Introduction:
Congenital urethral stenosis (CUS) in female infants is an exceptionally rare urological anomaly. Its occurrence in conjunction with vesicoureteral reflux (VUR) and solitary functional kidney poses significant diagnostic and therapeutic challenges. Prompt identification and management are crucial to safeguard renal function in such complex cases.
Case presentation:
We describe the case of a 33-month-old girl, born at 26 weeks’ gestation, with a complex medical history including omphalocele repair, ventriculitis, retinopathy of prematurity, and a chromosomal anomaly. She experienced recurrent urinary tract infections (UTIs) and progressive hydronephrosis affecting her only functional kidney. Catheterization attempts failed due to an abnormally narrowed urethral meatus. Examination under anesthesia confirmed congenital urethral stenosis. Urethral dilation permitted catheter insertion and voiding cystourethrogram (VCUG), which revealed a diverticular bladder, high-grade VUR, and ectopic ureteral insertion. Owing to persistent UTIs and difficulty performing clean intermittent catheterization, a vesicostomy was performed, leading to clinical stabilization, UTI resolution, and improved hydronephrosis and renal function.
Conclusion:
This case emphasizes the importance of considering urethral stenosis in the differential diagnosis of bladder outlet obstruction in female infants. For patients with a solitary kidney and neurodevelopmental challenges, individualized surgical approaches such as vesicostomy may offer an optimal strategy for preserving renal function.
Keywords: Urology, Child, Urethral obstruction, Vesicoureteral Reflux, Vesicostomy
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