Ureteral ectopia is a common condition presenting as incontinence in females and incidentally detected in males. The diagnosis requires thorough radiological investigations to delineate the anatomy and formulate the treatment. The prognosis is generally excellent. We presented a series of three cases of ureteral ectopia and discussion regarding their management.Keywords: Female; Urinary Incontinence; Ectopic Ureter; Duplex Moiety Kidney

Kumbhar, Vikrant V

Shah, Hemanshi S

Tiwari, Charu

Journal of Pediatric Nephrology

English

Journals Portal, Shahid Beheshti University of Medical Sciences

 Duplex Moiety Kidney with Ureteral Ectopia -Vikrant VK et al                                                  Journal of Pediatric Nephrology | Volume 4 | Number 1| 2016   41  Case Report  J Ped. Nephrology 2016;4(1):41-44         http://journals.sbmu.ac.ir/jpn  DOI: http://dx.doi.org/10.20286/jpn-040141  Duplex Moiety Kidney With Ureteral Ectopia; A Case Series  How to Cite This Article: Vikrant V K, Charu T, Hemanshi S. Duplex Moiety Kidney With Ureteral Ectopia; A Case Series. J Ped Nephrology2016;4(1):41-44.  Vikrant V Kumbhar Charu Tiwari Hemanshi S Shah*   MCh. Pediatric Surgery, Department of Pediatric Surgery, TNMC & BYL Nair Hospital, Mumbai Central, Mumbai-08 Department(s) and institution(s) where the work was carried out: Department of Paediatric Surgery, TNMC & BYL Nair Hospital. Mumbai -08, India.  *Corresponding Author  Dr Hemanshi S Shah hemanshisshah@gmail.com Ward no 24, 6th floor, OPD Building. TNMC & BYL Nair Hospital. Mumbai Central. Mumbai - 08  Ureteral ectopia is a common condition presenting as incontinence in females and incidentally detected in males. The diagnosis requires thorough radiological investigations to delineate the anatomy and formulate the treatment. The prognosis is generally excellent. We presented a series of three cases of ureteral ectopia and discussion regarding their management.  Keywords: Female; Urinary Incontinence; Ectopic Ureter; Duplex Moiety Kidney.  Received: Dec-2015 Revised: Dec-2015  Accepted: Dec-2015  Running Title: Duplex moiety kidney with ureteral ectopia   Introduction   An ectopic ureter is characterized by an orifice that is outside the posterolateral extremity of the bladder trigone [1]. They are associated with either duplex kidney or less commonly a single system (20-25%) [2,3]. They are usually asymptomatic or detected incidentally in males. The diagnosis is complex due to variations in the anatomy of the ectopic ureter. Therefore, we would like to present a series of three cases of ectopic ureters and discussing their management.                                              Case Report Case 1: A 6 year-old girl presented with incontinence. Examination had normal findings. CT scan-Intravenous pyelography (CT-IVP) showed left duplex kidney with upper moiety ureter opening ectopically in the anterior vaginal wall (Fig. 1-2). Voiding cystourethrography (VCUG) was unremarkable. There was adequate   function in the both kidneys on Renal Scan (separate function of upper and lower moiety was not performed).  On examination under general anesthesia, a distended tubular structure was seen on the left anterior vaginal wall; however, the opening could not be visualized. A left end to side ureteroureterostomy was performed (Fig. 3), where the adequately functioning upper moiety ureter was anastomosed to the side of the lower moiety ureter. Case 2: A 6 year-old female presented with continuous dampness of undergarments. Clinical examination had normal findings. Ultrasonography was suggestive of right complete duplex kidney with hydronephrosis of the upper moiety. VCUG had normal results. On CT-IVP, right complete duplex kidney with hydronephrosis of the upper moiety and hydroureter was seen (Fig. 4-5). The ectopic ureter was seen opening in the  Duplex Moiety Kidney with Ureteral Ectopia -Vikrant VK et al                                                  Journal of Pediatric Nephrology | Volume 4 | Number 1| 2016   42  upper part of the vagina. Renal scan showed 46.6% function in the left kidney with 53.4% function in the right kidney. Of this the upper moiety contributed to 12% function on the right, which was 6.4% of the total function. Cystogenitoscopy showed impression along the anterior wall of the vagina with flow of urine. Considering the above findings, right upper moiety heminephrectomy was performed (Fig. 6). Case 3: A 5 year-old female presented with dribbling of urine since birth. General examination and local examination had normal findings. Ultrasonography (USG) showed Duplex moiety on the right side with moderate hydronephrosis in upper moiety and mild hydronephrosis in lower moiety. Left kidney was not visualized. VCUG had normal results. Renal scan showed 15.52% function in the upper moiety and 84.48% function in the lower moiety. Magnetic resonance urography (MRU) showed left crossed fused kidney with its ureter draining normally in the bladder on the left side (Fig. 7-8). However, the right ureter had an ectopic opening. On Cystogenitoscopy the left ectopic ureter was visualized at its normal site in the bladder. Opening of the right ureter was not seen in the bladder or vagina. At surgery the right ectopic ureter was anastomosed to the pelvis of normally draining left crossed fused kidney (Fig. 9). All three patients had an uneventful postoperative recovery.    Figure 1. CT IVP showing left duplex moiety kidney  Figure 2. CT IVP showing the left ectopic ureter     Figure 3. Intra-operative picture of transected  upper moiety ureter   Figure 4.  CT IVP showing right duplex moiety   Duplex Moiety Kidney with Ureteral Ectopia -Vikrant VK et al                                                  Journal of Pediatric Nephrology | Volume 4 | Number 1| 2016   43   Figure 5.  CT IVP reconstruction showing  ectopic ureter alongside normal ureter   Figure 6.  Intra-operative image showing both  Duplex moieties   Figure 7.  MRU showing both kidneys on  right side    Figure 8.  MRU showing left ureter crossing to  drain in the bladder     Figure 9. Intra-operative image showing dilated  right renal pelvis with ureter along with left  crossed fused ureter.   Discussion Ureteral duplication is the most common urinary tract abnormality, occurring in approximately 1% of  the  population  [4].   Ureteral ectopia in the bladder is usually detected incidentally and warrants no further treatment. However in toilet trained girls with normal voiding pattern constant dribbling of urine is indicative of ectopic ureteric opening [5]. In contrast, boys rarely present with incontinence. This is because the ureteral bud is a Wolffian structure and would not get inserted directly into the mullerian duct structures, rather it courses alongside these structures resulting in various ectopic sites for its opening namely urethra below the neck, vagina or perineum. Because the Wolffian duct is not associated with any structures  Duplex Moiety Kidney with Ureteral Ectopia -Vikrant VK et al                                                  Journal of Pediatric Nephrology | Volume 4 | Number 1| 2016   44  below the pelvic floor in males, they do not present with dribbling of urine [6]. Furthermore, the relationship between ureteric openings of the duplex moieties was put forth by Carl Weigert and Meyer, popularly known as the Weigert-Meyer rule, which says that the ectopic ureter or the ureterocele associated with the upper moiety is caudal to the ureteral opening of the lower moiety [7]. The ectopic ureter is associated with dysplasia of the draining renal unit [8]. According to the Stephens hypothesis, the further the ectopic orifice located from the trigone, more severe is the dysplasia [9].  As discussed earlier, asymptomatic duplication can be left alone. Complex duplications warrant a thorough history taking and evaluation. Although USG and CT-IVU suffice to delineate the anatomy in most cases, MRU is the investigation of choice, limited by its cost and availability [10]. Because ureteral ectopia is associated with VUR, VCUG should be performed in every case [11]. However in all cases discussed above, there was no VUR. Renal scans are imperative to know the function of individual moieties as well the kidneys to plan surgical management and assess the prognosis.  The management in these cases is complex and has to be tailored according to the individual case scenario. There are various surgical options that can be broadly divided as renal level surgeries, ureter level surgeries or bladder level surgeries. As previously discussed, ectopy with duplication is associated with dysplasia hence upper moiety nephrectomy is advocated [12], after confirming the function on renal scans. In case the function in the dysplastic moiety is considerable, then ureteropyelostomy should be preferred. The third case discussed above is interesting because ectopia was associated with cross fused kidney, which is rare [13]. Ureteral ectopy with duplication although common are complex entities, which require a detailed clinical evaluation. MRU is the preferred diagnostic modality. The surgical principles of management are to treat ectopic ureter while preserving the renal function. The prognosis in most cases is excellent.         Conflict of Interest None declared  Financial Support None declared  References 1. Greene LF. Ureteral ectopy in females. Clin Obstet Gynaecol 1967;10: 147-150. 2. Burford CE, Glenn JE, Bumford EH. Ureteral ectopia: a review of the literature and 2 case reports. J Urol 1949;62: 211. 3. Skandalakis JE, Gray SW (eds). Embroyology for surgeons, 2nd edn. Williams & Wilkins, Baltimore Hong Kong London Sydney 1994; pp, 695-700. 4. John M Gatti, Harry P Koo, Jeffrey F Williams, J Patrick Murphy. Ureteral Duplication, Ureteral Ectopia, and Ureterocele. Retrieved November 20, 2015, from http://emedicine.medscape.com/article/1017202-overview.  5. Omer Oge, Burcin Ozeren, Ferah Sonmez. Nephron  sparing surgery in a duplex system associated with a vaginal ectopic ureter. Paediatr Nephrol 2001;16:1135-1136. 6. Craig A Peters, Richaed N Schlussel, Cathy Mendelsohn. Ectopic ureter, Ureterocoele and Ureteral anomalies. In: Wein AJ editor. Campbell-Walsh Urology. 9th ed. Philadelphia: Saunders; 2007; p, 3239-3240. 7. Farid S Haddad, Edmond E Griffin.  The Weigert – Meyer law demystified. JIMA 1996;28:16-19. 8. Yan YJ, Feng ZX, Min ZH, Jin YX. Single system ectopic ureters associated with renal dysplasia. Pediatr  Surg Intl 2004;20:851-854. 9. Li J, Hu T, Wang M, jiang X, Chen S, Huang L. Single ureteral ectopia with congenital renal dysplasia. J urol 2003;170: 558-559. 10. Thambidorai CR, Anuar Z. Magnetic resonance urography in duplex kidney with ectopic ureteral insertion. Journal of Indian Association of Pediatric Surgeons. 2011;16(3):115-117. doi:10.4103/0971-9261.83500. 11. Keating MA. Ureteral duplication anomalies: ectopic ureters and ureteral anomalies. In: Belman BA, King LR, Kramer SA (eds) Clinical Pediatric Urology, 4th edn, Martin Dunitz, London 2002; pp, 677-733. 12. Barret DM, Marek RS, Kelalis PP. Problems and solutions in 100 consecutiven ureteral duplications in children. J Urol 1975;114:126–130. 13. Bothra JM, Shah HS, Jayaswal S, Kumbhar V. Incontinence in crossed fused kidney: A rare association. Saudi Surg J 2015; 3:17-9.    

Duplex Moiety Kidney With Ureteral Ectopia; A Case Series

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