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Urinary tract reconstruction in prune belly syndrome
URINARY TRACT RECONSTRUCTION
IN
PRUNE BELLY SYNDROME
RON_4LD ?rlLARTIN JOHN
r\l. Il.
R4BINOM’ITZ BARKIN,
31. D.
F. SCHILLIN(;ER.
ROBERT
D. JEFFS.
31. I>. l1.D.
From the Division of Urology, The Hospital Sick Children. Toronto, Ontario. Canada
Considerable controwrsy exists regarding the rnanagen~t~nt of children with prune belly s!w drome. This entity, also called triad synclrome, is a cwngenital disorder almost universall) ~‘onfin(d to males and consists of deficienq~ of the abdolninal musculature, ureteral dilatation, and hilatcral cryptorchism. l-5 The literature is replettl with pleas for a nonoperative approach,’ as well as temporar) diwrsion in the form of ncphrostomy,’ cutaneous ureterostomy,x and v~sicostoin>~,” as well as permanent urinar), diversion and primar), reconstruction. ” Like so mail\. other entities, prune hell>, syndromt rrprestwts a spectrum of- a disease ranging from \rtar!’ nlild urinar!. tract involvement requiring no surgery to those with sevt:r-r wilw-y tract involwmc~nt. ” It is this latter group which is thtb suljec-t of this report.
1 PRUNE
BELLY
MEGAURETERS
f01
From 1965 through ISA, 25 ~nale\ with prune belly s!xdrome \\-ere cared fbr at The Hospital for Sick Children, Toronto. Fifteen of these children with massiveI> dilated upper tracts required surgical intell’eirtion lwcause of uncontrolled infection or progrt’ssi\.e loss of rcwal function. There were 27 Inassi\rtbly tliluted ureters in these 15 ho)x; 17 ‘,vc’I’c’associated \\ith reflux and 10 were not. Surgical treutment \vas initiated between tht, ages of one day and five years. Trn of these 15 hoyr s \vtArtJ treattd during the first month of life a11(1 6 during tht> first wcatlk of life. Snrgicd inter \.c,lrtion ~vas necessitatt~d 13,. uncont rollt~d iilfec*tioll in 7 (47 pt‘r cent) and/or progwssivc~ azotc~lnia iii 13 (93 per cent!.
1
Diversion
Reconstructed
Died
Tailo.red
Non-tailored
Reimplant
Reimplant
h!/.S rlf7)1)1(,
fc.illl
j”‘““”
hllr,
.S!/ll-
such a degree that nontailored ureteral reimplantation was performed, and the loop ureterostomies Lvere subsequently closed. Only 1 Case showed marked improvement; 2 continued to deteriorate, 1 with reflux. In 3 instances, reimplantation \vas supplemented b,; ureteral and all were successfd when uriuaq tailoring, tract continuity was restored. Five boys with 8 massively dilated ureters and severely dysplastic kidneys died of progressive azotemia in the first few weeks of life, despite adequate diversiou and control of infection. These children were found to have aLltops!.-prov~~l renal cl>ylasia. In 3 iustauces in \vhich contralateral function was satisfactory and reconstructable, prima) nel>hro~lreterectolrl~ for nonfunction and renal dysplasia was carried out. Six ureters Lvere managed by primary reimplantation ad tailoring without preliminary ureterostomy (Fig. 3). In one, this procedure was preceded by upper tract tailoring and nephrostomy. The results in this group were uniformly markedly successful. Follow-up has included physical examination, urinalysis and culture, intravenous urograin, voiding cystourethrogram, and renal function studies. These children have been followed for from one and one-half to ten years postreconstruction with an average length of pyelographic follow-up of four and one half years. As can be seen from Table I, wheu ureteral tailoring was combined with reilnplantation, whether as a primary procedure or as part of the reconstruction from loop cutaneous ureterostom); diversion, there was uniform success by
Treatment
and
TABLE
Results
The surgical management of these 27 pruue belly megaureters is summarized iu Figure 1. Neither cwtaneous vesicostmn~ nor teniporq uephrostom~ was carrid out in this group of patients. Ureteral dilatation mid tortuosity in thtw cliiltlrcw are so extensive that ~~~li~u diversion is required, it must he maintaiid for a long time. Under these circumstances, proximal urinar!. diversion is preferalde to distal diversion and nonintul~ated diversion is preferable to nephrostmn!. Loop cutaneous ureterostoni~ \vas performed in 18 instances. Despite the abdominal IIIUSC'Ulature deficiency these children tolerate loop cutaneous ureterostomy ~vell ant1 Lvithout troublesome prolapse (Fig. 2). In ‘7 cases ureteral caliber appeared to have improved to
‘3:31
Chnical
~lrinciry truct rc’c~ollstnrction in prune lady qnclrom
I.
Data
Reconstruction Intravenous pyelogram hlarked improvement Improved Stable Deteriorated No reflux Infection free Antibacterial prophylaxis Renal function Improved Stable Good result
Tailored ReimplauP
Nontailored Reimplant*
9
7
8 ( 89) I ( 11)
9 (100) 9 (100)
1 i 14 3 ( 43) I ( 14) 2 ( 29) 6 ( 86) 7 (100)
4 ( 44)
3 ( 71)
7( 78) 2 ( 22) 9 (100)
4 ( 57)
7 (100)
’~‘UIII~XTS in pwtwtht~sesindicate prccmtagt~.
C’HoI.o(;Y ! SEPTEhfBEH 19% i \‘oI,U\lt:
XII. \L’\iBIR
3
all parameters.
In those instancrs in nontailored rrinrplantation uxs pcrfmmd. reslilts m.(‘rc much less enco~iraging.
which the
<:ommeIlt
(:hildren \vith prune hell!. mc~gaureters should lx, waluated aggressively to dc~ter-mint, the statlls of their upper urinary trac+s. Those can do \vell withwith lthss sr\*ere involvement In those childrtxn whose out s~lrgicxl treatment. massi\.e upper tract dilatation was combined \vith progressive azotemia and/or im~ontrolletl infwtion. nonintuhatecl proximal di\x~rsion \vas pt~rformetl, hecause these ureters are markedl\~ and lvxaust~ the diredundant and tortuous,
\,prsion is required for long periods of tinlta. At the time of reconstruction, the ureterowoc!3tostomy should be augmented 11,. a tailoring procedurc~. histologic
It has lxw~
shown
that the degree
of
ant1 electron microscopic distiirlmKw of intrinsic uretrral structure art’ prtlseiit to ii grater extent in the more distal segmtwt of the prune bell>~ ureter. I2 The degree of urt+erectasis is also nlort’ se\wc in the distal urt~ttml segment. It ix thus apparent from both 21 clinical and a pathologic point of view that rc~cwnstru~tion of thy prunc~ belly megaureter must in~ludc c,xcision of the e?t~c:ss distal lm+eral lt>ngth mcl tailoring of‘ rweteral caliber at the tinrtl of wimI~]~l~~tatiol~. hlthough there are still ocusiolrs dtmairtl tcmporx~. uht.11 azottxinia and infection tlit> prmirnal portioii of the, prliiic’ tlivc,rsion, l,(lll!. lirc*tcAr is fiinc+ionull~~ and liistologicdl~~
IN
PRUNE BELLY SYNDROME
RON_4LD ?rlLARTIN JOHN
r\l. Il.
R4BINOM’ITZ BARKIN,
31. D.
F. SCHILLIN(;ER.
ROBERT
D. JEFFS.
31. I>. l1.D.
From the Division of Urology, The Hospital Sick Children. Toronto, Ontario. Canada
Considerable controwrsy exists regarding the rnanagen~t~nt of children with prune belly s!w drome. This entity, also called triad synclrome, is a cwngenital disorder almost universall) ~‘onfin(d to males and consists of deficienq~ of the abdolninal musculature, ureteral dilatation, and hilatcral cryptorchism. l-5 The literature is replettl with pleas for a nonoperative approach,’ as well as temporar) diwrsion in the form of ncphrostomy,’ cutaneous ureterostomy,x and v~sicostoin>~,” as well as permanent urinar), diversion and primar), reconstruction. ” Like so mail\. other entities, prune hell>, syndromt rrprestwts a spectrum of- a disease ranging from \rtar!’ nlild urinar!. tract involvement requiring no surgery to those with sevt:r-r wilw-y tract involwmc~nt. ” It is this latter group which is thtb suljec-t of this report.
1 PRUNE
BELLY
MEGAURETERS
f01
From 1965 through ISA, 25 ~nale\ with prune belly s!xdrome \\-ere cared fbr at The Hospital for Sick Children, Toronto. Fifteen of these children with massiveI> dilated upper tracts required surgical intell’eirtion lwcause of uncontrolled infection or progrt’ssi\.e loss of rcwal function. There were 27 Inassi\rtbly tliluted ureters in these 15 ho)x; 17 ‘,vc’I’c’associated \\ith reflux and 10 were not. Surgical treutment \vas initiated between tht, ages of one day and five years. Trn of these 15 hoyr s \vtArtJ treattd during the first month of life a11(1 6 during tht> first wcatlk of life. Snrgicd inter \.c,lrtion ~vas necessitatt~d 13,. uncont rollt~d iilfec*tioll in 7 (47 pt‘r cent) and/or progwssivc~ azotc~lnia iii 13 (93 per cent!.
1
Diversion
Reconstructed
Died
Tailo.red
Non-tailored
Reimplant
Reimplant
h!/.S rlf7)1)1(,
fc.illl
j”‘““”
hllr,
.S!/ll-
such a degree that nontailored ureteral reimplantation was performed, and the loop ureterostomies Lvere subsequently closed. Only 1 Case showed marked improvement; 2 continued to deteriorate, 1 with reflux. In 3 instances, reimplantation \vas supplemented b,; ureteral and all were successfd when uriuaq tailoring, tract continuity was restored. Five boys with 8 massively dilated ureters and severely dysplastic kidneys died of progressive azotemia in the first few weeks of life, despite adequate diversiou and control of infection. These children were found to have aLltops!.-prov~~l renal cl>ylasia. In 3 iustauces in \vhich contralateral function was satisfactory and reconstructable, prima) nel>hro~lreterectolrl~ for nonfunction and renal dysplasia was carried out. Six ureters Lvere managed by primary reimplantation ad tailoring without preliminary ureterostomy (Fig. 3). In one, this procedure was preceded by upper tract tailoring and nephrostomy. The results in this group were uniformly markedly successful. Follow-up has included physical examination, urinalysis and culture, intravenous urograin, voiding cystourethrogram, and renal function studies. These children have been followed for from one and one-half to ten years postreconstruction with an average length of pyelographic follow-up of four and one half years. As can be seen from Table I, wheu ureteral tailoring was combined with reilnplantation, whether as a primary procedure or as part of the reconstruction from loop cutaneous ureterostom); diversion, there was uniform success by
Treatment
and
TABLE
Results
The surgical management of these 27 pruue belly megaureters is summarized iu Figure 1. Neither cwtaneous vesicostmn~ nor teniporq uephrostom~ was carrid out in this group of patients. Ureteral dilatation mid tortuosity in thtw cliiltlrcw are so extensive that ~~~li~u diversion is required, it must he maintaiid for a long time. Under these circumstances, proximal urinar!. diversion is preferalde to distal diversion and nonintul~ated diversion is preferable to nephrostmn!. Loop cutaneous ureterostoni~ \vas performed in 18 instances. Despite the abdominal IIIUSC'Ulature deficiency these children tolerate loop cutaneous ureterostomy ~vell ant1 Lvithout troublesome prolapse (Fig. 2). In ‘7 cases ureteral caliber appeared to have improved to
‘3:31
Chnical
~lrinciry truct rc’c~ollstnrction in prune lady qnclrom
I.
Data
Reconstruction Intravenous pyelogram hlarked improvement Improved Stable Deteriorated No reflux Infection free Antibacterial prophylaxis Renal function Improved Stable Good result
Tailored ReimplauP
Nontailored Reimplant*
9
7
8 ( 89) I ( 11)
9 (100) 9 (100)
1 i 14 3 ( 43) I ( 14) 2 ( 29) 6 ( 86) 7 (100)
4 ( 44)
3 ( 71)
7( 78) 2 ( 22) 9 (100)
4 ( 57)
7 (100)
’~‘UIII~XTS in pwtwtht~sesindicate prccmtagt~.
C’HoI.o(;Y ! SEPTEhfBEH 19% i \‘oI,U\lt:
XII. \L’\iBIR
3
all parameters.
In those instancrs in nontailored rrinrplantation uxs pcrfmmd. reslilts m.(‘rc much less enco~iraging.
which the
<:ommeIlt
(:hildren \vith prune hell!. mc~gaureters should lx, waluated aggressively to dc~ter-mint, the statlls of their upper urinary trac+s. Those can do \vell withwith lthss sr\*ere involvement In those childrtxn whose out s~lrgicxl treatment. massi\.e upper tract dilatation was combined \vith progressive azotemia and/or im~ontrolletl infwtion. nonintuhatecl proximal di\x~rsion \vas pt~rformetl, hecause these ureters are markedl\~ and lvxaust~ the diredundant and tortuous,
\,prsion is required for long periods of tinlta. At the time of reconstruction, the ureterowoc!3tostomy should be augmented 11,. a tailoring procedurc~. histologic
It has lxw~
shown
that the degree
of
ant1 electron microscopic distiirlmKw of intrinsic uretrral structure art’ prtlseiit to ii grater extent in the more distal segmtwt of the prune bell>~ ureter. I2 The degree of urt+erectasis is also nlort’ se\wc in the distal urt~ttml segment. It ix thus apparent from both 21 clinical and a pathologic point of view that rc~cwnstru~tion of thy prunc~ belly megaureter must in~ludc c,xcision of the e?t~c:ss distal lm+eral lt>ngth mcl tailoring of‘ rweteral caliber at the tinrtl of wimI~]~l~~tatiol~. hlthough there are still ocusiolrs dtmairtl tcmporx~. uht.11 azottxinia and infection tlit> prmirnal portioii of the, prliiic’ tlivc,rsion, l,(lll!. lirc*tcAr is fiinc+ionull~~ and liistologicdl~~