Vaginal insertion of ureter with uterus didelphys and ipsilateral renal agenesis: A case report

A 45-year-old woman was referred to gynecology for surgical consultation regarding a complex left ovarian mass. Past medical history was notable for uterus didelphys (2 separate uteruses and cervices connected to a single vagina), congenital absence of the right kidney and ureter, endometriosis, and thalassemia. She had progressive abdominal discomfort and sharp pain in her lower left quadrant for 1 month before the pain began to radiate to her left flank and thigh. Abdominopelvic CT revealed a left adnexal lesion ( Fig. 1 ). Subsequent pelvic ultrasound (images not available for review) revealed a complex left adnexal mass concerning for ovarian malignancy. The patient began menarche at age 12 and had a history of dysmenorrhea with severe menstrual bleeding, which was managed with hormonal therapies including Depo Provera injections and OTC agents until 2018. She requested surgical consultation for a hysterectomy for cessation of menstrual symptoms and left salpingo-oophorectomy for removal of the ovarian mass. A pre-operative abdominopelvic MRI with and without contrast was performed which confirmed the presence of a T1 and T2 hyperintense left adnexal cystic lesion measuring 10.5 × 9.7 × 10.4 cm and left hydrosalpinx measuring 2.9 × 2.2 × 10 cm ( Fig. 3 , Fig. 5 ). A longitudinal vaginal septum was visualized as well as a small tubular structure inserting into the right vaginal fornix with concern raised for ectopic insertion of the right ureter ( Fig. 2 ). Uterus didelphys was also confirmed at the time of MRI ( Fig. 4 ). Fig. 1 Axial (A) and coronal (B) contrast-enhanced CT images of the abdomen and pelvis demonstrating a hypodense left adnexal mass (arrow). Congenital absence of the right kidney is also noted with elongation of the right adrenal gland (arrowheads).  Fig 1 dummy alt text Fig. 2 Coronal T2-weighted MR image of the pelvis demonstrating duplication of the cervix (arrowheads). A tubular structure is seen inserting in the right vaginal fornix (thin arrow), suspected right ureteral remnant. The patient’s left hydrosalpinx (thick arrow) is partially visualized. Fig 2 dummy alt text Fig. 3 Axial T2-weighted images of the pelvis demonstrating a curved, tubular structure posterior to the right ovary (arrow), terminating in the right lateral aspect of the upper vagina. Left adnexal mass (asterisk) and left hydrosalpinx (arrowhead) are present. Fig 3 dummy alt text Fig. 4 Axial T2-weighted image of the pelvis demonstrating uterus didelphys (asterisks). Fig 4 dummy alt text Fig. 5 Axial T1 noncontrast (A), T1 postcontrast (B), diffusion-weighted (C) and apparent diffusion coefficient map (D) images of the pelvis at the level of the left adnexal mass. Lesion is intrinsically T1 hyperintense with minimal peripheral enhancement and low-level diffusion restriction within the dependent portion, possibly within a small amount of retracted blood products (arrow). Fig 5 dummy alt text Axial (A) and coronal (B) contrast-enhanced CT images of the abdomen and pelvis demonstrating a hypodense left adnexal mass (arrow). Congenital absence of the right kidney is also noted with elongation of the right adrenal gland (arrowheads). Coronal T2-weighted MR image of the pelvis demonstrating duplication of the cervix (arrowheads). A tubular structure is seen inserting in the right vaginal fornix (thin arrow), suspected right ureteral remnant. The patient’s left hydrosalpinx (thick arrow) is partially visualized. Axial T2-weighted images of the pelvis demonstrating a curved, tubular structure posterior to the right ovary (arrow), terminating in the right lateral aspect of the upper vagina. Left adnexal mass (asterisk) and left hydrosalpinx (arrowhead) are present. Axial T2-weighted image of the pelvis demonstrating uterus didelphys (asterisks). Axial T1 noncontrast (A), T1 postcontrast (B), diffusion-weighted (C) and apparent diffusion coefficient map (D) images of the pelvis at the level of the left adnexal mass. Lesion is intrinsically T1 hyperintense with minimal peripheral enhancement and low-level diffusion restriction within the dependent portion, possibly within a small amount of retracted blood products (arrow). Diagnostic laparoscopy revealed a complex mass adherent to the left pelvic sidewall, the left ureter, and numerous retroperitoneal vessels. Extensive adhesiolysis was performed. The left retroperitoneal space was opened to identify the left ureter and infundibulopelvic ligament for resection of the mass. A modified radical hysterectomy was carefully performed to remove the adhered complex ovarian mass alongside the aberrant uterine vasculature. The mass was removed from the external and internal iliac vessels and left ureter. In addition, another 35 minutes was spent removing the ovarian mass from the left pelvic sidewall. Due to the unique vasculature surrounding the right uterus and uterine horn, the anterior limb of the right internal iliac and right uterine vessels were ligated to reduce the risk of bleeding. Both fallopian tubes and ovaries were removed as well as a portion of the upper vagina. Surgical pathology of removed left adnexal mass was negative for malignancy and confirmed to be an endometriotic cyst with reactive epithelial atypia. The left fallopian tube demonstrated tubo-ovarian adhesions. Both uteri were also negative for malignancy. No remnant right ureter was identified during surgery, however the right retroperitoneum was not explored. The patient tolerated the procedure well and recovered with minimal complications.

The patient reported in the manuscript signed the informed consent/authorization for participation in research which includes the permission to use data collected in future research projects including presented case details and images used in this manuscript.

This case reiterates the importance of preoperative imaging in patients with complex urogenital anomalies, specifically uterus didelphys, a rare congenital urogenital anomaly which may include accompanying abnormalities of the urinary tract including renal agenesis and ectopic ureteral insertion.

This case underscores the importance of pre-operative imaging in patients with complex genitourinary anatomy. Uterus didelphys represents approximately 11% of identified uterine anomalies and has an incidence of under 0.5% in the general population of female individuals [ 5 ]. Vaginal insertion of the ureter (also known as vaginal ectopic ureter) refers to another uncommon congenital anomaly where at least one ureter drains into the vagina instead of the bladder, occurring in approximately 0.05% of female individuals [ 6 ]. Due to the combined rarity of both conditions, vaginal insertion of ureter with uterus didelphys is extremely rare with unquantifiable incidence. Vaginal insertion of the ureter results from a deviant lateral origin of the ureteric bud from the mesonephric duct during the third to fourth week of embryogenesis [ 7 ]. A triad of associated developmental pathologies includes: the ureteric bud failing to separate and thus failing to migrate to the bladder, persistence of the distal mesonephric duct (also known as Garter’s duct) into the vaginal canal leading to an alternate pathway for urinary drainage, and renal dysplasia which includes maldevelopment or absence of the ipsilateral kidney [ 8 ]. Symptomatically, patients with vaginal insertion of the ureter typically present with lifelong, continuous urinary incontinence with normal micturition pattern [ 9 ]. However, atypical presentation without urinary incontinence has been previously documented as well [ 10 ]. In the case of this patient, it is likely that the triad of associated pathologies manifested and she remained asymptomatic as the upper portion of her right ureter and ipsilateral kidney failed to develop. In addition, due to the presence of an unobstructed hemivagina, Obstructed Hemivaginal and Ipsilateral Renal Anomaly (OHVIRA) Syndrome is ruled out. Pelvic ultrasonography is the first line imaging modality for patients with dysmenorrhea and menorrhagia. In patients with uterus didelphys, 2 separate uteri with endometrial echoes, 2 cervixes, and either 1 or 2 vaginas will be visualized [ 11 ]. In cases of equivocal uterine anatomy, pelvic MRI is recommended as a next step [ 12 ]. The challenges associated with diagnosis of uterus didelphys through imaging includes limitations of various imaging modalities and the clinician’s experience [ 13 ]. In the case of this patient, pelvic imaging completed years prior reported a bicornuate uterus instead of uterus didelphys, with the latter confirmed at the time of MRI and surgery. In pre-pubertal patients, small size of the uterus may limit evaluation of uterine anatomy [ 14 ]. In patients with known uterus didelphys or other Müllerian anatomical variants, renal imaging is recommended as a follow-up due to the developmental relationship between the urogenital tracts [ 15 ].

During the preparation of this work, the author(s) used OpenEvidence for assisting with review of the existing literature. The author(s) reviewed and edited the output as needed and take full responsibility for the content of the published article.

Amongst the different variations of Müllerian anomalies, uterine didelphys refers to a rare anatomical variant where the Müllerian ducts fail to fuse completely, resulting in the presence of a duplicate uterine horn, cervix, and vagina or longitudinal vaginal septum [ 1 ]. Since most uterine didelphys cases are asymptomatic, it is usually detected as an incidental finding [ 2 ]. However, when symptoms do occur, they tend to stem from associated anatomical variants such as a longitudinal vaginal septum, which may cause dyspareunia, dysmenorrhea, irregular menstrual bleeding, and lower abdominal pain [ 3 ]. In addition, due to the reciprocal relationship between the mesonephric (Wolffian) and paramesonephric (Müllerian ducts during embryological development, up to 40% of patients with Müllerian anomalies may also have related renal or ureteral anomalies [ 4 ]. We present a patient with vaginal insertion of a remanent ureter with uterus didelphys without urinary incontinence and with absence of the ipsilateral kidney.

Generally surgical intervention is not required solely in the basis of uterus didelphys with or without vaginal insertion of ureter, especially if the patient does not report urinary incontinence or other genitourinary symptoms. However, it is imperative that pre-operative imaging is performed prior to surgical intervention, such as when performing hysterectomy, due to the high prevalence of associated anatomical anomalies and the primary anatomical variation itself. A few special considerations include confirmation of aberrant ureteral course which may manifest as insertions into the reproductive organs of the pelvic cavity (such as the vaginal, cervix, or uterus) and a duplicated vascular supply (due to duplicated uteri) [ 16 ]. Intra-operative cystoscopy and ultrasound with Doppler assessment of ureteral jets are also suggested to confirm ureteral patency [ 17 ]. In cases of ureteral re-implantation with ectopic ureter and preserved renal function, the objective is to redirect the ectopic ureter(s) into the bladder. With cases of renal anomalies, pre-operative imaging of the urinary tract with thin slice MR urography may be of value [ 18 ]. When MR urography cannot be completed, intravenous ureteropyelgram (IVP) can be utilized to determine the anatomy of urinary tracts [ 19 ]. Two common surgical approaches include intravesical and extravesical implantation. In the former, an anterior cystotomy procedure is done to open the bladder. The ectopic ureter is then mobilized intravesically and drawn through a submucosal tunnel that has been created across the trigone [ 20 ]. Another technique, extravesical reimplantation, includes extravesical ureteral dissection and spatulation to suture the ectopic ureter to an incision on the posterolateral bladder [ 21 ]. Nonsurgical interventions for managing dysmenorrhea and menstrual bleeding associated with uterus didelphys with longitudinal vaginal septum include options for menstrual suppression such as continuous combination oral contraceptive pills, progesterone only pills, injections, and gonadotropin-releasing hormone agonists [ 22 ]. In this case the patient underwent surgery due to menstrual symptoms as well as presence of the adnexal mass.