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Table of Contents   
ORIGINAL ARTICLE
Year : 2012  |  Volume : 17  |  Issue : 3  |  Page : 111-115
 

Urethral duplication: Experience of four cases


Department of Pediatric Surgery, Vanivilas Hospital, Bangalore Medical College and Research Institute, Bangalore, India

Date of Web Publication6-Jul-2012

Correspondence Address:
Anand Alladi
Department of Pediatric Surgery, Vanivilas Hospital, Bangalore Medical College and Research Institute, Bangalore
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.98127

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   Abstract 

Aim: Our experience of 4 cases of urethral duplication is reported here. Materials and Methods: A retrospective chart review. Results: The age at presentation varied from newborn to 10 years. The clinical presentation ranged from prepubic sinus to diphallus urethra. There were 2 each incomplete duplication with only external openings (Type IA) and complete duplication of Effmann Type IIA2. All underwent complete excision of accessory urethra and corrections of associated anomalies. Conclusions: Urethral duplications have a varied presentation. At follow up, all are asymptomatic with good cosmetic result.


Keywords: Accessory urethra, preputial cyst, urethral anomalies, urethral duplication


How to cite this article:
Ramareddy RS, Alladi A, Siddappa O S. Urethral duplication: Experience of four cases. J Indian Assoc Pediatr Surg 2012;17:111-5

How to cite this URL:
Ramareddy RS, Alladi A, Siddappa O S. Urethral duplication: Experience of four cases. J Indian Assoc Pediatr Surg [serial online] 2012 [cited 2018 May 25];17:111-5. Available from: http://www.jiaps.com/text.asp?2012/17/3/111/98127



   Introduction Top


Urethral duplication (UD) is a rare anomaly with varied clinical manifestations such as deformed penis, twin streams, urinary tract infection (UTI), urinary incontinence, serous discharge from sinus, out flow obstruction and associated anomalies. [1],[2],[3],[4] It may be complete or incomplete. The accessory urethra often presents as dorsal or ventral midline openings and rarely opens eccentrically (Coronal/collateral). Dorsally opening accessory urethra is the most common type.


   Materials and Methods Top


Four cases of urethral duplication (from 2008 to 2010) were analyzed with respect to age, clinical features and management [Table 1]. Urethrography, sinogram, sonogram, and cystourethroscopy were performed on basis of clinical presentation.
Table 1: Age, clinical features and management

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   Results Top


Case 1: A 2 day-old boy presented with abdominal distention, dysuria and dribbling of urine from prepuce with saccular swelling proximal to the opening. The abdominal examination revealed palpable left kidney and bladder. The penile examination revealed dorsal preputial ballooning with purulent urine discharging from the opening on the prepuce and normal orthotopic meatus [Figure 1]. On attempting to catheterize the orthotopic urethra, an obstruction was encountered about 4 cms from meatus. The ultrasound of abdomen showed solitary left kidney with hydroureteronephrosis and distended bladder. Diagnostic cystoscopy could not negotiate the stenosis. Vesicostomy was done for obstructive urosepsis. The baby was subjected later to urethral dilatation with infant feeding tube up to 8 F and followed up on home urethral calibration. He started voiding per urethra in a good stream post-dilatation, and the vesicostomy spontaneously narrowed and closed by 2 months.
Figure 1: Dorsal accessory urethra with its terminal part mimicking a preputial cyst (arrow)

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Penile soft tissue scan revealed both dorsal accessory urethra starting from cyst and normal ventral dominant urethra, joining at membranous urethra as complete duplicate urethra [Figure 2]. Micturating cystourethrogram (MCU) also confirmed complete duplicate urethra with pooling of contrast in cyst and grade V left vesicoureteral reflux (VUR) [Figure 3]. At 9 months of age he underwent excision of the duplicate urethra. At 4 months post-operative follow-up he was voiding normally and urethral calibration was discontinued. He has been on prophylaxis for reflux and awaiting ureteric re- implantation in view of solitary kidney with grade V VUR.
Figure 2: Ultrasound picture with preputial cyst (vertical arrow), orthotopic urethra (narrow arrow) and Y-duplication of urethra merging at membranous urethra (heavy arrow)

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Figure 3: Y-duplication of urethra (line arrow) with left grade 5 VUR (hollow arrow) and dorsal accessory urethra with pooling of contrast in preputial sac (solid arrow)

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Case 2: An 8-year old boy presented with serous discharge from the root of penis since birth [Figure 4]. Local examination with probe and sinogram showed blind-ending sinus of 5 cms length. The ultrasound of genitourinary system and cystourethroscopy were normal. The sinus was found to be a blind- ending tract between corporal bodies behind pubic symphysis and excised completely [Figure 4].
Figure 4: Penopubic sinus (Type IA) [vertical arrow] and mobilization of accessory dorsal incomplete urethra (horizontal arrow)

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Case 3: A 3-year old boy was brought with history of deformed penis and voiding through normal orthotopic meatus. On examination he had dorsal chordee, ventral hood, abnormal opening at penopubic junction continuing distally as a urethral plate and ending in a wide grooved aborted glans, apart from normal orthotopic meatus [Figure 5]. Local examination with probe and sinogram revealed 7 cms blind-ending sinus tract. Ultrasound of genitourinary system and cystourethroscopy were normal. The entire duplicated urethral unit was excised as high as possible behind pubic sympysis and distally including the aborted glans. Glansplasty was done.
Figure 5: Dorsal incomplete epispadic duplicate (vertical arrow) with chordee and aborted glans (split arrow)

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Case 4: A 10-year old boy presented with twin streams. Local examination showed bifid scrotum, anteriorly placed ectopic anus, pubic diastasis, dorsal penile fistula and normal functioning ventral orthotopic urethra and meatus [Figure 6]. He had undergone excision of ectopic bowel and superior vesicle fissure repair in neonatal period and partial excision of accessory penis of diphallus at the age of 4 years. Retrograde urethrogram and cystoscopy confirmed normal dominant urethra and accessory dorsal urethra joining at posterior urethra as Y shaped duplicate urethra. A complete excision of accessory urethra was done.
Figure 6: Child with diphallus (post-accessory phallus excision earlier) with associated anomalies of bifid scrotum, anterior ectopic anus and pubic diastasis (plain radiograph)

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The age group of our patients at presentation ranged from newborn to 10 years. All children underwent complete excision of accessory urethra [Figure 7]. Post-operative period was uneventful. Follow up ranged from 6 months to 2 years. All have cosmetically normal looking and functioning penis [Figure 8]. Histologic examination revealed transitional epithelium with variable amount of smooth muscle in all.
Figure 7: Operative picture of mobilization and excision of accessory urethra (arrow)

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Figure 8: Pre- and Post-operative picture after excision of accessory urethra (horizontal arrow) voiding in a single good stream (vertical arrow)

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   Discussion Top


Urethral duplication is a rare anomaly with about 300 cases reported to date, usually seen in males and often associated with genitourinary and gastro-intestinal anomalies. [1],[2],[4] Embryogenesis of urethral duplication is not well understood and various hypothesis exist. [1],[4],[5] The components of the male external genitalia are developed from the cloacal folds, labioscrotal swelling, urogenital sinus and preputial swelling and is composed of all 3 germ layers with timely interaction of Sonic hedgehog signals, fibroblast growth factors, Hox genes, bone morphogenetic proteins signals and androgens. [6],[7],[8] According to most studies, which are histology based, the male urethra develops mostly or completely from the endoderm [7] except the distal most glanular part which is of ectodermal origin. [8] According to Hynes et al., the entire urethra develops from migration and fusion of the paramedian folds of mesoderm sub-epithelially. This starts proximally and moves distally and involves 4 pairs of folds which are urethral, frenular, preputial and lacuna. This is preceded by cleavage of cloacal membrane leading to the formation of the urethral groove . [9] .

The commonly quoted and accepted hypothesis for complete urethral duplication is that of Patten and Barry and is supposed to result from an abnormal relationship between the lateral folds of the genital tubercle and the ventral end of the cloacal membrane. [4],[8] But this does not explain all subtypes of urethral duplication.

Embryological etiology of Case 3 may be due to ischemic event to the urethral pate following initial splitting of the urethral anlage resulting in fibrotic dorsal chordee and persistent duplicate glans to different origin or preserved vascularity of the glans. [6] Failure of fusion of mesodermal bands or growth of mesoderm around 2 urethral anlages, duplication of cloacal membrane and defective mesenchymal proliferation around the cloacal membrane could explain the clinical presentations of our diphallus child. [10],[11]

Effmann et al., classified urethral duplication in to 3 types and gave the most exhaustive description. [12]

Type I: Blind-ending accessory urethra (incomplete urethral duplication) IA. Distal-duplicated urethras opening on the dorsal or ventral surface of the penis but not communicating with the urethra or bladder (the most common type) IB. Proximal-accessory urethra opening from the urethral channel but ending blindly in the periurethral tissues (rare)

Type II: Completely patent accessory urethra. It is divided into 2 parts: A (2 meatuses) and B (1 meatus) IIA1 Two noncommunicating urethras arising independently from the bladder IIA2 Second channel arising from the first and coursing independently into a second meatus (Y-type) IIB Two urethras arising from the bladder or posterior urethra and uniting into a common channel distally

Type III: Accessory urethras arising from duplicated or septated bladders.

Of our 4 patients, 2 were incomplete duplication with only external openings while the other 2 were complete duplication of Effmann Type IIA2 [Table 1]. Two of our patients had associated genitourinary anomalies where as 1 had gastrointestinal anomaly in addition.

Diphallus or penile duplication is a very rare congenital anomaly with about 100 cases reported to date. [13] It can be classified into 3 groups, namely; diphallus of glans alone, bifid diphallus and complete diphallus. [13] Superior vesicle fissure with diphallus urethra and ectopic bowel is a rare malformation which requires complete excision of accessory penis and correction of associated anomalies. [4],[10],[11],[13],[14] One of our patients had the rare spectrum described above.

Incomplete ventral accessory urethra often presents as a preputial sinus or swelling. [5],[12],[15] In one of our case, the accessory dorsal urethra was not only complete but presented as a dorsal prepuce cyst. Preputial cysts are usually either epidermoid or mucoid cyst with developmental or acquired etiology which can be differentiated from urethral diverticulum as in our case by voiding pattern or MCU. [16],[17] He also had congenital penile stenosis of orthotopic urethra and responded to dilatation. [18] Type IIA2(Y- type) duplications are associated with stenosis of the anterior portion of the normal urethra and many associated anomalies. [8] This was borne out in our patient (Case 1) who had stenosis of the anterior part of the orthotopic urethra, with Y- type duplication opening on dorsum and associated unilateral renal agenesis with VUR in solitary kidney.

The primary aim of surgical repair of Y-type urethral duplication is to preserve normal functioning orthotopic urethra with intact verumontanum, good caliber, and intact sphincter. [2] The progressive augmentation by dilating the urethra anterior (PADUA) procedure is done with soft catheter for the treatment of severe ventral urethral hypoplasia in cases of prune belly syndrome, urethral duplication and patent urachus. [19],[20] Gradual dilatation of orthotopic urethra and later native detrussor voiding pressure with urinary stream keeps the urethral patency and maintains the normal urethral development without morbidity of surgical procedures of urethrotomy or urethroplasty. [19],[20] In Case 1 of our series this technique was successfully used. Preputial island flap, buccal/bladder mucosal graft or combined grafts are used in a single or staged urethroplasty in cases of atretic penile orthotopic urethra and perineal duplication. [2]

Prepubic sinus of Case 2 (Type I A) often mimics the congenital prepubic sinus arising due to failure of midline fusion of anterior abdominal wall but later has abdominal wall defects and tract mostly lined by squamous epithelium while the former is lined by transitional epithelium. [3],[21]

Radiologic and endoscopic evaluation of urogenital system often delineates the anatomy well. [2],[4]


   Conclusion Top


Urethral duplication is a rare congenital anomaly; either isolated or associated with other anomalies, with varied presentations and requires radiologic and endoscopic workup to define the anatomy and planning the surgical approach to provide cosmetically and functioning normal penis.

 
   References Top

1.Arena S, Arena C, Scuderi MG, Sanges G, Arena F, Di Benedetto V. Urethral duplication in males: Our experience in ten cases. Pediatr Surg Int 2007; 23:789-4.  Back to cited text no. 1
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2.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;5:363-7.  Back to cited text no. 2
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3.Al-Wattar KM. Congenital prepubic sinus: An epispadiac variant of urethral duplication: Case report and review of literature. J Pediatr Surg 2003;38(4):E10.  Back to cited text no. 3
    
4.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. 4
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5.Abdur-Rahman LO, Abdul Rasheed A, Nasir John O, Agboola, Adeniran JO. Penile shaft sinus: A sequelae of circumcision in urethral duplication. Indian J Urol 2009;25:134-6.  Back to cited text no. 5
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6.Ratan SK, Rattan KN, Sehgal T, Sodhi PK. A rare presentation of abortive epispadiac urethral duplication and vanishing midurethra. Pediatr Surg Int 2005;21:689-1.  Back to cited text no. 6
    
7.Yamada G, Satoh Y, Baskin LS, Cunha GR. Cellular and molecular mechanisms of development of the external genitalia. Differentiation 2003;71:445-0.  Back to cited text no. 7
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8.Levin TL, Han B, Little BP. Congenital anomalies of the male urethra. Pediatr Radiol 2007;37:851-2.  Back to cited text no. 8
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9.Hynes PJ, Fraher JP. The development of the male genitourinary system: III. The formation of the spongiose and glandar urethra. Br J Plast Surg 2004;57:203-4.  Back to cited text no. 9
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10.Priyadarshi S. Diphallus with ectopic bowel segment: A case report. Pediatr Surg Int 2005;21:681-3.   Back to cited text no. 10
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11.Mukunda R, Bendre PS, Redkar RG, Hambarde S. Diphallus with anorectal malformation-Case report. J Pediatr Surg 2010;45:632-4.  Back to cited text no. 11
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12.Effmann EL, Lebowitz RL, Colodny AH. Duplication of the urethra. Radiology 1976; 119:179-5.  Back to cited text no. 12
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13.Mirshemirani A, Sadeghyian N, Mohajerzadeh L, Molayee H, Ghaffari P. Diphallus: Report on six cases and review of the literature. Iran J Pediatr 2010;20:353-7.  Back to cited text no. 13
    
14.Bhat HS, Sukumar S, Nair TB, Saheed CS. Successful surgical correction of true diphallia, scrotal duplication, and associated hypospadias. J Pediatr Surg 2006;41:E13-4.  Back to cited text no. 14
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15.Somuncu S, Caglayan F, Cakmak M, Ulusoy S. Embryologic preputial sinus remnant mimicking urethral duplication. Indian J Pediatr 2006;73:227-8.  Back to cited text no. 15
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16.Suwa M, Takeda M, Bilim V, Takahashi K. Epidermoid cyst of the penis: A case report and review of the literature. Int J Urol 2000;7:431-3.  Back to cited text no. 16
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17.De Mendonça RR, Silva Ii JL, Wroclawski ML, Sakuramoto PK, Pompeo AC. Mucoid cyst of the penis: Case report and literature review. Can Urol Assoc J 2010;4:E155-7.  Back to cited text no. 17
    
18.Banks FC, Griffin SJ, Steinbrecher HA, Malone PS. Aetiology and treatment of symptomatic idiopathic urethral strictures in children. J Pediatr Urol 2009;5:215-8.  Back to cited text no. 18
    
19.Passerini-Glazel G, Araguna F, Chiozza L, Artibani W, Rabinowitz R, Firlit CF. The P.A.D.U.A. (progressive augmentation by dilating the urethra anterior) procedure for the treatment of severe urethral hypoplasia. J Urol 1988;140:1247-9.  Back to cited text no. 19
    
20.Al-Bassam A, Sheikh MA, Al-Smayer S, Al-Boukai A, Al-Damegh S. Congenital H-type anourethral fistula with severe urethral hypoplasia: Case report and review of the literature. J Pediatr Surg 1998;33:1550-3.  Back to cited text no. 20
    
21.Kim HY, Shin OR, Jang ED, Yoon B, Chung MS, Chung YS, et al. Congenital prepubic sinus: A variant of urethra duplication. Korean J Urol 2007;48:881-4.  Back to cited text no. 21
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
 
 
    Tables

  [Table 1]


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