Query on Management of Concurrent presence of PUJO and VUJ anomalie

• 17 Mar 2020 07:16:46 PM
• |Pediatic Urology

1)Guidance regarding management in PUJO with VUR, which one to operate first PUJO or VUR and why? 

Comments(6)

• 

  Dr. Anil Takvani

  18 Mar 2020 08:34:08 AM

  "Guidance regarding management in PUJO with VUR, which one to operate first PUJO or VUR and why?"

  PUJO and VUR both can coexist in one patient.

  There can be :

  Primary pelviureteric junction obstruction with primary VUR

  Secondary PUJO coexisting because of severe primary VUR, where kink is fixed and PC system fails to empty in antegrade study or IVU or diuretic scan with catheter in bladder.

  Pseudo-PUJO; a kink at PUJ leading to appearance of PUJO but kink disappear with emptying of PC system when we do IVU or antegrade study with catheter in bladder. 

  Incidences:

  When primary diagnosis is PUJO, the incidence of VUR is around 10% but with diagnosis of primary VUR, only 0.75 -3.6% to have simultaneous PUJO.

  When both are primary and PUJO is significant, correcting PUJO is priority. Reflux can be treated on merit later either with AB prophylaxis or by surgery.

  Many of these reflux can resolve on its own.

  In cases of secondary PUJO because of severe primary VUR, where kink is fixed and PC system fails to empty in antegrade study or IVU or diuretic scan with catheter in bladder. PUJO need to be corrected first because correction of VUR in these cases can lead to acute decompensation of related to edema at anastomotic site following reimplantation.
  

  In case of pseudo PUJO, correcting high grade VUR is a first and pseudo PUJO will not require any correction.

  I am attaching images representing a kind of pseudo PUJO with primary high grade reflux. On correcting reflux successfully suspected PUJO was corrected on its own.

  Related article abstract is uploaded as a link

  http://europepmc.org/article/MED/2746763
  

  Coexisting ureteropelvic junction obstruction and vesicoureteral reflux: diagnostic and therapeutic implications. 

  Duckett JW

  Will upload images of both primary little later...

  

• 

  Dr. Anil Takvani

  18 Mar 2020 08:40:59 AM

  Description of above images:

  VCUG: with doubt of both coexisting.

  Limited plate IVU with catheter in place: straightening of kink with excellent drainage.

  Delayed EC scan plate with catheter in place, again excellent drainage, thus correction of VUR is priority in this situation.

• 

  Dr. Anil Takvani

  18 Mar 2020 08:51:05 AM

  Images of case suggestive of both coexisting as primary.

  We can see reflux grade is low and retention of most of the contrast in dilated PC system of lower moiety of duplex system on right renal unit suggestive of coexisting primary PUJO of lower moiety.

  This patient had crossing vessels, compressing from outside with responsible for increasing dilatation of pelvicalyceal system with obstruction.

  PUJO was corrected first, low grade reflux resolves with linear growth of child.

  

• 

  Venugopal P

  18 Mar 2020 09:31:31 AM

  Dear All,

  This is a subject close to Shyam’s heart. We have discussed this aspect on occasions during USICON’s of the past.

  I am providing a brief Correspondence of Shyam with BJUI in 2001 on this subject

  The question that poses considerable problem is whether PUJO is secondary to VUR or both can co exist together causing confusion regarding which among them need earlier attention.

  It is well known that PUJO can occur on one side and VUR on the contralateral side. There is no confusion regarding this. But when both PUJO like appearance and VUR occur on the same side, the confusion confounds itself. This situation is more with children having higher grade reflux, mostly 4 and 5. In earlier grades, it could be assumed that there could be coexistence but in Higher Grades there is a possibility of the PUJO like picture could be secondary. Shyam always maintained that in children, most PUJs, even in grotesque appearance, maintains its Funnel shaped configuration. This is lost and the PUJ is pushed upwards and more medially when VUR (high grades) are encountered suggesting a secondary nature for PUJO appearance. (I am hopeful that Shyam will correct me if my assumptions are not exactly right).

  The scenario today has changed considerably as we are detecting such combinations by performing Antenatal US. In Infants and neonates, US may not pick up the dilated Ureters. Even antenatally and in Infancy, fluctuating features of Renal Pelvic Dilatation is indicative of presence of Reflux. These children need be evaluated further for presence of Reflux.

  An article from Paediatric Surgery at PGI by Prema Menon et al, is addressing this issue.

  http://www.jiaps.com/article.asp?issn=0971-9261;year=2019;volume=24;issue=2;spage=109;epage=116;aulast=Hegde (PDF available)

  http://www.jiaps.com/temp/JIndianAssocPediatrSurg242109-1094083_030220.pdf

  Though she has risen the issue of whether PUJO or VUR need to be tackled first, their opinion seems to be for performing a Pyeloplasty and Observational management for VUR. This has to be considered with utmost caution as suggested earlier. When there is secondary PUJO with High Grade VUR, the possibility of Progressive Renal deterioration can occur during the interim period of waiting for definitive treatment for VUR.

  I do not know whether in low grade reflux and PUJ, endoscopic injection treatments have a place.

  I have said my say but I am confident that it may open up a Pandora’s box with many having alternate views and I welcome such dissents.

  With warm regards,

  Venu

   

  View Document

• 

  shriram joshi

  19 Mar 2020 06:04:26 PM

  Sorry for this delayed reply. My comments were made in early days when nuclear medicine was in its infancy and only I-131 Hippuran was available. In these situations with no usg, or nuclear scan, my comments were made. If you look at the first case of VUR with ? PUJ the pelvis is pushed up and medially and distortion of the conical shape., with Gr 4/5 VUR. The second case demonstrates classical PUJ with low grade reflux but conical shape is maintained. 

  
  

  Today we have both ultrasound and nuclear medicine to distinguish primary PUJ with reflux, and simulated PUJ with VUR. The reason you have to distinguish, you have to decide which takes a priority for surgery. You cannot operate on both simultaneously for fear of making the midureter ischaemic. 

  
  

  In primary PUJ with VUR a. the grade is lower, b. USG may show dilated calyces and thinning parenchyma, changes in AP diameter of pelvis on supine and prone position (Ref. Gyanendra Sharma), AP diameter of pelvis 30 mm or more. To prove this dilatation is obstructive, best imaging is Tch EC diuretic renogram with catheter in the bladder. In infants and children <3 years, I prefer to pass a 6/F Nealaton catheter and wrap it in a diaper, continuous drainage, this prevents the child from pulling out the catheter. With the catheter in the bladder, VUR is more or less eliminated. In diuretic renogram one should look out for 

  1. cortical transit time 2. differential renal function 3. drainage curve, 4. scanning at the end of one hour . Cortical transit time, although not specifically mentioned, all the other points are seen in both the cases demonstrated. One hour delayed picture shows one draining well and other showing retention of dye. 

  
  

  From the discussion above and other comments, if you prove there is primary PUJ obstruction, you procced with Pyeloplasty and treat VUR on its merit medically. On the other hand with Gr 4/5 and simulated PUJ treat the VUR first and follow up with repeat USG for PUJ obstruction. Even if you misjudge the PUJ obstruciton and treat VUR first, you have lost nothing. Repeat Tch EC diuretic renogram and USG at 3 months postop

  for reassessment. The renal function will not deteriorate and function will recover 

  
  

  I do hope I have cleared some of the doubts raised.

  SSJ

  
  

  I believe CT urography has little use in children. A tailored nuclear scan combined with an ultrasound will give you more information and lower radiation. CT scan requires either GA or heavy sedation. A renogram can done with oral pedichlor sedation. 

• 

  Kiran n g

  19 Mar 2020 07:47:15 PM

  @ Dr. Anil Sir - yes sir they are very much clear now...Thanks you very much Dr Anil sir ,Prof Dr Venugopal sir and Prof Dr Shriram Joshi sir.