Survey 67

Urinary Diversion in Children* H.], Pompino Chirurgische und L:rologische Ableilung der DRK-KinderkJinik Siegen (Leller. :'rof. Dr. H.-J. Pompino)

Resume

A.t the present time. urinary di\"er ion in children remains an last resort type of treatment that must be carried out in selected cases when there is no alternatiw available. A.11 forms of urinary di"ersion ha"e the common goal of gaining contral of a life-threatening situation; all, howe,'er, are fraught \I'ith serious disad'-antages for the affpr! ~'J children, The ne\l'er forms (undi'-ersion, pouch. ileocystopJasty, augmentation) undoubtedly impra"e the patienl's quality of life, but the risks of long-term metabolie and renal complications and malignant degeneration persisl.

.-\ ce jour, la di'-ersion des urine demeure un dernier recours dans certaine situations OU il n'y a plus d'autre alternati\"e. Toutes les deri,'ations des urines ont en commun l'a"antage de permettre le contr6le de situations "itales mais. en contre-partie presentent de nombreux desa"antages pour I'enfanl. Les nou\"elles techniques (dederi"ation, poche, iIeocystoplastie, agrandissement) incontestablement ameJiorentla qualM de "ie des patienls. .\\ais le risque a long terme de complications renales ou metaboliques et de degenerescence persiste,

Harzmann, who for years has concerned hirnself \lith urinal'}" di'-ersion and, in particular, \I-ith malignant change, has posed two difficult question : 1. ls urinary diversion using bo\\'el segmenls defensibJe for benign disorders, e, g., in children? 2, Which follow-up examinations should be performed after urinary di"ersion using bowel segmenls, and at what time intervals?

Harzmann qui s'est interesse pendant plusieurs annee aux deri,'ations urinaires et en palticulier au risque de degenerescence pose deux questions difficile : 1. La deri\"ation des urines utilisant un segment digestif se defend-elle pour les situations benignes, en particulier chez l'enfant? 2. Quels sontles examens post-operatoires qui doi\"ent elre utilises et a"ec quelle frequence apres diversion des urines utilisant un segment digestif?

Our goal should be to keep the nurnber of urinary diversions as sma]] as possible by means of appropriate lrealment, prophylaxis, and in particular the means and type of prirnary reconslructive therapy. Children have a Iifetime ahead of lhem, and the complications of urinary diversion increase decade by decade. This fact has been demonslrated in children who have undergone ureterosigmoidostomy or iIeaI conduil. Graunds for lhe assumption that fewer serious long-term complications are to be expected after colon conduit, pouch, neobladder, or augmentation procedures have not been established.

otre but doit eLre de limiter le nombre de derivations urinaires. Les enfanls ont un long devenir devant eux etles complications de la derivation des urines augmente decade apres decade. Ceci a ete bien demonlre chez les enfanls qui ont eulement une ureterosigmoi'doscopie ou une derivation iIeale. Les argumenls permettant de supposer que les complications serieuses a long terme seraient moins importantes apres derivation colique, poche, neovessie ou agrandissement n'ont pas encore ete demonlres.

Mols-eies Key words Crinary diversion - Cndi\"ersion - Pouch -

Diversion de urines - Dederivation - Poche - I1eocystoplastie - Agrandissement

Augmentation

Received \1arch 2-1. 1U9l Eur J Pedialr Surg ? (1 Oll?) 6- _-') VV_ "Ippokrales \'erlag Sluttgali \Iasson Editcur Paris

I!:)H'

* Prpscnted in part at the "Obermcderma,T \lemorial Lecture". Annual .\Ieeling of lhe IJcrman Socicty of Pedialric Surgeons, Seplember, 19UO.

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Summary

EurJ Pediatr Surg 2 (1992)

Zusammenfassung Die künstliche Harnableitung im Kindesalter ist derzeit noch eine operative Behandlungsmethode mit Ultima-ratio-Charakter, die in ausgesuchten Fällen durchgeführt werden muß, wenn keine Alternative zu ihr besteht. Wir verfolgen mit allen Formen der künstlichen Harnableitung das Ziel, eine lebensbedrohende Situation zu beherrschen. Alle Harnableitungen haben jedoch schwerwiegende Nachteile für die betroffenen Kinder. Die neuen Formen der Harnableitung (Undenision, Pouch, Ileozystoplastik, Augmentation) verbessern zweifellos die Lebensqualität, aber die Gefahren für metabolische und renale Langzeitkomplikationen sowie für die karzinomatöse Entartung bestehen um-erändert fort. Harzmann, der sich seit Jahren mit der künstlichen Harnableitung und vor allem mit der malignen Entartung beschäftigt hat, stellt zwei schwer zu beantwOltende Fragen: 1. Ist die künstliche Harnableitung über Darmsegmente bei benigner Grunderkrankung - z. B. bei Kindern - vertretbar?

Introduction The history of urinal'}' diversion is actually the history of reconstructive surgery of the upper and lower urinary tracts. Initial proposals date back to the previous century and were associated with names such as Syme, Trendelenburg, and Maydl, among others. The names Coffiy and Bricker are now synonymous with the extraordinary number of types of urinary diversion developed during the 1960s and 1970s (1, 6, 15). In the 1970s, Hendren achieved distinction in the field of urinary diversion by devising means of converting diversions back to undiversions. His preference for undiversion was due to the numerous disadvantages of artificial diversion, although the use of bowel segments continues to be employed in many types of undiversion even today (7, 8). Urinary diversions are being performed less often in children at the present time due to the POOl' long-term results, and consequently, fewer undiversions will be required in the future. According to Stephenson, primary reconstructive procedures have simultaneously been improving substantially, with the aim of reducing the need for diversions and undiversions (14, 19). While the indication for urinary diversion in adults is based mainly on age-related functional disorders and deficits or severe tuberculous diseases, in children it is employed most often for congenital malformations of the urinary tract, ,vith the goal of rehabilitation. The stormy course of urinary di"ersion in adult urology, with the multitude of new methods being proposed, is due to the general increase in life expectancy and the consequent need for more urologic-oncologic therapeutic concepts. The spherical low-pressure resen'oirs are created from ileum and colon with the chief aim being to achieve a dry stoma 01' "voluntary control" rather than to reduce the long-term meta-

H.j. Pompi/lO

2. Welche Kontrollen sollen nach Harnableitung über Darmsegmente und in welchen Intervallen vorgenommen werden? Unser Ziel sollte es sein, die Zahl künstlicher Harnableitungen durch sachgerechte Betreuung, Prophylaxe und vor allem die Art und Durchführung der primär rekonstruktiven Therapie so klein wie nur möglich zu halten. Kinder haben ihr Leben vor sich. Die Komplikationen bei künstlichen Harnableitungen nehmen von Jahrzehnt zu Jahrzehnt zu. Für Kinder, die mit Ureterosigmoideostomie oder Ileumkonduit versorgt worden sind, gilt diese Tatsache als bewiesen. Argumente für die Annahme, daß die Langzeitkomplikationen nach Kolonkonduit, Pouch, Neoblase oder Augmentation prinzipiell günstiger zu erwarten seien, sind kaum zu begründen.

Schlüsselwörter Künstliche Harnableitung duit - Ileumblase - Blasenvergrößerung

Kolonkon-

bolic, renal, and carcinogenic complications. Il is as yet uncer· tain whether a greater life expectancy can be attained by this means, particularly in comparison with the conventional forms of urinary diversion (1, 4, 5, 6, 11, 12).

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68

This constellation, and the difficult question of alternatives, can make the decision as to the procedure of choice extremely difficult in urgent cases (Table 1).

Indications for urinary diversion

The diagnoses most often involved in discussions on indications for urinary diversion are shown in Table 1 (3,9, 10, 14, 19). The same diagnoses reappear when the late results in children who have developed renal failure are observed: Table 2 shows the diagnoses from 2,372 such children. Table 1

Indications for possible urinary diversion.

1. Severe obstructive or refluxive uropathies 2. Uropathies subsequent to complicated or unsuccessful surgical proce' dures 3. Bladder exstrophy 4. Neurogenlc disorders of bladder emptYlng 5. Mallgnant tumors

Table 2 Dlagnoses In children < 15 years of age wlth terminal renal failure 1981-1985, RlzzonJ et al (1989) DIagnosIs (n

=

2,372 chlldren)

Glomerulonephntls Pyelonephntls Congenital hypo- and dysplasla Heredltary nephropathles Other etlologles, Inel. unknown

% 25.8 24.2

13.5 15.6 20.8

Urillary Diversion in Chi/dren

Eur J Pediatr Surg 2 (1992)

Etiology

% of 574 cases

Congemtal obstructlve uropathles WlthjWlthout reflux

41.3

Congenltal refluxlve uropathles wlthout obstructlon

32.1

Uropathies wlthout obstructlon or reflux, mcl. unknown

233

AcqUired obstructlve uropathles

33

Table 4

Prophylaxis of urlnary diversion.

Conservatlve 1 Medical therapy of reflux 2 Restrlctlve surglcal therapy of reflux 3 Bladder preservatlon In NBE (intermlttent catheterizatlon) 11. Timing and cholce of prlmary surglcal correctlon 1. Early plastlc reconstructlon of bladder exstrophy 2. Delayed prlmary operation of severe uropathles 3 Crltlcal consideration of what will be necessary temporarlly, e. g., nephrostomy, ureterostomy, veslcostomy, transurethral drainage, or combmatlons of these 4 Bladder preservatlon whenever posslble

The diagnoses of most concern to us are the second and third in Table 2. The causes of pyelonephritis are depicted in Table 3, which shows that congenital, obstructiye, and refluxi"e uropathies comprise 73.4 % of this grou p of diagnoses ( 18).

(.'lIeD) from a now 15-year-old boy after simple '-alve resection; Figure 2 shows his I\'P at the age of 12 years, .\lCUs ha,'e shown no further reflux for years, Follow-up studies had shown regression of reflux on both sides up to the age of -1 years.

What can be done to postpone the de,'elopment of definitiw renal failure as long as possible, and what role \\'ill be played by urinal}' di"ersion?

Although urinary diversions were performed in many children with neurogenie bladders into the 1970s, the predominant therapy at present is eonseryati"e, with intermittent eatheterizations and medications.

Prophylaxis of urinary diversion The prophylaxis of urinary di"ersion is of particular significance, Table..], shows the possible strategies for prophylactic and conservati"e treatment, which aim to decrease the need for urinary diversion. Children with urethral valves, for example, do not require neo implantation after valye resection if renal function improyes, Figure 1 shows the first intra"enous pyelogram (I\'P) and micturating cystourethrogram

Table -1 shows the timing and types of primary correetive proeedures that ean be useful in deereasing the number of urinary di"ersions required, The indieation for surgery in se,'ere obstrueti"e and refluxiye uropathies is no Ionger dependent on the radiologie findings alone, but also on the results of tests of funetion and isotope clearance on each side, Very early functionaJ reeonstruetion of bladder exstrophy by means of pelvie osteotomy and urethral augmentation can ob,-iate the need for primal}' urinal}" diversion (9, 19).

Fig, 1 Right First IVP In neonatal perlod, left first MCU Bilateral grade IV reflux, clearly vIsible urethral valves first year of Itfe

0 elevation of serum creatlnlne dUring

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Table 3 Causes of pyelonephrltls leadlng to termmal renal fallure in 574 children< 15 yearsof age (24.5 % of2,372 chlldren, Rizzonietal, 1989).

69

EurJ Pediatr Surg 2 (1992)

H. J. Pompino

The di cus ion of these various methods of diversion for children can be conden ed into three questions: (1) whether or not the bladder can be preserved; (2) whether cutaneou ureterostomy may regain more significance in selected cases as a means of gaining viable time; and (3) whether the use of detubulated segments of mall and large intestine can provide better long-term results, [t has been found that using tubulated bowel segments leads to excessively high press ures in the reservoir. Detubulated egments have been used Lo form spherical low-pressure reservoirs, wiLh substantially lower pressures, It may be expecled thatthis will also reduce the retrograde effects on the u pper urinary tract H, 5, 6, 9, 10, 11, 19),

Results The distribution of cases of se\'ere ob tructive and refluxi\'e uropathies in our own patient population is shown in Table 5. The incielence of second anel Lhirel operations and dh'er ion anel uneli\'ersions in infants anel chileIren \\'ith obstructi\'e and refluxi\'e uropathies is seen in Table 6. L:rinary di\'ersion was necessary in 9 % of Lhe 167 operateel ureterorenaJ units elue to unsalisfactory results from pre\'ious proceelures, Table '7 sho\\' lhe \'ariou operali\'e melhods and lale results in Fig, 2 IVP at 12 years 01 age No vIsible rellux on MCU slnce age 5 years No secondary operation or neolmplanta Ion performed

Table 5

Severe obstruc Ive and refluxlve uropatries. n (unlt)

DIagnosIs

Surgical options for urinary diversion Many of the forms of urinary diversion that have been propo ed since the turn of the century are no longer of importance today. The pouch, neobladder, and enterocystoplasty using stomach or small or large intestine have been developed, as weil as bladder augmentation and the MitrofanoffmeLhod, There is as yet no technique of choice for urinary diversion in chiJdren, a no single form of diversion can clearly be recommended (3, 5, 6, 19). The extensive long-term results of ileal conduits and ureterosigmoidostomies are so discouraging that boLh should better be avoided (1,4,6, 16,20), and the colon bladder and cutaneous ureterostomy cannot be recommended without reservations, Caution should al 0 be advised in opting for a pouch, neobladder, or enterocystoplasty in a chiJd, due to the lack of long-term results (10, 11, 14, 19,20), The Mitrofanoffprocedure has Lhe advantage that Lhe bladder is preserved but the disadvantage of numerous early complications, and Lhe long-term results of Lhis method are also unknown (13).

Op. method

Plastlc reconstructlon Plastlc reconstruc Ion Ureterostomy lIeal bladder Maydl Ureteroslgmoldostomy Ureteroslgmoldostomy Cystorectostomy ----

Time pertod

n=

1925-1940 1970- 984 1950-1970 1960-1984 1925-1935 1935-1955 1970-1984 1955-1967 - - --

7 7 7 4 2 20 6 34

87

Prlmary and secondary megaureter wlth rellux Prtmary and secondary megaureter wlthout rellux Ure eral outle stenosIs Double kldney Total

26

n (chlldren)

59

31

10

41

51 26 31

20 11 15

16 9 7

36 20 22

167

77

42

119

In

1:.

9 8.3 10.7

Planned second operations Unpla nned second operations Second and multiple procedures

35 14 18

Total

67

400

Diversion and undiversion

15

90

15/67

2

7 4 14

1975-1988



Table 6 Second and multiple procedures including diverSion and undiver' slon In Inlants wlth severe uropathies (119 patlents, 167 unlts).

Postoperative survlval 10 years 30 years

3

ci

5

t 5 0 0 0 2 15 0 2

22.4

Table 7 Long,term resul s In chil, dren W' h bladder exstrophy after vartous surglcal procedures 19251984 (Dr. v Haunersches Chlldren's Hospital, Unlversl y 01 Munlch, Dep!. 01 Pedla rlc Surgery, MunchenSchwablng City Hospitals, and Depts. 01 Surgery and Urology, German Red Cross Chlldren's Hospital Siegen), From Pompmo, "Ureter,Darmana' stomose nach Coffey bel Kindern, Langzeitergebnisse" Verhandlungs, bertch D Ges Urologie 35 (1984)

392.

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70

Eur J Pediatr Surg 2 (1992)

Urinary Diversion in Chi/dren

_---------I-A-ft-e-r_20....;.,ye_a_r_s_ _-+I_A_ft_er_3_0_y_e_a_rs_ Alive Kidneys normal (n Other diversion Rectal carclnoma Continent

=

19)

10 5 1 0 9

1

5 1/8 1 1 2

children with bladder exstrophy from three pediatric surgical departments*; 73 of these 87 infants and children required urinary di\'ersions. The majority of the children in this group underwent either cystorectostomy or ureterosigmoidostomy. Table 8 shows the long-term results over aperiod of 30 years in the children with ureterosigmostomies for bladder exstrophy: only 5 of these 20 patients are still alive. We observed one case of adenocarcinoma in this patient group (16).

Discussion In comparison to the risks of chronic pyelonephritis on renal function, factors 2-5 in Table 9 play subordinate roles that should nevertheless be taken seriously where ehildren are involved. The more urinary diversions and bladder augmentations that are performed, the more operations will be observed. The numbers of ease reports in the literature are inereasing, although the majority eoneern eases in adults. Thus far, the reports on augmented bladders and neobladders exeeed those on tubulated and detubulated reservoirs, amounting to about 3 % (I, 2, 3, 5, 19).

Table 9

Prognostic risks in children with urinary diversion.

1. Chronic pyelonephritis, scarring, hypertension, renal failure 2. 3. 4. 5.

Electrolyte imbalance Stomal complications Perforation Malignancy

How great is the eurrent risk of malignant degeneration for affected ehildren? Nitrosamine synthesis is eonsidered to be responsible for tumor induetion when feees and urine are diverted together. Chronie irritation of lhe bowel mueosa and urinary stasis in the reservoir are regarded, along wilh duration of time, as the eauses of tumor induetion. The magnitude of the ineidenee of eareinoma induction has to date been estimated only for ureterosigmoidostomies, at 25 %, but this phenomenon is eonsidered feasible in other forms of diversion as weil. In 1986, Stewart reported an ineidenee of 5 % po]yps and 5 % eareinomas among 117 ureter-to-intestine anastomoses (Table 10) from the extensiw patient population

Table 10 1986)

partment of Pediatric Surgery, .\\ünchen-Sch\\'abing City Hospitals; and the Departments of Surgery and L'rology, German Hed Cross Children's Hospital, Siegen.

Stewart,

Tumor histology I Adenocarclnoma

Operation

I Polyp

I

Ureterosigmoidostomy Veslcorectostomy Maydl procedure

117

7

7 =

Table 11 1986)

10%

Tumor hlstology after ureteroslgmoldostomy (after

Histology

n

Polyp AdenocarclOoma Other

48

=

81

23 10

Stewart,

% 28.4 592 12.4

atthe Great Ormond Street Hospital in London. He estimated the risk of eareinoma in ehildren \\'ith these anastomoses at80100 times that of the normal population (I, 2, 3, 5, 12, 20). Tumor development has only rarely been observed during the first 10 postoperati\'e years; itthen inereases deeade by deeade. In 81 of 94 eases of tumor subsequent to ureterointestinal anastomoses, the urinary diversion was performed for a benign disorder. Table 11 shows the histologie distribution of these 81 tumors (20). Urologists reeommend regular endoseopie follow-up examinations in adults 5 years after urinary di\'ersion utilizing bowel segments for the early diagnosis of tumors (4, 5). II is obvious thatthe risk of tumor induetion in ehildren, who have a life expeetaney of 60-80 years ahead of them, presents a different set of problems than that in urologie oneology patients in their 6th to 8th decades. All in all, the long-term prognosis after urinary diversion in ehildren must be regarded as unfavorable. There is generally a severe primary disorder of funetion assoeiated with a eongenital malformation. Urinary diversion is not a prophylaetie measure. Serious long-term eomplications sueh as ehronie pyelonephritis with subsequent renal faiIure or malignant degeneration must be expeeted. Every pediatrie surgeon who performs urologie proeedures should strive to drastiealIy reduee the number of urinary diversions utilizing small and Iarge intestine in ehildren, to undivert lhem when possible, and to investigate whether these diversions ean be replaeed by superior methods. Robert MitchelI, in Indianapolis, has aplly stated that it is to be hoped thatthe eurrently standard teehniques of urinary diversion \\'ill be of merely historieal interest in the future (4, 5, 6, 16, 19).

References Egglzart )-. Bacher R. l1allbllLlll1l RE' Die Ileum '\;eoblase. !-:Iinikarzt 11 (1988) 2 FilII/er RB, SpeIlcer J R . .\\alignancies in bladder augmentation, and intestinal conduits. J l'rology 1 1:1 (1990) 61 I :l Clee,oll JIJ, Griffith DP.· L'rinar.\ dil'Crsion. Erit J L'ml ßö (I D90) 113 I llarzmanll R: Harnableitungskarzinom - Fiktion oder !{ealitii(? ,\ktuelle L' rologie 20 (1989) 119 I

* The Dr. \'. Haunersches Children's Hospital, l'ni\'Cf'sity of .\\unich; the De

Tumor risk and ureterolntestlnal anastomoses (after

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Table 8 Late results in 20 children after Goffey procedure for bladder exstrophy 1935-1955. From: Pompino, .Ureter-Darmanastomose nach Coffey bei Kindern, Langzeitergebnisse. " VerhandlungsberIcht Dt. Ges. Urologie 35 (1983) 392.

71

Eur J Pediatr Surg 2 (1992)

H. j. Pompino

5 Harzmann R: Komplikationen und Spätfolgen nach urologischen Eingriffen am Darm. \'erhandlungsbericht der Deutschen Gesellschaft für Urologie, 40. Tagung (1989) 139 6 Hautmann R: Harnableitung 1989. Crologe 28 (1989) 177 7 Hendren WH: Techniques for urinary undiversion. In: Bladder Reconstruction and Continent Urinary Diversion. LR King, AR Stone and GD Webster, Chicago: Year Book .\Iedical Publishers (1987). chapl. 9, pp 101-126 8 Hendren WH: Bladder augmentation: Experience \\'ith 129 children and young adults. J Urol 14-1 (1990) -115 9 jejJs RD, GerhartjP. Augmentation cystoplasty in the fai1ed exstroph~ reconstruction. J Urol 139 (1988) 790 10 Koek SG: Continent ileo tom\". J l'rol 128 (1982) -169 11 Koek SG: l'rinary di\'ersion \:ia a continent ileal reser\Oir (Kock·Pouch). J lJrol part 2 (198-1) 131 12 Leadbetter G 11', Ziekenllalln P, Pieree E l'reterosigmoidostom~ and car· cinoma of the colon. J erol 121 (1979) ,32-,35 13 .1/itrofanoffP Kontinente Cystostomie. Chir Pediatr 21 (1980) 29, 11 .1/lIlldy AR Augmentation and Substitution Cystoplasty. In: Frank jD and johns/on jH Operati\'e Pediatric l'rology. Lendon. Churchill Li\'ing~tone 1990 15 Pa/liek C, Cartwright, ßren/ 11', Snow' Partial detrusorectomy: .\ug menting the pediatric bladder without 00\\ el. J l'rol 139 (I \)38) 231 16 Pompmo Ilj l'reter·Darmanastomose nach Coffe~' bei Kindern, Langzeitergebnisse. \'erhandlungsbericht Dt. Ges. l'rologie :15 (I \)8~) 392 r; Pompillo Hj Komplikationen in der ,\Iegaureterchirurgie. In: Komplikationen in der Kmderchirurgie. Stuttgart Thieme 1l!\1I 18 Ri::olli G, Dello S/rologo L: Ätiologie terminaler :--Jiereninsuffizienz bei Kindern: ein epidemiologischer Cberblick. Annales :'\estle l'i (1989) 139 19 ::'/ephensoll TP l'ndi\'ersion - Indications and patient assessment. In: Frank jD and johnstoll jJl: Operative Pediatric l'rology. London. Churchill Li\'ingstone 1990 20 S/eu.'al1 .\l: l'rinary di\"ersion and OOwel cancer. ,\nnals of the Hoyal Col· lege of Surgeons of England 68 (19 6) 98

Prof Dr. H.]. Pompino Chirurgische und Urologische Abteilung der DRK-Kinderklinik Siegen Wellersbergstr. 60 D·5900 Siegen

Chirurgische Anatomie der Hand

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Von

H,- \1, SCH\1IDT. ßonn

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19

Urinary diversion in children.

At the present time, urinary diversion in children remains an last resort type of treatment that must be carried out in selected cases when there is n...
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