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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 3  |  Issue : 2  |  Page : 122-124

A surprise during hypospadias repair: A rare case of urethral duplication with review of literature


Department of Pediatric Surgery, Pondicherry Institute of Medical Science, Puducherry, India

Date of Submission10-Sep-2017
Date of Acceptance06-Oct-2017
Date of Web Publication8-Jan-2018

Correspondence Address:
Dr. Mamatha Basavaraju
C Block-14, Pondicherry Institute of Medical Science Hospital Quarters, Kalapet, Puducherry
India
S K Pavithra
Department of Pediatric Surgery, Pondicherry Institute of Medical Science, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcrsm.jcrsm_57_17

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  Abstract 

Urethral duplication (UD) is a rare congenital anomaly mainly affecting boys. This article highlights a case of UD which was incidentally diagnosed during hypospadias repair. It was an incomplete UD with an accessory channel opening orthotopically and primary urethral opening being hypospadiac. The patient underwent glansplasty and excision of distal septum between the two urethrae to make it into a single channel following which he was asymptomatic.

Keywords: Excision of septum, glanular hypospadias, urethral duplication


How to cite this article:
Ashok AJ, Basavaraju M, Zachariah N, Pavithra S K. A surprise during hypospadias repair: A rare case of urethral duplication with review of literature. J Curr Res Sci Med 2017;3:122-4

How to cite this URL:
Ashok AJ, Basavaraju M, Zachariah N, Pavithra S K. A surprise during hypospadias repair: A rare case of urethral duplication with review of literature. J Curr Res Sci Med [serial online] 2017 [cited 2018 Jan 20];3:122-4. Available from: http://www.jcrsmed.org/text.asp?2017/3/2/122/222423


  Introduction Top


Urethral duplication (UD) is a rare anomaly with a variety of clinical presentations such as deformed penis, twin streams, urinary tract infection (UTI), urinary incontinence, outflow obstruction, and serous discharge from sinus.[1],[2] Although a number of theories have been proposed to describe this condition, the actual mechanism of this disorder is still not clear. Treatment of UD is individualized based on the anatomic variant and also clinical symptoms and severity of anomaly. We report a case of UD incidentally found during hypospadias repair in a 3-year-old male child.


  Case Report Top


A 3-year-old male child presented with a complaint of passing thin stream of urine since birth. On examination, the child had a dorsal-hooded prepuce and meatal opening was narrow located slightly ventrally over the glans. He was passing very thin stream of urine from the stenosed meatus. There was no history of recurrent UTI or dribbling of urine during micturition. Physical examination revealed no other associated congenital abnormality and testes were bilaterally present in scrotum. During surgery, attempted catheterization with infant feeding tube (IFT) 5fr could be passed up to 4 cm and was blind ending at mid-shaft [Figure 1]A. On careful examination, there was another meatal opening ventrally. A narrow stenosed meatus was found ventrally over the glans. When this was dilated and opened, the meatus almost retracted to coronal position. It now allowed an IFT to be easily inserted into urethra till bladder [Figure 1]B. He was diagnosed to have UD of incomplete variety. The accessory urethra was opening dorsal to primary urethra and was blind ending. The primary urethral meatus was stenotic and hypospadiac. Because of the proximity of the two urethrae, the septum between the two urethrae was divided and made into a single channel at the distal part relieving his symptoms. The hypospadias were repaired with glansplasty. The postoperative period was uneventful and the child has a normal urinary stream from tip of glans [Figure 2].
Figure 1: urethral duplication with two meatal openings, (A) infant feeding tube in blind ending accessory urethra and (B) infant feeding tube in primary urethra with hypospadiac meatus. Arrow shows dorsal hooded prepuce

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Figure 2: Postoperative appearance showing single meatus at glans tip

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  Discussion Top


UD is an infrequent congenital malformation with an estimate of 300 reported cases worldwide.[3] Many hypotheses have been proposed to explain this unusual condition, including partial failure or an irregularity of the ingrowth of lateral mesoderm,[4] abnormal termination of mullerian duct,[5] and asymmetry in the closure of urorectal septum.[6] However, no single theory explains all the various types of anomalies.

The most widely used classification for UD is Effmann Classification [Figure 3].[7] The most common subtype is type IA.
Figure 3: Effmann classification of urethral duplication. Type I is incomplete urethral duplication (accessory urethra); Type IA, distal; Type IB, proximal; Type II, complete urethral duplication; Type IIA1, two noncommunicating urethras arising independently from bladder and opening in two different meatus; Type IIA2, a second channel arising from the first and coursing independently to a second meatus; Type IIA2 (Y-type duplication); Type IIB, two urethras arising from the bladder or posterior urethra and uniting to form a common channel; and Type III, urethral duplication associated with duplication of bladder and penis

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Investigation and treatment of UD are usually individualized based on the anatomic variant and also clinical symptoms and severity of anomaly. Diagnosis is made during a physical examination, supplemented by voiding cystourethrography, retrograde urethrogram, and urethrocystoscopy. Ultrasound aids in diagnosis of other associated renal anomalies.

Our case was a variant of Effmann Type 1A since the accessory urethra opened at tip of glans and the primary urethra was opening ventrally on glans [Figure 4]. Literature search revealed a new classification proposed by AbouZeid et al. where dorsal accessory urethra was divided into subgroup A and B.[8] In subgroup A, the ventral meatus is orthotopic, and in subgroup B, the ventral meatus is hypospadiac like in our case. In the authors' experience, subgroup A was more common with very good prognosis as it did not have any associated renal anomalies whereas subgroup B was associated with major renal anomalies such as vesicoureteral reflux, crossed renal ectopia, ectopic ureteric insertion which impacted on the prognosis and final outcome.
Figure 4: In type 1A, the accessory urethra opens dorsally and primary urethra is usually at tip of glans. In our child, there was variation since the accessory urethra opened at the tip of glans and the primary urethra was opening ventrally on glans and was stenotic (A)

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In our child, the UD was incidentally detected while repairing a glanular hypospadias. These types of duplications may be missed during circumcision and later present with a discharging sinus dorsally like reported by Abdur-Rahman et al.[2] Sometimes, they may present with a prepubic discharging sinus.[3] In such cases, the accessory urethra may be excised completely. Some patients do not require treatment if they are free from infection and incontinence. Use of thermocoagulation or the injection of caustic substances into the accessory duct for them to scar and scleroses were done in the past but have been abandoned now for fear of fibrosis of corpora cavernosa.[9]

In our child, division of distal septum and glansplasty relieved his symptoms. On postoperative period, he had a normal meatus at the tip of glans and was passing good stream of urine. During follow-up, ultrasound was done to look for any associated renal anomalies and was normal.

Literature search revealed a similar case report by Carvalho et al.[10] While repairing a distal hypospadias, catheter stopped at mid-shaft similar to our case. They tried dilatation without success. Later on careful inspection, another opening was found ventrally. Their management slightly differed as they used Koff technique where primary urethra is mobilized and later divided the septum to make it into a single channel. We did not mobilize the urethra instead did a glansplasty and divided the septum.

We want to highlight that even glanular hypospadia which is a very minor form of hypospadias can be associated with UDs. Urethral dilatation should not be tried when unable to catheterize. In such cases, to look more vigilantly for the presence of any other openings, if the two urethrae are adjacent to each other, just division of septum is sufficient instead of excision of the accessory urethra.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parent/guardian has given his consent for his images and other clinical information to be reported in the journal. The patient's parent/guardian understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Onofre LS, Gomes AL, Leão JQ, Leão FG, Cruz TM, Carnevale J, et al. Urethral duplication – A wide spectrum of anomalies. J Pediatr Urol 2013;9:1064-71.  Back to cited text no. 1
    
2.
Abdur-Rahman LO, Nasir AA, Agboola JO, Adeniran JO. Penile shaft sinus: A sequalae of circumcision in urethral duplication. Indian J Urol 2009;25:134-6.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Ramareddy RS, Alladi A, Siddappa OS. Urethral duplication: Experience of four cases. J Indian Assoc Pediatr Surg 2012;17:111-5.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Casselman J, Williams DI. Duplication of the urethra. Acta Urol Belg 1966;34:535-41.  Back to cited text no. 4
    
5.
Das S, Brosman SA. Duplication of the male urethra. J Urol 1977;117:452-4.  Back to cited text no. 5
    
6.
Rice PE, Holder TM, Ashcraft KW. Congenital posterior urethral perineal fistula: A case report. J Urol 1978;119:416-7.  Back to cited text no. 6
    
7.
Effmann EL, Lebowitz RL, Colodny AH. Duplication of the urethra. Radiology 1976;119:179-85.  Back to cited text no. 7
    
8.
AbouZeid AA, Safoury HS, Mohammad SA, El-Naggar O, Zaki AM, Hassan TA, et al. The double urethra: Revisiting the surgical classification. Ther Adv Urol 2015;7:76-84.  Back to cited text no. 8
    
9.
Abbate B, Centonze N, Danti DA. Urethral duplication in pediatric age. A case report. Pediatr Med Chir 2002;24:387-9.  Back to cited text no. 9
    
10.
Carvalho BC, Talini C, Antunes LA, Del Valle MH, Aranha AA Jr., Moreira Amarante AC, et al. Our experience on urethral duplication in children: 4 case reports and literature review. Pediatr Urol Case Rep 2017;4:378-82.  Back to cited text no. 10
    


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



 

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