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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
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CASE REPORT
Year : 2005  |  Volume : 10  |  Issue : 2  |  Page : 108-111
 

Unilateral ureteral triplication with duplex kidney and megaureter


N.R.S. Medical College and Hospital, Kolkata, India

Correspondence Address:
S Bhattacharyya
Flat No. 3B, Krishnakunj Apartment, 38D, East Road, Santoshpur, Kolkata - 700 075
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.16475

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   Abstract 

We report a case of duplex kidney with Type-2 ureteral triplication, with one megaureter having stenosis at its lower end and dysplasia of the upper moiety of the kidney. The child presented with lump abdomen and recurrent attacks of urinary tract infection. The child underwent excision of the dysplastic part of the kidney and megaureter with an ureteroureterostomy connecting the normal caliber lower ureter (U3) draining in trigone with the mildly dilated middle ureter (U2) draining into the megaureter (U1) of the upper moiety. The patient is well 2 months postoperatively with no urinary infection and stable renal function.


Keywords: Duplex kidney, megaureter, ureteral triplication


How to cite this article:
Bhattacharyya S, Basu K S, Samanta N. Unilateral ureteral triplication with duplex kidney and megaureter. J Indian Assoc Pediatr Surg 2005;10:108-11

How to cite this URL:
Bhattacharyya S, Basu K S, Samanta N. Unilateral ureteral triplication with duplex kidney and megaureter. J Indian Assoc Pediatr Surg [serial online] 2005 [cited 2017 Jul 22];10:108-11. Available from: http://www.jiaps.com/text.asp?2005/10/2/108/16475


Ureteral triplication is a rare congenital anomaly of the urinary system, more so when associated with ipsilateral duplex kidney, and megaureter. Since the first description by Wrany in 1870, only about 100 cases of ureteral triplication have been reported in the literature. Paucity of symptoms often lead to missed or delayed diagnosis of the entity, where a proper diagnosis, and timely intervention can save any functioning renal tissue from progressive destruction.


   Case report Top


A 4-year-old boy presented with occasional haziness of urine since 7 months of age and occasional pain in the abdomen localized in the right side of the abdomen since 1.5 years of age.

Physical examination revealed an intra-abdominal lump occupying the right lumbar and iliac region, ballotable, 4 cm by 3 cm in size, globular, cystic, with smooth surface and rounded margins, moving with respiration, with slight mobility in both axes, retroperitoneal and nontender.

Routine blood examinations revealed normal peripheral blood count, serum urea, and creatinine level. Urine examinations revealed the presence of urinary infection, which was managed by antibiotics.

Special investigations done included ultrasonography (USG), intravenous urography (IVU), micturating cystourethrogram (MCU), isotope renogram, and cystoscopy.

Ultrasonogram found both the kidneys to be of normal size and cortical thickness with normal corticomedullary differentiation, with moderate pelvicalyceal dilatation of right kidney. The left ureter was not visualized. The right ureter appeared as a grossly dilated and convoluted tubular structure with a large communicating cyst in the middle of the ureter (raising the suspicion of megaureter). The right ureter showed echogenic debris within it [Figure - 1].

Intravenous urography showed the presence of normal caliber ureters draining into the bladder on either side along with persistent presence of a triangular area of dye hold up in the right renal area [Figure - 2].

Micturating cystourethrogram showed a urinary bladder of normal outline with no filling defect or pressure effect. No vesicoureteral reflux was noted on either side.

Isotope renogram showed the right kidney to be of smaller size than the left kidney with slight tracer retention till the end of study. Lasix challenge, however, increased clearance, and washed out the retained tracer. The left kidney function was within normal limits. The relative function of each kidney was 63 and 27% in the left and right side, respectively, and GFR was 57.2 ml/min in the left and 34 ml/min in the right kidney.

Cystoscopy revealed a single, normal-sized ureteric orifice on the left side, but on the right side there were two ureteric orifices; one of normal size and the other (i.e., lower one) dilated.

Armed with the above findings and with a suspicion of duplex system on the right side, the patient was explored. The right renal system was approached extraperitoneally with an oblique incision extending from posterior axillary line at the level of the umbilicus up to the lateral border of the right rectus. A huge cystic swelling in the right hilar region was obscuring the anatomy, which became clear after aspiration of the cyst. The presence of a duplex renal system with ureteral triplication was visualized. The smaller upper moiety of the right kidney was dysplastic, draining into a megaureter (U1), which tapered down gradually to the ureterovesical junction, where it was found to be stenosed. The normal kidney (in the lower moiety) on the right side had two ureters. The one from the upper segment (U2) was slightly dilated and was draining into the megaureter of the upper moiety. The lower segment of the normal kidney had a separate, normal-sized ureter (U3) draining at the normal site into the bladder [Figure - 3].

The dysplastic upper moiety was excised along with U1. The U1 was intimately related with U3 near the bladder. Every possible care was taken to protect the vascularity of U3 while dissecting U1, which was excised close to the bladder wall. The U2 was dissected from U1 and was anastomosed with U3, over a stent [Figure - 4]. The stent was kept for 14 days. The patient had no significant postoperative problems and was discharged on the 15th postoperative day with a 3-month course of urinary antiseptic. He is doing well 2 months postoperatively with no urinary infection and maintaining a stable renal function.


   Discussion Top


Ureteral triplication is an extremely uncommon congenital urinary anomaly, with only about a 100 cases reported worldwide since the first description by Wrany in 1870.[1] Ureteral triplication was classified into four types by Smith:[2] (i) three ureters and three ureteral orifices (triple ureter), (ii) three ureters and two ureteral orifices (double ureter with one bifid), (iii) three ureters and one ureteral orifice (trifid ureter), and (iv) two ureters and three ureteral orifices (double ureter with inverted Y bifurcation of one ureter) [Figure - 5]. Our case represents Type-2 triplication.

The embryological event resulting in ureteral triplication derives from multiple ureteral buds arising from the mesonephric duct with fissuring of one or more of them.

The positions of the ureteral orifices of duplex systems usually follow the  Weigert-Meyer law More Details. The upper orifice drains the lower renal segment and the lower orifice drains the upper segment. This principle does not apply uniformly in ureteral triplication. [3],[4],[5] Out of the 13 patients with ureteral triplication studied by Mark R. Zaontz,[6] this was true only in seven. In our case, the position of the ureteral orifice did not conform to the above principle. Perkins et al .[7] described eight patients with Type-1 ureteral triplication; out of them, five patients did not conform to the Weigert-Meyer law.

Presentation of ureteral triplication is often delayed due to paucity of symptoms and signs. The most common presentations are recurrent urinary infection and enuresis. Enuresis occurs in the case of a female child with ectopic ureteric orifice. Stephens reviewed 16 cases of Type-1 triplication. The presenting symptoms included urinary infection - six cases, enuresis - six, hematuria following trauma - one, pain without infection - one, and accidental diagnosis during routine examination in one case.[8] Our patient presented with an abdominal lump caused by the obstructed megaureter. Lump abdomen is a very uncommon form of presentation of the triplicate system.

The common urological anomalies associated with ureteral triplications are contralateral duplication in 37% and ureteral ectopia in 28% of cases.[9] Ureterocele and vesicoureteral reflux are other associations found with the entity.[10] Renal dysplasia is found associated in 8% cases.[9] The upper moiety of the right kidney in our patient was found to be dysplastic. The interesting association that our case had along with ureteral triplication was stenosis of the proximal ureteral orifice along with obstructed megaureter. The obstructed megaureter in a triplicate system is very uncommon and only a few cases have been reported worldwide.[11]

While many patients are asymptomatic[12] or have only mild symptoms, diagnosis of triplication is important to avoid complications and progressive renal damage. In our case, diagnosis and timely intervention lead to prevention of any further kidney damage.[13]



 
   References Top

1.Li J, Hu T, Wan M, Chen S, et al. Ureteral triplication: The first report in China. J Pediatr Sung 2004;39:E38-9.  Back to cited text no. 1    
2.Smith I. Triplicate Ureter. Br J Surg 1946;34:182-5.  Back to cited text no. 2    
3.Lugue-Mialdea R, De TE, Arrojo F, et al. Ureteral triplication: Double extravesical ureteral ectopia. J Urol 1991;145:109-11.  Back to cited text no. 3    
4.Rich MA, Heimler A, Waber L, et al. Autosomal dominant transmission of ureteral triplication and bilateral amastia. J Urol 1987;137:102-5.  Back to cited text no. 4    
5.Ireland EF Jr, Chute R. A case of triplicate - duplicate ureters. J Urol 1955;74:342.  Back to cited text no. 5  [PUBMED]  
6.Zaontz MR, Maizels M. Type I Ureteral triplication: An extension of the Weigert-Meyer, law. J Urol 1985;134:949-50.  Back to cited text no. 6  [PUBMED]  
7.Perkins PJ, Kroovand LR, Evans AT. Radiology 1973;108:533.  Back to cited text no. 7    
8.Stephens FD. Triplicate and quadruplicate ureters. In: Congenital malformations of the urinary tract. New York: Praeger Publishers; 1983. p. 350.  Back to cited text no. 8  [PUBMED]  
9.Gosalbez R Jr, Gosalbez R, Piro C, et al. Ureteral triplication and ureterocele: Report of 3 cases and review of the literature. J Urol 1991;145:105-8.  Back to cited text no. 9    
10.Youngson GG. Ureteral triplication, contralateral duplication and bilateral extravesical ectopic ureter. J Urol 1985;134:533-5.  Back to cited text no. 10  [PUBMED]  
11.Merlini E. Trifid obstructed megaureter. Urology 1983;22:62-3.  Back to cited text no. 11  [PUBMED]  
12.Blumberg N. Ureteral triplication. J Pediatr Surg 1976;11:579-80.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]
13.Kohri K, Nagai N, Kanebo S, et al. Bilateral Trifid ureters associated with fused kidney, ureterovesical stenosis, left cryptorchidism and angioma of the bladder. J Urol 1978;120:249-50.  Back to cited text no. 13    


Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]


This article has been cited by
1 Ureter triplication with contra-lateral partial duplex system
Mubarak, M.Y., Zainun, A.R., Rohaya, M.
Medical Journal of Malaysia. 2009; 64(3): 236-237
[Pubmed]



 

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