Challenges in the management of bilateral single-system ectopic ureters in male infants

To describe our experience of managing bilateral single-system ectopic ureters in boys.

We discuss difficulties in diagnosis, importance of appropriate preoperative imaging, and the individualized surgical management for this rare congenital malformation.

The first patient aged 3 months presented with urosepsis-subsequent imaging demonstrated bilateral single-system ectopic ureters, which were reimplanted at age 7 months. The second case was an antenatal diagnosis of bilateral hydronephrosis. Postnatal imaging identified bilateral ectopic single-system ureters. A suprapubic catheter was inserted to ensure optimal bladder drainage. He underwent a Cohen reimplantation at age 7 months because of recurrent urinary tract infections. The third patient was born with a duodenal atresia (repaired in the neonatal period) and a complex anorectal malformation, including a pouch colon with a colourethral fistula, a dilated, ectopic left ureter, and a hypoplastic urethra. He was initially managed with a colostomy and suprapubic catheter. At age 14 months, he underwent a left nephrouretectomy for a nonfunctioning kidney and posterior sagittal anorectoplasty. The right ureter was noted to be ectopic at this time and was reimplanted.

Our series highlights the many challenges for diagnosis and management in boys with this condition because of the diverse presentation and need for appropriate preoperative imaging. Treatment is by bilateral ureteric reimplantation at an appropriate age.