Duplex collecting systems are the most common congenital renal anomaly, affecting approximately 0.8% of the population1. Ectopic ureters are rarer (0.025–0.05%) but are associated with duplex systems in up to 80% of cases2, predominantly involving the upper pole moiety according to the Weigert-Meyer rule, whereby the upper pole ureter inserts medially and inferiorly and frequently drains ectopically into the vagina, urethra, or vestibule in females. Embryological maldevelopment of the ureteric bud leads to ectopic insertion, obstruction, hydronephrosis, and continuous urinary leakage (Figure 1). In females, vaginal drainage commonly manifests as persistent incontinence, often misdiagnosed as neurogenic bladder, vaginal discharge, or fistula—especially in adulthood, where nearly 30% of cases are diagnosed after age 183.
Standard treatment for poorly functioning moieties is surgical excision, with robotic-assisted laparoscopic heminephroureterectomy increasingly preferred over open or laparoscopic approaches due to enhanced precision, minimized blood loss, decreased hospitalization duration, and minimal complications4,5. Indocyanine green (ICG) fluorescence further improves vascular identification and tissue preservation6,7. This article is suitable for specialists who treat uncommon congenital conditions in intricate cases.
Case Presentation:
A 41-year-old female, wheelchair-dependent due to progressive relapsing-remitting multiple sclerosis (EDSS 6.5) and bilateral hip arthroplasties, presented with continuous vaginal urinary leakage, recurrent urinary tract infections (multidrug-resistant Escherichia coli), intermittent lower abdominal pain, and episodic fever (up to 38.5 °C). Symptoms were initially attributed to neurogenic bladder secondary to multiple sclerosis, leading to initiation of intermittent self-catheterization and reliance on incontinence pads. This conservative approach exacerbated social withdrawal, psychological distress, and recurrent infections. Diagnostic evaluation included contrast-enhanced CT urography demonstrating a right duplex kidney with severe hydronephrosis of a non-functioning upper pole moiety and a tortuous, dilated ectopic megaureter (30 mm) inserting into the vaginal vault (Figure 2), retrograde pyelography confirming the ectopic insertion, and MAG3 scintigraphy verifying 0% differential function of the upper pole (preserved lower pole function: 33% of the total renal function). Percutaneous nephrostomy placement achieved decompression of hydronephrosis over four weeks.
Diagnosis, Assessment, Plan:
Right-sided duplex kidney with vaginal ectopic ureter from a non-functioning upper pole moiety causing continuous incontinence and recurrent pyelonephritis. Differential diagnosis included neurogenic bladder dysfunction, vesicovaginal fistula, or single-system ectopic ureter. Multimodal imaging (CT urography, retrograde pyelography, MAG3 scintigraphy) showing obstruction, megaureter, absent upper pole function, and vaginal insertion bypassing sphincter control. Initial temporary diversion with percutaneous nephrostomy and targeted antibiotics to resolve infection and inflammation, followed by definitive robot-assisted upper pole heminephroureterectomy. The robotic approach was selected for superior visualization and precision. ICG fluorescence was planned to safeguard the lower pole vascular supply. Anticipated complications (bleeding, lower pole ischemia, infection recurrence) were minimized through fluorescence-guided dissection and an experienced robotic team.