Congenital Uro-Nephropathy: Beyond the Urinary Tract

Background: The developing urinary and reproductive tracts are closely related due to the intricate interaction between mesonephric and paramesonephric ducts. Given to this embryologic association of both systems, disorders observed in one may be a harbinger for an anomaly of the other.

Case Presentation Summary: We report the case of a girl with a suspected prenatal left polycystic kidney that turned out to be a left ureteral duplication with functional exclusion of the upper left kidney’s pole and a left ureterocele in the neonatal period. Uro-nephrologic family history was also present. A left uretero-heminefrectomy was performed 6 months after birth. After the surgery, several urinary tract infections were diagnosed, although the urinary tract imaging tests didn’t show anomalies that could explain it. All the episodes were associated with abnormal vaginal discharge. At three years old, the patient was admitted with vulvar pain and foul-smelling vaginal discharge for three days. Fever and urinary symptoms were denied. On admission, a vulvar erythema and a vaginal introitum swelling were observed. Urinary tract infection was excluded. Pelvic ultrasound showed a bifid uterus with a slightly distended left cavity, and two vaginal canals, one of them distended by vaginal discharge. For further detailed anatomic assessment, a pelvic MRI was performed, revealing complete bicornis bicollis uterus, with uterine laps’ fusion, and vaginal canal duplicity, which corresponds to an U3bC2V2 congenital gynaecological disorder according to the European Society of Human Reproduction and Embryology and European Society for Gynaecological Endoscopy Classification. No invasive treatment was performed.

Discussion: Bicornis bicollis uterus with associated vaginal canal duplicity is a rare congenital disorder that can be related to several urinary tract abnormalities. This case emphasizes the importance of ruling out reproductive tract disorders in patients with congenital urinary tract abnormalities, which should be ideally done in neonatal period, in order to avoid possible future complications.