Cross-fused dystopia of the right kidney with cystic dysplasia of the non-functioning left kidney associated with an ureterocele

Abstract

Cross-dystopia is a rare type of congenital anomaly and is characterized by a displacement of the kidney to the opposite side; as a result, they are both located on the same side, and in approximately 85% of such cases, a fusion of the parenchyma of two kidneys can be observed. We have not found reports of cases in which a nonfunctioning orthotopic cystic dysplastic kidney associated with ureterovesical segment pathology (ureterocele) was fused with the lower pole of a cross-dystopian normally formed kidney in the literature. The patient, 8 days old, had a preliminary diagnosis of agenesis of the right kidney, doubling of the left kidney, cystic dysplasia, doubled left kidney with an ureterohydronephrosis in the lower half and ureterocele. To restore the urine outflow and function of the lower half of the presumably doubled left kidney, a cystourethroscopy was performed. In a typical place on the right, there was a correctly formed ureteral orifice, i.e., an ureterocele on the left, which occupies half of the bladder volume. With a holmium laser, an artificial orifice was formed in the ureterocele to restore the urine passage. At 9 months old, tomography urinary tract was performed, which revealed cross-dystopia of the right kidney with a fusion of the lower pole and a cystic dysplastic orthotopic left kidney. Laparoscopic nephroureterectomy of a non-functioning orthotopic kidney was performed.

The control examination indicated complete clinical remission and social adaptation of the patient after discharge. Cross-dystopia of the kidney with fusion, cystic dysplastic kidney, and a ureterocele are rare congenital anomalies that require timely examination in a specialized clinic and drawing up an individual treatment plan. The surgical method should focus on a symptomatic urological problem, emphasizing preserving kidney function.

Keywords

urology / cross-dystopia / cystic dysplasia / ureterocele / nephroureterectomy / children

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