Case Report

Urethral Duplications in Children – A Case Series

Vuille-dit-Bille RN1,2*#, Leu S3#, R. Delcont M4, Ulf Kessler5, Zeino M5 and Klimek P1
1Department of Pediatric Surgery, Cantonal Hospital of Aarau, Switzerland
2Department of Pediatric Surgery, Children’s Hospital Colorado, USA
3Department of Pediatric Surgery, University of Zurich, Switzerland
4Department of Pediatric Surgery, University of Colorado School of Medicine, USA
5Department of Pediatric Surgery, Bern University Hospital, Switzerland
#Contribute equally for the manuscript

*Corresponding author: Raphael N Vuille-dit-Bille, Department of Pediatric Surgery, Children’s Hospital Colorado, 13123 E 16th Ave, Aurora, CO 80045, USA

Published: 14 Sep, 2018
Cite this article as: Vuille-dit-Bille RN, Leu S, R. Delcont M, Ulf Kessler, Zeino M, Klimek P. Urethral Duplications in Children – A Case Series. Clin Surg. 2018; 3: 2099.


Background: Urethral duplications are rare congenital anomalies with multiple anatomical variants. They mostly occur in the sagittal plane and can be associated with other congenital urogenital malformations. According to Effmann urethral duplications are classified into incomplete (type I), complete (type II), and complete associated with caudal duplication (type III).
Methods: We hereby describe three cases of urethral duplications and their respective treatment. Results: Two cases were type I duplications. One patient was treated by complete resection of the blind ending secondary urethra, whereas no treatment was performed in the second patient. The third case was a IIA-2 Y-subtype duplication that needed complex reconstruction.
Conclusions: There is no need for surgical management in asymptomatic patients with type I urethral duplications. Type IIA-2 Y-subtype duplications are extremely rare, and their treatment must be individualized according to anatomical and physiological features.
Keywords: Urethral duplication; Y-duplication; Urethral anomaly


Urethral duplications are seldomly seen urinary tract variants consisting of aberrant partial or complete development of an additional urethra [1]. They may be associated with different urogenital abnormalities, including bladder duplication and bladder exstrophy [2]. The etiology of this disease is not well understood. In the majority of cases, urethral duplications occur in the sagittal plane with the accessory urethra located dorsally to the orthotopic urethra [3]. The most commonly applied classification according to Effmann et al. divides urethral duplications into incomplete duplications (type I), complete duplications (type II), and duplications as a component of partial or complete caudal duplication (type III) [4]. Types I and II are further subdivided (Table 2) [5]. Whereas Type I duplications are typically clinically silent without urinary discharge or infections of the accessory urethra, complete duplications may be the cause for urinary incontinence and repeat urinary tract infections.
The aim of this case series is to describe the clinical presentation and respective treatment of this rare disease in three male children suffering from different types of urethral duplication.

Materials and Methods

We hereby present three male patients born between September 2008 and November 2013 with urethral duplications (Table 1). Thereof, two children had type IA duplications according to Effmann et al [4]. In one child, the ectopic urethra was successfully excised, whereas there was no treatment in the other child who also had Down syndrome. The third child presented with IIA- 2 Y-subtype urethral duplication which consisted of a dystopic urethral orifice into the anus and a dysplastic dorsal urethra. A three-staged procedure was performed to restore the anatomy. All children were treated at two pediatric surgery centers in Switzerland (the University Hospital of Bern and the Cantonal Hospital of Aarau).

Figure 1

Another alt text

Figure 1
Type 1A urethral duplication. Unrepaired blind ending secondary orifice in the axial plane lateral to the orthotopic urethra.

Figure 2

Another alt text

Figure 2
Type 1A urethral duplication. (A) Preoperative image of accessory pore originating 2 cm proximal to the orthotopic urethra. (B) Sounding of pore and marking of secondary urethra with betadine injection. (C) Complete accessory urethral resection (D) Histology showing fistula tract with urothelium and low-grade chronic inflammation.


Case 1
Case 1 describes a boy with Down syndrome who suffered from spontaneously resolving Myelodysplastic Syndrome (MDS) in the neonatal period. A secondary urethral orifice was found incidentally by the child’s paediatric oncologist at 5 years old (Figure 1). No urinary tract or local infections were noted. Ultrasound showed an unaffected bladder without wall thickening, as well as proportionately sized and normally structured kidneys and ureters. Micturating cystourethrogram demonstrated an anatomically positioned penile urethra arising from the common bladder neck. No vesicoureteral reflux was noted, and the bladder could be fully emptied. The secondary orifice was blind ending without connecting to the primary urethra or the bladder, corresponding to Type IA (Table 2). Because it was a short, incomplete and hence asymptomatic duplication, surgical excision was not performed in accordance with the child’s mother.
Case 2
Case 2 describes a boy with a blind-ending accessory urethral orifice incidentally discovered at an inguinal hernia repair follow-up when he was 10 months old (Figure 2A). The boy was asymptomatic, without discharge of urine from the accessory orifice, and otherwise healthy. Ultrasound revealed a blind ending secondary urethra 2 cm proximal to the orifice, along with unaffected intra and retroperitoneal organs. Eleven months after first recognition, complete circumcision due to symptomatic phimosis in combination with excision of the secondary urethra was performed. The pore was sounded (Figure 2B), and betadine solution was injected to mark the course of the secondary urethra. The pore was closely cut down to the orthotopic urethra and resected completely (Figure 2C). The operation was performed in an outpatient setting. Histologic examination confirmed a fistula tract with urothelium and low-grade chronic inflammation (Figure 2D). Postoperative follow-up 1.5 months after excision showed complete wound healing without relapse.
Case 3
Case 3 presented as a newborn boy with severe pyelonephritis. The patient passed urine rectally with only a few drops from the dorsal glandular meatus (Figure 3A), concomitant with IIA-2 Y-subtype urethral duplication. Further diagnostic work-up (renal ultrasound, micturating cystourethrogram, Tc-99m dimercaptosuccinic acid renal scintigraphy, and magnetic resonance urography) showed right-sided grade 4 vesicoureteral reflux with 10% function of the right kidney. Unilateral right-sided loop cutaneous ureterostomy [6] was performed without complication at 3 months old allowing retrograde urination from the bladder, and the child was discharged 4 days later after an uneventful post-operative course.
Separation and mobilization of the ventral urethra was performed at 14 months old using an anterior sagittal trans-anorectal approach. The hypoplastic distal urethra was resected, and urethral bed substitution using a pedicle preputial flap (first stage of the twostage repair according to Bracka [7]) was performed (Figure 3B). Surgery was uneventful. At 1.5 years old, the second stage of urethral reconstruction, consisting of tubularization of the graft, was done together with right-sided nephroureterectomy due to worsening of right-sided kidney function to less than 10% (Figure 3C and 3D). The scrotum bifidum was also corrected with scrotal transposition, a urethral stent was inserted, and a suprapubic catheter was placed.
The patient was anuric during the immediate postoperative period, so emergent abdominal ultrasound was performed. This showed an intraperitoneal urinoma together with pyelectasis of the remaining left kidney. Emergent reoperation revealed a left-sided ureteral injury by virtue of crossed dystopia of the kidney (left to right) that was not diagnosed before this operation. The ureter was mobilized to the left side, and ureteroneocystostomy using the psoas hitch ureteral reimplantation technique [8] was undertaken. Voiding cystourethrogram performed 18 days after the second stage of urethral reconstruction showed a small leakage at the middle section of the penile urethra. The suprapubic catheter was hence left in place. Repeat voiding cystourethrogram one week thereafter showed no fistula and a normal sized urethra, so the suprapubic catheter was removed. The subsequent five-month follow-up showed good cosmetic and functional results without any complications.

Figure 3

Another alt text

Figure 3
Urethral duplication IIA-2 Y-subtype repair. (A) Preoperative picture of hypoplastic, nonfunctional dorsal glandular meatus. (B) Resection of hypoplastic distal urethra and urethral bed substitution using a pedicle preputial flap, according to Bracka et al [7]. (C) Tubularization of graft, insertion of urethral stent, and correction of scrotum bifidum with scrotal transposition (D) completed urethroplasty.


Urethral duplication reflects a very rare urogenital malformation that occurs more frequently in males than females. Only a few more than 325 cases have been reported in the literature to date [9]. They typically occur in the sagittal plane [10] and consist of squamous epithelia surrounded by smooth muscle identical to the orthotopic urethra. The aetiology of urethral duplication is still not clear and is attributed to altered embryogenesis. The most commonly quoted hypothesis is the one of Patten and Barry, which purports a misalignment of the lateral folds of the genital tubercle and the ventral end of the cloacal membrane. Other authors describe urethral duplications as due to Mullerian duct termination and growth arrest of the urogenital sinus [10]. However, no single theory is able to explain all different subtypes of urethral duplication. Different anatomical variants of urethral duplication led to several different classification systems [11], with that proposed by Effmann and colleagues being the most used [3]. Effmann divides urethral duplications into three different types (Table 2). In a series of pediatric urology case reports, the relative frequency of type I, type II, and type III duplications was 25%, 63%, and 12%, respectively [9]. In the present case series, two type IA cases with blind ending accessory urethras and one IIA-2 Y-subtype are shown.
Most blind-ending accessory urethras are clinically asymptomatic and rarely associated with other anomalies. If symptoms exist, they may include sinus tract infection and mucus discharge. One child with type IA duplication in the present study was otherwise healthy, while the other child had Down syndrome. Both duplications were incidentally noted by health professionals during physical examination. There’s no need for therapeutic management in asymptomatic patients apart from cosmetic issues. Accordingly, the child with type IA duplication and symptomatic phimosis was treated by complete excision of the blind ending urethra, whereas the other child with Down syndrome was not treated.
In contrast to type I duplications, type II and type III duplications are associated with clinical symptoms including a doubled urinary stream, urinary incontinence, recurrent urinary tract infections, bladder outflow tract obstruction, and passing urine per anus with voiding [3,9,12-14]. Type II and III duplications occur along with more severe congenital anomalies. A variant of type IIA-2 duplication, referred to as the ‘IIA-2 Y-subtype’, describes a secondary urethra coursing into a second meatus that opens posteriorly into the rectum/ perineum [1]. This type is extremely rare with less than 20 cases being reported until the year 2000 and is associated with hypospadias, epispadias, cleft palate, congenital heart disease, tracheoesophageal fistula, and imperforate anus [9,10,14]. Type III, which describes complete or partial caudal urethral duplication, occurs most often with bladder and/or penis duplication, as well as the afore-mentioned abnormalities [3].
These duplications (type II and III) are hence typically diagnosed directly at birth [15]. Correspondingly, the child presenting with the IIA-2 Y-subtype duplication in this study showed repeated urinary tract infections, as well as higher grade vesicoureteral reflux with reduced kidney function. Surgical treatment was necessary to relieve the kidney and to restore anatomy. A three-step procedure was thereby chosen consisting of (i) unilateral loop cutaneous ureterostomy at 3 months old, (ii) separation and mobilization of the ventral urethra at 14 months old, and (iii) urethral reconstruction at 18 months old.


In general, there is no need for therapeutic management in asymptomatic patients with type I urethral duplications apart from cosmetic issues. Type IIA-2 Y-subtype duplications are extremely rare, and their treatment must be individualized according to anatomical and physiological features. Vesicoureteral reflux and repeated urinary tract infections are common co-findings of this subtype of urethral duplication.


This work was supported by the Research Council Cantonal Hospital Aarau [Grant 1410.000.076 to P.K., R.N.V., and M.Z].


  1. Alanee S, Gupta P, Gleich P, Shukla AR. Complete urethral duplication: Description of surgical approach mimicking distal epispadias repair. J Pediatr Urol. 2012;8(4):343-7.
  2. Baid M, Dutta A. Urethral duplication in a 15-year-old: case report with review of the literature. Rev Urol. 2014;16(3):149-51.
  3. Bracka A. A versatile two-stage hypospadias repair. Br J Plast Surg. 1995;48(6):345-52.
  4. Coleman RA, Winkle DC, Borzi PA. Urethral duplication: Cases of ventral and dorsal complete duplication and review of the literature. J Pediatr Urol. 2010;6(2):188-91.
  5. Gupta NP, Ansari MS, Aron M, Mandal S. Y duplication of urethra with complete atresia of the orthotopic channel: 1-stage reconstruction. J Urol. 2000;163(3):949-50.
  6. Haleblian G, Kraklau D, Wilcox D, Duffy P, Ransley P, Mushtaq I. Y-type urethral duplication in the male. BJU Int. 2006;97(3):597-602.
  7. Levin TL, Han B, Little BP. Congenital anomalies of the male urethra. Pediatr Radiol. 2007;37(9):851-62.
  8. Manassero F, Mogorovich A, Fiorini G, Di Paola G, De Maria M, Selli C. Ureteral reimplantation with psoas bladder hitch in adults: A contemporary series with long-term followup. Scientific World J. 2012;379316.
  9. Mane SB, Obaidah A, Dhende NP, Arlikar J, Acharya H, Thakur A, et al. Urethral duplication in children: our experience of eight cases. J Pediatr Urol. 2009;5(5):363-7.
  10. Mori K, Shin T, Tobu S, Noguchi M, Sumino Y, Sato F, et al. A case of urethral duplication arising from the posterior urethra to the scrotum with urinary stone in a 6-year-old male. Case Rep Pediatr. 2014;290623.
  11. Onofre LS. Urethral duplication--a wide spectrum of anomalies. J Pediatr Urol. 2013;9(6, Pt B):1064-71.
  12. Podesta ML, Medel R, Castera R, Ruarte AC. Urethral duplication in children: surgical treatment and results. J Urol. 1998;160(5):1830-3.
  13. Singh S, Rawat J. Y-type urethral duplication in children: Management strategy at our center. J Indian Assoc Pediatr Surg. 2013;18(3):100-4.
  14. Wagner JR, Carr MC, Bauer SB, Colodny AH, Retik AB, Hendren WH. Congenital posterior urethral perineal fistulae: A unique form of urethral duplication. Urology. 1996;48(2):277-80.
  15. Zilberman DE, Golomb J, Kitrey ND, Inbar Y, Heyman Z, Kleinnbaum Y, et al. Long-term urinary bladder function following unilateral refluxing low loop cutaneous ureterostomy. Korean J Urol. 2012;53(5):355-9.