2001-01-24-16 Ureterocele © Sleurs www.thefetus.net/
Elke Sleurs, MD*&, Philippe Jeanty, MD, PhD&
*Vrije Universiteit Brussel and &Women’s Health Alliance, Nashville TN
A ureterocele is the herniation of the cystic end of a lower ureter, lying between the mucosa and the bladder muscle, ballooning inside the bladder. The abnormality leads to urinary retention and recurrent urinary tract infection, which can cause irreversible damage to the kidney. Four types of ureteroceles are described:
- ureterocele with total duplication of the ureter and extra-vesical development (62%);
- ureterocele with total duplication and intra-vesical development (10%);
- ureterocele with single ureter (10%);
- ureterocele with ectopic ureter (3%)
Ureteroceles are highly specific markers for the diagnosis of duplex renal system.
A routine examination at 15 weeks of gestation demonstrated a bilateral pyelectasis (left = 7mm, right = 5mm). Control examination at 24 weeks revealed a duplicated collecting system with ectopic ureterocele in the bladder. No pejorative evolution occurred during the pregnancy. The baby had resection of the lower pole moiety and ureter at 6 months and has been doing fine since. She never had an urinary tract infection.
There is a 2.1 MB videoclip.
The left kidney"s upper pole
and lower pole
The ureterocele in the bladder
1. male: seminal vesicular cyst
· Gartner’s duct cyst,
· pseudo-ureterocele: an ectopic ureter draining into a mesonephric duct cyst can be confused with an ectopic ureterocele,
Aneuploidies: a one-month-old boy, which presented with hydronephrosis due to an intravesical uretrocele was found to have a 49, XXXXY karyotype. Ureteroceles are also associated with the DiGeorge syndrome characterized by renal anomalies (hydroureteronephrosis with ureterocele, multicystic kidneys, renal agenesis), cardiac abnormalities (ventricular septal defect, double-outlet right ventricle) and absence of the thymus due to a deletion of chromosome 22q11.
Ureteroceles appear as thin-walled cyst-like structures within or about the bladder.
Hence ureteroceles can be missed depending on:
- the filling conditions of the bladder: when the bladder is empty the cyst is apposed to the bladder wall, and a full bladder can result in effacement of the ureterocele
- the localization of the ureterocele: low in the fetal bladder or in the proximal urethra, making it difficult to visualize.
Prenatal diagnosis of duplex renal system can be made in utero during the second half of pregnancy in the presence of two or more of the following signs:
- hydronephrosis limited to one pole in a kidney with two separate, non-communicating renal pelves;
- ipsilateral megaureter; and ureterocele.9
The observation of hydronephrosis should prompt a search for an unaffected lower pole moiety, a dilated ureter, or a visible ureterocele.
Damage from obstruction is a progressive process, which may develop into bilateral renal dysfunction. As such it is important to perform serial ultrasonographic evaluations of fetal obstructive uropathies.
Ureteroceles can be become very large and can cause prenatal hydronephrosis from bladder outlet obstruction. It is the most common cause of bladder outlet obstructions in infant girls1. The common clinical presentations include urinary tract infections, pyelonephritis, urinary septicemia and urinary incontinence. The advantage of prenatal diagnosis is that the postnatal urinary antibiotic prophylaxis prevents urinary infection.
The prenatal diagnosis of ureteroceles allows postnatal urinary antibiotic prophylaxis and appropriate complementary investigations12. Post-natal management of ureteroceles should be individualized.
Several authors report the endoscopic incision of ectopic and single-system ureteroceles13 as a first choice for prenatally diagnosed ureteroceles14,15,16,17,18. Prompt intervention allows successful decompression of the ureterocele and usually improves renal function. Repeat puncture may be required to ensure adequate decompression in a minority of cases19.
Surgical procedures in cases of complete ureteric duplication are upper pole nephroureterectomy in case of a nonfunctioning moiety. Ureteropyelostomy or ureteric reimplantation can be considered for functioning segments20,21. The results are satisfactory, but long-term follow-up is necessary.
Other techniques are early unroofing of ureteroceles or the endoscopic injection of Teflon, which causes the vesico-ureteral reflux to disappear in most cases (70%).22
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 Brueziere J. Ureteroceles. Ann Urol 1992;26(4):202-11
 Hammadeh MY, Dhillon HK, Duffy PG, Ransley PG. Antenatally diagnosed seminal vesicular cyst. Fetal Diagn Ther 1994 Jan-Feb;9(1):62-4
 Sheih CP, Li YW, Liao YJ, Huang TS, Kao SP, Chen WJ. Diagnosing the combination of renal dysgenesis, Gartner’s duct cyst and ipsilateral mullerian duct obstruction. J Urol 1998 Jan;159(1):217-21
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