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CASE REPORT
Year : 2012  |  Volume : 11  |  Issue : 3  |  Page : 186-189  

Urethral duplication: Case report and literature review


Department of Surgery, Urology Division, Ahmadu Bello University Teaching Hospital, Shika, Zaria, Nigeria

Date of Web Publication5-Jun-2012

Correspondence Address:
Hussaini Y Maitama
Department of Surgery, Ahmadu Bello University Teaching Hospital, Shika, PO Box 1210 Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1596-3519.96883

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   Abstract 

Urethral duplication is a rare congenital genitourinary abnormality. The purpose of this report is to add to the scanty literature on the subject. We emphasize the significance of high index of suspicion, thorough evaluation, and to discourage circumcision performed by unqualified individuals. A six year old with a finding of urethral duplication is presented along with the review of the literature.The patient presented with of passage of urine from 2 orifices on the penis following circumcision, six months prior presentation. He voids with double streams - clear urine and the other haematuria. Examination revealed a normal primary urethral meatus and an accessory opening on the corona of the dorsal surface of the phallus. No other abnormality detected. Patient had single stage urethroplasty with good outcome. Urethral duplication is rare. Diagnosis requires a high index of suspicion. Optimal knowledge of the duplication is significant for the managing surgeon in order to avoid complications. The overall outcome is good.

   Abstract in French 

Duplication urétrale est une anomalie génito-urinaires congénitale rare. Le but de ce rapport est d'ajouter à la littérature rares sur le sujet. Nous tenons à souligner l'importance du haut indice de suspicion, évaluation approfondie et à décourager la circoncision effectuée par des personnes non qualifiées. A six ans, avec une conclusion de duplication urétrale est présenté avec la revue de la littérature.Le patient a présenté avec du passage de l'urine de 2 orifices sur le pénis après la circoncision, présentation préalable de six mois. Il invalide avec double flux - urine claire et l'autre hématurie. L'examen a révélé un normal méat primaire et un accessoire d'ouverture sur la Couronne de la surface dorsale du phallus. Aucune autre anomalie détectée. Patient avait seule étape urethroplasty avec bons résultats. Duplication urétrale est rare. Diagnostic exige un indice élevé des soupçons. Connaissance optimale de la duplication est importante pour le chirurgien de gestion afin d'éviter des complications. Le résultat global est bon.
Mots clés: Duplication urétrale; Anomalies urétrales; urètre accessoire

Keywords: Urethral Duplication; Urethral anomalies; accessory urethra


How to cite this article:
Maitama HY, Mbibu HN, Tella UM. Urethral duplication: Case report and literature review. Ann Afr Med 2012;11:186-9

How to cite this URL:
Maitama HY, Mbibu HN, Tella UM. Urethral duplication: Case report and literature review. Ann Afr Med [serial online] 2012 [cited 2020 Aug 5];11:186-9. Available from: http://www.annalsafrmed.org/text.asp?2012/11/3/186/96883


   Introduction Top


Urethral duplication (UD) is generally an uncommon congenital abnormality in urological practice. It is more common in the boys than girls. UD are of various types with several classification systems reported. The pattern of presentation depends on the type. The etiology is poorly understood and various theories have been proposed that of. Casselman and Williams, [1] which states that a partial failure or an irregularity of the in growth of the lateral mesoderm between the ectodermal and endodermal layers of the cloacal membrane in the midline accounts for the form with a dorsal epispadiac channel. Das and Brosmas [2] reported that abnormal termination of the Mullerian duct was responsible for urethral duplication. While Rica et al, [3] suggested that asymmetry in the closure of the urorectal septum results in an urethroperineal fistula. Despite the numerous theories proposed to explain this anomaly, no single theory explains all the various types of anomalies.

We report a case of UD in a six-year-old boy, with an initial impression of urethra-cutaneous fistula secondary to circumcision mishap.


   Case Report Top


A six-year-old boy presented with six months history of passage of urine from two orifices in the penis, which has been persistent. There were episodes of painful terminal hematuria with increased urinary frequency which subsided with antibiotics treatment. There was no urinary incontinence or other lower urinary tract symptoms. His parents noticed the voiding with double streams six months after a circumcision performed by a traditional barber. There was no significant history on antenatal, perinatal or developmental milestones.

Examination revealed a healthy looking boy. There was a circumcised phallus of normal size. In addition to the normal urethral meatal opening, there was another opening on the corona of the dorsal surface of the phallus as shown in [Figure 1]. There was no chordee. Both testes were descended, and the scrotum was normal. There was no fistula or sinus in the perineum. An initial impression of urethrocutaneous fistula secondary to circumcision mishap was made.
Figure 1: Physical examination

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The urine was sterile, genotype was 'AA', full blood count and the blood chemistry were within normal limits. Micturating cysto-urethrography was not done, because this rare condition was not initially thought of.

Operative technique

Patient was planned for urethroplasty. Under general anesthesia, in supine position, cleaning and draping, exposing the penis were carried out. Examination under anesthesia and cannulation of both urethral orifices were performed. Both drained urine as shown in [Figure 2], [Figure 3] and [Figure 4]. The penis was degloved through a circumcising subcoronal incision proximal to the accessory urethral orifice. The main urethra was grossly normal as shown in [Figure 2]a. The accessory urethra was mobilized up to its origin at the bladder neck while the neurovascular bundle had been safe guarded on either side. Both urethrae remain parallel to one another. The accessory urethra was then ligated using vicryl 2/0 and excised. Degloved penis was reconstructed and the urethral stent kept and maintained for five days post operative before removal. The observed stream of voiding was satisfactory [Figure 4].
Figure 2: Voiding with single stream

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Figure 3: Both normal and duplicated urethra draining urine

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Figure 4: Observed urine stream after surgery

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Histology of the excised urethra revealed normal urothelium lined tissue. At three months, the boy has remained symptom-less, continent and voiding with satisfactory stream. Flowmetric facilities are not available in our center.

Discussion and the review of the literature

Urethral duplication (UD) is rare congenital abnormality, and hence poses a great challenge in its diagnosis as in the index case reported. There is need for high index of suspicion. This anomaly is unique to males, with few cases reported in females. Approximately, only over 200 cases have been reported worldwide [4],[5] Different classifications of this anomaly have been done, such as the ones by Effmann et al, [6] Williams and Kenawi, [7] and by Das and Brosman. [2] The classification proposed by Effmann et al is functional, which represents all clinical aspects of urethral duplication, and is the most commonly accepted classification. It classified the male UD into three main types as shown in [Table 1].
Table 1: Effmann classification of urethral duplication[6]

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However, it does not distinguish sagittal from coronal collateral duplication. Our patient fits into Effmann's type 2A2 (the non Y variant). Absence of urinary incontinence in this patient suggests that union of the two sister urethrae is below the sphincter. Effmann type 2A2 with Y- variant is the most common type reported [8],[9],[10] The Y- type as compared to other forms of UD, is commonly associated with severe congenital anomalies like inperforate anus, cloacal atrophy, conjoined twins, early amnion rupture syndrome, prune belly syndrome and hand-foot genital syndrome. [11]

Das and Brosman [2] classified duplicated urethra into three types. Type I is a complete accessory urethra arising from a separate or confluent opening within the bladder and extending to an external orifice. Type II includes accessory urethras that arise from the primary urethra and may or may not extend to a distal orifice. Type III arises from the bladder neck or prostatic urethra and opens onto the perineum. The main urethra may be atretic. Firlit classified duplication as a urethra that arises proximally from the bladder, bladder neck or duplicated bladder. [12] Its distal course usually is dorsal to the main urethra. The complete form extends from the bladder to the glans. Most duplication occurs in the same sagittal plane, on top of the other. Less commonly, openings of UD lie collaterally (side by side) in the frontal plane. The classification of Williams and Kenawi includes epispadiac, hypospadiac, spindle and collateral types. According to Urakami et al, [13] collateral urethral duplication in the frontal plane has been reported in only nine cases in the literature.

Radiological investigation includes voiding cystourethrography and retrograde urethrography (RUG) which should be carried out in lateral projections for visualization of the size, shape and position of the two channels. [14] Effman et al[6] showed that catheterization of a ventrally placed urethra was easier. IVU may demonstrate a wide symphysis pubis in case of epispadiac accessory urethra. Other associated anomalies are unilateral renal agenesis, ureteral duplication and a duplicated bladder. USG can demonstrate the exact length of any stricture segment or any extra luminal abnormal soft tissue or diverticulations. However, USG is user-dependent. The advantage is the lack of radiation exposure. MRI is an excellent investigation for the evaluation of duplicated urethras and the periurethral soft tissues. [15] MRI can demonstrate with precision the sizes, shapes and positions of the two urethras as well as other associated genitourinary abnormalities. However, till recently MRI has only been used as an adjunct for the visualization of the urethra. Urethrocystoscopy is required in assessing the duplicated urethra especially when the imaging results are inconclusive. The danger in poor demonstration of tract is the possibility of inadequate and improper preparation for surgery leaving a residual tract or damage to adjoining tissues during surgery. Differential diagnoses include congenital anterior urethrocutneous fistula, Post circumcision urethrocutneous fistula, lacuna magna (which is a problem especially in patients with hypospadias, when the lacuna lies distal to the meatus), urethral diverticulum and dilated Cowper's gland.

Indications for surgery include cosmesis, annoying symptoms such as a double stream, urinary incontinence, recurrent urinary tract infection, obstructive symptoms and associated genitourinary or other anomalies. Treatment of UD would depend on the type of urethral duplication and associated malformations. [16] All efforts should be made to preserve the sphincter. The simplified treatment scheme proposed by Salle et al[16] is given in [Table 2].
Table 2: Treatment scheme proposed by Salle et al.[16]


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   Conclusion Top


Urethral duplication is a rare congenital anomaly presenting in various types and some with associated other malformations, and hence management of these patients must be individualized. The patient presented with type2A1 UD. He remains symptom free three months post-operation.

 
   References Top

1.Casselman J, Williams DI. Duplication of the urethra. Acta Urol Belg 1996;34:535-41.   Back to cited text no. 1
    
2.Das S, Brosman SA. Duplication of the male urethra. J Urol 1997;117:452-4.  Back to cited text no. 2
    
3.Rica PE, Holder TM, Ashcraft KW. Congenital posterior urethral perineal fistula: A case report. J Urol 1978;199:416-7.   Back to cited text no. 3
    
4.Okeke LI, Aisuodionoe-Shadrach OI, Adekanye AO. Urethral duplication with a perineal opening in a four-year-old boy. J Natl Med Assoc 2006;98:284-6.  Back to cited text no. 4
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5.Lukman O. Abdura-Rahman, Abur-Rasheed A Nasir, Agboola OJ. Penile shaft sinus: A sequelae of circumcision in Urethral duplication. Indian J Urol 2009;25:134-6.   Back to cited text no. 5
    
6.Effmann EL, Lebowitz RL, Colodny AH. Duplication of the urethra. Radiology 1976;119:179-85.  Back to cited text no. 6
[PUBMED]    
7.Williams DI, Kenawi MM. Urethral Duplication in the male. Eur Urol 1975;1:209-15.  Back to cited text no. 7
[PUBMED]    
8.Podesta ML, Medel R, Castera R, Ruarte AC. Urethra duplication in children: Surgical treatment and results. J Urol 1998;160:1830-33.   Back to cited text no. 8
[PUBMED]  [FULLTEXT]  
9.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;48:277-80.   Back to cited text no. 9
[PUBMED]  [FULLTEXT]  
10.Slavov C, Donkov I, Popov E. Case of duplication of the urethra in an adult male, presenting with symptoms of bladder outlet obstruction. Eur Urol 2007;52:1249-51.   Back to cited text no. 10
[PUBMED]  [FULLTEXT]  
11.Stephens FD. Abnormal Embryology- cloacal dysgenesis. Congenital Malformations of the Urinary Tract. Part 1, Chapter 2. New York: Praeger Scientific; 1983. p. 15-52.  Back to cited text no. 11
    
12.Firlit CF. Urethral abnormalities. Urol Clin North Am 1978;5:31-55.  Back to cited text no. 12
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13.Urakami S, Igawa M, Shiina H, Shigeno K, Yoneda T, Yagi H. Congenital collateral urethral duplication in the frontal plane. J Urol 1999;162:2097-8.   Back to cited text no. 13
[PUBMED]    
14. Kim HJ, Lim JW, Lee DH, Ko YT. Radiological Findings of Male Urethral Duplication Associated with Bladder Duplication: Case Report. J Korean Radiol Soc 2004;51:645-8.   Back to cited text no. 14
    
15.Bhadury S, Parashari UC, Singh R, Kohli N. MRI in congenital duplication of urethra. Indian J Radiol Imaging 2009;19:232:4.  Back to cited text no. 15
    
16.Salle JL, Sibai H, Rosenstein D, Brzezinski AE, Corcos J. Urethral duplication in the male: review of 16 cases. J Urol 2000;163:1936-40.  Back to cited text no. 16
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]



 

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