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Renal colic in a young woman

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Q1: What are the findings on the plain and cystogram films of the IVU?

In the plain film (fig 1, see p 511) a small radio-opaque shadow (calculus) is seen in the area of the bladder to the right of the midline. On the cystogram film a typical cobra head appearance characteristic of a ureterocele is seen.The wall of the ureterocele is radiolucent whereas the contrast present within the ureterocele and in the bladder is radio-opaque. This gives the typical cobra head appearance of the ureterocele. The diagnosis in this case is an intravesical stenotic ureterocele containing a calculus.

Q2: What are the types of ureteroceles and how does an ureterocele present?

A ureterocele is a cystic dilatation of the terminal ureter and is a developmental abnormality. They are broadly classified into two categories: 1, intravesical and 2, ectopic. An ureterocele is termed ectopic if any portion of the ureterocele extends to the bladder neck or the urethra. Ureteroceles are classified further according to the number of systems (single or duplex) and the type of orifice (for example, stenotic, sphincteric, sphincterostenotic). This classification was established by the American Academy of Pediatrics and is most frequently used today. Clinical presentation varies considerably (see box 1). Patients commonly present with infection but calculi can develop secondary to urinary stasis and are often seen in the distal ureter.

Box 1: Clinical presentation of a ureterocele

  • Urinary tract infection.

  • Ureteric colic due to a calculus.

  • Bladder outflow obstruction.

  • Urinary incontinence.

  • Mass prolapsing through female urethra.

  • Haematuria.

  • Palpable abdominal mass may be detectable in some children (hydronephrotic kidney).

Q3: How do you manage this condition?

In our case the ureterocele was intravesical, single with a stenotic orifice and there was a calculus in it. An endoscopic transurethral incision of ureterocele with a Collin's knife and extraction of the calculus was performed under general anaesthesia.

Learning points

  • Ureterocele is a cystic dilatation of lower end of ureter (congenital abnormality).

  • Intravesical ureteroceles are generally associated with single ureters, whereas ectopic ureteroceles most often are associated with the upper pole moiety of duplex ureters.

  • Seven times more common in girls. 10% of cases are bilateral. Ectopic ureteroceles are four times more common.

  • Commonest presentation is urinary tract infection.

  • Excretory urography is usually diagnostic.

  • Transurethral endoscopic incision of ureterocele is the treatment of choice for symptomatic intravesical ureterocele.

  • Prenatal diagnosis of the ureterocele with ultrasound scan enables neonatal transurethral incision of ureterocele which effectively decompresses the ureterocele with preservation of renal parenchyma.

Discussion

The term ureterocele is used to describe the cystic dilatation of the terminal portion of the ureter. It may be either intravesical or ectopic. Ureterocele has been attributed to incomplete dissolution of the Chwalle's membrane which divides the early ureteric bud from the urogenital sinus.1 The cystic dilation forms between the superficial and deep muscle layers of the trigone. Ureteroceles of a single ureter are characteristically of the intravesical type and are less prone to severe obstruction and dysplasia. Ectopic ureteroceles are most commonly encountered in paediatrics and usually develop with ureteral duplication and are often responsible for serious complications.2 Stasis of urine in this obstructed system can lead not only to urinary tract infection but also to calculus formation. Some children may present with a palpable mass in their abdomen, which is a hydronephrotic kidney. An ectopic ureterocele can prolapse out of the urethra and present as a vaginal mass. This is termed a prolapsing ureterocele. A large ureterocele can obstruct the bladder neck or even the contralateral ureteric orifice causing hydronephrosis. Ectopic ureteroceles can cause urinary incontinence by interfering with the normal sphincteric function at or distal to the bladder neck. Excretory urography often demonstrates the characteristic cobra head (or spring onion) deformity—an area of increased density similar to the head of a cobra with a halo or a less dense shadow around it. At cystoscopy the ureterocele usually expands rhythmically with each peristaltic wave that fills it and then shrinks as a thin jet of urine drains through the thin orifice. Treatment of ureterocele must be individualised. Single system ureteroceles are more amenable to endoscopic incision3 and are less likely to exhibit postoperative reflux. Treatment of ectopic ureterocele associated with an upper pole moiety of a duplex system is more complicated. In most instances the upper pole moiety of the duplex system is obstructed, hydronephrotic and contributes little to overall renal function. This involves upper pole nephrectomy and partial ureterectomy.4 Less commonly, when significant upper pole function is present a ureteropyelostomy is advocated. This should decompress the ureterocele, with return of the trigone to a more normal configuration and resolution of ipsilateral lower pole reflux. However if the patient has high grade reflux into the ipsilateral lower pole ureter, a combination of ureterocele excision with or without nephroureterectomy, and lower pole ureteral reimplantation (common sheath) may be necessary. Separation of the duplicated ureters during intravesical dissection should be discouraged because it can lead to sacrifice of the common blood supply running longitudinally in between the ureters, hence the need for common sheath reimplantation of the ureters. With the advent of frequent prenatal ultrasonography early diagnosis of ureteroceles is possible. Treatment of ureterocele in the neonatal period by endoscopic incision enables effective decompression of the ureterocele, preservation of renal function in the neonate and reduces the risk of severe infection in the neonate.5

Final diagnosis

Intravesical stenotic ureterocele containing a calculus.

References

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