Co-existing obstructed and refluxing lower moiety megaureter in a female without incontinence: a perplexing myriad in a duplex system with ectopic lower moiety ureter

Abstract

Ectopic ureter is a rare congenital anomaly that predominantly affects females and typically manifests as urinary incontinence due to atypical ureteral openings in structures such as the vagina or cervix. This condition is estimated to occur in approximately 1 in 4000-10,000 female births. An exceptional variant, the intrasphincteric ectopic ureter, poses significant diagnostic challenges due to its atypical presentation, as it may cause dynamic obstruction and reflux phenomena without resulting in incontinence. A 28-year-old female presented with severe left flank pain, fever, and chills persisting for 10 days. Her medical history included intermittent dull, aching left flank pain over 7 years, exacerbated during voiding, but no urinary incontinence. Physical examination revealed marked tenderness in the left flank, and laboratory investigations showed elevated leukocyte count and normal serum creatinine levels. Computed tomography urography revealed a left duplex moiety with upper moiety infected hydronephrosis, necessitating percutaneous nephrostomy placement. Subsequent nephrostogram and micturating cystourethrogram identified a grossly dilated and tortuous upper moiety ureter with intrasphincteric ectopic ureteral opening, confirming the diagnosis of obstructed and refluxing megaureter. Following infection resolution, the patient underwent laparoscopic extravesical non-refluxing reimplantation of the upper moiety ectopic ureter with DJ stenting. The postoperative course was uneventful, and the patient reported no flank pain or fever upon stent removal. Periodic evaluations showed no increase in hydronephrosis, indicating a successful intervention. This case underscores the diagnostic complexity of intrasphincteric ectopic ureter, a rare anomaly presenting with obstructed and refluxing megaureter without incontinence. Heightened clinical vigilance and comprehensive radiological evaluation are imperative for timely diagnosis and intervention. Preservation of continence further complicates the clinical picture, necessitating early intervention to prevent renal function deterioration and improve long-term outcomes.