Solitary kidney with POM with prostatic uterical cyst

• 20 Feb 2020 07:34:53 PM
• |Pediatic Urology

Comments(14)

• 

  Gyanendra Sharma

  21 Feb 2020 06:48:46 AM

  Dear Dr. Chirag

  Some more information  needed

  1. What was the level of obstruction on Right Side during RGP
  2. Did he have a vesical diverticulum on cystoscopy
  3. Sr. Creat now
  4. USG findings now as the MCU shows a ? malrotated Left Kidney ectopically placed
  5. Have you done any functional study now?

  
  

• 

  Dr. Anil Takvani

  22 Feb 2020 10:47:09 AM

  I congratulate Chirag Shah to post very rare and interesting case for discussion.

  He is second trainee after Utsav to post on uroacademy.

  Can you please describe or post the report of VCUG?

  Also provide details asked by Dr. Gyanendra.

• 

  shriram joshi

  22 Feb 2020 01:20:02 PM

  Dear Chirag,

  It is always difficult when the presentation is incomplete, as Gyanendra Sharma and Anil have already raised the queries. This appears to be your first attempt on the web site so don't get discouraged just reply to the querries one by one.

   SSJ

• 

  Chirag shah

  22 Feb 2020 07:33:03 PM

  Apologies for late reply.

  1. Obst at VUJ

  2.no vesical diverticulum. Prostatic utericle was large

  3.0.3

  4. Left kidney is absent. What is seen is utericle.right kidney has mild HUN

  5.no functional study is done 

  
  

• 

  Gyanendra Sharma

  23 Feb 2020 02:41:11 PM

  Dear Chirag

  Thanks for the inputs

  This is such a rare case that I doubt if anyone has experience of dealing with it

  The approach to it would be based on the principle of taking care of the problem and taking care that new problems do not occur--this would entail searching the literature and applying common sense

  I can think of the following strategy and would want others to give their inputs also

  1. DMSA Scan as  functional evaluation of the solitary right kidney as it will give idea regarding the presence of scarring--as the child had presented with urospesis
  2. CT Scan/ MRI for better anatomical evaluation of the prostatic utricle, as from the MCU certain things are not clear and especially in the last image I feel as if there in a pelvi calyceal system seen
  3. Cystoscopy for evaluation of the lower tract, evaluation of the prostatic utricle and evaluation of capacity of bladder
  4. If it is a large prostatic utricle only then its excision & then later on Right ureteric reimplantation

  I am attaching links to few articles which  I got when I serached for management of such a case

  https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4439243/
  

  https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3754401/
  

  http://dx.doi.org/10.1136/bcr-2012-008123
  

• 

  Dr. Anil Takvani

  23 Feb 2020 08:52:14 PM

  I completely concur with Dr. Gyanendra.

  Only thing I want to re-emphasis is not to do hurry for right reimplantation and uretrostomy closure.

  
  

• 

  Ravindra Sabnis

  24 Feb 2020 08:59:43 AM

  He also needs TRUS. He is now 13 yrs. Something needs to be done now.  Even though it is sobers uretrostomy, & some amount of urine is going down, bladder needs to get activated now.  It is long time.  All investigations as suggested & then plan for definative surgery. Post the pictures of utricle. 

• 

  Dr. Anil Takvani

  24 Feb 2020 09:06:00 AM

  Prof. Sabnis Sir, Thanks.

  I would like to put on your notice current age of patient- 1.3 years ( 13 Months).

  Is it right Dr. Chirag?

• 

  Chirag shah

  24 Feb 2020 03:09:40 PM

  Yes sir. Age is 15 months

• 

  Chirag shah

  28 Feb 2020 01:41:43 PM

  We got MRI of this patient. It shows shrunken dysplastic   ectopic left kidney in pelvis opening in prostatic urethra. Patient was admitted with sepsis 5days ago. Stable at present.we are planning for left nephrectomy with right ureterostomy closure. Opinion of house please...

  

• 

  Chirag shah

  28 Feb 2020 01:43:49 PM

  Suspected urecal cyst was ectopic kidney with dilated ureter. Ureter opens in prostatic urethra.

  

• 

  Dr. Anil Takvani

  28 Feb 2020 02:57:32 PM

  Have you confirmed status of right lower ureter? In your initial post you mentioned gross right HUN and for that right ring uretrostomy was done at age of 1 month.

  Is it obstructed?

  I think ureterogram from distal loop can help.

  Right ureterostomy can be closed keeping stent but later on we need to do diuretic renal scan study on removal of stent after 6 weeks as there was doubt of obstructed megaureter on right side.

  Regarding left side you need to do nephroureterectomy. Remove ureter as down as possible without going too far behind the bladder neck.

  Thanks for update and interesting case.

• 

  shriram joshi

  29 Feb 2020 04:28:44 PM

  The first thing one has to do is to prove there is a UVJ obstructuion on the rt. side. As Anil has suggested a descending ureterogram will decide this issue. But this is only for the diagnosis. 

  Priority should be for nephroureterectomy on the left side. It appears this is a refluxing ectopic ureter hence the appearance of a prostatic urtricle. The acute sepsis is probably from this and needs to be eliminated. Chirag you don't have to go all way down to prostatic urethra, for disection at this level may lead to neuropraxia of the bladder. You also have to be careful of the vas deferens, it is very thin and not as hard as in adults, when you dissect the ureter in the pelvis. For the ureteric stump don't block with a ligature, but just burn the mucosa with bipolar cautery and allow free drainage. You will need a bladder catheter for drainage to avoid refluxing urine in the ectopic ureteric stump, for about 5 days. 

  When the child settles down from this one can tackle the right side depending on descending ureterogram. Could be either simple closure of ureterostomy or closure of ureterostomy with a DJ stent (3/F) and UVJ surgery after 6 weeks. 

  AS Gynendra has suggested a DMSA scan now is an excellent idea for function in the right kidney and scars if any.

  SSJ

  
  

• 

  Ravindra Sabnis

  02 Mar 2020 02:30:48 PM

  Very sorry for asuming 13 years. It was actually 1.3 yrs.  We have learnt from Dr  SSJ sir so my plan is exactly like what he has described.  Trying to remove full.lower end till prostate is harmful. Sir has mentioned all points. So nothing more to add. 

  One should not be in hurry for Rt side.  RT obst may be because if left dilated ureter.