Orthotopic Ureterocele

Case History: A 12-year-old girl admitted to our hospital for dysuria. Physical examination was unremarkable. Urianalysis was suggestive of lower urinary tract infection. Urinary ultrasound was indicated.

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Figure 1

Bladder ultrasound showed a fluid-filled cystic intravesical mass contiguous with a mildly dilated left ureter and arised from a normally positioned ureteral orifice (arrow). The wall of the mass also showed continuity with the bladder wall. These findings were consistent with an orthotopic ureterocele. Intravenous urography (IVU) was then indicated.

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Figure 2

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Figure 3

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Figure 4

IVU demonstrated the sign of “cobra head” within the bladder consistent with single system ureterocele. Mild hydroureteronephrosis was also noted. Bilateral renal function remained intact with normal bladder emptying. No vesicoureteral reflux was seen. Left ureterocele was confirmed on cystoscopy.

Diagnosis: Orthotopic ureterocele

Discussion: Ureteroceles occur in approximately 1 in every 4,000 children. Females are affected 4-7 times more often than males. A slight left-sided preponderance appears to exist, and approximately 10% of ureteroceles are bilateral. In the adult population, ureteroceles also occur more frequently in females.

Orthotopic ureteroceles occur in 17-35% of cases, with an incidence of ectopic ureteroceles of approximately 80% in most pediatric series [1]. Similarly, approximately 80% of ureteroceles are associated with the upper pole moiety of a duplex system [2]. This congenital defect is the obstruction of the meatus, and ureterocele is simply a hyperplastic response to this obstruction. The outer wall is composed of bladder epithelium and the inner wall of ureteral epithelium, with connective tissue and muscle fiber in between [3].

Stephens and colleagues classified ureteroceles as stenotic ureteroceles and ureteroceles associated with duplex ureters. Stenotic ureterocele is the most common type in single ureter system, occurring more often in adults and always intravesical [2].

Urinary tract infection is the most common clinical manifestation. Surgical therapy for both pediatric and adult ureteroceles may include endoscopic puncture, incision or transurethral unroofing of the ureterocele, upper pole heminephrectomy, excision of ureterocele and ureteral reimplantation, and nephroureterectomy. Indications for surgical treatment for ureteroceles depend on the site of the ureterocele, the clinical situation, associated renal anomalies, and the size of the ureterocele. Observation alone is rarely a good option in symptomatic ureteroceles [1].

References
1. Cooper C.S, Kim E.D, Ureterocele treatment and mangement, Medscape, http://emedicine.medscape.com/article/451105-treatment#a1127.
2. Berrocal et al, Anomalies of the distal ureter, bladder and urethra in children: Embryologic, Radiologic and Pathologic features. Radiographics 2002; 22: 1139-1164
3. Shamsa A. et al, Bilateral simple orthotopic ureteroceles with bilateral stones in an adult, a case report and review of the literature. Urology Journal 2010; 7 (3).

Le Trong Binh, MD
Department of Radiology
Hue University College of Medicine and Pharmacy, Hue, Vietnam