Urethral duplication

3 year old boy voids through two separate external urethral orifices (photographer attached) on Ventral aspect more through scrotal opening. Only Dysuria but no documented UTI. MCU film attached.
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Comments(10)

  • Dr. Md. Mazedur Rahman
    Dr. Md. Mazedur Rahman
    12 Mar 2020 11:14:22 PM

    Photograph... 

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  • Ashish parikh
    Ashish parikh
    13 Mar 2020 05:03:14 PM

    Thank you for sharing such rare case

    I didn't see a case of urethral duplication so far
    I have sent an article for reference purposes.
    Seniors and teacher - pl share your experiences and guide for further management.
    Thanks
    Ashish

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  • Dr Prashant Mulawkar
    Dr Prashant Mulawkar
    15 Mar 2020 12:15:47 AM

    This is a urethral duplication. It is a rare anomaly. Around 500 cases have been reported so far.

    Effman classification is the most commonly used one [1]. But this does not include all types [9]

    Effmann has classified this in three types.  Type I, Type II and Type III

    Type I is Blind incomplete urethral duplication. (accessory urethra)  This is further subclassified in

    Type IA: Distal: Opens on dorsal or ventral surface of the penis but does not communicate with urethra or bladder (this is the commonest type)

    Type IB: Proximal: Opens from urethral channel and ends blindly in the periurethral tissue. It is difficult to differentiate this one from urethral diverticula or Cowper’s ducts. This has been reported. But is quite rare (I personally think that this subtype does not exist because it is difficult to diagnose and document)

    Type II : This is a complete patent urethral duplication. This is further subclassified in 

    Type IIA: Two meati (the word meatus has two pleural forms and both are in use: meati and meatuses). Type IIA has two subtypes

    Type IIA1: Two noncommunicating urethras arising from bladder

    Type IIA2: One urethra arises from bladder and the second channel arises from the first and courses independently into a second meatus

    Type IIB: One meatus: Two urethras arise from the bladder or from posterior urethra and unite into a common channel (this is rare)

    Type III: Urethral duplication as a component of partial or complete caudal duplication.

    Your case fits into Type IIA2.

    As a general rule the ventrally positioned urethral channel regardless of the absolute position of its meatus is the more functional one. But I personally think that the urethral duplication is such a rare entity, that such generalizations should not be made. Each case should be managed on its merit. 

    As the Effmann classification system is not ‘complete’, many other classification systems have been proposed.

    Another classification is proposed by Williams and Kenawi [2]. In this the duplication is classifies into sagittal type (more common)  and collateral type (less common). But this lacks many of the anatomical details. 

    Third classification is proposed by AbouZeid[3]. This includes all the types but is bit complex and less commonly used. 

    Lima et al have also proposed a classification system. It is worth reading [8].

    Management plan:

    The ventral urethral plate seems to be the ventral urethra. But many things are not clear from what is presented here. You need to examine this child under anesthesia. Canulate both the openings and see which one ‘looks’ like a normal urethra. Assess how easy (or difficult) would be the reconstruction. The possible plans of reconstruction are:

    1. Excision of the ‘accessory’ channel. While deciding about this, please note that we may be jeopardising the blood supply of ‘native’ urethra.

    2. Marsupialization of the two urethras into one. This depends on the thickness of the ‘septum’. Looking at the urethrogram, this seems to be a difficult option.

    The possible complications are:

    Leaving a blind segment, which can be source of infection and stone formation

    Damage to the blood supply of native urethra leading to stricture

    Fistula formation


    I think we should discuss the plan of action after we examine the child under anesthesia and do scopy if possible.

    Leaving him just like that can also be an option

    Please see following articles for different surgical approaches and devise your own approach to ‘fit’ to your patient. [4: nice surgical diagrams in this reference] [5], [6]


    Collateral urethral duplication is the most rare one. We have presented one case [7]. You may have a look at this one if interested. 


    Ref

    1. Effmann EL, Lebowitz RL, Colodny AH. Duplication of the urethra. Radiology. 1976 Apr;119(1):179-85.

    2. Williams DI, Kenawi MM. Urethral duplications in the male. European urology. 1975;1:209-15.

    3. AbouZeid AA, Safoury HS, Mohammad SA, El-Naggar O, Zaki AM, Hassan TA, Hay SA. The double urethra: revisiting the surgical classification. Therapeutic advances in urology. 2015 Apr;7(2):76-84.

    4. Middleton Jr AW, Melzer RB. Duplicated urethra: an anomaly best repaired. Urology. 1992 Jun 1;39(6):538-42.

    5. Şencan A, Yıldız M, Ergin M, Hoşgör M. A new variation of urethral duplication. Urology. 2013 Aug 1;82(2):451-3.

    6. Coleman RA, Winkle DC, Borzi PA. Urethral duplication: cases of ventral and dorsal complete duplication and review of the literature. Journal of pediatric urology. 2010 Apr 1;6(2):188-91.

    7. Mulawkar P, Tapre P, Mulawkar U, Bhat D. A new variant of urethral duplication-collateral urethral duplication with paraspadiac meatus: A case report. Pediatric Urology Case Reports. 2017 Dec 14;5(1):22-7.

    8. Lima M, Destro F, Maffi M, Proietti DP, Ruggeri G. Practical and functional classification of the double urethra: A variable, complex and fascinating malformation observed in 20 patients. Journal of pediatric urology. 2017 Feb 1;13(1):42-e1.

    9. Abraham MK, Garge S, Sudarshan B, Vishwanath N, Puzhankara R, Paliwal A, Prabhakaran A, Naaz A, Narasimhan K, Prakash D. An unusual variant of urethral duplication: an addition to the Effman classification. International urology and nephrology. 2013 Jun 1;45(3):601-6.



  • Venugopal P
    Venugopal P
    15 Mar 2020 09:00:01 AM

    Dear All,

    Tikenjit Mazumdar had posted a case ‘Double Urethra’ on 4th march 2020 in Uroacademy for which I had posted a long winding reply on 8-3-2020. I had provided a link for a Proposed New Classification for Urethral duplication. I had also discussed briefly on Prepubic Sinus which is another rare anomaly seen in this region.

    I must compliment Prashant for having given us detailed information concerning Urethral Duplication. He has mentioned about a case that he treated of Collateral Urethral duplication (hi sis the sixth case in Literature) along with Paraspadiac Meatus which is probably the first combination with Collateral urethral duplication.

    The article is worth reading with good demonstration of the various points highlighted. I am providing the link for those interested.

    http://pediatricurologycasereports.com/ojs/index.php/pucr/article/view/326 (PDF available)

    I am sure all will benefit from reading both the discussion in tandem.

    With warm regards

    Venu

     

  • shriram joshi
    shriram joshi
    15 Mar 2020 04:23:13 PM

    Dear Prashant and Venus,

    Thank you for the detailed discussion on this rare anamoly. I also feel this is type IIA2. But as Prashant said the delineation of the origin of the urethra from prostatic urethra is not clear and it appears ventral urethra is opening correctly into the bladder neck.
    Instead taking the child under GA where in apart from passing guide wires it may be difficult to negotiate a cystoscope. 
    Hence I suggest we do an ascending urethrogam in one of the urethra whichever is easily available for this. Then compare the two urethrograms (AGU and MCUG) and make an intelligent guess as plan of surgery.
    SSJ

  • Amilal Bhat
    Amilal Bhat
    18 Mar 2020 11:04:15 AM

    11 years old male child presented with complaint of voiding urine per rectum since birth with no per urethral voiding.  He had normal external urethral meatus, bifid scrotum and dorsal aplastic urethra  even 5 Fr ureteric catheter was not negotiable proximal to the glans .  RGU was not possible due to Aplastic dorsal urethra, IVP cystogram showed Large capacity UB & MCU was inconclusive. Antegrade Cysto-urethroscopy showed single Bladder neck, normal verumontanum, posterior urethra negotiated just distal to the veru, flushed saline came out from the anus. He was diagnosed as Y duplication of urethra & managed by mobilisation of the patent ventral urethra from rectum to its proximal part up to membranous urethra, then inner prepucial pedicled tube was used for end to end anastomosis  with mobilised part of ventral urethra, neourethra brought out from perineum to penoscrotal junction.

    Case 2 

    14 years old male child presented with complaint of voiding urine per rectum since birth with no per urethral voiding.  Examination showed normal position of external urethral meatus and per urethral catheter could be negotiated up to 12cm . MCU revealed large capacity bladder with delineation of small part of posterior urethra. Urethrocystoscopy revealed  n. calibre anterior urethra  up to mid bulbar part, proximal to this complete closure of urethral  opeAntegrade  Scopy showed single bladder neck, normal veru ,  post. urethra.   On evaluation he was diagnosed as type II A2, Y duplication of urethra, hypoplastic dorsal urethra at proximal part and with normal calibre of penile urethra. The ventral urethra was communicating with rectum.     we mobilised the ventral urethra from the rectum and anastomosed to the patent distal part of dorsal urethra ( end to end urethro-urethrastomy).

    Amilal Bhat

  • Amilal Bhat
    Amilal Bhat
    18 Mar 2020 11:07:58 AM

    Pictures case 1 ,2

  • Amilal Bhat
    Amilal Bhat
    18 Mar 2020 11:50:28 AM

    Find attached pictures 

  • Amilal Bhat
    Amilal Bhat
    18 Mar 2020 11:53:28 AM

    picture

  • Amilal Bhat
    Amilal Bhat
    18 Mar 2020 01:40:50 PM

    Better to combine opening the septum between two urethrae to deal with the proximal segment. Distal segment to be augmented with BMG or inner prepucial flap which is still available  to complete the distal urethroplasty . Or go for staged urethral for distal segment after augmenting the urethral plate .

    Amilal  Bhat

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