561760 research-article2014
TAU0010.1177/1756287214561760Therapeutic Advances in UrologyA. A. AbouZeid et al.
Therapeutic Advances in Urology
Original Research
The double urethra: revisiting the surgical classification Amr AbdelHamid AbouZeid, Hesham Soilman Safoury, Shaimaa AbdelSattar Mohammad, Osama El-Naggar, Ahmed Medhat Zaki, Tarek Ahmed Hassan and Sameh Abdel Hay
Ther Adv Urol 2015, Vol. 7(2) 76–84 DOI: 10.1177/ 1756287214561760 © The Author(s), 2014. Reprints and permissions: http://www.sagepub.co.uk/ journalsPermissions.nav
Abstract Aim: The aim of this article was to describe our experience with 14 patients with double urethra. Patients and methods: We retrospectively examined the patients’ records including their clinical presentations, investigations, operative findings, and outcome. In addition to Effmann’s classification, we used a newly proposed classification that depends on the orientation of the double urethral channels. Results: During the last 15 years, 18 patients were diagnosed to have double urethra at our pediatric surgical unit. We excluded four patients with ‘Y-type’ urethral duplication. The remaining 14 patients were divided into either sagittal or collateral duplication. Their age at presentation ranged from the neonatal period to 9 years. The sagittal urethral duplication included 12 male patients. All patients had two urethral channels, one above the other. The dorsal urethral channel was always characterized by poor function and ectopic course. The ventral channel was always the more functioning urethra, with a normal course from the urinary bladder to end either at an orthotopic meatus (subgroup A), or more proximally in a hypospadiac location (subgroup B). The collateral urethral duplication included two patients. Both patients were associated with duplication of the urinary bladder and the external genitalia as a part of caudal duplication syndrome. In this group, both urethrae had comparable function, lying side by side, and each draining a separate urinary bladder. Conclusion: The double urethra is a diverse spectrum comprising different pathologies. Our proposed classification system of duplicated urethras is clinically relevant as it guides surgical management and allows prognostication of outcome.
Keywords: accessory urethra, epispadias, hypospadias, urethral duplication
Introduction The double urethra is a rare anomaly which a pediatric urologist might face occasionally in their career. The disease comprises such a wide spectrum of anatomical variations that most authors would agree to manage each case on individual basis [Prasad et al. 1999; Salle et al. 2000]. A single embryological explanation cannot be applied for all of the different types of double urethra [Casselman and Williams, 1996]; this would suggest that the different entities included under the term ‘double urethra’ are not just a single pathology. Several classifications have been proposed trying to distinguish between the different types of
urethral duplication, and to define an appropriate plan of management [Podesta et al. 1998]. Based on the location of the external urinary meatii, Williams and Kenawi described epispadiac, hypospadiac, spindle, and collateral types [Williams and Kenawi, 1975]. However, their classification has been criticized for lacking many of the anatomical details [Salle et al. 2000]. A year later, Effmann and colleagues introduced their famous classification which was based on detailed radiological anatomy as seen in the patients’ urethrograms [Effmann et al. 1976]. The latter has been described as the most exhaustive classification [Mane et al. 2009], and has gained widespread acceptance
Correspondence to: Amr AbdelHamid AbouZeid, MD Ain-Shams University, Lotefy El-Sayed, Abbassia, Cairo, Egypt amrabdelhamid@hotmail. com Hesham Soilman Safoury, MD Shaimaa AbdelSattar Mohammad, MD Osama El-Naggar, MD Ahmed Medhat Zaki, MD Tarek Ahmed Hassan, MD Sameh Abdel Hay, MD Ain-Shams University, Cairo, Egypt
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AA AbouZeid, HS Safoury et al. among radiologists and urologists as well [Salle et al. 2000]. Here, we report our experience with a group of patients with double urethra that were managed at our unit over recent years; in addition to a suggested classification that is inspired from the old surgical classifications [Williams and Kenawi, 1975; Woodhouse and Williams, 1979; Stephens, 1983]. Patients and methods The study included all patients with double urethra diagnosed at our unit during the last 15 years. We excluded patients with the so-called ‘Y-type’ urethral duplication, since it is debatable whether to be classified as a subtype of urethral duplication or a congenital urethroperineal fistula [Bates and Lebowitz, 1995; Stephens and Donnellan, 1977; Salle et al. 2000]. Also, we excluded mild distal types (abortive types) often encountered with hypospadias. We retrospectively examined the patients’ records including their clinical presentations, investigations, operative findings, and outcome. Investigations included renal ultrasonography to screen for possible renal anomalies, ascending, and micturating cystourethrograms. In addition to Effmann and colleagues’ classification [Effmann et al. 1976], we used a newly proposed classification that is inspired from old surgical classifications [Williams and Kenawi, 1975] and depends on the orientation of the double urethral channels (Figure 1). First we classified the double urethra into either sagittal type (one urethra above the other) or collateral type (lying side by side). The collateral type represents a part of partial or complete caudal duplication; while in the sagittal type, the dorsal urethral channel was always the accessory one. Results During the last 15 years, 18 patients were diagnosed to have double urethra at our pediatric surgical unit. We excluded four patients with congenital urethro-anal communication, also known as ‘Y-type’ urethral duplication (these cases were discussed in another separate report). The study included the remaining 14 patients that were divided into either sagittal or collateral duplication. Their age at presentation ranged from the neonatal period to 9 years.
The sagittal urethral duplication group included 12 male patients. All patients had two urethral channels, one above the other. The dorsal urethral channel (whether it was complete or incomplete, communicating or blind) was always characterized by poor function and ectopic course; in other words, it was always the accessory one (Figures 2, 3 and 4(e,f)). On the other hand, the ventral channel was always the one with better function: wider caliber, and containing the veru and sphincteric control. The ventral urethra had a normal course from the urinary bladder to end either at an orthotopic meatus (subgroup A), or more proximally in a hypospadiac location (subgroup B). A degree of preputial deficiency either dorsal or ventral (in subgroups A and B, respectively) was noticed in all uncircumcised patients (Figures 4e and 5). The subgroup (A) was more common and included nine male patients (Table 1). The dorsal accessory meatus was located on the dorsum of the glans in four patients, dorsum of coronal sulcus in two, peno-pubic in one, and an epispadiac urethral plate replaced the dorsal accessory channel in two. The treatment was directed towards the dorsal accessory urethra which was either excised, anastomosed to the ventral urethra (urethro-urethrotomy), or left untreated. Excision of the dorsal accessory urethra was found to be both feasible and safe, and has the advantage of correcting associating dorsal chordae that was evident in two cases (Figure 6). Lately, the author has applied a new modification for excising the dorsal accessory urethra by stripping (Figure 7). Whatever the procedure, the outcome was generally very good and satisfactory due to the absence of other associating complex urological anomalies. An exception was in the last two cases that were associated with an ‘open’ epispadiac dorsal urethral plate as a part of epispadias-exstrophy complex. Even in the latter two cases, the prognosis was still good. After excision of the dorsal epispadiac accessory urethral plate and closure of the urinary bladder, the patients were continent to urine through the spared ventral urethral channel (Figure 8) [Salle et al. 2001]. The subgroup (B) included three male patients (Figures 3 and 4(e,f)). Urethroplasties were needed to correct the position of the hypospadiac ventral urethral meatus. In addition, the condition was usually complicated by the presence of other complex urological anomalies that had a major impact on the prognosis in these
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Peno-pubic
An epispadiac urethral plate replacing the accessory dorsal channel. An epispadiac urethral plate replacing the accessory dorsal channel and continuous with exstrophy of the urinary bladder.
4 months
6 months
3 months
2 years
Neonate
5 (Figures 6 and 7)
6 (Figure 5(a–c))
7 (Figure 5(d,e)) 8
9 (Figure 8)
Dorsum of the coronal sulcus
Dorsum of the coronal sulcus
Dorsum of the glans
5 years
Dorsum of the glans
Dorsum of the glans
4
6 months
2 (Figure 2b)
Dorsum of the glans
6 months
6 months
1 (Figure 2a)
Position of dorsal accessory meatus
3 (Figures 2c and 4c,d)
Age
Case
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Dorsal preputial deficiency
Circumcised at presentation; presence of dorsal chordae. Dorsal preputial deficiency helped in detecting the accessory channel. Mild dorsal chordae. Dorsal preputial deficiency helped in detecting the accessory channel Dorsal preputial deficiency Dorsal preputial deficiency
Circumcised at presentation
Circumcised at presentation Circumcised at presentation
Penile anatomy
Type IIA1
Type I
Type I
Type I
Type I
Type IIA1 (complete patent accessory urethra with separate origin from bladder) Type IIA2 (the two urethral channels are communicating with a common origin from the bladder) Type I
Type I (blind accessory urethral tube)
Effmann classification
Bladder exstrophy
Ano-rectal stenosis
Associated anomalies
Excision of the accessory epispadiac dorsal urethral plate + bladder closure.
Excision of peno-pubic sinus Excision of the accessory epispadiac dorsal urethral plate
Just circumcision
Excision of dorsal accessory urethra by stripping Excision of dorsal accessory urethra by stripping
Urethro-urethrotomy + glanuloplasty
Excision of dorsal accessory urethra
No treatment
Management
Table 1. Subgroup A of the sagittal urethral duplication. All patients have a main ventral urethra ending at an orthotopic external urinary meatus.
Therapeutic Advances in Urology 7(2)
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AA AbouZeid, HS Safoury et al.
The double urethra
Y-type (congenital urethroperineal fistula)
True duplica on
Dorsal accessory urethra
(4 cases)
(collateral type)
(sagi al type)
(2 cases)
(12 cases)
excluded from the study
part of caudal duplica on syndrome
This group is usually associated with other organ duplica ons (urinary bladder, external genitalia, gastrointes nal). Careful planning and collabora on of other special es are required in their management.
Subgroup A ventral meatus is orthotopic
Subgroup B ventral meatus is hypospadiac
(9 cases) (Excellent prognosis)
Managment: Excision of dorsal accessory urethra can be offered to all cases irrespec ve to their different radiological anatomy
(3 cases) (Gaurded prognosis according to the presence of associa ng urological anomalies)
Managment: Managment of other associa ng urological anomalies. Urethroplasty for hypospadiac ventral meatus + excision of dorsal accessory channel.
Figure 1. Newly proposed classification and algorithm for the double urethra.
Figure 2. Urethrograms of three male patients with sagittal urethral duplication (subgroup A). The three patients have a main ventral urethra ending at an orthotopic external urinary meatus, and another dorsal accessory urethral channel. The dorsal accessory urethra has a different anatomy in each case: (a) blind dorsal accessory urethra (Effmann type I); (b) complete patent dorsal accessory urethra with separate origin from bladder (Effmann type IIA1), note that the ventral urethral channel appears wider and contain the veru (white arrow pointing to the filling defect in the posterior urethra); (c) a common posterior urethra arises from the bladder which then bifurcates into a dorsal accessory urethra, and a ventral main urethra (Effmann type IIA2). The three patients share the same therapeutic decision (excision of the dorsal accessory urethra), and the good prognosis due to absence of other associating urological anomalies.
patients (vesico-ureteric reflux with marked hydro-ureteronephrosis and renal dysplasia, crossed renal ectopia, ectopic ureteral insertion in posterior urethra).
On the other hand, the collateral urethral duplication group included two patients, one of whom was a female (the only female in this series). Both patients were associated with duplication of the
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Therapeutic Advances in Urology 7(2) classification in distinguishing between the different types of the anomaly in a way that would be reflected in the therapeutic decision and prognosis.
Figure 3. Male patient with sagittal urethral duplication (subgroup B). (a) The ventral urinary meatus is hypospadiac (arrow), and the dorsal is orthotopic. (b) The patient urethrogram showing the ectopic course of the dorsal urethral channel from the urinary bladder. Despite having its meatus in a normal location, the dorsal urethra is still the accessory one (ectopic course and poor function). Also, there is reflux of contrast into a hugely dilated ectopic ureter.
urinary bladder and the external genitalia as a part of caudal duplication syndrome (Figure 9). In this group, both urethrae had comparable function, lying side by side, and each draining a separate urinary bladder (two separate urinary systems). Treatment of such complex anomalies required staged operations that are beyond the scope of this report. Discussion Reviewing the double urethra is a tedious work that has been described by Boissonnat as a thankless task for the many confusing titles and unclear case reports leading to errors that are passed from one author to another when quoted and requoted [Boissonnat, 1961]. The several attempts to classify the double urethra have been considered unsatisfactory, until Effmann and colleagues presented their classification that included many details based on the radiological anatomy [Effmann et al. 1976]. By avoiding the various theories of embryogenesis, their classification has been described as a practical one. In this report, we retrospectively gathered our experience with cases of the double urethra. We proposed a classification that is inspired from old surgical classifications and highlighting the embryological explanations. We believe that it may be superior to the currently widely accepted
First we excluded patients with the so-called Y-type urethral duplication, also known as congenital urethro-anal fistula [Bates and Lebowitz, 1995; Stephens and Donnellan, 1977]. These were discussed in a separate report. The embryological explanation postulates the occurrence of a vascular accident leading to the maldevelopment of the penile urethra [Williams and Kenawi, 1975]. This would have a backward effect on the developing uro-rectal septum causing misalignment of its components that results in the persistence of a cloacal lumen communicating between the urethra and the anal canal. Williams and Kenawi considered this communicating urethroanal tract to be primarily a fistula that developed early enough to acquire some of the characteristics of the urethra [Williams and Kenawi, 1975]. The second group is the collateral urethral duplication. The fact that the plane of the double urethrae in the collateral duplication is at a right angle to that of the sagittal duplication should indicate that the two conditions must be of different origins [Williams and Kenawi, 1975]. Midline structures (such as the urinary bladder) are formed by fusion of lateral components derived from each side. It appears that each of these lateral components is capable of forming a complete organ upon failure of midline fusion [Williams and Kenawi, 1975]. This represents a true duplication in which both urethrae are functionally comparable. Representing a part of the caudal duplication syndrome, this group is usually associated with other organ duplications (urinary bladder, external genitalia, gastrointestinal). Careful planning and collaboration of other specialties are required in their management. The most common group was the sagittal urethral duplication or accessory urethra. This is characterized by the presence of two urethral channels, one above the other. Embryologically, the genital tubercle arises from two lateral components (analgens). It has been postulated that a posterior shift of the lateral analgens of the genital tubercle would result in their fusion behind the uro-genital membrane [Stephens, 1983]. The latter will break down exposing the epithelium on the dorsum of the penis. The gross posterior misplacement of the analgens of the genital tubercle
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AA AbouZeid, HS Safoury et al.
Figure 4. Clinical photographs (a,c,e) and their respective urethrograms (b,d,f) of three male patients diagnosed to have double urethra. Their urethrograms share a common radiological anatomy (a common posterior urethra arising from the bladder which then bifurcates into two channels, Effmann type II A2). However, each case has a different therapeutic decision and prognosis. (a, b) An 18-month old male passing urine per anus during otherwise normal micturition (Y-type duplication or congenital urethro-anal fistula; excluded from this study). The therapeutic decision was excision of the posterior ‘ventral’ tract communicating between the urethra and the anal canal. (c, d) Six-month-old male with double urinary stream (ventral urethral meatus is orthotopic ‘subgroup A’). The therapeutic decision is to excise the dorsal accessory urethra or perform an urethro-urethrotomy, with excellent prognosis due to absence of associating urological anomalies. (e, f) A 2-year-old male with double urethra (ventral urethral meatus is hypospadiac ‘subgroup B’). Although the dorsal meatus is in a more normal location on the dorsum of the glans, yet the dorsal channel still represents the accessory urethra with its narrow caliber (poor function) and ectopic course (arising from the dorsum of the ventral main urethra). The prognosis is deeply affected in this subgroup by the presence of other associating complex urological anomalies (urinary reflux in an ectopically inserted right ureter with renal dysplasia).
results in complete epispadias. However, intermediate degrees of misplacement can result in the formation of two urethral plates on the dorsal and ventral aspects of the genital tubercle [Stephens, 1983]. What distinguishes our classification is using a single term ‘the dorsal accessory urethra’ for all of the sagittal duplications. This is in contrast to previous classifications that mentioned epispadiac and hypospadiac types according to the location of the external urinary meatii [Williams and Kenawi, 1975; Woodhouse and Williams, 1979]. The dorsal channel had always a poor function and an ectopic epispadiac course even when its meatus was located in a more normal position (Figures 3 and 4(e,f)). On the other
hand, the ventral channel was always the more functioning urethra, with a normal course from the urinary bladder to end either at an orthotopic meatus (subgroup A), or more proximally in a hypospadiac location (subgroup B). Subclassifying the sagittal duplication group was not only for anatomical reasons (location of the ventral urinary meatus), but also for the different prognosis in both subgroups. The prognosis was excellent in subgroup A in contrast to subgroup B that was associated with other complex urological anomalies. In this report, we will focus on the treatment of the sagittal urethral duplication (accessory
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Therapeutic Advances in Urology 7(2)
Figure 5. Dorsal preputial deficiency associating the sagittal urethral duplication (subgroup A). (a, b, c) Six-month-old male patient diagnosed at circumcision to have a dorsal accessory urethra; this was suspected due to the presence of dorsal preputial deficiency. (d, e) One-year-old male patient with dorsal preputial deficiency, underwent excision of peno-pubic sinus (arrow) at the age of 3 months.
Figure 6. Four-month-old male with dorsal accessory urethra (subgroup A): (a) associating dorsal chordae; (b) release of fibrous bands related to the dorsal accessory urethra at the base of penile shaft; (c) after excision of accessory urethra, correction of penile chordae, and circumcision.
urethra). Individually tailored treatment is advised by most authors [Prasad et al. 1999]. Also, there is a consensus on treating only symptomatic patients (significant functional or cosmetic problems) [Salle et al. 2000; Arena et al. 2007; Coleman et al. 2010]. The aim of the treatment is to preserve renal function, achieve continence and the best cosmetic outcome. The ventral channel is always the functioning urethra (containing the veru and sphincters), and has to be preserved. In subgroup B, the ventral urethra is hypospadiac, and a suitable urethroplasty is needed as for hypospadias; in addition to other necessary procedures to deal with commonly
associating urological anomalies. The treatment in subgroup A is simpler, and is directed towards the dorsal accessory channel (only for symptomatic patients with significant functional or cosmetic problems). Obliteration of the accessory channel by injection of sclerosing agent or other ablative interventions has been reported; however, these techniques have been abandoned for fear of corporeal thrombosis and fibrosis [Salle et al. 2000]. When both meatii are near each other in an apical position (Figure 4(c)), an urethro-urethrotomy can be performed by joining both urethral openings into a single orthotopic meatus [Alanee et al. 2012]; however, there is the
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Figure 7. Steps of excision of the dorsal accessory urethra by stripping (a) midline longitudinal incision in the tunica for dissecting out the proximal end of the dorsal accessory channel; (b, c) the proximal end of the accessory urethra is tied to the catheter inside, which is then pulled, stripping the urethra out; (d, e) the accessory urethra is excised with minimal disturbance to the glans and collar of the foreskin.
Figure 8. Male neonate with bladder exstrophy and sagittal urethral duplication. This combination has greatly improved the prognosis of bladder exstrophy in this patient: (a) the dorsal accessory epispadiac urethral plate has spared a functioning ventral urethra; (b) excision of the dorsal accessory epispadiac urethral plate and closure of the urinary bladder.
Figure 9. Collateral urethral duplication (caudal duplication syndrome). (a) Male patient with double penis, omphalocoele, and meningiocoele. (b) Cystogram showing double urinary bladder. (c) Female patient with double urethra, double external genitalia, and lipomeningiocoele. (d) MRI, axial T2WI showing double urinary bladder and double rectum.
potential risk for meatal stenosis and obliteration of dorsal meatus. Surgical excision of the dorsal accessory urethra has been reported as the best therapeutic option, and should be performed in a delicate way to avoid damage to the external
sphincter and neurovascular bundle [Salle et al. 2000]. Significant dorsal penile curvature which is sometimes associating the condition can also be corrected during the operation. The author has applied a modification for excising the dorsal
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Therapeutic Advances in Urology 7(2) accessory urethra by stripping, which eliminates the need for distal dissection with minimal disturbance to the glans. Like other studies on urethral duplication, this one is limited by being a retrospective study over a long period of time, and by the relative small number of cases for such a diverse anomaly. Also, some rare variants have not been encountered, such as the spindle type (Effmann type IIB). Searching through the literature, we have found very few case reports describing the latter type [Williams and Kenawi, 1975; Effmann et al. 1976]; with a warning to exclude the possible iatrogenic causes (false passage as the cause of such rare anatomical arrangement). However, to the best of the authors’ knowledge, this is one of the largest case series described in the literature. We believe our newly proposed classification can provide a comprehensive guide for managing most cases of the double urethra. Conclusion The double urethra is a diverse spectrum comprising different pathologies. Our proposed classification system of duplicated urethras is clinically relevant as it guides surgical management and allows prognostication of outcome. Conflict of interest statement The authors declare that there is no conflict of interest. Funding This research received no specific grant from any funding agency in the public, commercial, or notfor-profit sectors.
References
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SAGE journals
Alanee, S., Gupta, P., Gleich, P. and Shukla, A. (2012) Complete urethral duplication: description of surgical approach mimicking distal epispadias repair. J Pediatr Urol 8: 343–347. Arena, S., Arena, C., Scuderi, M., Sanges, G., Arena, F. and Di Benedetto, V. (2007) Urethral duplication
in males: our experience in ten cases. Pediatr Surg Int 23: 789–794. Bates, D. and Lebowitz, R. (1995) Congenital urethroperineal fistula. Radiology 194: 501–504. Boissonnat, P. (1961) Two case reports of complete double urethra with a single bladder. Br J Urol 33: 453–461. Casselman, J. and Williams, D. (1996) Duplication of the urethra. Acta Urol Belg 34: 535–541. Coleman, R., Winkle, D. and Borzi, P. (2010) Urethral duplication: cases of ventral and dorsal complete duplication and review of the literature. J Pediatr Urol 6: 188–191. Effmann, E., Lebowitz, R. and Collodny, A. (1976) Duplication of the urethra. Radiology 119: 179–185. Mane, S., Obaidah, A., Dhende, N., Arlikar, J., Acharya, H., Thakur, A. et al. (2009) Urethral duplication in children: our experience of eight cases. J Pediatr Urol 5: 363–367. Podesta, M., Medel, R., Castera, R. et al. (1998) Urethral duplication in children: surgical treatment and results. J Urol 160: 1830–1833. Prasad, N., Vivekanandhan, K., Ilangovan, G. and Prabakaran, S. (1999) Duplication of the urethra. Pediatr Surg Int 15: 419–421. Salle, J., Sibai, H., Jacobson, A., Fehri, M., Brzezinski, A. and Homsy, Y. (2001) Bladder exstrophy associated with complete urethral duplication: a rare malformation with excellent prognosis. J Urol 165: 2434–2437. Salle, J., Sibai, H., Rosenstein, D., Elias, B. and Jacques, C. (2000) Urethral duplication in the male: review of 16 cases. J Urol 163: 1936–1940. Stephens, F. (1983) Abnormal embryology - cloacal dysgenesis. In: Stephens, F. (ed.), Congenital Malformations of the Urinary Tract. New York, NY: Praeger Scientific, pp. 15–52. Stephens, F. and Donnellan, W. (1977) “H-type” urethroanal fistula. J Ped Surg 12: 95–102. Williams, D. and Kenawi, M. (1975) Urethral duplications in the male. Eur Urol 1: 209–215. Woodhouse, C. and Williams, D. (1979) Duplications of the lower urinary tract in children. Br J Urol 51: 481.
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TAU0010.1177/1756287214561760Therapeutic Advances in UrologyA. A. AbouZeid et al.
Therapeutic Advances in Urology
Original Research
The double urethra: revisiting the surgical classification Amr AbdelHamid AbouZeid, Hesham Soilman Safoury, Shaimaa AbdelSattar Mohammad, Osama El-Naggar, Ahmed Medhat Zaki, Tarek Ahmed Hassan and Sameh Abdel Hay
Ther Adv Urol 2015, Vol. 7(2) 76–84 DOI: 10.1177/ 1756287214561760 © The Author(s), 2014. Reprints and permissions: http://www.sagepub.co.uk/ journalsPermissions.nav
Abstract Aim: The aim of this article was to describe our experience with 14 patients with double urethra. Patients and methods: We retrospectively examined the patients’ records including their clinical presentations, investigations, operative findings, and outcome. In addition to Effmann’s classification, we used a newly proposed classification that depends on the orientation of the double urethral channels. Results: During the last 15 years, 18 patients were diagnosed to have double urethra at our pediatric surgical unit. We excluded four patients with ‘Y-type’ urethral duplication. The remaining 14 patients were divided into either sagittal or collateral duplication. Their age at presentation ranged from the neonatal period to 9 years. The sagittal urethral duplication included 12 male patients. All patients had two urethral channels, one above the other. The dorsal urethral channel was always characterized by poor function and ectopic course. The ventral channel was always the more functioning urethra, with a normal course from the urinary bladder to end either at an orthotopic meatus (subgroup A), or more proximally in a hypospadiac location (subgroup B). The collateral urethral duplication included two patients. Both patients were associated with duplication of the urinary bladder and the external genitalia as a part of caudal duplication syndrome. In this group, both urethrae had comparable function, lying side by side, and each draining a separate urinary bladder. Conclusion: The double urethra is a diverse spectrum comprising different pathologies. Our proposed classification system of duplicated urethras is clinically relevant as it guides surgical management and allows prognostication of outcome.
Keywords: accessory urethra, epispadias, hypospadias, urethral duplication
Introduction The double urethra is a rare anomaly which a pediatric urologist might face occasionally in their career. The disease comprises such a wide spectrum of anatomical variations that most authors would agree to manage each case on individual basis [Prasad et al. 1999; Salle et al. 2000]. A single embryological explanation cannot be applied for all of the different types of double urethra [Casselman and Williams, 1996]; this would suggest that the different entities included under the term ‘double urethra’ are not just a single pathology. Several classifications have been proposed trying to distinguish between the different types of
urethral duplication, and to define an appropriate plan of management [Podesta et al. 1998]. Based on the location of the external urinary meatii, Williams and Kenawi described epispadiac, hypospadiac, spindle, and collateral types [Williams and Kenawi, 1975]. However, their classification has been criticized for lacking many of the anatomical details [Salle et al. 2000]. A year later, Effmann and colleagues introduced their famous classification which was based on detailed radiological anatomy as seen in the patients’ urethrograms [Effmann et al. 1976]. The latter has been described as the most exhaustive classification [Mane et al. 2009], and has gained widespread acceptance
Correspondence to: Amr AbdelHamid AbouZeid, MD Ain-Shams University, Lotefy El-Sayed, Abbassia, Cairo, Egypt amrabdelhamid@hotmail. com Hesham Soilman Safoury, MD Shaimaa AbdelSattar Mohammad, MD Osama El-Naggar, MD Ahmed Medhat Zaki, MD Tarek Ahmed Hassan, MD Sameh Abdel Hay, MD Ain-Shams University, Cairo, Egypt
76 http://tau.sagepub.com
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AA AbouZeid, HS Safoury et al. among radiologists and urologists as well [Salle et al. 2000]. Here, we report our experience with a group of patients with double urethra that were managed at our unit over recent years; in addition to a suggested classification that is inspired from the old surgical classifications [Williams and Kenawi, 1975; Woodhouse and Williams, 1979; Stephens, 1983]. Patients and methods The study included all patients with double urethra diagnosed at our unit during the last 15 years. We excluded patients with the so-called ‘Y-type’ urethral duplication, since it is debatable whether to be classified as a subtype of urethral duplication or a congenital urethroperineal fistula [Bates and Lebowitz, 1995; Stephens and Donnellan, 1977; Salle et al. 2000]. Also, we excluded mild distal types (abortive types) often encountered with hypospadias. We retrospectively examined the patients’ records including their clinical presentations, investigations, operative findings, and outcome. Investigations included renal ultrasonography to screen for possible renal anomalies, ascending, and micturating cystourethrograms. In addition to Effmann and colleagues’ classification [Effmann et al. 1976], we used a newly proposed classification that is inspired from old surgical classifications [Williams and Kenawi, 1975] and depends on the orientation of the double urethral channels (Figure 1). First we classified the double urethra into either sagittal type (one urethra above the other) or collateral type (lying side by side). The collateral type represents a part of partial or complete caudal duplication; while in the sagittal type, the dorsal urethral channel was always the accessory one. Results During the last 15 years, 18 patients were diagnosed to have double urethra at our pediatric surgical unit. We excluded four patients with congenital urethro-anal communication, also known as ‘Y-type’ urethral duplication (these cases were discussed in another separate report). The study included the remaining 14 patients that were divided into either sagittal or collateral duplication. Their age at presentation ranged from the neonatal period to 9 years.
The sagittal urethral duplication group included 12 male patients. All patients had two urethral channels, one above the other. The dorsal urethral channel (whether it was complete or incomplete, communicating or blind) was always characterized by poor function and ectopic course; in other words, it was always the accessory one (Figures 2, 3 and 4(e,f)). On the other hand, the ventral channel was always the one with better function: wider caliber, and containing the veru and sphincteric control. The ventral urethra had a normal course from the urinary bladder to end either at an orthotopic meatus (subgroup A), or more proximally in a hypospadiac location (subgroup B). A degree of preputial deficiency either dorsal or ventral (in subgroups A and B, respectively) was noticed in all uncircumcised patients (Figures 4e and 5). The subgroup (A) was more common and included nine male patients (Table 1). The dorsal accessory meatus was located on the dorsum of the glans in four patients, dorsum of coronal sulcus in two, peno-pubic in one, and an epispadiac urethral plate replaced the dorsal accessory channel in two. The treatment was directed towards the dorsal accessory urethra which was either excised, anastomosed to the ventral urethra (urethro-urethrotomy), or left untreated. Excision of the dorsal accessory urethra was found to be both feasible and safe, and has the advantage of correcting associating dorsal chordae that was evident in two cases (Figure 6). Lately, the author has applied a new modification for excising the dorsal accessory urethra by stripping (Figure 7). Whatever the procedure, the outcome was generally very good and satisfactory due to the absence of other associating complex urological anomalies. An exception was in the last two cases that were associated with an ‘open’ epispadiac dorsal urethral plate as a part of epispadias-exstrophy complex. Even in the latter two cases, the prognosis was still good. After excision of the dorsal epispadiac accessory urethral plate and closure of the urinary bladder, the patients were continent to urine through the spared ventral urethral channel (Figure 8) [Salle et al. 2001]. The subgroup (B) included three male patients (Figures 3 and 4(e,f)). Urethroplasties were needed to correct the position of the hypospadiac ventral urethral meatus. In addition, the condition was usually complicated by the presence of other complex urological anomalies that had a major impact on the prognosis in these
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Peno-pubic
An epispadiac urethral plate replacing the accessory dorsal channel. An epispadiac urethral plate replacing the accessory dorsal channel and continuous with exstrophy of the urinary bladder.
4 months
6 months
3 months
2 years
Neonate
5 (Figures 6 and 7)
6 (Figure 5(a–c))
7 (Figure 5(d,e)) 8
9 (Figure 8)
Dorsum of the coronal sulcus
Dorsum of the coronal sulcus
Dorsum of the glans
5 years
Dorsum of the glans
Dorsum of the glans
4
6 months
2 (Figure 2b)
Dorsum of the glans
6 months
6 months
1 (Figure 2a)
Position of dorsal accessory meatus
3 (Figures 2c and 4c,d)
Age
Case
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Dorsal preputial deficiency
Circumcised at presentation; presence of dorsal chordae. Dorsal preputial deficiency helped in detecting the accessory channel. Mild dorsal chordae. Dorsal preputial deficiency helped in detecting the accessory channel Dorsal preputial deficiency Dorsal preputial deficiency
Circumcised at presentation
Circumcised at presentation Circumcised at presentation
Penile anatomy
Type IIA1
Type I
Type I
Type I
Type I
Type IIA1 (complete patent accessory urethra with separate origin from bladder) Type IIA2 (the two urethral channels are communicating with a common origin from the bladder) Type I
Type I (blind accessory urethral tube)
Effmann classification
Bladder exstrophy
Ano-rectal stenosis
Associated anomalies
Excision of the accessory epispadiac dorsal urethral plate + bladder closure.
Excision of peno-pubic sinus Excision of the accessory epispadiac dorsal urethral plate
Just circumcision
Excision of dorsal accessory urethra by stripping Excision of dorsal accessory urethra by stripping
Urethro-urethrotomy + glanuloplasty
Excision of dorsal accessory urethra
No treatment
Management
Table 1. Subgroup A of the sagittal urethral duplication. All patients have a main ventral urethra ending at an orthotopic external urinary meatus.
Therapeutic Advances in Urology 7(2)
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The double urethra
Y-type (congenital urethroperineal fistula)
True duplica on
Dorsal accessory urethra
(4 cases)
(collateral type)
(sagi al type)
(2 cases)
(12 cases)
excluded from the study
part of caudal duplica on syndrome
This group is usually associated with other organ duplica ons (urinary bladder, external genitalia, gastrointes nal). Careful planning and collabora on of other special es are required in their management.
Subgroup A ventral meatus is orthotopic
Subgroup B ventral meatus is hypospadiac
(9 cases) (Excellent prognosis)
Managment: Excision of dorsal accessory urethra can be offered to all cases irrespec ve to their different radiological anatomy
(3 cases) (Gaurded prognosis according to the presence of associa ng urological anomalies)
Managment: Managment of other associa ng urological anomalies. Urethroplasty for hypospadiac ventral meatus + excision of dorsal accessory channel.
Figure 1. Newly proposed classification and algorithm for the double urethra.
Figure 2. Urethrograms of three male patients with sagittal urethral duplication (subgroup A). The three patients have a main ventral urethra ending at an orthotopic external urinary meatus, and another dorsal accessory urethral channel. The dorsal accessory urethra has a different anatomy in each case: (a) blind dorsal accessory urethra (Effmann type I); (b) complete patent dorsal accessory urethra with separate origin from bladder (Effmann type IIA1), note that the ventral urethral channel appears wider and contain the veru (white arrow pointing to the filling defect in the posterior urethra); (c) a common posterior urethra arises from the bladder which then bifurcates into a dorsal accessory urethra, and a ventral main urethra (Effmann type IIA2). The three patients share the same therapeutic decision (excision of the dorsal accessory urethra), and the good prognosis due to absence of other associating urological anomalies.
patients (vesico-ureteric reflux with marked hydro-ureteronephrosis and renal dysplasia, crossed renal ectopia, ectopic ureteral insertion in posterior urethra).
On the other hand, the collateral urethral duplication group included two patients, one of whom was a female (the only female in this series). Both patients were associated with duplication of the
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Therapeutic Advances in Urology 7(2) classification in distinguishing between the different types of the anomaly in a way that would be reflected in the therapeutic decision and prognosis.
Figure 3. Male patient with sagittal urethral duplication (subgroup B). (a) The ventral urinary meatus is hypospadiac (arrow), and the dorsal is orthotopic. (b) The patient urethrogram showing the ectopic course of the dorsal urethral channel from the urinary bladder. Despite having its meatus in a normal location, the dorsal urethra is still the accessory one (ectopic course and poor function). Also, there is reflux of contrast into a hugely dilated ectopic ureter.
urinary bladder and the external genitalia as a part of caudal duplication syndrome (Figure 9). In this group, both urethrae had comparable function, lying side by side, and each draining a separate urinary bladder (two separate urinary systems). Treatment of such complex anomalies required staged operations that are beyond the scope of this report. Discussion Reviewing the double urethra is a tedious work that has been described by Boissonnat as a thankless task for the many confusing titles and unclear case reports leading to errors that are passed from one author to another when quoted and requoted [Boissonnat, 1961]. The several attempts to classify the double urethra have been considered unsatisfactory, until Effmann and colleagues presented their classification that included many details based on the radiological anatomy [Effmann et al. 1976]. By avoiding the various theories of embryogenesis, their classification has been described as a practical one. In this report, we retrospectively gathered our experience with cases of the double urethra. We proposed a classification that is inspired from old surgical classifications and highlighting the embryological explanations. We believe that it may be superior to the currently widely accepted
First we excluded patients with the so-called Y-type urethral duplication, also known as congenital urethro-anal fistula [Bates and Lebowitz, 1995; Stephens and Donnellan, 1977]. These were discussed in a separate report. The embryological explanation postulates the occurrence of a vascular accident leading to the maldevelopment of the penile urethra [Williams and Kenawi, 1975]. This would have a backward effect on the developing uro-rectal septum causing misalignment of its components that results in the persistence of a cloacal lumen communicating between the urethra and the anal canal. Williams and Kenawi considered this communicating urethroanal tract to be primarily a fistula that developed early enough to acquire some of the characteristics of the urethra [Williams and Kenawi, 1975]. The second group is the collateral urethral duplication. The fact that the plane of the double urethrae in the collateral duplication is at a right angle to that of the sagittal duplication should indicate that the two conditions must be of different origins [Williams and Kenawi, 1975]. Midline structures (such as the urinary bladder) are formed by fusion of lateral components derived from each side. It appears that each of these lateral components is capable of forming a complete organ upon failure of midline fusion [Williams and Kenawi, 1975]. This represents a true duplication in which both urethrae are functionally comparable. Representing a part of the caudal duplication syndrome, this group is usually associated with other organ duplications (urinary bladder, external genitalia, gastrointestinal). Careful planning and collaboration of other specialties are required in their management. The most common group was the sagittal urethral duplication or accessory urethra. This is characterized by the presence of two urethral channels, one above the other. Embryologically, the genital tubercle arises from two lateral components (analgens). It has been postulated that a posterior shift of the lateral analgens of the genital tubercle would result in their fusion behind the uro-genital membrane [Stephens, 1983]. The latter will break down exposing the epithelium on the dorsum of the penis. The gross posterior misplacement of the analgens of the genital tubercle
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Figure 4. Clinical photographs (a,c,e) and their respective urethrograms (b,d,f) of three male patients diagnosed to have double urethra. Their urethrograms share a common radiological anatomy (a common posterior urethra arising from the bladder which then bifurcates into two channels, Effmann type II A2). However, each case has a different therapeutic decision and prognosis. (a, b) An 18-month old male passing urine per anus during otherwise normal micturition (Y-type duplication or congenital urethro-anal fistula; excluded from this study). The therapeutic decision was excision of the posterior ‘ventral’ tract communicating between the urethra and the anal canal. (c, d) Six-month-old male with double urinary stream (ventral urethral meatus is orthotopic ‘subgroup A’). The therapeutic decision is to excise the dorsal accessory urethra or perform an urethro-urethrotomy, with excellent prognosis due to absence of associating urological anomalies. (e, f) A 2-year-old male with double urethra (ventral urethral meatus is hypospadiac ‘subgroup B’). Although the dorsal meatus is in a more normal location on the dorsum of the glans, yet the dorsal channel still represents the accessory urethra with its narrow caliber (poor function) and ectopic course (arising from the dorsum of the ventral main urethra). The prognosis is deeply affected in this subgroup by the presence of other associating complex urological anomalies (urinary reflux in an ectopically inserted right ureter with renal dysplasia).
results in complete epispadias. However, intermediate degrees of misplacement can result in the formation of two urethral plates on the dorsal and ventral aspects of the genital tubercle [Stephens, 1983]. What distinguishes our classification is using a single term ‘the dorsal accessory urethra’ for all of the sagittal duplications. This is in contrast to previous classifications that mentioned epispadiac and hypospadiac types according to the location of the external urinary meatii [Williams and Kenawi, 1975; Woodhouse and Williams, 1979]. The dorsal channel had always a poor function and an ectopic epispadiac course even when its meatus was located in a more normal position (Figures 3 and 4(e,f)). On the other
hand, the ventral channel was always the more functioning urethra, with a normal course from the urinary bladder to end either at an orthotopic meatus (subgroup A), or more proximally in a hypospadiac location (subgroup B). Subclassifying the sagittal duplication group was not only for anatomical reasons (location of the ventral urinary meatus), but also for the different prognosis in both subgroups. The prognosis was excellent in subgroup A in contrast to subgroup B that was associated with other complex urological anomalies. In this report, we will focus on the treatment of the sagittal urethral duplication (accessory
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Therapeutic Advances in Urology 7(2)
Figure 5. Dorsal preputial deficiency associating the sagittal urethral duplication (subgroup A). (a, b, c) Six-month-old male patient diagnosed at circumcision to have a dorsal accessory urethra; this was suspected due to the presence of dorsal preputial deficiency. (d, e) One-year-old male patient with dorsal preputial deficiency, underwent excision of peno-pubic sinus (arrow) at the age of 3 months.
Figure 6. Four-month-old male with dorsal accessory urethra (subgroup A): (a) associating dorsal chordae; (b) release of fibrous bands related to the dorsal accessory urethra at the base of penile shaft; (c) after excision of accessory urethra, correction of penile chordae, and circumcision.
urethra). Individually tailored treatment is advised by most authors [Prasad et al. 1999]. Also, there is a consensus on treating only symptomatic patients (significant functional or cosmetic problems) [Salle et al. 2000; Arena et al. 2007; Coleman et al. 2010]. The aim of the treatment is to preserve renal function, achieve continence and the best cosmetic outcome. The ventral channel is always the functioning urethra (containing the veru and sphincters), and has to be preserved. In subgroup B, the ventral urethra is hypospadiac, and a suitable urethroplasty is needed as for hypospadias; in addition to other necessary procedures to deal with commonly
associating urological anomalies. The treatment in subgroup A is simpler, and is directed towards the dorsal accessory channel (only for symptomatic patients with significant functional or cosmetic problems). Obliteration of the accessory channel by injection of sclerosing agent or other ablative interventions has been reported; however, these techniques have been abandoned for fear of corporeal thrombosis and fibrosis [Salle et al. 2000]. When both meatii are near each other in an apical position (Figure 4(c)), an urethro-urethrotomy can be performed by joining both urethral openings into a single orthotopic meatus [Alanee et al. 2012]; however, there is the
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Figure 7. Steps of excision of the dorsal accessory urethra by stripping (a) midline longitudinal incision in the tunica for dissecting out the proximal end of the dorsal accessory channel; (b, c) the proximal end of the accessory urethra is tied to the catheter inside, which is then pulled, stripping the urethra out; (d, e) the accessory urethra is excised with minimal disturbance to the glans and collar of the foreskin.
Figure 8. Male neonate with bladder exstrophy and sagittal urethral duplication. This combination has greatly improved the prognosis of bladder exstrophy in this patient: (a) the dorsal accessory epispadiac urethral plate has spared a functioning ventral urethra; (b) excision of the dorsal accessory epispadiac urethral plate and closure of the urinary bladder.
Figure 9. Collateral urethral duplication (caudal duplication syndrome). (a) Male patient with double penis, omphalocoele, and meningiocoele. (b) Cystogram showing double urinary bladder. (c) Female patient with double urethra, double external genitalia, and lipomeningiocoele. (d) MRI, axial T2WI showing double urinary bladder and double rectum.
potential risk for meatal stenosis and obliteration of dorsal meatus. Surgical excision of the dorsal accessory urethra has been reported as the best therapeutic option, and should be performed in a delicate way to avoid damage to the external
sphincter and neurovascular bundle [Salle et al. 2000]. Significant dorsal penile curvature which is sometimes associating the condition can also be corrected during the operation. The author has applied a modification for excising the dorsal
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Therapeutic Advances in Urology 7(2) accessory urethra by stripping, which eliminates the need for distal dissection with minimal disturbance to the glans. Like other studies on urethral duplication, this one is limited by being a retrospective study over a long period of time, and by the relative small number of cases for such a diverse anomaly. Also, some rare variants have not been encountered, such as the spindle type (Effmann type IIB). Searching through the literature, we have found very few case reports describing the latter type [Williams and Kenawi, 1975; Effmann et al. 1976]; with a warning to exclude the possible iatrogenic causes (false passage as the cause of such rare anatomical arrangement). However, to the best of the authors’ knowledge, this is one of the largest case series described in the literature. We believe our newly proposed classification can provide a comprehensive guide for managing most cases of the double urethra. Conclusion The double urethra is a diverse spectrum comprising different pathologies. Our proposed classification system of duplicated urethras is clinically relevant as it guides surgical management and allows prognostication of outcome. Conflict of interest statement The authors declare that there is no conflict of interest. Funding This research received no specific grant from any funding agency in the public, commercial, or notfor-profit sectors.
References
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