Bilateral hydroureteronephrosis: A neonatal presentation of bladder duplication

Abstract

In a male neonate with bilateral hydroureteronephrosis, the most common surgical diagnosis is posterior urethral valves. This case report describes a male infant with the same presentation, but caused by a very uncommon congenital anomaly. The summation of different imaging modalities allowed a multidisciplinary team of colleagues to define the anatomy: bilateral duplex kidneys draining into separate urinary bladders. Only one of the bladders had an outlet, hence the obstructive uropathy to the right kidney led to total loss of function. The distended tortuous ureters produced a mass effect at presentation. This case acts as a reminder that complex congenital anomalies can mimic the presentation of more common conditions, and that they often require input from various specialists to diagnose the condition and guide its management.

Keywords: Bladder duplication; Congenital anomaly; Hydroureteronephrosis; Neonatal.

Fig. 1

(a) Antenatal ultrasound at 28 weeks gestation showing left hydronephrosis. (b) Antenatal ultrasound at 28 weeks gestation showing right hydronephrosis.

Fig. 2

Plain abdominal radiograph on day 0 of life demonstrating a right-sided abdominal mass displacing the bowel to the patients left (asterix at the center of the opacity).

Fig. 3

(a) Longitudinal ultrasound image showing the duplex right kidney, and suggesting a loss of cortico-medullary differentiation. (b) Axial ultrasound image showing a section of the cystic mass (asterix) posterior to the catheterized orthotopic bladder. (c) Sagittal ultrasound image showing the ectopic bladder (asterix) not decompressing despite urethral catheterization, which is visualized superiorly.

Fig. 4

Axial, coronal, and sagittal sections of a T2-weighted MRI showing the duplex right kidney and the mass effect of the dilated tortuous right sided obstructed ureters (asterix on axial and sagittal images).

Fig. 5

Lateral view of the micturating cystourethrogram, showing contrast in the orthotopic bladder, and the normal caliber urethra (asterix).

Fig. 6

(a) Right kidney antegrade nephrostogram (prone): guidewires in each moiety, with contrast draining into the ectopic bladder and then draining out via vesicostomy. (b) Retrograde cystogram (prone): right upper and lower pole nephrostomies in situ, retrograde cystogram through the urethral catheter, showing reflux into the left ureter.

Fig. 7

MAG3 renogram with left/right differential function, showing no functional activity in the right kidney (0%), and adequate drainage of the left kidney on the drainage curve.