Urethral atresia

• 04 Feb 2020 09:15:46 PM
• |Pediatic Urology

7 years old male child presented with vesicostomy, done when child was 4 days old for urinary retention.

Comments(15)

• 

  Rahul Kapoor

  04 Feb 2020 09:18:54 PM

  Urethroscopy could not be done due to very narrow calibre, SPC Scopy veru was seen and a pinpoint hole. Glide wire could be passed as seen. 

  ASU - quality is not very good but it will give some idea about the urethra.

• 

  Dr. Anil Takvani

  04 Feb 2020 09:25:56 PM

  Dear Rahul, 

  Thanks for an extremely challenging case. 

  Have you attempted VCUG? 

  thanks 

• 

  Rahul Kapoor

  04 Feb 2020 09:27:26 PM

  Sir

  No. I attempted SPC scopy. 

• 

  Dr. Anil Takvani

  04 Feb 2020 09:32:31 PM

  I think, VCUG can give us some more insight... Thanks 

• 

  Amilal Bhat

  05 Feb 2020 12:14:55 PM

  Did the child had history of urinary leak per rectum

  Do inspect the rectum

  VCUG is the key investigation 

  Pass a guide wire through  SPC scopy and then tube and do VCUG

• 

  Rahul Kapoor

  05 Feb 2020 01:25:57 PM

  Sir

  No urinary leak per rectum.

  On SPC scopy, bladder looks normal. 

  VCUG not done.

• 

  shriram joshi

  05 Feb 2020 10:03:12 PM

  Dear Rahul,

  Thank you for presenting this rare case. Amilalji is thinking of a duplex urethra with a major urethral segment opening in the rectum. 

  I think this is failure of urethral plate fusion. Prostatic urethra is developed with trigone of the bladder and urethral buds give the penile and bulbar urethra. 

  this is a stenosis of the penile urethra from prostatomembranous urethra to the tip. 

  Kidneys have been saved with the properly done vesicostomy as seen in the picture. Good location, no pouting bladder mucosa. 

  You  will have to open the urethra and see the width of the urethra. Open urethra with a guide wire in situ. If less than 6 mm will need excision and replacement. Doing a ventral onlay on a narrow urethra has poor results in the long run, with stenosis, urethral diverticulii etc. I

  Will leave this replacement of urethra to Amilalji as he probably has more experience than me. I have some thoughts, but will comment only after I hear from Amilalji.

  altrnatively we could ask Dr. S Kulkarni from Pune for his expert opinion. 

  SSJ

• 

  Dr Sanjay Balwant Kulkarni

  06 Feb 2020 12:43:26 PM

  Dear Dr Sham Joshi

  This boy has urethral atresia.MCU suggests normal posterior urethra.

  I would like to use flexible ureterorenoscopescope(RIRS) from SPC to check the proximal urethra.

  Through the meatus I will try to pass 4.5 fr rigid ureteroscope.

  If the urethra is reasonable caliber one stage dorsal bmg urethroplasty.

  I think this boy will need two stage urethroplasty. Inverted U perineal incision and Johanson's first stage with perineal urethrostomy. Six months later second stage Asopa Dorsal inlay

  Whenever I have treated duplex urethra,there is an atretic segment and the results of augmentation urethroplasty only are poor.

  For truely atretic urethra dorsal bmg with ventral skin flap may be required. Schreiter's mesh graft can be considered. Enterourethroplasty is the last choice.

  
  

• 

  Dr. Anil Takvani

  06 Feb 2020 12:54:09 PM

  Dear Rahul,

  After couple of excellent inputs you need to give us VCUG.

  Dr. Amilala Bhat will write his approach after looking at VCUG.

• 

  Rahul Kapoor

  06 Feb 2020 04:54:56 PM

  Unfortunately i lost follow up with this patient. 

  I discussed the options and need for stage.

  I will try to follow this patient and if possible get back with VCUG

• 

  Amilal Bhat

  07 Feb 2020 10:15:19 AM

  If confirmed no Urethral duplication then 

  1.Lay open the urethra augment with buccal. Mucoasa and tubularization after 6 Months when buccal mucos stabilized.

  2.Lay open urethra urethral plate reasonable wide onlay flap urethroplasty

  3.Urethral plate narrow n poor excise urethral plate n flap tube urethroplasty

• 

  Dr. Anil Takvani

  07 Feb 2020 11:39:47 AM

  Thanks to all stalwarts for their very much valuable comments and suggestions.

  I have deleted female case. Rahul is requested to post that case in a separate thread.

  
  

• 

  Venugopal P

  08 Feb 2020 05:59:02 PM

  Dear All,

  This post may not be as a reply to the discussions hitherto made.

  Antenatally, 2 types of Lower Urinary Tract Obstructions (LUTO) can be discerned. Of which we discuss PUV quite frequently and hence no further discussion on PUV is made. The other two types though uncommon are Urethral Stenosis and Urethral Atresia. Among these two Urethral Stenosis is rarer than Urethral Atresia. Those who practice Foetal Cystoscopy mention that a catheter could be passed down the urethra and out of external Urethral meatus in Urethral stenosis and PUV but not in Urethral Atresia.

  In the presentation that we are discussing, we have been using the term Urethral Atresia rather loosely.

  I am providing few Images of these conditions as observed with Foetal cystoscope (PDF).

  The term Urethral Atresia should be limited to a situation where the child even prenatally has not voided through the urethra. Urethral Atresia is usually fatal unless there is some other egress for the urine to escape the bladder, such as patent urachus or an Uro-Rectal communication, and these lesions are not compatible with renal development. The point regarding Uro-Rectal communication has been raised by Amilal Bhat. This child presented did not have any such communications. Atresia of urethra often presents on routine antenatal ultrasound with Oligohydramnios or Anhydramnios which in turn affects the development of the lungs and causes features of Potter sequence.

  The other condition that we have to understand is Urethral Stenosis. The urethra is often stenosed at the Bladder neck region or rarely at the prostato-membranous region and could be short segment stenosis or the entire urethra could be narrow. As mentioned earlier there is usually a communication between External meatus and Bladder. This could be hypoplastic. Such a scenario of hypoplastic urethra could be observed in Prune belly syndrome as well. But the case provided does not suggest this.

  Extensive surgeries have been recommended by Sanjay and others. But the saving grace is an attempt at a Urethroscopy s prior to planning surgery.

  Abdol-Mohammad Kajbafzadeh et al (2010, he has described various types of PUV that can be observed) has suggested Hydrodistension of Urethra in such hypoplastic Urethra prior to decision of surgical interventions.  Five milliliters of normal saline was pushed via a 22-gauge plastic angio catheter into the Urethra and this was repeated with higher volumes of the solution (up to 20 mL). The procedure was continued until a 6F or 8F feeding tube catheter confirmed the urethral patency. Hydrodistension was repeated in 3-month intervals till complete patency was confirmed by imaging. It usually takes few sittings to achieve success.

  May be this could be performed for this child though the child is now seven and needs higher volumes for hydrodistension. This is only a suggestion.

  Anil and others have suggested VCUG, a procedure that could be performed by occluding the vesicostomy. But can a child who has never voided per urethra be able to void per urethra when attempt at VCUG is made. I do not know.

  With warm regards,

  Venu

   

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• 

  Dr. Anil Takvani

  09 Feb 2020 07:44:34 AM

  Thanks for concluding remarks Sir. 

  Simply great... 

• 

  Rahul Kapoor

  09 Feb 2020 03:07:15 PM

  Thanks for wonderful inputs. 

  Whats this Schreiter's graft.