Vol 13 No 01   January-February 2016   2471Vol 13 No 01   January-February 2016   2579

PICTORIAL

Bladder Agenesis Associated with Crossed Fused Renal Ectopia and Verte-
bral Anomalies: A Rare Entity

Stephen Lalfakzuala Sailo,1* Laltanpuii  Sailo2

A 9-month-old girl presented with dribbling urine and no normal voiding since birth. There was no history of 
urinary tract infection. On examination, the child appeared healthy. An abdominal examination was normal. On 
separation of the labia, a single opening with leaking urine was seen; no separate urethral opening was identified 
(Figure 1). Abdominal ultrasonography revealed a crossed left renal ectopic kidney. Computed tomography also 
showed a normally excreting crossed fused ectopic kidney/lump kidney (Figure 2A), ureters draining into a uro-
genital sinus, and butterfly and block lumbar vertebrae (Figure 2B). The serum creatinine level was normal (0.3 
mg/dL). Endoscopy using a pediatric cystoscope inserted through the genital opening revealed a urogenital sinus in 
which both ureteric openings were located (Figure 3); both ureters refluxed when the sinus was filled with contrast 
(Figure 4). We plan to divert the ureters to a cutaneous stoma. Agenesis of the urinary bladder is extremely rare; 
only 23 living cases have been reported. Agenesis is attributable to injury to the urogenital sinus at weeks 5~7 of 
embryogenesis.(1) The condition is associated with renal and skeletal anomalies. Young age is not a contra-indica-
tion for continent urinary diversion using a self-catheterizable pouch.(2)

Figure 1. Genital opening filled with urine (arrow).

Figure 2. Computed tomographic images of A) the crossed fused ectopic kidneys/lump kidney (arrow) and, 
B) the crossed fused ectopic kidneys, ureters draining into the urogenital sinus (triangle), block vertebra 
(arrow), and butterfly vertebra (star).

Department of Urology,1 and Anaesthesia,2 North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, 
Shillong, Meghalaya, India.
*Correspondence: Department of Urology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, 
Shillong, Meghalaya, India.
Tel: +91 364 2538006. Fax: +91 364 2538010. E-mail: stephensailo@gmail.com.
Received: June 2016 & Accepted: September 2016



REFERENCES
1. Pacheco-Mendoza BA, Gonzalez-Ledón FJ, Diaz-Pardo M, Soto-Blanquel JL, Castelán-Martínez OD. 

Bladder agenesis and incomplete kidney duplication: Ileal reservoir with continent diversion as definitive 
treatment. Can Urol Assoc J. 2015;9:e142-4.

 2. Pfister D, Sahi D, Heidenreich A, Rohrmann D. A continent urinary diversion in a female with agenesis 
of the bladder: A 5-year follow-up. Urology. 2012;80:437-9.

Figure 3. Endoscopy using a pediatric cystoscope shows the urogenital 
sinus and left ureteric orifice (arrow).

Figure 4. Both ureters and the pelvi-calyceal systems became opacified 
when the urogenital sinus was filled with contrast.

Bladder Agenesis and Vertebral Anomalies-Sailo and Sailo.

Pictorial Urology  2580