|Year : 2013 | Volume
| Issue : 3 | Page : 100-104
Y-type urethral duplication in children: Management strategy at our center
Sunita Singh, Jiledar Rawat
Department of Pediatric Surgery, King George Medical University, Lucknow, Uttar Pradesh, India
|Date of Web Publication||3-Aug-2013|
Department of Pediatric Surgery, King George Medical University, Lucknow-226 003, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Aims: Report of seven children with Y-type urethral duplication (YUD). Materials and Methods: (A) Four staged operations were performed in patients having extensive perineal dissection (for rectourinary fistula separation and anterior mobilization of ventral urethra (VU)), tension rectocutaneous anastomosis, and children who were not toilet trained). These stages are (1) diversion sigmoid colostomy with anterior mobilization of VU as perineal urethrostomy via anterior sagittal approach; (2) Orthotopic urethral (OU) reconstruction; (3) anastomosis of OU and perineal urethra; (4) colostomy closure with management of complications. (B) The patients having VU onto the perineum underwent single stage urethral reconstruction. Results: The VU was urethrorectal/urethroanal in five and urethroperineal in two. Low anorectal malformation and upper urinary tract anomalies were present in 57.1% (4/7) and 14.7% (1/7) patients, respectively. Buccal mucosa free graft, transverse inner preputial flap, and perineal skin were tubularized for OU reconstruction. Mean age at 1 st , 2 nd , 3 rd , and 4 th surgery was 5 ± 0.78, 28 ± 0.78, 36 ± 0.78, 49 ± 0.78 months respectively. Three patients needed surgery for complications (urethrocutaneous fistula in two and urethral diverticulum in one) in a mean 3.12 ± 0.34 years of follow-up. Final uroflowmetry and fecal continence were good in all patients. Conclusions: The YUD is a difficult entity to manage. Although, staged procedure appears to be time consuming, but good and promising results can be achieved by staging the procedure.
Keywords: Anourinary fistula, perineal urethrostomy, rectourinary fistula, urethral duplication, Y-type urethral duplication
|How to cite this article:|
Singh S, Rawat J. Y-type urethral duplication in children: Management strategy at our center. J Indian Assoc Pediatr Surg 2013;18:100-4
|How to cite this URL:|
Singh S, Rawat J. Y-type urethral duplication in children: Management strategy at our center. J Indian Assoc Pediatr Surg [serial online] 2013 [cited 2020 Dec 2];18:100-4. Available from: https://www.jiaps.com/text.asp?2013/18/3/100/116042
| Introduction|| |
The Y-type urethral duplication (YUD) is a rare congenital anomaly, constituting 6-30% of all urethral duplications (UD).  The Williams and Kenawi had classified UD as epispadiac, hypospadiac, spindle, and YUD. According to Effmann's classification, YUD is categorized under type IIA2 anomaly [Figure 1]. , In YUD, ventral urethra (VU) originates from bladder neck/or posterior urethra and opens either onto perineum or within rectum/anus. The orthotopic urethra (OU) is characteristically stenotic, thus VU is the only functional urethra. ,,,,, The clinical presentation is perineal urination or urination through the anus with urination in thin stream/drops or no urination through the thin/aplastic/atretic OU [Figure 2]. ,, Elderly patients can also present with recurrent urinary tract infection, epididymitis, and perineal abscess. ,, In YUD, both urethras are continent. ,
|Figure 1: Effmann's classification: Type IA: Incomplete distal UD Type IB: Incomplete proximal UD; Type IIA1 (Complete UD): Two-meatus non-communicating urethras arising independently from the bladder, Type IIA2 (Complete UD): Second channel arising from the first and exiting independently (including YUD), Type IIB: Complete UD bifurcate and rejoining at one meatus; Type III: UD as a component of caudal duplication|
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|Figure 2: Clinical presentation showing (a) perineal dominant urethra (ventral urethra) and atretic native urethra (orthotopic urethra), (b) micturation through rectum with anal stenosis and bifid scrotum|
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YUD and congenital urethra-cutaneous fistula (CUCF) have same clinico-radiological appearance, but latter had dominant OU. In YUD, VU can't be compromised as it is the only functional urethra (contrary to CUCF). ,,,
Till date, there is no definitive management strategy for YUD. ,,,,, Here, the author discussed their management strategy with discussion and relevant review of the literature.
| Materials and Methods|| |
This is a retrospective analysis of seven patients who underwent surgery for YUD from 2002 to 2012. The patients' characteristics are shown in [Table 1]. All patients were operated by single surgeon (second author). Four staged operations were performed in patients having extensive perineal dissection (for rectourinary fistula separation and anterior mobilization of ventral urethra (VU)), tension rectocutaneous anastomosis, and in children who were not toilet trained.
Stage 1: First stage surgery was performed at minimum age of 3 months. Through anterior sagittal approach, separation and anterior mobilization of VU from rectum/anal canal was done as perineal urethrostomy. Concurrent anoplasty was performed for associated anal stenosis with covering loop sigmoid colostomy.
Stage 2: Second stage surgery/orthotopic urethral reconstruction was performed at minimum age of one year. Preoperatively, intramuscular testosterone was given thrice, at dosage of 2 mg/kg at 3 weeks interval on outdoor visits. , The hypoplastic/stenotic/atretic OU was excised and simultaneous urethral reconstruction performed. The author left the anastomosis of long neourethral tube and perineal urethrostomy at this stage to prevent urinary contamination of graft/flaps.
The choice of technique was based on the site of dysgenic urethra. Tubularization of free buccal mucosa graft (BMGT) was used for the reconstruction of dysgenic penile urethra, and atretic proximal penile and bulbar urethra. Transverse inner preputial island flap based on dorsal penile dartos flap (TIPF-DD) was used for reconstruction of distal penile urethra. Perineal skin was tubularized in continuity with TIPF-DD for dysgenic anterior urethra [Figure 3]. The urethroplasty was augmented by vascular flaps (1 st and 3 rd cases by penile dartos flap, 6 th case by mid scrotal septal dartos flap). Glanuloplasty and meatoplasty was done. The Byar's skin flaps were used for penile coverage.
|Figure 3: (a) ventral urethral meatus (shown by vertical arrow), (b) Catheterization of ventral urethra, (c) anterior mobilization of rectal urethra through anterior sagittal approach, (d) patent distal urethra, agenesis of proximal penile and bulbar urethra, perineal urethrostomy with catheter in situ (right black arrow) and neoanus (left black arrow), (e, f) urethral reconstruction through perineal skin, scrotoplasty and anastomosis with perineal urethrostomy|
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In bifid scrotum, midline ventral penile incision was extended up to perineal urethrostomy. Bilateral scrotal flaps was raised, bulbar urethra reconstructed and over it scrotoplasty performed by multilayer closure of scrotal flaps. Thus, scrotoplasty also provides several layers of coverage over the neourethra.
A light compression dressing was done for 5 days. The urethral stent was kept in reconstructed neourethra by folding on itself as shown in [Figure 3]. The patients were put on intravenous antibiotics (ceftriaxone and amikacin) for 5 days and discharged on oral antibiotics with urethral stent in neourethra for 14 days. The neourethral stent was removed on 14 th postoperative day. During follow-up, neourethra was calibrated every month and urethrography performed before next stage surgery [Figure 4].
|Figure 4: (a) Urethrocystography showing patent neourethra, (b) postoperative VCUG showing urethral diverticulum|
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Stage 3: At an average of 6 months, urethrocystoscopy was done to ascertain the patency of neourethra. Thereafter, spatulation of proximal end of neourethra and perineal urethrostomy were done with end-to-end anastomosis over urethral stent. Mid scrotal septal dartos flap was used for augmentation of anastomosis in 1 st , 2 nd , 3 rd , and 5 th patients. The patients were discharged on 5 th postoperative day on oral antibiotics and urethral stent in situ. The urethral stent was removed on 10 th postoperative day.
Stage 4: At an average of 8 months, colostomy closure was done with appropriate management of complications of previous operations (urethrocutaneous fistula repair in 1 st and 4 th patients, diverticulecomy and urethral repair in 2 nd patient).
The patients having VU onto the perineum (6 th and 7 th case) were assured at birth and single stage urethral reconstruction was done after 1 year of age. The outcome was assessed 1 year after final surgery by uroflowmetry, voiding cystourethrogram (VCUG), and Kelly's fecal continence evaluation questionnaires. The data were analyzed using Statistical Package for the Social Sciences (SPSS) 17.0 version for Windows. Continuous variables were expressed as mean values with two standard deviation, median and ranges minimum to maximum, as well as in percentages.
| Results|| |
Total seven patients (five in neonatal, two beyond infancy) underwent surgery over 10 years. All were male, having sagittal type YUD. The median age of presentation was 20 days (ranged two days to one year). Mean age at 1 st , 2 nd , 3 rd , and 4 th surgery was 5 ± 0.78, 28 ± 0.78, 36 ± 0.78, and 49 ± 0.78 months, respectively. Preoperative voiding cystourethrogram (VCUG) was possible in only 6 th and 7 th patient; other patients underwent VCUG through perineal urethrostomy. The patient having multicystic dysplastic kidney (MCDK) was kept on the surveillance. The patients having atretic proximal penile and bulbar urethra (two patients) also had bifid scrotum. Low anorectal malformation (anal stenosis) was present in 57.1% (4/7) patients. The BMGT was used each for anterior urethra reconstruction and atretic (proximal penile + bulbar) urethra reconstruction. Perineal skin was tubularized along with TIPF-DD for long urethral reconstruction (dysgenic anterior urethral reconstruction). The TIPF-DD was used for distal penile urethra reconstruction in two patients.
Two patients needed urethrocutaneous fistula repair (one proximal penile, one penoscrotal junction) at a mean 3.12 ± 0.34 years of follow-up. The patient having long urethral reconstruction by BMGT developed distal penile ventral urethral diverticulum after 2 yrs of final surgery. The diverticulum was not associated with meatal stenosis. The diverticulum was excised and urethral repair was done. Final, uroflowmetry and VCUG were normal in all patients. In mean 3.12 ± 0.34 years of follow-up, fecal continence was good in patients having rectourinary/rectoanal fistula.
| Discussion|| |
The UD was first described by Aristotele and Vesalius.  Wagner differentiated YUD from the H-type anorectal malformation by the presence of transition epithelium in the fistulous tract. , The YUD is often associated withgenito-urinary and gastro-intestinal abnormalities. ,,,,, We have 14.7% (1/7 patients) of upper urinary tract anomalies contrary to 80% described in literature.  Contrary to the series of Mishra et al., (30%) we had higher (57.1%) association of low anorectal malformation. We didn't find any other anomaly (cloacal exstrophy, conjoined twins, VACTERL complex etc). 
The options for management of YUD are: (i) Anterior mobilization of fistulous tract, its anastomosis to midline scrotal skin tube, laying open of OU in first stage followed by penile urethroplasty in second stage;  (ii) laying open of OU, transposition of rectal urethra to the perineum in first stage followed by staged construction of OU;  (iii) covering colostomy, lengthening of ectopic rectal urethra (by strip of anterior anorectal wall), reconstruction of OU in first stage followed by scrotal skin tubularization (for mid urethra), securing of whole urethra in continuity, and colostomy closure in second stage;  (iv) progressive augmentation by dilation of the urethra anterior' (PADUA);  (v) mobilization of VU to the penoscrotal junction, tubularization of mid scrotal skin flap (bulbar and membranous urethra) and preputial flap (for penile urethra) in single stage. The authors do not agree with PADUA as repeated trauma leads to stricture formation. The authors believe that if native urethra is not good in quality or caliber, it must be discarded. ,
Although diversion colostomy is not universally advisable, it is authors' choice in patients having extensive perineal dissection (for rectourinary fistula separation, anterior mobilization of VU), tension rectocutaneous anastomosis, and children who are not toilet trained. Fecal diversion enhances healing of anorectum and survival of flaps/free grafts (in toilet untrained children). Perineal urethrostomy also enhances survival of flaps/free grafts. The strategy is especially useful in children <3 years of age, where voluntary bladder bowel control is not achieved, thus, it is very difficult to take care of the perineal wound, in close proximity to the anal canal. This may be the reason of good survival of free buccal mucosa graft.
We believe that planned one-stage reconstruction of long OU can offer comparable results. The advantage is that, if there is chance of secondary procedures (redourethroplasty/urethral diverticulum/stricture repair), approximately two-third of penile and scrotal vascular tissue is preserved for subsequent procedures.  The authors experience with primary BMGT is not satisfactory as development of urethral diverticulum (1/3 patients) occurred because of poor tensile strength of BMG (as it was not associated with meatal stenosis).  Importantly, urethral strictures, perhaps the most feared complication was not found in any. Although, the staged procedure appears to take long time, but overall stages of surgery were less than Haleblean et al., series with comparable results. 
To conclude, the complexity of rectourinary type YUD is because of difficult separation of VU from rectum, need for long urethral reconstruction and the care of perineal wound/flaps/grafts in toilet untrained children. In these situations, authors staged approach might help in good and promising results.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]