Long-term results of treatment of single-system ectopic ureters

Single-system ureteral ectopia (UE) encompasses a spectrum of malformations involving the bladder trigone, ureter, and kidney. The clinical presentati on is variable, and both diagnostic and therapeutic problems are common. Re duced renal function in these patients may result from primary dysplasia, o bstruction, vesicoureteral reflux, or recurrent infection. Based on our exp erience of seven patients, suggestions for diagnostic procedures and criter ia for renal saving versus nephrectomy are offered. The relationship betwee n ostium localization, renal function, and longterm results was investigate d. From 1972 to 1990, five female and two male patients were studied. Durin g the same period, 31 patients with UE and duplex kidneys were seen. Ages r anged from 1 day to 7 years. A ureteric opening into the bladder neck was a ssociated with dilatation of the ureter and renal pelvis. Two patients had vaginal ectopia and severe renal dysplasia. In one, a cyst of the vaginal w all (Gardner's cyst) was detected at birth. A male newborn had multicystic renal dysplasia on the left and ureteric ectopia to the ductus deferens on the right side. To our knowledge, he is the first patient reported with ren al function totally dependent on a kidney with severe UE. Follow-up ranged from 4 to 9 years. One patient died in the postoperative period because of renal failure and sepsis. All the others are well and have normal creatinin e values. Improvement of renal function was noted after ureteral reimplanta tion (URI) in patients with bladder-neck ectopia. The numbers of infections were also drastically reduced. Our observations suggest that the combinati on of ultrasound, cyst urethrography, and cystoscopy will be diagnostic in most patients. A suspicion of UE should be raised in symptomatic patients w ith apparently solitary kidneys, enuresis ureterica, or atypical obstructiv e uropathy. Reduced renal function in some patients with ectopia to the bla dder neck will improve after URI. This may be of importance in patients wit h bilateral anomalies and marginal renal function.