Obstructive uropathy in a female infant with a single kidney: Unmasking congenital urethral stenosis: A case report and review of the literature

Abstract

Congenital urethral stenosis (CUS) in female infants is an exceptionally rare urological anomaly. When combined with vesicoureteral reflux (VUR) and a solitary functional kidney, it presents a significant risk for renal deterioration. Early recognition is essential to avoid irreversible damage, especially in complex cases with multiple comorbidities. We report the case of a 33-month-old female born prematurely at 26 weeks, with a history of omphalocele repair, ventriculitis, retinopathy of prematurity, and a chromosomal abnormality. She presented with recurrent febrile urinary tract infections and worsening hydronephrosis of her only functional kidney. Multiple catheterization attempts failed due to a pinhole-sized urethral meatus. Examination under anesthesia revealed congenital urethral stenosis, which was managed with serial dilations allowing catheter placement and voiding cystourethrogram (VCUG). Imaging showed a trabeculated bladder with diverticula, grade V VUR, and laterally displaced ureteral orifice. Due to persistent infections and poor compliance with catheterization, a vesicostomy was performed. The patient subsequently remained infection-free, with improved renal function and resolution of hydronephrosis. This case highlights the diagnostic challenge posed by CUS in females, particularly in the context of solitary kidney and developmental delay. The absence of obvious obstructive symptoms may delay diagnosis. In such complex scenarios, vesicostomy provides effective bladder drainage, protects upper tract function, and simplifies care when clean intermittent catheterization is not feasible. CUS should be included in the differential diagnosis of bladder outlet obstruction in female infants, particularly those with recurrent UTIs and solitary kidney. In carefully selected patients, vesicostomy remains a valuable interim or long-term solution to preserve renal function and improve quality of life . • Congenital urethral stenosis (CUS) in females is extremely rare but clinically significant, often presenting with obstructive voiding symptoms and recurrent UTIs. • Clinical presentation may include weak stream, straining, dribbling, enuresis, and recurrent UTIs—often mistaken for functional disorders in early childhood. • Severe CUS can lead to serious complications like vesicoureteral reflux (VUR), hydronephrosis, and even end-stage renal disease, especially in patients with a solitary kidney. • Diagnosis remains challenging and often requires examination under anesthesia, urethroscopy, urethral calibration, and imaging studies like VCUG. • Treatment strategies include urethral dilation, meatotomy, and in refractory cases, urethrotomy or meatoplasty. Outcomes vary, and risk of restenosis exists.