SURGICAL CARE AND PERSPECTIVES

Yves Aigrain, Paris, France

Chair: Eric Girardin, Geneva, Switzerland

Adrian Woolf, London, United Kingdom

Dr Yves Aigrain AP-HP Hôpital Robert Debré UFR Denis Diderot Paris, France

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Slide 1

Thank you Mr Chairman and thank you for inviting me to participate to this meeting. So I had difficulty to figure how I could overview the surgical care of urinary tract malformations in 20 minutes but I chose to try to figure out what has been changing in the past years with these babies and infants.

Slide 2

What is sure and what has been nicely shown in the previous paper is that the surgical care of congenital anomalies of the urinary tract has been deeply modified by their prenatal diagnosis. This allows us to know better today their natural history and has changed some of our practice.

Slide 3

As you know and as it has already been said, these anomalies are usually classified in upper urinary tract abnormalities: PUJ, wide ureters, duplex system and the most frequent of them vescicoureteric reflux. Lower urinary tract abnormalities where the most severe one on which I’m going to talk a little bit more at the end of this communication are posterior urethral valves.

Slide 4

PUJ are very often diagnosed prenatally but if I may add my answer to the one that was just raised by the Chairman about the difference between the babies that we see and the adult cases that urologists are seeing, I don’t think that these are exactly the same disease. One anatomic argument is the frequency of the lower pole vessels associated with PUJ, which is much higher in the cases that we see in adolescences or young adults than in the newborn babies. Most of the cases that we see in childhood are diagnosed prenatally. Many of these dilated kidneys will have resolved spontaneously during the first month of life or even before birth or will remain asymptomatic with deterioration of the ipsilateral kidney function.

Slide 5

It is for sure that in the early eighties at the beginning of prenatal diagnosis we have been operating on much too many of these babies. It is very rare today that a neonatal surgery is requested in a baby with a palpable mass of the left flank corresponding to this huge dilated kidney above a PUJ obstruction. A nephrostomy stent was inserted allowing to evaluate the ipsilateral kidney function, which was nicely preserved before the baby was repaired.

Slide 6

So if we come to surgical indications these are, of course, indicated in cases of symptoms and the most frequent of them is UTI, more rarely hematuria or pain. Increasing dilatation is the second major cause of surgical indications, which lead me to say that one of our best tools to follow up these babies is still ultrasound, even if we do not have the nice 3D ultrasound that was shown just before.

Slide 7

Most of paediatric urologists do not believe very much in the elimination curve of the MAG3 or the MRI, which is more reflecting the compliance of the urinary tract than the pressure and its effect on the ipsilateral function. So what we used in most cases is the ipsilateral function on two successive evaluations whether this is done by the actual gold standard, the MAG3 scan or by the MRI studies and we are just now starting a prospective study to compare these two tools, which still needs to be done. Prognosis is good in these babies and even on the cosmetic point in very young babies we can do bilateral surgery without any scars either by doing posterior approach or by using modern tools like retroperitoneoscopy, which has been developed in our department.

Slide 8

Second anomaly I want to talk about are the wide ureters and as you know, they might be primary or secondary to PUV, neurogenic bladder or duplex system. Primary wide ureters maybe refluxing or not. The prenatal diagnose of non-refluxing primary wide ureters has shown that most of them will resolve spontaneously during the first months of life.

Slide 9

Here is an old study by IVP of a baby at 3 months of age with these huge wide ureters, which were non-refluxing in this VUJ.

Slide 10

And the same baby a few months later who has good improvement of his upper urinary tract and return to normal of his ureter a few months later.

Slide 11

The Philadelphia group published in 2000 their review of primary wide ureters prenatally diagnosed and you see that very few of these wide ureters were operated on for progressive deterioration of the ipsilateral function or for an increasing dilatation of the upper urinary tract. Surgery more was often indicated because of febrile UTI  than decreased renal function.

Slide 12

Duplex system. It maybe associated, as everyone knows, with an obstruction of the upper pole either by ectopic insertion of the upper pole ureter or by an ectopic ureterocele and it can also be associated with a lower pole reflux.

Slide 13

What is now known but it is a rather recent discovery is that the natural history of lower pole refluxing ureter is the same as in single ureters. The chance of spontaneous resolution of reflux associated to a duplex system is not different than in single ureters, which means that the management will be exactly the same.

Slide 14

Upper pole dilatations. First our studies among others have shown that the dilated upper pole is seldom dysplastic, it’s more often with inflammatory damages than with dysplastic histology, which has led us to propose very often a conservative approach each time that there was residual function in the upper pole kidney. In case of an ectopic insertion of the upper pole, this conservative treatment may either be a reimplant or an anastomosis between the upper pole and the lower pole ureter.

Slide 15

In case of ureterocele it maybe developed either in the bladder or protruding itself in the ureter. Intravesical ureterocele are in most cases easily treated by endoscopic incision and in more than 2/3 of the cases this will be the only treatment that these babies will need. Extravesical ureterocele will more often require a complex surgical approach including upper pole heminephrectomy, ureterocelectomy and reimplantation of the lower pole ureter.

Slide 16

Primary VUR. What have we learnt about primary VUR in recent years? I will not come back about the genetic history and we have been doing studies, prospective studies in VUR II but what I would like to add to what has already been said about the familial character of this disease is that what our study has shown is that it is of no benefit to the siblings of index cases to be looked at for reflux by VCUJ. So our concern to these families nowadays is to do very attentive ultrasound prenatally and postnatally on these babies and not to do VCUJ unless they would have febrile UTI but this maybe discussed. We have also learned by prenatal diagnosis that VUR is more frequent in males than female babies with a sex ratio of 3:1 in the prenatally diagnosed cases.

Slide 17

The reflux nephropathy is more the consequence of congenital lesions than of UTI scars. The DMSA scans on prenatally diagnosed reflux often show abnormalities before any UTI has occurred and this has been shown by C.K. Yeung in Great Ormond Street.

Slide 18

The so-called primary VUR is, in fact, very often a secondary one or associated to some degree of bladder dysfunction and dyselimination syndrome. Koff in Ohio has emphasised the importance of treating both the voiding problems and the stool retention often present in children with VUR.

Slide 19

The spontaneous resolution of VUR is the rule. Both in prenatally diagnosed cases in the absence of DMSA scan damage even in high grade reflux and this has been shown also by the Great Ormond Street group. In most symptomatic cases if the reflux is a low grade 1, from 1-3.

Slide 20

It is well-established from 1992 that surgery is not better than medical treatment in preventing renal scars. So surgery is indicated in cases of breakthrough infection despite an adequate medical treatment, which is not only antibioprophylaxis but also bladder management. It may consist either in endoscopic treatment and our tool of choice is now the Deflux® or ureteral reimplantation.

Slide 21

Now, I would like to finish this brief overview of paediatric urology about the posterior urethral valves. They are really mostly diagnosed prenatally. In the last 20 years in our department we have been taking care of 105 cases of PUV among which 80 were prenatally diagnosed. 15 of these 80 were electively terminated because of early anamnios, dysplastic cystic kidneys or poor biochemical data on foetal urine or foetal serum.

Slide 22

63 of 65 newborns with prenatally diagnosed PUV were treated at birth during the 24 first hours of life by primary valve ablation. In most of these patients this was the only surgical treatment.
53 of these 65 had the bilateral dilatation of the upper urinary tract on the first Ultrasound and 36 of them had an associated reflux.
43 have experienced at least one episode of UTI despite initial treatment of their obstruction.

Slide 23

PUV remains a uropathy with a poor prognosis both in terms of renal and bladder function. It is also a difficult pathology for the transplant surgeon and Salomon et al in Paris have shown on a very large series that 10 years after transplantations, PUV patients have a poorer result with higher creatinine level than children transplanted for other causes of end-stage renal failure. 

Slide 24

So in conclusion Ladies and Gentlemen the prenatal diagnosis of urinary tract anomalies has modified the surgical approach of these children and our knowledge of the natural history of these malformations. Surgery is seldom indicated at birth but a careful monitoring by the paediatric nephrologist and urologist is required. Thank you.

Slide 25

 

Chairman: Thank you very much for this talk. It’s open now for discussion. Any comment or question from the audience?

Question: I’m Doctor Serin from India. How would you compare your experience with deflux as you mentioned with ureteral reimplantation? Would you suggest we go in for deflux therapy and which patients would you chose for deflux and which patients would you choose for ureteral reimplanatation? That is question number 1. Number 2 would you like to dwell a little on hyopsedias? I recently had a patient with urethral hyposedias and we did a kidney transplantation, the hypospadias was well dealt surgically pretransplant but yet the patient had a lot of UTI. So hypospadia in transplantation. Would you like to say something of that?

Prof Aigrain: I’m not sure I got correctly the second question so please stay on the microphone because I may need your help. For the first one between endoscopic treatment and reimplant. We have bad experience in duplex cases but this is not the case for all groups with endoscopic treatments, so nowadays we would consider endoscopic treatment in every case requiring surgery from grade 1-4 reflux on single ureters either unilateral or bilateral as the first step of the treatment and we would repeat these injections in case of failure once before going to reimplantation.

Question: Secondly I wanted you to dwell a little on hypospadias of the urethra.

Prof Aigrain: Hypospadias of the urethra and transplantation?

Question: I mean what are your experiences with hypospadia in relation to chronic kidney failure?

Prof Aigrain: Well hypospadias, we have a very large experience because as most of you may know hypospadias is a malformation which incidence is increasing in every developed country for reasons which may include environmental factors but this is not sure yet so we have a large experience with  hypospadia surgery. This is never a minor surgery. This should be done by people used to have this type of surgery but you now in cases where there is no anomaly in the size of the appendix you can now obtain good functional results both for micturation and in anterior cases for sexual development of these children. I would be much more careful on the long-term results for posterior hypospadias about sexual outcome.

Question: You mentioned 3:1 male: female ratio in VUR. What is the explanation? And to build the bridge with the first communication, is it the same figure in the familial form of VUR and what again could be the explanation?

Prof Aigrain: I don’t know and maybe Doctor Wolf will complete my answer but it’s for sure that before prenatal diagnosis most urologists and I guess paediatric nephrologists would have answered that VUR was most frequently seen in girls and that we saw them from 18 months to 3-5 years with UTI but as the prenatal diagnosis has shown that boys have more frequent reflux that they have higher grade reflux but also that this reflux may resolve spontaneously. How you can figure this with the genetics? I prefer that Doctor Wolf would answer.

Question: Well, you raised an interesting point and I think it’s true that if you look at all children with primary VUR, male and female are about equally affected and in the screening genetic studies that have been done historically you have again about equal numbers of males and females. Of course, you’re both talking about a relatively newly realised population, the boys born with very severe VUR. I don’t believe that anyone has done a systematic familial screening study on that very special population but you know one would think are there modifying genes on the you know X-chromosome for example, or one might think is it different in boys because the urine has to get out through the urethra as well. So does that induce a degree of resistance? But you would be able to…

Prof Aigrain: Yes. Somehow I’m wondering but I have no scientific data to give to you if in boys the cause of the reflux may very well be a transient disinertia between the bladder and the sphincter. A group in London is advocating this very much and this may also be somehow linked to some genetic factors and this will evolve spontaneously during the first year of life with correcting this disinertia and the reflux disappearing.

Question: What do you do with a child that has a megaureter but who also appears to have ureteric stenosis at the vescicoureteric junction other than just following them up?

Prof Aigrain: Well, there is very often a narrow end of these wide aortas at the junction, ureteral vescical junction that’s very often the case and it may even be very difficult to pass a catheter in this wide ureter and despite this fact, this ureter may very well improve during the growth of the child. So very often we do not know when we are taking care of these babies at the beginning if there is a real stricture or if it is only a dynamic process, which is giving difficulty to the ureter to push the urine into the bladder due to some muscular arrangements or so. So we on all of them, we follow them very carefully mainly during the first year to see if they are improving or not. If UTI is happening during the very first months of life of these babies what we are trying to do now but this is on a prospective basis so I cannot tell you that this is what you should do, is to insert doubles chase stents in these babies to avoid reimplanting while they have a tiny bladder where this surgery is a major one.