Original article 277
20 Years of Functional Reconstructive Surgery in Bladder Exstrophy - Balance 10 Years After a Preliminary Report P. acher, M. chwöbel, U. G. Stauffir Depaltmenl of Surgery, eniversily Children 's Hospital, Zurich, Swilzerland
This is an update of a consecutive series of 19 patients with bladder exstrophy presented as a preliminary report 10 years aga with a follow-up of 6 years. The average time of follow-up now is 14 years. In difference to the former reporl everal additional procedures have been performed in most patients. 3 earl)" "good" patients stay "good and continent". Onl)" 3 out of 5 patients categorized as "fair" are no\\" "good and continent" and only one \\'ithout furlher inten·ention. 3 out of 6 patients who were c1assified as "poor" are now diverted. The overall rate of secondary di\'ersions is 40 % including 2 patients \\ith ureteroigmoidostomies. All patients have normal renal function and normal blood pressures. These results show that an early "good" result generally remains "good" but additional surgery may be warranted. The initially "fair" results usually necessitate a secondary procedure, e. g. augmentation cystoplasty or/and bladder neck tightening to show an improvement. Patients with early "poor" results unfortunately remain "poor" and have a great chance to get diverted. Key words Bladder exstrophy - Urinary diversion Lang-term results Resume Les auteurs presentent une revue d'une serie consecutive de 19 malades atteints d 'exstrophie vesicale et deja presentee en rapport preliminaire 10 annees auparavant avec un follow-up de 6 ans. La periode moyenne du followup actuel est de 14 ans. Quelques interventions additionnelles ont ete reali ees chez de nornbreux patients. Trois ca consicteres comme bons resultats, restent bons et continents. eulement 3 des 5 patients consideres comme mediocres sont maintenant bons et continents et eulement 1 sans intervention ulterieure. 3 des 6 patients qui ont ete classes comme mauvais sont maintenant derives. Le pourcentage general de derivation secondaire est de 40 % incluant 2 patients avec ureterosigmo'idostomie. Tous les patients ont une fonction renale normale et une pression sanguine normale. Ces constatations demontrent qu 'un resultat primitivement bon reste habituellement bon mais que des inten'entions chirurgicales complementaires peuvent elre necessaires. Le resultat
L
Received Oclober 20. 1991 Eur J Pedialr Surg 2 (1992) 211-280 Hippokrales "erlag Slullgarl ..\Iasson Edileur Paris
(!:)
initialement mediocre necessite habituellement une inten'ention iterative, par exemple cystoplastie d'aggrandissement ou elargissement du col vesical pour amener une amelioration. Les patients, avec un resultat primitivement mauvais, restent malheureusement definitivement mauvais et ont un grand risque de e voir derives. Mots-cll~s
Exstrophie vesicale - Derivation urinaire Resultats
a long terme Zusammenfassu ng - - - -
Es werden 19 Patienten, bei denen wegen einer Ekstrophie eine Blasenrekonstruktion durchgeführt worden war, 10 Jahre nach einer ersten Bestandsaufnahme mit einer mittleren Nachbeobachtungszeit von 6 Jahren nochmals vorgestellt. Die durchschnittliche Nachbeobachtungszeit beträgt nun im Durchschnitt 14 Jahre. Bei der Mehrzahl der Patienten wurden seit dem letzten Bericht weitere Eingriffe durchgeführt. 3 initial "gute" Fälle sind weiterhin "gut und kontinent". ur 3 von 5 Patienten, die vor 10 Jahren als "genügend" beurteilt wurden, sind nun "gut und kontinent", nur einer davon ohne weitere Eingriffe. 3 von 6 Patienten mit einern primär "ungenügenden" Resultat haben nun eine Umleitungsoperation. Die Rate von sekundären Umleitungen beträgt 40 %, mit eingeschlossen 2 Patienten mit einer Ureterosigmoidostomie, Alle Patienten haben eine normale ierenfunktion und einen normalen Blutdruck. Die Resultate dieser kleinen konsekutiven Serie zeigen, daß ein initial gutes Resultat meistens gut bleibt. Die Patienten mit initial genügendem Resultat benötigen in den meisten Fällen weitere Eingriffe, wie eine Augmentationszystoplastik und! oder eine Kontinenzplastik, um sich zu verbessern. Diejenigen Patienten mit einern initial ungenügenden Resultat bleiben leider häufig ungenügend. Bei den meisten dieser Patienten muß das initiale Management der Blasenrekonstruktionschirurgie verlassen und eine sekundäre Umleitungsoperation vorgenommen werden. Schlüsselwörter Blasenekstrophie - Harnumleitungsoperationen - Langzeitresultate
Downloaded by: Universite Laval. Copyrighted material.
Summary
Eur J Pediatr Surg 2 (J 992)
P. Sacher et al
low-pressure reservoir and attalnment of a satisfactory cosmetic and functional phallu in boys (6).
Inlroduction This report deals wiLh lhe ame series of 19 patients wiLh bladder exstrophy that were presented 10 years ago (11). The aim of this article is to elucidate the evolution of each case untiJ puberty, i. e. definite correction of Lhe malformation, and to asse s whether a follow-up of 6 years chosen for Lhe first report in 1982 is safe enough or not.
We were especially interested to ee Lhe individual evolution of every patient since the last report. With one exception, all patients have now reached or passed puberty.
Results .\talerials and meLhods Our series of 19 patients includes 15 boys and -I girls. All 19 patients had slaged funcLional c10sure of bladder exstrophy, no primary urinary diversion was undeltaken. Ages at bladder c10sure ranged from 10 days to 6 Ih years. Reconstruction consisted of a staged proced ure: (I) posLerior peh'ie osteotomy according to Schultz, (2) Turn-in-operation simultaneously wiLh bladder neck reconsLruction and a ureteral reimplant mostly by Politano-Leadbeller technique and (3) eloure of epi padias if necessary eombined with a ) ollllg-DeesLeadbeller bladder neck tightening. In 1981 Lhe resuJts were categorized as "good", "fair" or "POOl''' (li). PaLients in whom time elapse since turnin-operation \\'as less than 3 real' \\'ere not been da sified. Criteria for a "good ,. result \\'ere normal renal function, no signs of obstructive uropathy, no recurrent urinary infections and a urinary continence of at least 2 hours. Actual data and radiographs or ultrasound examinations of all patients ha\'e been collected and analyzed. A questionnaire has been sent to those patients who are not treated at our institution any longer. Patients Lhat were suitable for analysi have been classified as "good/continent", "diverted" or "incontinent". Nowadays criteria of a "good/continent" result consist of preservation of renal function, creation of a continent
Table 1
5 5 6 3
Pat.
Sex
17 9/ 12
Sch. S M. P
RR AJ St L E
FF V E
RA L S B S
J eh. D I. KG D AG W St
Time since
m m m I m I m m m m m m m m ~~-
17 7/12 14 6/ 12 18 1/ 12 13 6 / 12 17 2 12 14 2/ 12 17 5/ 12 13 5 12 14 3 12 12 I ~2 15 le 12 11 5/12 10 6/:2
10 11 4/ 12
11 out of all 19 patients are till treated at OUf institution. Sinee our last report foul' patients ha\'e been operated on at other institutions. Three patients living in foreign countries were lost for follow-up. Therefore Lhe results of 16 patients are presented. The a\'erage time of follow-up i no\\' 14 (min. 10, max. 18) years. 6 out of 16 patients (38 %), --l boy and 2 girls, may be categorized as "good/continent" according to Lhe above mentioned criteria of HlISlllan et al (6).
Six patients haw an external or internal urinary diversion. Foul' of them ha\'e a continent diversion i. e. twiee a ureterosigmoidostomy and a MitrofanojJ'bladder and a .\1ainz pouch one eaeh. In one patient the eutaneou ureterostomie ha\'e not yet been taken down and in another one construction of a cutaneous ureterostomy has been necessary due to obstructive uropathy in a single kidney. Three patients are till incontinent. OnJy one of them is treated aL OUf institution any longer. A recently performed augmentationcystopla ty did not alter urinary incontinence ignificantly. A bladder neck tightening is planned. None of our patients has lost a kidney. All 16 patients have normal renal function and normal blood pressures. The clinicaJ and laboratory data at present follow-up are summarized in Table 2.
19 patlents wlth bladder exstrophy 1971-1980.
good lalr poor no classllied
The resuJts of our first report in 1982 (11) with an a\'erage follo\\'-uP of 6 Ih year are ummarized in Table 1. At that time 2 patients had cutaneous ureterostomies as a secondary di\'ersion. No patient had had an augmentationcystoplasty or ureterosigmoidostomy or an undiversion.
(31 %) (31 %) (38%)
Figure 1 shows Lhe individual evolution of OUf 19 patients since the last report. Three out of 5 patients who
were categorized as "fair" are now "good/continent". One \vith-
um-In
Renal lunctlon
Blood pressure
IVP/US
Table 2 Time 01 ollow-up and data o 16 patlents wlth bladder exs rophy
normal normal normal normal normal normal normal normal normal norma' norma' normal normal normal normal normal
normal normal normal normal normal normal normal norma' norma norma' normal norma normal norma norma' normal
normal normal normal mild hydronephrosis normal normal normal normal normal normal m Id hydronephrosis normal mild hydronephrosis normal mild hydronephrosis normal
(1990)
Downloaded by: Universite Laval. Copyrighted material.
278
20 Years ofFunctional Rewnstructive Surgery in Bladder Exstrophy - Balance 10 Years After a Preliminary Report
600D/CONTINENT
[600D ? ?
p.M .• m
O.M.A .•m 5ch.5 .• 1 M.P .• I Iu/ .. m
augmentation cysloplasly Voung-Oees-Le.dbeller
I
I~
FAIR
A.J .• m
5LM.m
LE..I
6ugmenlatlOn cystoplasty
F.F .m V E .1
~IDIVERTEDI ureterosl gmoi dostomy
R.A m
l.J.m (C:\Jt~n.ous ur.t'r'"os10ml.s:) Sl.Ch.m (CU1Ml,ousurttfor-ostomws)
NOT
----
MA I NZ-pouch cutaneous ureterostomles
~
Mllrol.noff-blodder cutnneous ureterostomies
?
LG.m B.o.• m 5.I.m
I
_
"-.... '-........~
CLASS~IE~ß....
K.Gm
O·AG .• m
~~
/'
W.51.m
~
IINCONTINENT
I
~
augmentation cysloplasly
Fig.l
out further inten'ention, one with an additional augmentation cystopla ty and one with augmentation cy toplasty combined with a secondary bladder neck tightening. Three out of 6 patients who \-\'ere c1assified as "poor" are divelted. One has a ureterosigmoidostomy, one a 1\1ainz pouch and one has still cutaneous ureterostomies. Two of the formerly unclassified cases are now diverted. One has a Mitrofanoffbladder and the other one a cutaneous ureterostomy resulting in an overall rate of 40 % of secondary diversions. Since our last report an augmentation cystoplasty has been performed in 4 boys. 5 boys underwent orthoplasty of the penis according to Ransley and Kramer. A vaginoplasty has been performed in two girls,
Discussion 10 years ago we stated that the ideal result of recon tructive urgery in children \\ith bladder ex trophy would be a child with total urinary continence, free of recurrent urinary tract infections, normal kidneys and normal renal function, . owadays the goals of treatment - presen'ation of renal function and creation of a continent low-pressure resen'oir are upplemented \\'ith attainment of a satisfactory cosmetic and functional phallus in boy (6), -
E\'en in the light of these criteria 6 out of 16 patients (38 %) show a "good/continent" result. This lies in between the 25 % of the BAPS re\'ie\\' b\' de lu IIlI/zt et al (3) and the 86 % reported by Oesterling andJ;jJs (10). According to our consecutive series of 19 patients with bladder exstrophy, the time of bladder c10sure seems
279
not to be of importance for a succes ful outcome since onJy one ofthe 6 "good/continent" patients ha been operated on as a neonate. All the other 5 children were older than one year when the turn-in-operation was undertaken. One girl was even 6 1/2 year old, This is in contra t to the statement of Montagnani (9) that a prolonged exposure of the exstrophied bladder to mechanical irritation and infection wouJd be a main factar for unsuccessful outcome, . evertheless most authors including our group tend today towards closure of the bladder during the first 48 hours of life, the main reason being that pehic osteotomy may not be necessary and bladder closure is much easier, In the present series an early diversion, i. e, cutaneous ureterostomy, has been necessary in two cases, probably due lo an obstructive uropathy following a primary ureteral reimplantation in a thickened bladder wall simultaneously with the turn-in-operation, Therefore, primary reimplantation is no Ionger performed at our institution, The ureters are only reimplanted no\-\' at the time of the Young-Dees-Leadbetterprocedure (8, 10). Reconstructi\'e surgery has an imminent risk for an obstructi\'e uropathy and consequent renal damage and insufficiency, A few centers therefore still favour primary urinary diwrsion, e. g. a ureterosigmoidostomy as a beller solution for the problem, One reason for thi management is the fact thatthe upper urinal)' tract is primar')' normal, i. e. there is no hydronephrosis or ureteral dilatation. Fröhneberg et aI (4) demonstrated in a review of 38 patients that after a ureterosigmoidostomy no deterioration is to be expected prO\ided that the upper urinal')' tract is preoperati\'ely normal. Howe\'er, Husmann et aI (6) shO\\'ed that in patients managed \\ith a uri-' nary diversion the occurrence of renal damage was notably higher even with antireflux techniques. This is in agreement with Mollard (8) who states that in long-term view the risks of reconstructive surgery for the kidneys eems to be minor compared to internal and external diver ions. One major problem that has been argued again t ureterosigmoidostomy is the possibility of neoplastic complications (6, 7, 12), Therefore many authars believe that ureterosigmoidostomy is no more an acceptable means of urinary diversion. However, there are recent reports about carcinoma arising after ileocystoplasty (5) and indeed "it is not unreasonable to believe that the incidence of cancer in augmented bladder may not be appreciably different from that seen in ureterosigmoidostomy" as Allen (1) states in an Editorial Comment in the Journal of Urology in 1989. In the light of these facts we believe that ureterosigmoidostomy is still an alternative for a limited number of patients \\ith bladder exstrophy, especially when taking into consideration the necessity of the patient's compliance to intermillent catheterization, the possibility of the bladder needing augmentation and the p ychological impact of wet tomata. \\'e think that the individual history of a patient after reconstruction is of special interest and imporlance, .'.lost reports, ho\\'e\'er, deal \\'ith "numbers and percentages". Our indi\'idual re ults ho\\ that an early "good" re ult gene rally remains "good". The initially "fair" results usually necessitate a secondary procedure, e. g. augmentation cy toplasty and/or bladder neck tightening, to show an im-
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1990
1981
Eur J Pediatr Surg 2 (1992)
Eur J Pediatr Surg 2 (1992)
P. Sacher et al
provement. Patients with early "poor" results remain unfortunately poor and have a great chance to get diverted. Girl have a rar better chance for a successful reconstruction of bladder exstrophy than boys. In more than half of the boys in oUf eries reconstructive surgery had to be abandoned. Ideally, the reconstruction of the bladder should be achieved at the age of school entrance.
References ----
lienls wiLh bladder exslrophy. J Urol 139 (1988) 719-722 8 Mollard P, Basset T, Oeseubis .11, el al: Re ultals de la reeon truetion \'e icale el urelrale paur exslrophie. Chir Pedialr 27 (1986) 27-32 9 ,\lontagnani CA: Funelional reeonstruelion of ex lrophied bladder. Timing and leehnique. Follo\\-up of 39 eases. Z Kinderehir 43 (1988) 322330 10 Oesterling} E.}effi RO: The imporlanee of a ueees ful inilial bladder do-
11
rillen Tri Edilorial eommenl. J Crol I 12 (1989) 795 TI 1', Lutlimer}/\, et al: Lang-term follo\\up of 207 patienls \\ith bladder exstrophy: An e\'olution in treatment J l 'rol I 12 ( 1989) 793-796 J de /a Hunt ,\t.\'. ODollllell B Clirrent managemenl of bladder exstrophy: A BAPS eolleeti\'e rede\\ from eight eentre of 81 patienls born bet\\een 1975 and 1985. J Pediatr Slirg 2-1 (1989) 58-1-585 I Frö/lIleberg Oll. Hohellfellller R. Strul/b E' l'reterosigmoidoslomy in bladder exstrophy. Eur L'rol 9 (1983) 133-1::18 5 Hu,egawa S, O,hima S. Killukawa T cl al: Adenoeareinoma of the bladder 29 years after ileoey,toplasty Hrit J l'rol 61 (1988) 162 li lJ",mullll DA .. \IcLoJie G.·\, C/lltrtlzilll3.\t. A eompam.on of renal fune lion in the ex
20 Years of Functional Reconstructive Surgery in Bladder Exstrophy - Balance 10 Years After a Preliminary Report P. acher, M. chwöbel, U. G. Stauffir Depaltmenl of Surgery, eniversily Children 's Hospital, Zurich, Swilzerland
This is an update of a consecutive series of 19 patients with bladder exstrophy presented as a preliminary report 10 years aga with a follow-up of 6 years. The average time of follow-up now is 14 years. In difference to the former reporl everal additional procedures have been performed in most patients. 3 earl)" "good" patients stay "good and continent". Onl)" 3 out of 5 patients categorized as "fair" are no\\" "good and continent" and only one \\'ithout furlher inten·ention. 3 out of 6 patients who were c1assified as "poor" are now diverted. The overall rate of secondary di\'ersions is 40 % including 2 patients \\ith ureteroigmoidostomies. All patients have normal renal function and normal blood pressures. These results show that an early "good" result generally remains "good" but additional surgery may be warranted. The initially "fair" results usually necessitate a secondary procedure, e. g. augmentation cystoplasty or/and bladder neck tightening to show an improvement. Patients with early "poor" results unfortunately remain "poor" and have a great chance to get diverted. Key words Bladder exstrophy - Urinary diversion Lang-term results Resume Les auteurs presentent une revue d'une serie consecutive de 19 malades atteints d 'exstrophie vesicale et deja presentee en rapport preliminaire 10 annees auparavant avec un follow-up de 6 ans. La periode moyenne du followup actuel est de 14 ans. Quelques interventions additionnelles ont ete reali ees chez de nornbreux patients. Trois ca consicteres comme bons resultats, restent bons et continents. eulement 3 des 5 patients consideres comme mediocres sont maintenant bons et continents et eulement 1 sans intervention ulterieure. 3 des 6 patients qui ont ete classes comme mauvais sont maintenant derives. Le pourcentage general de derivation secondaire est de 40 % incluant 2 patients avec ureterosigmo'idostomie. Tous les patients ont une fonction renale normale et une pression sanguine normale. Ces constatations demontrent qu 'un resultat primitivement bon reste habituellement bon mais que des inten'entions chirurgicales complementaires peuvent elre necessaires. Le resultat
L
Received Oclober 20. 1991 Eur J Pedialr Surg 2 (1992) 211-280 Hippokrales "erlag Slullgarl ..\Iasson Edileur Paris
(!:)
initialement mediocre necessite habituellement une inten'ention iterative, par exemple cystoplastie d'aggrandissement ou elargissement du col vesical pour amener une amelioration. Les patients, avec un resultat primitivement mauvais, restent malheureusement definitivement mauvais et ont un grand risque de e voir derives. Mots-cll~s
Exstrophie vesicale - Derivation urinaire Resultats
a long terme Zusammenfassu ng - - - -
Es werden 19 Patienten, bei denen wegen einer Ekstrophie eine Blasenrekonstruktion durchgeführt worden war, 10 Jahre nach einer ersten Bestandsaufnahme mit einer mittleren Nachbeobachtungszeit von 6 Jahren nochmals vorgestellt. Die durchschnittliche Nachbeobachtungszeit beträgt nun im Durchschnitt 14 Jahre. Bei der Mehrzahl der Patienten wurden seit dem letzten Bericht weitere Eingriffe durchgeführt. 3 initial "gute" Fälle sind weiterhin "gut und kontinent". ur 3 von 5 Patienten, die vor 10 Jahren als "genügend" beurteilt wurden, sind nun "gut und kontinent", nur einer davon ohne weitere Eingriffe. 3 von 6 Patienten mit einern primär "ungenügenden" Resultat haben nun eine Umleitungsoperation. Die Rate von sekundären Umleitungen beträgt 40 %, mit eingeschlossen 2 Patienten mit einer Ureterosigmoidostomie, Alle Patienten haben eine normale ierenfunktion und einen normalen Blutdruck. Die Resultate dieser kleinen konsekutiven Serie zeigen, daß ein initial gutes Resultat meistens gut bleibt. Die Patienten mit initial genügendem Resultat benötigen in den meisten Fällen weitere Eingriffe, wie eine Augmentationszystoplastik und! oder eine Kontinenzplastik, um sich zu verbessern. Diejenigen Patienten mit einern initial ungenügenden Resultat bleiben leider häufig ungenügend. Bei den meisten dieser Patienten muß das initiale Management der Blasenrekonstruktionschirurgie verlassen und eine sekundäre Umleitungsoperation vorgenommen werden. Schlüsselwörter Blasenekstrophie - Harnumleitungsoperationen - Langzeitresultate
Downloaded by: Universite Laval. Copyrighted material.
Summary
Eur J Pediatr Surg 2 (J 992)
P. Sacher et al
low-pressure reservoir and attalnment of a satisfactory cosmetic and functional phallu in boys (6).
Inlroduction This report deals wiLh lhe ame series of 19 patients wiLh bladder exstrophy that were presented 10 years ago (11). The aim of this article is to elucidate the evolution of each case untiJ puberty, i. e. definite correction of Lhe malformation, and to asse s whether a follow-up of 6 years chosen for Lhe first report in 1982 is safe enough or not.
We were especially interested to ee Lhe individual evolution of every patient since the last report. With one exception, all patients have now reached or passed puberty.
Results .\talerials and meLhods Our series of 19 patients includes 15 boys and -I girls. All 19 patients had slaged funcLional c10sure of bladder exstrophy, no primary urinary diversion was undeltaken. Ages at bladder c10sure ranged from 10 days to 6 Ih years. Reconstruction consisted of a staged proced ure: (I) posLerior peh'ie osteotomy according to Schultz, (2) Turn-in-operation simultaneously wiLh bladder neck reconsLruction and a ureteral reimplant mostly by Politano-Leadbeller technique and (3) eloure of epi padias if necessary eombined with a ) ollllg-DeesLeadbeller bladder neck tightening. In 1981 Lhe resuJts were categorized as "good", "fair" or "POOl''' (li). PaLients in whom time elapse since turnin-operation \\'as less than 3 real' \\'ere not been da sified. Criteria for a "good ,. result \\'ere normal renal function, no signs of obstructive uropathy, no recurrent urinary infections and a urinary continence of at least 2 hours. Actual data and radiographs or ultrasound examinations of all patients ha\'e been collected and analyzed. A questionnaire has been sent to those patients who are not treated at our institution any longer. Patients Lhat were suitable for analysi have been classified as "good/continent", "diverted" or "incontinent". Nowadays criteria of a "good/continent" result consist of preservation of renal function, creation of a continent
Table 1
5 5 6 3
Pat.
Sex
17 9/ 12
Sch. S M. P
RR AJ St L E
FF V E
RA L S B S
J eh. D I. KG D AG W St
Time since
m m m I m I m m m m m m m m ~~-
17 7/12 14 6/ 12 18 1/ 12 13 6 / 12 17 2 12 14 2/ 12 17 5/ 12 13 5 12 14 3 12 12 I ~2 15 le 12 11 5/12 10 6/:2
10 11 4/ 12
11 out of all 19 patients are till treated at OUf institution. Sinee our last report foul' patients ha\'e been operated on at other institutions. Three patients living in foreign countries were lost for follow-up. Therefore Lhe results of 16 patients are presented. The a\'erage time of follow-up i no\\' 14 (min. 10, max. 18) years. 6 out of 16 patients (38 %), --l boy and 2 girls, may be categorized as "good/continent" according to Lhe above mentioned criteria of HlISlllan et al (6).
Six patients haw an external or internal urinary diversion. Foul' of them ha\'e a continent diversion i. e. twiee a ureterosigmoidostomy and a MitrofanojJ'bladder and a .\1ainz pouch one eaeh. In one patient the eutaneou ureterostomie ha\'e not yet been taken down and in another one construction of a cutaneous ureterostomy has been necessary due to obstructive uropathy in a single kidney. Three patients are till incontinent. OnJy one of them is treated aL OUf institution any longer. A recently performed augmentationcystopla ty did not alter urinary incontinence ignificantly. A bladder neck tightening is planned. None of our patients has lost a kidney. All 16 patients have normal renal function and normal blood pressures. The clinicaJ and laboratory data at present follow-up are summarized in Table 2.
19 patlents wlth bladder exstrophy 1971-1980.
good lalr poor no classllied
The resuJts of our first report in 1982 (11) with an a\'erage follo\\'-uP of 6 Ih year are ummarized in Table 1. At that time 2 patients had cutaneous ureterostomies as a secondary di\'ersion. No patient had had an augmentationcystoplasty or ureterosigmoidostomy or an undiversion.
(31 %) (31 %) (38%)
Figure 1 shows Lhe individual evolution of OUf 19 patients since the last report. Three out of 5 patients who
were categorized as "fair" are now "good/continent". One \vith-
um-In
Renal lunctlon
Blood pressure
IVP/US
Table 2 Time 01 ollow-up and data o 16 patlents wlth bladder exs rophy
normal normal normal normal normal normal normal normal normal norma' norma' normal normal normal normal normal
normal normal normal normal normal normal normal norma' norma norma' normal norma normal norma norma' normal
normal normal normal mild hydronephrosis normal normal normal normal normal normal m Id hydronephrosis normal mild hydronephrosis normal mild hydronephrosis normal
(1990)
Downloaded by: Universite Laval. Copyrighted material.
278
20 Years ofFunctional Rewnstructive Surgery in Bladder Exstrophy - Balance 10 Years After a Preliminary Report
600D/CONTINENT
[600D ? ?
p.M .• m
O.M.A .•m 5ch.5 .• 1 M.P .• I Iu/ .. m
augmentation cysloplasly Voung-Oees-Le.dbeller
I
I~
FAIR
A.J .• m
5LM.m
LE..I
6ugmenlatlOn cystoplasty
F.F .m V E .1
~IDIVERTEDI ureterosl gmoi dostomy
R.A m
l.J.m (C:\Jt~n.ous ur.t'r'"os10ml.s:) Sl.Ch.m (CU1Ml,ousurttfor-ostomws)
NOT
----
MA I NZ-pouch cutaneous ureterostomles
~
Mllrol.noff-blodder cutnneous ureterostomies
?
LG.m B.o.• m 5.I.m
I
_
"-.... '-........~
CLASS~IE~ß....
K.Gm
O·AG .• m
~~
/'
W.51.m
~
IINCONTINENT
I
~
augmentation cysloplasly
Fig.l
out further inten'ention, one with an additional augmentation cystopla ty and one with augmentation cy toplasty combined with a secondary bladder neck tightening. Three out of 6 patients who \-\'ere c1assified as "poor" are divelted. One has a ureterosigmoidostomy, one a 1\1ainz pouch and one has still cutaneous ureterostomies. Two of the formerly unclassified cases are now diverted. One has a Mitrofanoffbladder and the other one a cutaneous ureterostomy resulting in an overall rate of 40 % of secondary diversions. Since our last report an augmentation cystoplasty has been performed in 4 boys. 5 boys underwent orthoplasty of the penis according to Ransley and Kramer. A vaginoplasty has been performed in two girls,
Discussion 10 years ago we stated that the ideal result of recon tructive urgery in children \\ith bladder ex trophy would be a child with total urinary continence, free of recurrent urinary tract infections, normal kidneys and normal renal function, . owadays the goals of treatment - presen'ation of renal function and creation of a continent low-pressure resen'oir are upplemented \\'ith attainment of a satisfactory cosmetic and functional phallus in boy (6), -
E\'en in the light of these criteria 6 out of 16 patients (38 %) show a "good/continent" result. This lies in between the 25 % of the BAPS re\'ie\\' b\' de lu IIlI/zt et al (3) and the 86 % reported by Oesterling andJ;jJs (10). According to our consecutive series of 19 patients with bladder exstrophy, the time of bladder c10sure seems
279
not to be of importance for a succes ful outcome since onJy one ofthe 6 "good/continent" patients ha been operated on as a neonate. All the other 5 children were older than one year when the turn-in-operation was undertaken. One girl was even 6 1/2 year old, This is in contra t to the statement of Montagnani (9) that a prolonged exposure of the exstrophied bladder to mechanical irritation and infection wouJd be a main factar for unsuccessful outcome, . evertheless most authors including our group tend today towards closure of the bladder during the first 48 hours of life, the main reason being that pehic osteotomy may not be necessary and bladder closure is much easier, In the present series an early diversion, i. e, cutaneous ureterostomy, has been necessary in two cases, probably due lo an obstructive uropathy following a primary ureteral reimplantation in a thickened bladder wall simultaneously with the turn-in-operation, Therefore, primary reimplantation is no Ionger performed at our institution, The ureters are only reimplanted no\-\' at the time of the Young-Dees-Leadbetterprocedure (8, 10). Reconstructi\'e surgery has an imminent risk for an obstructi\'e uropathy and consequent renal damage and insufficiency, A few centers therefore still favour primary urinary diwrsion, e. g. a ureterosigmoidostomy as a beller solution for the problem, One reason for thi management is the fact thatthe upper urinal)' tract is primar')' normal, i. e. there is no hydronephrosis or ureteral dilatation. Fröhneberg et aI (4) demonstrated in a review of 38 patients that after a ureterosigmoidostomy no deterioration is to be expected prO\ided that the upper urinal')' tract is preoperati\'ely normal. Howe\'er, Husmann et aI (6) shO\\'ed that in patients managed \\ith a uri-' nary diversion the occurrence of renal damage was notably higher even with antireflux techniques. This is in agreement with Mollard (8) who states that in long-term view the risks of reconstructive surgery for the kidneys eems to be minor compared to internal and external diver ions. One major problem that has been argued again t ureterosigmoidostomy is the possibility of neoplastic complications (6, 7, 12), Therefore many authars believe that ureterosigmoidostomy is no more an acceptable means of urinary diversion. However, there are recent reports about carcinoma arising after ileocystoplasty (5) and indeed "it is not unreasonable to believe that the incidence of cancer in augmented bladder may not be appreciably different from that seen in ureterosigmoidostomy" as Allen (1) states in an Editorial Comment in the Journal of Urology in 1989. In the light of these facts we believe that ureterosigmoidostomy is still an alternative for a limited number of patients \\ith bladder exstrophy, especially when taking into consideration the necessity of the patient's compliance to intermillent catheterization, the possibility of the bladder needing augmentation and the p ychological impact of wet tomata. \\'e think that the individual history of a patient after reconstruction is of special interest and imporlance, .'.lost reports, ho\\'e\'er, deal \\'ith "numbers and percentages". Our indi\'idual re ults ho\\ that an early "good" re ult gene rally remains "good". The initially "fair" results usually necessitate a secondary procedure, e. g. augmentation cy toplasty and/or bladder neck tightening, to show an im-
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provement. Patients with early "poor" results remain unfortunately poor and have a great chance to get diverted. Girl have a rar better chance for a successful reconstruction of bladder exstrophy than boys. In more than half of the boys in oUf eries reconstructive surgery had to be abandoned. Ideally, the reconstruction of the bladder should be achieved at the age of school entrance.
References ----
lienls wiLh bladder exslrophy. J Urol 139 (1988) 719-722 8 Mollard P, Basset T, Oeseubis .11, el al: Re ultals de la reeon truetion \'e icale el urelrale paur exslrophie. Chir Pedialr 27 (1986) 27-32 9 ,\lontagnani CA: Funelional reeonstruelion of ex lrophied bladder. Timing and leehnique. Follo\\-up of 39 eases. Z Kinderehir 43 (1988) 322330 10 Oesterling} E.}effi RO: The imporlanee of a ueees ful inilial bladder do-
11
rillen Tri Edilorial eommenl. J Crol I 12 (1989) 795 TI 1', Lutlimer}/\, et al: Lang-term follo\\up of 207 patienls \\ith bladder exstrophy: An e\'olution in treatment J l 'rol I 12 ( 1989) 793-796 J de /a Hunt ,\t.\'. ODollllell B Clirrent managemenl of bladder exstrophy: A BAPS eolleeti\'e rede\\ from eight eentre of 81 patienls born bet\\een 1975 and 1985. J Pediatr Slirg 2-1 (1989) 58-1-585 I Frö/lIleberg Oll. Hohellfellller R. Strul/b E' l'reterosigmoidoslomy in bladder exstrophy. Eur L'rol 9 (1983) 133-1::18 5 Hu,egawa S, O,hima S. Killukawa T cl al: Adenoeareinoma of the bladder 29 years after ileoey,toplasty Hrit J l'rol 61 (1988) 162 li lJ",mullll DA .. \IcLoJie G.·\, C/lltrtlzilll3.\t. A eompam.on of renal fune lion in the ex
