Objective: To document the array of diagnostic modalities, the variety of errant diagnoses and treatments. and the time from initial presentation to ultimate diagnosis in girls with an ectopic single-system ureter draining an ectopic hypoplastic and/or dysplastic kidney.
Patients and methods: Between 1990 and 1997, seven females were identified who had an ectopic hypoplastic and/or dysplastic kidney with an ectopically draining ureter, and who were treated at our institutions, either initially or upon referral. The nature and number of all diagnostic evaluations, previous diagnoses and treatments, and the time from presentation to definitive diagnosis were recorded.
Results: All seven females had a classical history of successful toilet training, a normal voiding pattern and continuous urinary incontinence. Typically, a solitary kidney was noted on the initial diagnostic evaluation by ultrasonography and/or intravenous urography. The mean (range) age at initial presentation was 3.2 (2-6) years. Additionally, voiding cysto-urethrography, urodynamics, radionuclide scintigraphy, computed tomography, magnetic resonance imaging and endoscopy were performed. The age at definitive diagnosis was 3-16.5 years and the mean (range) time from initial presentation to diagnosis was 5.7 (1-10) years. Nephroureterectomy was curative and all kidneys were dysplastic.
Conclusion: Continuous urinary incontinence in females with a normal voiding pattern should prompt an evaluation for ureteric ectopia. When the initial evaluation yields the diagnosis of a solitary kidney, clinicians should be aware of the possibility of a hypoplastic and/or dysplastic, often ectopic, contralateral kidney with an ectopically draining ureter. Identification of this entity should allow curative surgical treatment.