Ureter duplication

Facial dysmorphism, pterygium colli, distended abdomen, absence of external genitalia and of anal and urethral openings, polycystic kidneys, dysmorphism of lumbar spine Unilateral ureteral duplication with one ureter ending in the vagina, hydronephrosis, hydroureters... 

Fig. 1.1.1. US axial scan of the bladder in a 10-year-old girl. Dilated left upper pole ureter identified through a full bladder in a child with duplicated ureter and ectopic implantation of the upper pole ureter...

Fig. 1.1.4. Accidental introduction of the tube in the ectopic orifice of the upper pole ureter of a duplicated kidney. No consequence was observed. Catheter was withdrawn, then introduced again in the bladder. This type of accident illustrates the importance of sterility in any maneuver while performing VCU...

Table 3.5. Syndromes with duplication of kidneys, renal pelvis and ureters (selection)...

Ectopic ureterocele develops at the lower end of the upper pole ureter of a duplicated kidney. It is a cyst-like thin-walled structure that is known to be mobile and variable in shape. During fetal life, the ureterocele can prolapse into the posterior urethra and create obstruction (Fig. 6.9). Bilateral hydronephrosis and megacystis can subsequently develop. Clinical diagnosis can be made at birth in girls with a perineal soft tissue mass, megacystis and bilateral urinary tract obstruction. Sonographic diagnosis can be difficult when the ectopic ureterocele has... 

Fig. 6.8. Sagittal US view of the bladder neck in a male neonate with left duplicated ureter. The echogenic mass visible in the bladder neck is a spontaneously ruptured ectopic ureterocele, draining the dysplastic upper pole of the duplicated left kidney. Urethral polyp is the main differential diagnosis...

Fig. 6.9. VCU in a female neonate with prenatal diagnosis of megacystis and hydronephrosis. Vesicoureteric reflux into the lower pole of a left duplicated kidney. Left upper pole ureter is obstructed by an ectopic ureterocele that is prolapsed in the urethra during micturition...

Fig. 8.3. IVU. Bilateral duplicated dysplastic kidney systems with megaureters and ureter dysmotility. Note dysplastic sacrum...

Another causal factor in enuresis nocturna might be rapid bladder Ailing (N0rgaard et al. 1989). In this context the rare anatomic abnormality of a completely duplicated ureteral system in which an ectopic ureter empties directly outside the bladder has to be kept in mind. In this case the upper renal moiety drains through a ureter that empties below the urethral sphincter or at some other ectopic site such as the vagina, uterus or even the perineum. Hence, these children are wet all of the time. This condition occurs exclusively in girls. 

In most cases incomplete ureteric duplication is an incidental finding. However, it is occasionally possible that there is yo-yo reflux leading to stasis and ureteric dilatation. This can lead to flank pain and infection. In the rare cases requiring surgery, the type of operation depends on the level of duplication. If this is very low, it may be possible to carry out a reimplantation of the ureters with separate ureteric orifices into the bladder. If the duplication is higher, a high ureteropyelostomy or ureteroureterostomy with excision of most of the duplicated ureter is the treatment of choice. 

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