*Vrije Universiteit Brussel  and ^&Women’s Health Alliance, Nashville TN

Synonyms: none

Definition:

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[1]. 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%)[2]

Ureteroceles are highly specific markers for the diagnosis of duplex renal system.

Case report:

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.

Both kidneys:

The left kidney"s upper pole

and lower pole

The ureterocele in the bladder

Differential diagnosis:

1.       male: seminal vesicular cyst[3]

2.       female:

·         Gartner’s duct cyst[4],

·         pseudo-ureterocele: an ectopic ureter draining into a mesonephric duct cyst can be confused with an ectopic ureterocele[5],

·         hydro(metro)colpos.

Associated anomalies:

Aneuploidies: a one-month-old boy, which presented with hydronephrosis due to an intravesical uretrocele was found to have a 49, XXXXY karyotype[6]. 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[7].

Sonographic findings:

Ureteroceles appear as thin-walled cyst-like structures within or about the bladder[8].

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[9].

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.[10]

Prognosis:

Ureteroceles can be become very large and can cause prenatal hydronephrosis from bladder outlet obstruction[11]. 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.

Management:

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

References

[1] Farrokh D. Early diagnosis of congenital ureterocele. J Belge Radiol 1992 Feb;75(1):25-8

[2] Brueziere J. Ureteroceles. Ann Urol 1992;26(4):202-11

[3] Hammadeh MY, Dhillon HK, Duffy PG, Ransley PG. Antenatally diagnosed seminal vesicular cyst. Fetal Diagn Ther 1994 Jan-Feb;9(1):62-4

[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

[5] Sumfest JM, Burns MW, Mitchell ME. Pseudoureterocele: potential for misdiagnosis of an ectopic ureter as a ureterocele. Br J Urol 1995 Mar;75(3):401-5

[6] Kojima Y, Hayashi Y, Maruyama T, Sasaki S, Mogami T, Kohri K.  49XXXXY syndrome with hydronephrosis caused by intravesical ureterocele. Urol Int 2000;63(3):212-49

[7] Goodship  J, Robson SC, Sturgiss S, Cross IE, Wright C. Renal abnormalities on obstetric ultrasound as a presentation of DiGeorge syndrome. Prenat Diagn 1997 sept;17(9):867-70

[8] Malisch TW. Jeanty P. Ureterocoele, ectopic.  www.thefetus.net/

[9] Vergani P, Ceruti P, Locatelli A, Mariani E, Paterlini G, Zorloni C, Ghidini A. Accuracy of prenatal ultrasonographic diagnosis of duplex renal system.J Ultrasound Med 1999 Jul;18(7):463-67

[10] Kang AH, Bruner JP. Antenatal ultrasonographic development of ureteroceles. Implications for management. Fetal Diagn Ther 1998 May-Jun;13(3):157-61

[11] Austin PF, Cain MP, Casale AJ, Hiett AK, Rink RC Prenatal bladder outlet obstruction secondary to ureterocele.Urology 1998 Dec;52(6):1132-5

12 Roy GT, Desai S, Cohen RC. Ureteroceles in children: an ongoing challenge. Pediatr Surg Int 1997;12(1):44-8

13 Di Benedetto V, Morrison-Lacombe G, Bagnara V, Monfort G. Transurethral puncture of ureterocele associated with single collecting system in neonates. J Pediatr Surg 1997 Sep;32(9):1325-7

14 Jayanthi VR, Koff SA. Long-term outcome of transurethral puncture of ectopic ureteroceles: initial success and late problems. J Urol 1999 Sep;162(3 Pt 2):1077-80

15 Di Benedetto V, Monfort G. How prenatal ultrasound can change the treatment of ectopic ureterocele in neonates? Eur J Pediatr Surg 1997 Dec;7(6):338-40

16 De Gennaro M, De Grazia E, Nappo S. The prenatal diagnosis and early endoscopic section of ureterocele. Pediatr Med Chir 1996 Nov-Dec;18(6):585-9

17 Barret E, Pfister C, Dunet F, Liard A, Mitrofanoff P. Endoscopic treatment of prenatally diagnosed ureteroceles. Prog Urol 1996 Aug-Sep;6(4):529-34

18 Blyth B, Passerini-Glazel G, Camuffo C, Snyder HM 3d, Duckett JW. Endoscopic incision of ureteroceles: intravesical versus ectopic. J Urol 1993 Mar;149(3):556-9

19  Jayanthi VR, Koff SA. Long-term outcome of transurethral puncture of ectopic ureteroceles: initial success and late problems. J Urol 1999 Sep;162(3 Pt 2):1077-80

20 Peng HC, Chen HC. Surgical management of complete ureteric duplication abnormalities in children. Chung Hua I Hsueh Tsa Chih (Taipei) 2000 Mar;63(3):182-8

21 Encinas Goenechea A, Gomez Fraile A, Aransay Bramtot A, Lopez Vazquez F, Matute de Cardenas JA. Surgery of ectopic ureterocele in children: strategy based on prenatal function. Cir Pediatr 1995 Jul;8(3):108-12

22 Dodat H, Beck F, el Khoury G, Takvorian P. Development of the diagnosis and treatment of pyelo-ureteral duplication in children. Reflections on 179 cases. Pediatrie 1990;45(2):115-22