optimize outcomes and reduce the risk of long-term complications and improve patient’s quality of life [ [11] , [12] ].

Herlyn-Werner-Wunderlich syndrome (HWW) or obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, is a rare congenital disorder resulting from abnormal development of the Müllerian and Wolffian ducts [ [1] , [5] ]. Initially reported in 1922, this condition arises from a disruption in the embryological development of the paramesonephric (Müllerian) and mesonephric (Wolffian) ducts [ [6] , [8] ]. Obstructive Müllerian anomalies are estimated to occur in approximately 0.1% to 3.8% of the general population [ [1] , [11] ]. It manifests with a triad of uterus didelphys, obstructed hemivagina, and renal agenesis on the same side as the obstructed structures [ [2] , [10] ]. OHVIRA syndrome is due to abnormal development of the Müllerian and Wolffian ducts. A defect in the Wolffian duct leads to ipsilateral renal agenesis and disrupts the normal fusion of the Müllerian ducts, resulting in a uterus didelphys [ 8 ]. The displaced Müllerian duct fails to connect with the urogenital sinus, forming an obstructed hemivagina [ [5] , [8] ]. Renal agenesis consistently occurs on the same side as the vaginal obstruction and is always seen on the side with the obstructed hemivagina [ [4] , [7] ]. Although this condition is typically diagnosed in adolescence due to cyclic pelvic pain, delayed presentations in adulthood are rare but carry significant morbidity [ [3] , [11] ]. This case describes a 46-year-old woman who presented with acute hypogastric pain and was initially admitted for suspected renal obstruction. Further radiological evaluation revealed left renal agenesis and a tubo-ovarian abscess related to a non-previously diagnosed OHVIRA syndrome. This report underscores the pivotal role of imaging, particularly Magnetic resonance imaging (MRI), in differentiating complex pelvic pathologies [ 5 ] and underscores the need for early recognition to prevent complications such as endometriosis and infertility [ [9] , [12] ].