British JournalofUrology (1992), 70, 113-120
01992 British Journal of Urology
REVIEW
Lower Urinary Tract Reconstruction in Young Patients C.R . J. WOODHOUSE Institute of Urology; Departments of Urology, St George‘s Hospital and Royal Marsden Hospital, London
There are now very few indications for a permanent cutaneous urinary diversion in a child. Many congenital anomalies of the lower urinary tract can be completely reconstructed. The neuropathic bladder can be made continent and “safe” for the kidneys. For some patients continent diversion avoids the need for a cutaneous appliance. In the past, however, diversion was sometimes a life-saving procedure and in other patients the only practical method of managing incontinence or recurrent urinary infection. In young adults, the reconstructive techniques that are used now in children can be applied to get rid of the bag. Almost anyone can have a reconstruction, but as the original surgery may have included resection of some of the urinary tract, more extensive and imaginative techniques may be needed. Before undertaking such surgery it is important to consider the possible long-term benefits. The only certain improvement is cosmetic : the patient will have no external appliance. If the bladder is neurologically normal the long-term results of undiversion should be good. Some patients will be able to void normally but others may need to empty the bladder by intermittent clean self-catheterisation (ICSC). The long-term results of cutaneous diversion are poor, whilst the late results of complex reconstructions, especially those using bowel, are yet unknown. Some infective and metabolic complications are becoming apparent. It is salutary to remember that ureterosigmoidostomy had been used for nearly 100 years before the risk of neoplasia was really appreciated. Those who undertake such surgery must be sure that the parents and patients know of the uncertain future and the need for careful follow-up.
Older patients should not be deprived of such surgery just on grounds of age. However, care is needed to select patients who are both physically and emotionally fit enough.
Principles of Lower Urinary Tract Reconstruction When one or more components of the lower urinary tract are unusable, imagination is needed to devise a system that avoids an external bag. Reconstruction should be tailored for each patient. There are 3 components to be considered and they are largely independent of each other: a reservoir for the urine, an outlet to the surface and a continence mechanism. The anatomical structures that have been used for each of these are shown in the Table. It can be seen that the reservoir may be made from one or more of the structures in the first column and the outlet from one in the second column (lengthened by a skin tube if necessary); the continence mechanism is indicated from column 3. The reconstructed system may be emptied by ICSC or by spontaneous voiding. Simple reconstruction or undiversion has now become routine and will not be considered further. Even for complex reconstructions, however, the reservoir should include as much normal bladder as Table Tissues Used in Reconstruction of the Lower Urinary Tract Reservoir
Conduit
Control system
Bladder Stomach Ileum Caecum Colon
Urethra Appendix Fallopian tube Ureter Skin tube Ileum Umbilical vein
Urethral sphincters Mitrofanoff Kock Ileocaecal valve Benchekroun Artificial sphincter
Accepted for publication 4 February 1992
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114 possible. The urethra remains the best outlet. The natural sphincters, if they can retain urine, are the best control mechanism. Otherwise the choice of reconstruction depends on the material available, the needs of the patient, the possibilities for follow-up and the skill of the surgeon. It is a mistake to confine oneself to a single systeia, such as a particular type of pouch, because none is suitable for all patients. Furthermore, there are many occasions when a planned reconstruction must be changed mid-operation if a component is found to be unusable.
Choice of Reservoir Bladder is the natural reservoir for urine and should only be abandoned if irreversibly diseased, a rare circuinstance in children and adolescents. The bladder may be removed for cancer or when damaged by radiotherapy or cyclophosphamide. In endemic areas, bilharzia or tuberculosis can destroy the bladder. Chronic interstitial cystitis is occasionally seen in adolescents. In exstrophy and its variants the bladder may be too small (particularly after a failed reconstruction) to be worth saving. Adolescent females with a congenital neuropathic bladder are an exceptional group in whom it is not wise to retain a large bladder remnant. Five of 16 in my own series had problems from the residual native bladder. In one there was persistent hyper-reflexia. In 4 patients hyper-reflexia was unmasked by sexual activity: orgasm in 3 and sexual arousal in 1. Therefore, if bladder emptying is likely to be by ICSC, a good case can be made for using a substitution cystoplasty (Woodhouse, 1992). If the bladder is small, poorly compliant or at high pressure, some bowel may be added (augmentation cystoplasty) or may completely replace the bladder (substitution cystoplasty). A sinall bladder must be considered in relation to the ureteric and urethral junctions. If there is no ureteric reflux or obstruction and the outlet is continent, the small bladder may be an ideal base for reconstruction. It is usually difficult to reimplant the ureters into such a bladder, though it may be possible to reconstruct the outlet for incontinence. If there is no bladder (or if it is to be abandoned), a new reservoir must be constructed from intestine. If there is an existing conduit, it should be incorporated. There is no consensus on the choice of bowel for a urinary reservoir, except that jejunum is too metabolically active for general use; 40 cm of small intestine, 20 cm of large intestine or a
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combination can make a low pressure substitute of adequate volume. The stomach may be useful in children with renal failure because of the acid that is secreted (Adam et al., 1988). However, if the child then becomes anuric, the continued secretion of acid into an otherwise “dry” bladder may cause irreversible damage. The intrinsic peristaltic contractions must be abolished. I believe that this is best achieved by longitudinal incision through the full thickness of the bowel wall. The opened-out bowel is then reconfigured to form a reservoir which is as near spherical as possible (Hinman, 1988). Gonzales et al. (1986) used bowel without transection of the muscle and gave anticholinergics to suppress peristalsis in the 50% of patients who had unacceptably high pressures. Alternatively, a very long segment of bowel may be used, so that the volume of urine stored is never large enough to stimulate high pressure contractions (Mundy, 1988a). Urodynamic studies have shown that the lowest pressures are achieved with an ileal patch augmentation of a colonic bladder (Lytton and Green, 1989). It is not always possible to abolish peristalsis even by complete transection of the circular muscle. I have also encountered 2 patients who re-established high pressure peristalsis after a period of bowel inertia. I have not found anticholinergic drugs to be effective. In follow-up, the ileum appears to have a greater metabolic activity but a lesser risk of spontaneous rupture than colon. However, the differences are insufficient to dictate a particular choice. As lower urinary tract reconstruction becomes more complex in its surgery, so the choice of technique becomes more difficult.
The Outlet The urethra is the natural outlet for urine and the only one that will allow spontaneous voiding. The urethra is more dependent on its continence mechanism than other possible outlets. Together, the urethra and its sphincters form the ideal system, but it can be difficult to predict whether they will be competent or usable after bladder reconstruction. Grossly abnormal bladders are often associated with other major abnormalities. Firstly, the substituted bladder cannot always be emptied to completion, so that ICSC may be
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necessary. Patients with a neuropathic bladder which is so poor that substitution is necessary are often severely affected with myelomeningocele. They may lack the manual dexterity to selfcatheterise. The problem is worse if they walk with 2 crutches or are wheel-chair bound. If the bladder is not neuropathic but has been damaged, for example, by cyclophosphamide or surgery, the urethra, especially in the male, may be too sensitive for ICSC. Secondly, the urethral sphincters may be incompetent, so that either an artificial sphincter (AUS) or a bladder neck reconstruction will be necessary. The feasibility and desirability of these procedures must be taken into account before deciding to anastomose the pouch to the urethra. Thirdly, if an adolescent boy has had a lifelong diversion, his penis will only ever have been a sexual organ. If, after undiversion, he needs to selfcatheterise, the philosophical question arises as to whether he should catheterise his sexual organ. It seems unlikely that there is a “right” answer to this question. The site of a catheterisable stoma should be chosen chiefly for the patient’s convenience : the urethra should only be used as the outlet if it is clearly superior to a suprapubic continent stoma. If there is no usable urethra the choice lies between constructing one or using a technique of continent diversion with a suprapubic catheterisable stoma. It is possible completely to reconstruct the urethra from labia, penile skin or pedicled ureter. The tube is then tunnelled into the reservoir to make a continent outflow. The bladder is usually emptied by ICSC but spontaneous voiding is sometimes (Mitchell et al., 1988; Mundy, 1988b). Such intricate reconstruction is only occasionally indicated. A new outlet can be constructed from almost any tube. If a wide bore tube (such as ileum) is used, its continence system must involve some kind of intussusception, unless it is tailored. Narrow tubes must be tunnelled into the reservoir to produce continence. Of the narrow tubes the appendix is the easiest to use and appears to be the most resilient. A slightly dilated ureter is also satisfactory, but a completely normal one may be difficult to dilate to a diameter suitable for catheterisation. The lateral two-thirds of the fallopian tube are also satisfactory but are seldom available. A tube can also be made from pedicled skin to use alone or with anastomosis. The ileum is the standard wide tube and is generally the easiest to catheterise.
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Continence Systems If the urethra is the ideal outlet, the urethral sphincters should be the best continence system. In diverted patients, they must be tested and preparation should include an examination under anaesthesia and insertion of 2 suprapubic catheters. Several days can then be spent filling the bladder with water and testing the sphincter function-socalled “bladder cycling”. Urodynamics are performed when a stable state is reached. Neurologically normal sphincters should allow complete emptying after enterocystoplasty. Even in patients with the neuropathic bladder of spina bifida, I have found that function was correctly predicted in 79% of patients by this simple investigation (Woodhouse, 1992). Using the more sophisticated techniques of fluoroscopy of the internal sphincter and electromyography of the external sphincter, correct function was predicted in 93%of patients (Gonzalez and Sidi, 1985). In assessing the bladder/sphincter function as a whole, especially in patients with neuropathies, Wang et al. (1988) emphasised the importance of the “leak point pressure”. That is the intravesical pressure at which leakage occurs through the urethral sphincters. If the leak point pressure is above 40 cm of water, upper tract deterioration is likely, probably because of progressive loss of bladder compliance. Conversely, as a bladder reconstructed by enterocystoplasty usually has a resting pressure below 30 cm of water, such powerful sphincters should maintain continence. In my experience, young adults with normal sphincters have been continent after bladder substitution. However, in older patients some authors have found day or night incontinence. The main cause has been persistence of mass peristalsis even in detubularised bowel. Most of the reported patients were elderly and all had had a cystectomy; both of these factors may have had an adverse effect on sphincter function (Lytton and Green, 1989). If the sphincters are continent but do not allow complete emptying, ICSC is generally the best management. There is sometimes pressure on the surgeon to arrange for “normal” voiding. To achieve this it would be necessary to destroy the natural sphincters and to implant an AUS. In most cases this seems unwise. Even with an AUS, complete voiding cannot be guaranteed after enterocystoplasty and ICSC may still be necessary. The long-term results of AUS are unknown. Should the AUS become infected and require removal, the
116 patient would be continually incontinent and worse off than he was originally: the options for further reconstruction would then be limited. Finally, in units performing large numbers of these operations, many of the patients come from remote parts of the world where “servicing” of a faulty AUS might be impossible. If the sphincters are incontinent, the options are to reconstruct the bladder outlet to create continence or to use an AUS. A good case can be made for either of these. However, units have tended to use either one or the other and so it is difficult to establish indications for each. For example, Gonzalez and Sidi (1985) reported the use of the YoungDees bladder neck reconstruction in 8 patients with incompetent sphincters, 7 of whom were continent on ICSC. The AUS had been used in other patients with success, but the need for “servicing” and doubts about the mechanism’s longevity favoured the Young-Dees. Mitchell and Piser (1987) reserve the AUS for patients who can demonstrate complete bladder emptying. Both Mundy et al. (1986) and Galloway et al. (1987) have reported good results with undiversion using an AUS (Brantley Scott 792 or 800). There were 36 patients in their 2 series. Virtually all were able to void to completion. Up to 7 were at least partly incontinent. There were 8 sphincter complications that required reoperation. At least in patients with neuropathic bladder, the trend has been away from sphincterotomy and the AUS in favour of ICSC (Parry et al., 1990). The long-term results of ICSC are good. When Diokno et al. (1983) reviewed patients after 10years’ follow-up, only 2 of 60 patients had given up catheterising because of a complication. Thirty others had been lost to follow-up, died or suffered a change in neurological status. However, the urethra can be damaged so badly that the technique has to be abandoned. The risk is greater if the urethra has no sensation. Development of a false passage is a particular problem and can be difficult to treat: 2 of 9 patients in one series had to have a continent diversion because of this complication (Koleilat et al., 1989). Attempts to make a continent bladder neck and urethra which also allow complete spontaneous voiding have met with mixed results. It is difficult to balance the outflow resistance against the intravesical pressure. Furthermore, there must be enough good bladder tissue to allow the reconstruction to be done (not a common circumstance in children presenting for reconstruction). In a 10- to 22-year follow-up of patients who had
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bladder neck reconstruction (but who had not been diverted), 19 of 27 children were completely dry and 1 had stress incontinence. One child emptied by ICSC, but the rest voided normally (Leadbetter, 1985). This is probably the best result and longest follow-up available for such reconstructions. Those who claim success for more complex methods must compare their results with it. Without the urethral sphincters, there are 2 basic principles that may be applied to create continence : either a flutter valve made by the intussusception of a wide bore tube (Kock, 1971) or a flap valve made by the tunnelling of a narrow tube into the wall of the reservoir (Mitrofanoff, 1980). There are several variations on these themes, in some cases amounting to completely new designs (Gleeson and Griffith, 1990). The Indiana system is based on the competence of the ileocaecal valve (Rowland et al., 1987).However, the terminal ileum which forms the conduit is tailored and may even be buried in the pouch wall, which makes the whole design very similar to that of the Mitrofanoff procedure. In the Benchekroun system a length of ileum is intussuscepted but with the serosal surface being turned inwards to form the catheterisable tract, unlike the Kock procedure where the mucosal surface forms the tract (Benchekroun et al., 1989). Our own studies have shown that the resting pressure generated within a Mitrofanoff conduit is 2 to 3 times the highest pressure generated by the reservoir. Furthermore, the conduit responds to pouch pressure rises with a considerable pressure rise of its own. The difference between the conduit pressure and the pouch pressure is large, so that continence is reliably maintained. In Kock nipples the difference between conduit and pouch pressures is small and the response to pouch pressure rises is limited, so that continence is tenuous. These experimental observations .in patients have mirrored my clinical experience: it is much easier to make a continent Mitrofanoff than a continent Kock (Cumming et al., 1987). Nonetheless, all of the various systems are used with enthusiasm by their proponents. They can all be made to work but there must be lessons in construction that can only be learnt with personal practice. It can be difficult to assess the continence rates from the published data because some authors give a complication rate without specifying details and others give a continence rate after revision operations. The latest published continence rates for the various systems range from 86 to 100%. As a last resort, the AUS may be considered to
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give continence to a reconstructed outlet. The cuff of an artificial sphincter is designed to encircle a normal urethra or bladder neck. Experimental evidence suggests that AUS cuffs can be placed safely around intestine providing the pressure is low (Engelmann et al., 1985). An AUS with a cuff pressure of 71 to 80 cm has been used successfully around large bowel in 3 of 4 children with a maximum follow-up of 11 years (Light, 1989). In the reconstructed urethra the greatest experience is in patients with exstrophy. In the series reported by Decter et al. (1988), 10 of 16 patients retained their device, 9 of whom were continent. Ten revision procedures were necessary to achieve even this result. Although these results are poor, the patients were a difficult group, having failed numerous other attempts to achieve continence. As with undiversion, imagination must be used to produce the best system for each patient. It is a mistake to become addicted to a single complete system and to use it exclusively: the problems encountered in children and adolescents are varied and the correct combination of reservoir, outlet and valve must be chosen for each.
General Consequences of Reconstruction Storage and emptying In the short term, it has been shown that the reconstructed bladders of children and adolescents can store urine and can be emptied spontaneously or by ICSC. Those that have been diverted can be undiverted. If all else fails a continent internal diversion is an acceptable alternative. The surgery required is technically taxing. The complication rate is 25 to 30%. Most of the complications requiring reoperation have been related to the continence mechanism rather than to the cystoplasty (Gonzales et al., 1986). As always with the young, it is essential to consider the long-term results. For the complex reconstructions and undiversions, there seem to be only about 76 children in the literature who have been followed up for at least 5 years. It is difficult to pick out their details from the mass of data about a variety of different patient types (Woodhouse, 1991). The good results for continence are maintained but complications related to storage are becoming apparent. Rupture of reservoir The incidence of spontaneous rupture varies between different units. When it does occur, the
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consequences are devastating. There may be delay in diagnosis, although the history of sudden abdominal pain and diminished or absent urine drainage should make it obvious. The patient rapidly becomes very ill with generalised peritonitis. “Cystogram” is commonly normal. Diagnosis is best made by ultrasonography and peritoneocentesis of fluid collections. If diagnosed early, catheterisation and broad spectrum antibiotics may lead to recovery. If the patient fails to respond rapidly to this regime or if there has been delay in diagnosis, laparotomy should be performed at once. In one series 1 of 4 patients died (Rushton et al., 1988). Patients and their families should be warned of this possible complication and advised to return to hospital at once if there are any symptoms of acute abdomen, especially if the reservoir stops draining its usual volume of urine.
Pregnancy When reconstructing females it is essential to consider the possibility of a future pregnancy. The “bladder” should be made to lie anteriorly in the abdomen. The bowel pedicle should be fixed on one side to allow enlargement of the uterus on the other. Pregnancies after any type of undiversion or lower tract reconstruction with bowel are rare so far. They may be complicated and require the joint care of obstetrician and urologist (Hill and Kramer, 1989). Particular problems include upper tract obstruction and changes in bladder control as the uterus enlarges, and damage to the bladder outlet during vaginal delivery. It is tempting to recommend caesarean section but only with a urologist present who can protect the bowel pedicle from damage. Pregnancies after continent diversion have not yet been reported in the literature. There has been a successful one in my own series and another from Philadelphia, both following Mitrofanoff procedures (Duckett, personal communication). Metabolic changes Metabolic changes are common when urine is stored in intestinal reservoirs and must be carefully monitored. Nurse and Mundy (1989a) made an experimental study of the handling of electrolytes instilled into intestinal reservoirs and subsequent arterial blood gas analysis. All patients were found to absorb sodium and potassium from the reservoirs but the extent was variable. A third of all patients
118 (but 50% of those with an ileocaecal reservoir) had hyperchloraemia. All patients had abnormal blood gases, the majority having metabolic acidosis with respiratory compensation. The findings were unrelated to either renal function or to the time that had elapsed since the reservoir was constructed. In 106 children and adolescents at St Peter’s Hospitals who had any form of enterocystoplasty, hyperchloraemic acidosis (HCA) was found in 14 (13%) and borderline HCA in 25 (24%). The incidence was lower in reservoirs with ileum as the only bowel segment when compared with those containing some colon (9 us 20%). When arterial blood gases were measured in 29 of these children no consistent pattern was found (Wagstaff et al., 1991). A further area that might give concern is the consequence of removing the ileocaecal valve and terminal ileum from the intestinal tract to use in the bladder reconstruction. Although the remainder of the ileum is known to have considerable ability to adapt, there have been fears that patients would develop macrocytic anaemia or chronic diarrhoea. In practice these fears seem to have been unfounded. There have been no clinical problems in my own patients: 8% were found to be anaemic but none had macrocytaemia or folate deficiency. In an experimental study in patients with reservoirs between 8 months and 8 years old, no disturbance of BIZor carotene metabolism was found (Canning et al., 1989). In Kock pouch patients followed up for 5 to 1 1 years, 6 of 17 patients were found to have borderline or subnormal BIZlevels requiring supplements (Akerlund et al., 1989). It is particularly important to note that the reservoirs retain their ability to alter body metabolism for years. Where Nurse and Mundy (1989a) detected changes they were unaffected by the age of the reservoir. Renalfunction In the follow-up so far available, undiversion or continent diversion seem not to have affected renal function. When function has improved after such surgery it is likely to be the result of eliminating obstruction or high bladder storage pressure. In the longer term, renal deterioration has been related to obstruction, reflux and stone formation. In one long-term study of Kock pouch patients, these complications occurred at the same rate as that found in patients with ileal conduits : 29% at 5 to 11 years (Akerlund et al., 1989).
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Infection and stones Stones are a particular problem in reservoirs containing metal staples (such as the Kock). Otherwise, stones in the reservoir are associated with infection and retained mucus. In our patients, 28% have troublesome urinary infections and a further 20% have occasional infections. Renal stones have developed in 1.6% of patients of all ages and reservoir stones in 12% (Wagstaff et al., 1991). The significance of infection alone is uncertain. In the long-term follow-up of patients practising ICSC on non-reconstructed bladders, 11% were found to have chronic bacteriuria (Diokno et al., 1983). It has been said that such infection is harmless except in the presence of high grade reflux, when 60% of patients suffer renal damage (Kass et al., 1981). In ileal conduits, however, persistent infection, especially with Escherichia coli, has been associated with progressive renal scarring (Bergman et al., 1979). Growth The most worrying consequence of enterocystoplasty in children has been delayed growth in height. The data are far from complete. In a series of 60 children, Wagstaff et al. (1992) found delay in linear growth in 20%, while growth in weight was normal. There were no metabolic or infective complications to account for this finding. Mundy (personal communication) reported 3 other children who had delayed growth after ileocystoplasty. Cancer It is the constant fear of surgeons who carry out these operations on the young that history will repeat itself: the bowel segments will develop neoplasia in the same way that the ureterosigmoidostomy does. Evidence from animal studies suggests that faecal and urinary streams must be mixed for neoplasia to occur. However, if it is chronic mixed bacterial infection, rather than the faeces per se, that is required, the bowel urinary reservoir may be at risk. In 23 patients with colonic and ileal cystoplasties, high levels of nitrosamines were found in the urine (Nurse and Mundy, 1989b). Biopsies of the ileal and colonic segments showed changes similar to those found in ileal and colonic conduits and in ureterosigmoidostomies. More severe histological changes and higher levels of nitrosamines correlated with heavy mixed bacterial growth on urine culture.
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Single cases of pouch neoplasms have been reported. In a review of the literature, 14 cases were identified by Filmer and Spencer (1990). Special features could be found in nearly all cases. For example, 10 patients had been reconstructed for tuberculosis;4 tumours were not adenocarcinomas ; 1 patient had a pre-existing carcinoma; 6 patients were over 50 years o!d. Nonetheless, cancer does occur in enterocystoplasties at a mean of 18 years from construction. This is a few years earlier than the mean time at which malignant neoplasms are seen in ureterosigmoidostomies. Cancer must remain a cause for great vigilance in the follow-up of patients. It is interesting to note that no cases have been reported from units routinely performing large numbers of these operations. Psychological consequences There has been no study of the psychological consequences of lower urinary tract reconstruction in children. Apart from the trauma of major surgery, it seems probable that successful reconstruction in a young child could only be beneficial. Similarly good results could be expected when getting rid of a cutaneous diversion in a child. In adolescents, particularly those who have been diverted all of their lives, the generally good outcome has been tempered with the occasional and unexpected disaster. Most surgeons with wide experience of undiversion have a patient who, while not wishing to have the bag back, is disturbed by the new arrangement. Conversely, a problem arises with the “disaffected teenager”. His many social and psychological problems are blamed by his family and doctors on “growing up” and by the patient on his stoma. The greatest care must be taken in the assessment of these patients, but occasionally I have found that the patient’s view was correct: all was solved by getting rid of the stoma. In a formal psychological assessment of their patients, Boyd et al. (1987) found some surprising facts. They compared those who had had ileal conduits with those who had had a Kock pouch. A small subgroup had been converted from conduit to Kock. The majority of patients had cancer and were past middle age. There was little difference in the levels of satisfaction and of physical activity between the 2 main groups. Significantly for the adolescent, however, was the finding that the Kock patients had a better self image, had more physical contact with others and more sexual activity. The subgroup who had had a conversion from conduit
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to Kock were almost uniformly extremely satisfied by the result. References Adams, M. C., Mitchell, M. E. and Rink, R. C. (1988). Gastrocystoplasty : an alternative solution to the problem of urological reconstruction in the severely compromised patient. J. Urol., 140, 1152-1156. Akerlund, S., D e b , K., Kock, N. G. e t d (1989). Renal function and upper urinary tract configuration following urinary diversion to a continent ileal reservoir (Kock pouch). J . Urol., 142,964968. Benchekroun, A., Essekali, N., Faik, M. et d (1989). Continent urostomy with hydraulic ileal valve in 136 patients: 13 years ofexperience. J. Urol., 142,46-51. Bergman, B., Kaijser, B. and Nilson, A. E. (1979). Urinary diversion and urinary tract infection. Scand. J. Urol. Nephrol., 13,65-70. Boyd, S. D., Feinberg, S. M., Skinner, D. G. e t d (1987). Quality of life survey of urinary diversion patients. J. Urol., 138,13861389. Canning, D. A., Perman, J. A., Jeffs, R. D. et d (1989). Nutritional consequences of bowel segments in the lower urinary tract. J. Urol., 142,509-51 1. Cumming, J., Worth, P. H. L. and Woodhouse, C. R. J. (1987). The choice of suprawbic continent catheterisable stoma. Br. J. Urol., 60,227z236. Decter. R. M.. Roth. D. R.. Fishman. I. J. et d (1988). Use of the AS800 device ’in exstrophy and epispadias: J. urol., 140, 1202- 1203. Dmkno, A. C., Sonda, L. P., Hollander, J. P. et d. (1983). Fate of patients started on clean intermittent self catheterisation therapy 10 years ago. J. Urol., 129,112&1122. Engelmann, U. H., Felderman, T. P. and Scott, F. B. (1985). The use of the AMS-AS800 artificial sphincter for continent urinary diversion. 1. Investigations including pressure flow studies using rabbit intestinal loops. J. Urol., 134, 183-190. Filmer, R. B. and Spencer, J. R. (1990). Malignancies in bladder augmentations and intestinal conduits. J. Urol., 143,671-678. Galloway, N. T. M., Heathcote, P. S., Gately, C. et aL (1987). The longer-term results of undiversion. Br. J. Urol., 60, 5153. Gleeson, M. J. and Griffith, D. P. (1990). Urinary diversion. Br. J. Urol., 66,113-122. Gonzalez, R. and Sidi, A. A. (1985). Preoperative prediction of continence after enterocystoplasty or undiversion in children with neurogenic bladder. J. Urol., 134,705-707. Gonzalez, R., Sidi, A. A. and Zhang, G. (1986). Urinary undiversion : indications, technique and results in 50 cases. J . Urol., 136, 13-16. Hill, D. E. and Kramer, S. A. (1989). Pregnancy after augmentation cystoplasty. J. Urol., 144,457459. Hinman, F. (1988). Selection of intestinal segments for bladder substitution: physical and physiological characteristics. J. Urol., 139, 519-523. Kass, E. J., Koff, S. A., Diokno, A. C. et d (1981). The significance of bacilluria in children on long term intermittent catheterisation. J. Urol., 126,223-235. Kock, N. G. (1971). Ileostomy without external appliances: a survey of 25 patients provided with intra-abdominal reservoir. Ann. Surg., 173,545-550. Koleilat, N., Sidi, A. A. and Gonzalez, R. (1989). Urethral false passage as a complication of intermittent catheterisation. J. Urol., 142, 1216-1217.
120 Leadbetter, G. W. (1985). Surgical reconstruction for complete urinary incontinence: a 10 to 22 year follow-up. J. Urol., 133, 205-206. Light, J. K. (1989). Long-term clinical results using the artificial sphincter around bowel. Br. J . Urol.,64,56-60. Lytton, B. and Green, D. F. (1989). Urodynamic studies in patients undergoing bladder replacement surgery. J . Urol., 141,13941397. Mitchell, M. E., Adams, M. C. and Rink, R. C. (1988). Urethral replacement with ureter. J . Urol.,139, 1282-1285. Mitchell, M. E. and Piser, J. A. (1987). Intestinocystoplasty and total bladder replacement in children and young adults : follow-up of 129 patients. J. Urol., 138, 579-584. Mitrofanoff, P. (1980). Cystostomie continente trans-appendiculaire dans le traitement des vessies neurologiques. Chir. Pediatr., 21,297-305. Mundy, A. R. (1988a). Cystogasty. In Current OperativeSurgeryUrology,ed: Mundy A. R. Chapter 11, pp. 140-159. London: Bailliere Tindall. Mundy, A. R. (1988b). A technique for total substitution of the lower urinary tract without the use of a prosthesis. Br. J . Urol., 62,334338. Mundy, A. R., Nurse, D. E., Dick, J. A. et al. (1986). Complex urinary undiversion. Br. J . Urol., 58,640-643. Nurse, D.E. and Mundy, A. R. (1989a). Metabolic complications of cystoplasty. Br. J. Urol., 63, 165-170. Nurse, D. E. and Mundy, A. R. (1989b). Assessment of the malignant potential of cystoplasty. Br. J . Urol., 64,489492. Parry, J. R. W., Nurse, D. E., Boucaut, H. A. P. et aL (1990). Surgical management of the congenital neuropathic bladder. Br. J.Urol., 65, 164-167.
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Rowland, R. G., Mitchell, M. E., Bhrle, R. et al. (1987). Indiana continent urinary reservoir. J . Urol.,137, 1136-1 139. Rushton, H. G., Woodward, J. R., Parrott, T.S. et al. (1988). Delayed bladder rupture after augmentation enteroplasty. J. Urol.,140, 344346. Wagstaff, K. E., Woodhouse, C. R. J., Duffy, P. G. et aL (1992). Delayed linear growth in children with enterocystoplasties. Br. J. Urol., 69, 314317. Wagstaff, K. E., Woodhouse, C. R. J., Rose, G. A. etaL (1991). Blood and urine analysis in patients with intestinal bladders. Br. J. Urol., 68, 311-316. Wang, S. C., McGuire, E. J. and Bloom, D. A. (1988). A bladder pressure management system for myelodysplasia-clinical outcome. J. Urol., 140, 1499-1502. Woodhouse, C. R. J. (1991). Undiversion and alternatives to a cutaneous urinary diversion. In Long Term Paediatric Urology. Pp. 97-1 15. Oxford: Blackwell. Woodhouse, C. R. J. (1992). Reconstruction of the lower urinary tract for neurogenic bladder: lessons from the adolescent age group. Br. J. Urol., 69,589-593.
The Author C. R. J. Woodhouse, MB, FRCS, Senior Lecturer, Institute of Urology; Consultant Urologist, St George’s Hospital and the Royal Marsden Hospital; Honorary Consultant Urologist, St Peter’s Hospitals and the Hospital for Sick Children, London. Requests for reprints to: C. R. J. Woodhouse, Institute of Urology and Nephrology, University College and Middlesex School of Medicine, Riding House Street, London W1P 7PN.
01992 British Journal of Urology
REVIEW
Lower Urinary Tract Reconstruction in Young Patients C.R . J. WOODHOUSE Institute of Urology; Departments of Urology, St George‘s Hospital and Royal Marsden Hospital, London
There are now very few indications for a permanent cutaneous urinary diversion in a child. Many congenital anomalies of the lower urinary tract can be completely reconstructed. The neuropathic bladder can be made continent and “safe” for the kidneys. For some patients continent diversion avoids the need for a cutaneous appliance. In the past, however, diversion was sometimes a life-saving procedure and in other patients the only practical method of managing incontinence or recurrent urinary infection. In young adults, the reconstructive techniques that are used now in children can be applied to get rid of the bag. Almost anyone can have a reconstruction, but as the original surgery may have included resection of some of the urinary tract, more extensive and imaginative techniques may be needed. Before undertaking such surgery it is important to consider the possible long-term benefits. The only certain improvement is cosmetic : the patient will have no external appliance. If the bladder is neurologically normal the long-term results of undiversion should be good. Some patients will be able to void normally but others may need to empty the bladder by intermittent clean self-catheterisation (ICSC). The long-term results of cutaneous diversion are poor, whilst the late results of complex reconstructions, especially those using bowel, are yet unknown. Some infective and metabolic complications are becoming apparent. It is salutary to remember that ureterosigmoidostomy had been used for nearly 100 years before the risk of neoplasia was really appreciated. Those who undertake such surgery must be sure that the parents and patients know of the uncertain future and the need for careful follow-up.
Older patients should not be deprived of such surgery just on grounds of age. However, care is needed to select patients who are both physically and emotionally fit enough.
Principles of Lower Urinary Tract Reconstruction When one or more components of the lower urinary tract are unusable, imagination is needed to devise a system that avoids an external bag. Reconstruction should be tailored for each patient. There are 3 components to be considered and they are largely independent of each other: a reservoir for the urine, an outlet to the surface and a continence mechanism. The anatomical structures that have been used for each of these are shown in the Table. It can be seen that the reservoir may be made from one or more of the structures in the first column and the outlet from one in the second column (lengthened by a skin tube if necessary); the continence mechanism is indicated from column 3. The reconstructed system may be emptied by ICSC or by spontaneous voiding. Simple reconstruction or undiversion has now become routine and will not be considered further. Even for complex reconstructions, however, the reservoir should include as much normal bladder as Table Tissues Used in Reconstruction of the Lower Urinary Tract Reservoir
Conduit
Control system
Bladder Stomach Ileum Caecum Colon
Urethra Appendix Fallopian tube Ureter Skin tube Ileum Umbilical vein
Urethral sphincters Mitrofanoff Kock Ileocaecal valve Benchekroun Artificial sphincter
Accepted for publication 4 February 1992
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114 possible. The urethra remains the best outlet. The natural sphincters, if they can retain urine, are the best control mechanism. Otherwise the choice of reconstruction depends on the material available, the needs of the patient, the possibilities for follow-up and the skill of the surgeon. It is a mistake to confine oneself to a single systeia, such as a particular type of pouch, because none is suitable for all patients. Furthermore, there are many occasions when a planned reconstruction must be changed mid-operation if a component is found to be unusable.
Choice of Reservoir Bladder is the natural reservoir for urine and should only be abandoned if irreversibly diseased, a rare circuinstance in children and adolescents. The bladder may be removed for cancer or when damaged by radiotherapy or cyclophosphamide. In endemic areas, bilharzia or tuberculosis can destroy the bladder. Chronic interstitial cystitis is occasionally seen in adolescents. In exstrophy and its variants the bladder may be too small (particularly after a failed reconstruction) to be worth saving. Adolescent females with a congenital neuropathic bladder are an exceptional group in whom it is not wise to retain a large bladder remnant. Five of 16 in my own series had problems from the residual native bladder. In one there was persistent hyper-reflexia. In 4 patients hyper-reflexia was unmasked by sexual activity: orgasm in 3 and sexual arousal in 1. Therefore, if bladder emptying is likely to be by ICSC, a good case can be made for using a substitution cystoplasty (Woodhouse, 1992). If the bladder is small, poorly compliant or at high pressure, some bowel may be added (augmentation cystoplasty) or may completely replace the bladder (substitution cystoplasty). A sinall bladder must be considered in relation to the ureteric and urethral junctions. If there is no ureteric reflux or obstruction and the outlet is continent, the small bladder may be an ideal base for reconstruction. It is usually difficult to reimplant the ureters into such a bladder, though it may be possible to reconstruct the outlet for incontinence. If there is no bladder (or if it is to be abandoned), a new reservoir must be constructed from intestine. If there is an existing conduit, it should be incorporated. There is no consensus on the choice of bowel for a urinary reservoir, except that jejunum is too metabolically active for general use; 40 cm of small intestine, 20 cm of large intestine or a
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combination can make a low pressure substitute of adequate volume. The stomach may be useful in children with renal failure because of the acid that is secreted (Adam et al., 1988). However, if the child then becomes anuric, the continued secretion of acid into an otherwise “dry” bladder may cause irreversible damage. The intrinsic peristaltic contractions must be abolished. I believe that this is best achieved by longitudinal incision through the full thickness of the bowel wall. The opened-out bowel is then reconfigured to form a reservoir which is as near spherical as possible (Hinman, 1988). Gonzales et al. (1986) used bowel without transection of the muscle and gave anticholinergics to suppress peristalsis in the 50% of patients who had unacceptably high pressures. Alternatively, a very long segment of bowel may be used, so that the volume of urine stored is never large enough to stimulate high pressure contractions (Mundy, 1988a). Urodynamic studies have shown that the lowest pressures are achieved with an ileal patch augmentation of a colonic bladder (Lytton and Green, 1989). It is not always possible to abolish peristalsis even by complete transection of the circular muscle. I have also encountered 2 patients who re-established high pressure peristalsis after a period of bowel inertia. I have not found anticholinergic drugs to be effective. In follow-up, the ileum appears to have a greater metabolic activity but a lesser risk of spontaneous rupture than colon. However, the differences are insufficient to dictate a particular choice. As lower urinary tract reconstruction becomes more complex in its surgery, so the choice of technique becomes more difficult.
The Outlet The urethra is the natural outlet for urine and the only one that will allow spontaneous voiding. The urethra is more dependent on its continence mechanism than other possible outlets. Together, the urethra and its sphincters form the ideal system, but it can be difficult to predict whether they will be competent or usable after bladder reconstruction. Grossly abnormal bladders are often associated with other major abnormalities. Firstly, the substituted bladder cannot always be emptied to completion, so that ICSC may be
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necessary. Patients with a neuropathic bladder which is so poor that substitution is necessary are often severely affected with myelomeningocele. They may lack the manual dexterity to selfcatheterise. The problem is worse if they walk with 2 crutches or are wheel-chair bound. If the bladder is not neuropathic but has been damaged, for example, by cyclophosphamide or surgery, the urethra, especially in the male, may be too sensitive for ICSC. Secondly, the urethral sphincters may be incompetent, so that either an artificial sphincter (AUS) or a bladder neck reconstruction will be necessary. The feasibility and desirability of these procedures must be taken into account before deciding to anastomose the pouch to the urethra. Thirdly, if an adolescent boy has had a lifelong diversion, his penis will only ever have been a sexual organ. If, after undiversion, he needs to selfcatheterise, the philosophical question arises as to whether he should catheterise his sexual organ. It seems unlikely that there is a “right” answer to this question. The site of a catheterisable stoma should be chosen chiefly for the patient’s convenience : the urethra should only be used as the outlet if it is clearly superior to a suprapubic continent stoma. If there is no usable urethra the choice lies between constructing one or using a technique of continent diversion with a suprapubic catheterisable stoma. It is possible completely to reconstruct the urethra from labia, penile skin or pedicled ureter. The tube is then tunnelled into the reservoir to make a continent outflow. The bladder is usually emptied by ICSC but spontaneous voiding is sometimes (Mitchell et al., 1988; Mundy, 1988b). Such intricate reconstruction is only occasionally indicated. A new outlet can be constructed from almost any tube. If a wide bore tube (such as ileum) is used, its continence system must involve some kind of intussusception, unless it is tailored. Narrow tubes must be tunnelled into the reservoir to produce continence. Of the narrow tubes the appendix is the easiest to use and appears to be the most resilient. A slightly dilated ureter is also satisfactory, but a completely normal one may be difficult to dilate to a diameter suitable for catheterisation. The lateral two-thirds of the fallopian tube are also satisfactory but are seldom available. A tube can also be made from pedicled skin to use alone or with anastomosis. The ileum is the standard wide tube and is generally the easiest to catheterise.
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Continence Systems If the urethra is the ideal outlet, the urethral sphincters should be the best continence system. In diverted patients, they must be tested and preparation should include an examination under anaesthesia and insertion of 2 suprapubic catheters. Several days can then be spent filling the bladder with water and testing the sphincter function-socalled “bladder cycling”. Urodynamics are performed when a stable state is reached. Neurologically normal sphincters should allow complete emptying after enterocystoplasty. Even in patients with the neuropathic bladder of spina bifida, I have found that function was correctly predicted in 79% of patients by this simple investigation (Woodhouse, 1992). Using the more sophisticated techniques of fluoroscopy of the internal sphincter and electromyography of the external sphincter, correct function was predicted in 93%of patients (Gonzalez and Sidi, 1985). In assessing the bladder/sphincter function as a whole, especially in patients with neuropathies, Wang et al. (1988) emphasised the importance of the “leak point pressure”. That is the intravesical pressure at which leakage occurs through the urethral sphincters. If the leak point pressure is above 40 cm of water, upper tract deterioration is likely, probably because of progressive loss of bladder compliance. Conversely, as a bladder reconstructed by enterocystoplasty usually has a resting pressure below 30 cm of water, such powerful sphincters should maintain continence. In my experience, young adults with normal sphincters have been continent after bladder substitution. However, in older patients some authors have found day or night incontinence. The main cause has been persistence of mass peristalsis even in detubularised bowel. Most of the reported patients were elderly and all had had a cystectomy; both of these factors may have had an adverse effect on sphincter function (Lytton and Green, 1989). If the sphincters are continent but do not allow complete emptying, ICSC is generally the best management. There is sometimes pressure on the surgeon to arrange for “normal” voiding. To achieve this it would be necessary to destroy the natural sphincters and to implant an AUS. In most cases this seems unwise. Even with an AUS, complete voiding cannot be guaranteed after enterocystoplasty and ICSC may still be necessary. The long-term results of AUS are unknown. Should the AUS become infected and require removal, the
116 patient would be continually incontinent and worse off than he was originally: the options for further reconstruction would then be limited. Finally, in units performing large numbers of these operations, many of the patients come from remote parts of the world where “servicing” of a faulty AUS might be impossible. If the sphincters are incontinent, the options are to reconstruct the bladder outlet to create continence or to use an AUS. A good case can be made for either of these. However, units have tended to use either one or the other and so it is difficult to establish indications for each. For example, Gonzalez and Sidi (1985) reported the use of the YoungDees bladder neck reconstruction in 8 patients with incompetent sphincters, 7 of whom were continent on ICSC. The AUS had been used in other patients with success, but the need for “servicing” and doubts about the mechanism’s longevity favoured the Young-Dees. Mitchell and Piser (1987) reserve the AUS for patients who can demonstrate complete bladder emptying. Both Mundy et al. (1986) and Galloway et al. (1987) have reported good results with undiversion using an AUS (Brantley Scott 792 or 800). There were 36 patients in their 2 series. Virtually all were able to void to completion. Up to 7 were at least partly incontinent. There were 8 sphincter complications that required reoperation. At least in patients with neuropathic bladder, the trend has been away from sphincterotomy and the AUS in favour of ICSC (Parry et al., 1990). The long-term results of ICSC are good. When Diokno et al. (1983) reviewed patients after 10years’ follow-up, only 2 of 60 patients had given up catheterising because of a complication. Thirty others had been lost to follow-up, died or suffered a change in neurological status. However, the urethra can be damaged so badly that the technique has to be abandoned. The risk is greater if the urethra has no sensation. Development of a false passage is a particular problem and can be difficult to treat: 2 of 9 patients in one series had to have a continent diversion because of this complication (Koleilat et al., 1989). Attempts to make a continent bladder neck and urethra which also allow complete spontaneous voiding have met with mixed results. It is difficult to balance the outflow resistance against the intravesical pressure. Furthermore, there must be enough good bladder tissue to allow the reconstruction to be done (not a common circumstance in children presenting for reconstruction). In a 10- to 22-year follow-up of patients who had
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bladder neck reconstruction (but who had not been diverted), 19 of 27 children were completely dry and 1 had stress incontinence. One child emptied by ICSC, but the rest voided normally (Leadbetter, 1985). This is probably the best result and longest follow-up available for such reconstructions. Those who claim success for more complex methods must compare their results with it. Without the urethral sphincters, there are 2 basic principles that may be applied to create continence : either a flutter valve made by the intussusception of a wide bore tube (Kock, 1971) or a flap valve made by the tunnelling of a narrow tube into the wall of the reservoir (Mitrofanoff, 1980). There are several variations on these themes, in some cases amounting to completely new designs (Gleeson and Griffith, 1990). The Indiana system is based on the competence of the ileocaecal valve (Rowland et al., 1987).However, the terminal ileum which forms the conduit is tailored and may even be buried in the pouch wall, which makes the whole design very similar to that of the Mitrofanoff procedure. In the Benchekroun system a length of ileum is intussuscepted but with the serosal surface being turned inwards to form the catheterisable tract, unlike the Kock procedure where the mucosal surface forms the tract (Benchekroun et al., 1989). Our own studies have shown that the resting pressure generated within a Mitrofanoff conduit is 2 to 3 times the highest pressure generated by the reservoir. Furthermore, the conduit responds to pouch pressure rises with a considerable pressure rise of its own. The difference between the conduit pressure and the pouch pressure is large, so that continence is reliably maintained. In Kock nipples the difference between conduit and pouch pressures is small and the response to pouch pressure rises is limited, so that continence is tenuous. These experimental observations .in patients have mirrored my clinical experience: it is much easier to make a continent Mitrofanoff than a continent Kock (Cumming et al., 1987). Nonetheless, all of the various systems are used with enthusiasm by their proponents. They can all be made to work but there must be lessons in construction that can only be learnt with personal practice. It can be difficult to assess the continence rates from the published data because some authors give a complication rate without specifying details and others give a continence rate after revision operations. The latest published continence rates for the various systems range from 86 to 100%. As a last resort, the AUS may be considered to
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give continence to a reconstructed outlet. The cuff of an artificial sphincter is designed to encircle a normal urethra or bladder neck. Experimental evidence suggests that AUS cuffs can be placed safely around intestine providing the pressure is low (Engelmann et al., 1985). An AUS with a cuff pressure of 71 to 80 cm has been used successfully around large bowel in 3 of 4 children with a maximum follow-up of 11 years (Light, 1989). In the reconstructed urethra the greatest experience is in patients with exstrophy. In the series reported by Decter et al. (1988), 10 of 16 patients retained their device, 9 of whom were continent. Ten revision procedures were necessary to achieve even this result. Although these results are poor, the patients were a difficult group, having failed numerous other attempts to achieve continence. As with undiversion, imagination must be used to produce the best system for each patient. It is a mistake to become addicted to a single complete system and to use it exclusively: the problems encountered in children and adolescents are varied and the correct combination of reservoir, outlet and valve must be chosen for each.
General Consequences of Reconstruction Storage and emptying In the short term, it has been shown that the reconstructed bladders of children and adolescents can store urine and can be emptied spontaneously or by ICSC. Those that have been diverted can be undiverted. If all else fails a continent internal diversion is an acceptable alternative. The surgery required is technically taxing. The complication rate is 25 to 30%. Most of the complications requiring reoperation have been related to the continence mechanism rather than to the cystoplasty (Gonzales et al., 1986). As always with the young, it is essential to consider the long-term results. For the complex reconstructions and undiversions, there seem to be only about 76 children in the literature who have been followed up for at least 5 years. It is difficult to pick out their details from the mass of data about a variety of different patient types (Woodhouse, 1991). The good results for continence are maintained but complications related to storage are becoming apparent. Rupture of reservoir The incidence of spontaneous rupture varies between different units. When it does occur, the
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consequences are devastating. There may be delay in diagnosis, although the history of sudden abdominal pain and diminished or absent urine drainage should make it obvious. The patient rapidly becomes very ill with generalised peritonitis. “Cystogram” is commonly normal. Diagnosis is best made by ultrasonography and peritoneocentesis of fluid collections. If diagnosed early, catheterisation and broad spectrum antibiotics may lead to recovery. If the patient fails to respond rapidly to this regime or if there has been delay in diagnosis, laparotomy should be performed at once. In one series 1 of 4 patients died (Rushton et al., 1988). Patients and their families should be warned of this possible complication and advised to return to hospital at once if there are any symptoms of acute abdomen, especially if the reservoir stops draining its usual volume of urine.
Pregnancy When reconstructing females it is essential to consider the possibility of a future pregnancy. The “bladder” should be made to lie anteriorly in the abdomen. The bowel pedicle should be fixed on one side to allow enlargement of the uterus on the other. Pregnancies after any type of undiversion or lower tract reconstruction with bowel are rare so far. They may be complicated and require the joint care of obstetrician and urologist (Hill and Kramer, 1989). Particular problems include upper tract obstruction and changes in bladder control as the uterus enlarges, and damage to the bladder outlet during vaginal delivery. It is tempting to recommend caesarean section but only with a urologist present who can protect the bowel pedicle from damage. Pregnancies after continent diversion have not yet been reported in the literature. There has been a successful one in my own series and another from Philadelphia, both following Mitrofanoff procedures (Duckett, personal communication). Metabolic changes Metabolic changes are common when urine is stored in intestinal reservoirs and must be carefully monitored. Nurse and Mundy (1989a) made an experimental study of the handling of electrolytes instilled into intestinal reservoirs and subsequent arterial blood gas analysis. All patients were found to absorb sodium and potassium from the reservoirs but the extent was variable. A third of all patients
118 (but 50% of those with an ileocaecal reservoir) had hyperchloraemia. All patients had abnormal blood gases, the majority having metabolic acidosis with respiratory compensation. The findings were unrelated to either renal function or to the time that had elapsed since the reservoir was constructed. In 106 children and adolescents at St Peter’s Hospitals who had any form of enterocystoplasty, hyperchloraemic acidosis (HCA) was found in 14 (13%) and borderline HCA in 25 (24%). The incidence was lower in reservoirs with ileum as the only bowel segment when compared with those containing some colon (9 us 20%). When arterial blood gases were measured in 29 of these children no consistent pattern was found (Wagstaff et al., 1991). A further area that might give concern is the consequence of removing the ileocaecal valve and terminal ileum from the intestinal tract to use in the bladder reconstruction. Although the remainder of the ileum is known to have considerable ability to adapt, there have been fears that patients would develop macrocytic anaemia or chronic diarrhoea. In practice these fears seem to have been unfounded. There have been no clinical problems in my own patients: 8% were found to be anaemic but none had macrocytaemia or folate deficiency. In an experimental study in patients with reservoirs between 8 months and 8 years old, no disturbance of BIZor carotene metabolism was found (Canning et al., 1989). In Kock pouch patients followed up for 5 to 1 1 years, 6 of 17 patients were found to have borderline or subnormal BIZlevels requiring supplements (Akerlund et al., 1989). It is particularly important to note that the reservoirs retain their ability to alter body metabolism for years. Where Nurse and Mundy (1989a) detected changes they were unaffected by the age of the reservoir. Renalfunction In the follow-up so far available, undiversion or continent diversion seem not to have affected renal function. When function has improved after such surgery it is likely to be the result of eliminating obstruction or high bladder storage pressure. In the longer term, renal deterioration has been related to obstruction, reflux and stone formation. In one long-term study of Kock pouch patients, these complications occurred at the same rate as that found in patients with ileal conduits : 29% at 5 to 11 years (Akerlund et al., 1989).
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Infection and stones Stones are a particular problem in reservoirs containing metal staples (such as the Kock). Otherwise, stones in the reservoir are associated with infection and retained mucus. In our patients, 28% have troublesome urinary infections and a further 20% have occasional infections. Renal stones have developed in 1.6% of patients of all ages and reservoir stones in 12% (Wagstaff et al., 1991). The significance of infection alone is uncertain. In the long-term follow-up of patients practising ICSC on non-reconstructed bladders, 11% were found to have chronic bacteriuria (Diokno et al., 1983). It has been said that such infection is harmless except in the presence of high grade reflux, when 60% of patients suffer renal damage (Kass et al., 1981). In ileal conduits, however, persistent infection, especially with Escherichia coli, has been associated with progressive renal scarring (Bergman et al., 1979). Growth The most worrying consequence of enterocystoplasty in children has been delayed growth in height. The data are far from complete. In a series of 60 children, Wagstaff et al. (1992) found delay in linear growth in 20%, while growth in weight was normal. There were no metabolic or infective complications to account for this finding. Mundy (personal communication) reported 3 other children who had delayed growth after ileocystoplasty. Cancer It is the constant fear of surgeons who carry out these operations on the young that history will repeat itself: the bowel segments will develop neoplasia in the same way that the ureterosigmoidostomy does. Evidence from animal studies suggests that faecal and urinary streams must be mixed for neoplasia to occur. However, if it is chronic mixed bacterial infection, rather than the faeces per se, that is required, the bowel urinary reservoir may be at risk. In 23 patients with colonic and ileal cystoplasties, high levels of nitrosamines were found in the urine (Nurse and Mundy, 1989b). Biopsies of the ileal and colonic segments showed changes similar to those found in ileal and colonic conduits and in ureterosigmoidostomies. More severe histological changes and higher levels of nitrosamines correlated with heavy mixed bacterial growth on urine culture.
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Single cases of pouch neoplasms have been reported. In a review of the literature, 14 cases were identified by Filmer and Spencer (1990). Special features could be found in nearly all cases. For example, 10 patients had been reconstructed for tuberculosis;4 tumours were not adenocarcinomas ; 1 patient had a pre-existing carcinoma; 6 patients were over 50 years o!d. Nonetheless, cancer does occur in enterocystoplasties at a mean of 18 years from construction. This is a few years earlier than the mean time at which malignant neoplasms are seen in ureterosigmoidostomies. Cancer must remain a cause for great vigilance in the follow-up of patients. It is interesting to note that no cases have been reported from units routinely performing large numbers of these operations. Psychological consequences There has been no study of the psychological consequences of lower urinary tract reconstruction in children. Apart from the trauma of major surgery, it seems probable that successful reconstruction in a young child could only be beneficial. Similarly good results could be expected when getting rid of a cutaneous diversion in a child. In adolescents, particularly those who have been diverted all of their lives, the generally good outcome has been tempered with the occasional and unexpected disaster. Most surgeons with wide experience of undiversion have a patient who, while not wishing to have the bag back, is disturbed by the new arrangement. Conversely, a problem arises with the “disaffected teenager”. His many social and psychological problems are blamed by his family and doctors on “growing up” and by the patient on his stoma. The greatest care must be taken in the assessment of these patients, but occasionally I have found that the patient’s view was correct: all was solved by getting rid of the stoma. In a formal psychological assessment of their patients, Boyd et al. (1987) found some surprising facts. They compared those who had had ileal conduits with those who had had a Kock pouch. A small subgroup had been converted from conduit to Kock. The majority of patients had cancer and were past middle age. There was little difference in the levels of satisfaction and of physical activity between the 2 main groups. Significantly for the adolescent, however, was the finding that the Kock patients had a better self image, had more physical contact with others and more sexual activity. The subgroup who had had a conversion from conduit
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to Kock were almost uniformly extremely satisfied by the result. References Adams, M. C., Mitchell, M. E. and Rink, R. C. (1988). Gastrocystoplasty : an alternative solution to the problem of urological reconstruction in the severely compromised patient. J. Urol., 140, 1152-1156. Akerlund, S., D e b , K., Kock, N. G. e t d (1989). Renal function and upper urinary tract configuration following urinary diversion to a continent ileal reservoir (Kock pouch). J . Urol., 142,964968. Benchekroun, A., Essekali, N., Faik, M. et d (1989). Continent urostomy with hydraulic ileal valve in 136 patients: 13 years ofexperience. J. Urol., 142,46-51. Bergman, B., Kaijser, B. and Nilson, A. E. (1979). Urinary diversion and urinary tract infection. Scand. J. Urol. Nephrol., 13,65-70. Boyd, S. D., Feinberg, S. M., Skinner, D. G. e t d (1987). Quality of life survey of urinary diversion patients. J. Urol., 138,13861389. Canning, D. A., Perman, J. A., Jeffs, R. D. et d (1989). Nutritional consequences of bowel segments in the lower urinary tract. J. Urol., 142,509-51 1. Cumming, J., Worth, P. H. L. and Woodhouse, C. R. J. (1987). The choice of suprawbic continent catheterisable stoma. Br. J. Urol., 60,227z236. Decter. R. M.. Roth. D. R.. Fishman. I. J. et d (1988). Use of the AS800 device ’in exstrophy and epispadias: J. urol., 140, 1202- 1203. Dmkno, A. C., Sonda, L. P., Hollander, J. P. et d. (1983). Fate of patients started on clean intermittent self catheterisation therapy 10 years ago. J. Urol., 129,112&1122. Engelmann, U. H., Felderman, T. P. and Scott, F. B. (1985). The use of the AMS-AS800 artificial sphincter for continent urinary diversion. 1. Investigations including pressure flow studies using rabbit intestinal loops. J. Urol., 134, 183-190. Filmer, R. B. and Spencer, J. R. (1990). Malignancies in bladder augmentations and intestinal conduits. J. Urol., 143,671-678. Galloway, N. T. M., Heathcote, P. S., Gately, C. et aL (1987). The longer-term results of undiversion. Br. J. Urol., 60, 5153. Gleeson, M. J. and Griffith, D. P. (1990). Urinary diversion. Br. J. Urol., 66,113-122. Gonzalez, R. and Sidi, A. A. (1985). Preoperative prediction of continence after enterocystoplasty or undiversion in children with neurogenic bladder. J. Urol., 134,705-707. Gonzalez, R., Sidi, A. A. and Zhang, G. (1986). Urinary undiversion : indications, technique and results in 50 cases. J . Urol., 136, 13-16. Hill, D. E. and Kramer, S. A. (1989). Pregnancy after augmentation cystoplasty. J. Urol., 144,457459. Hinman, F. (1988). Selection of intestinal segments for bladder substitution: physical and physiological characteristics. J. Urol., 139, 519-523. Kass, E. J., Koff, S. A., Diokno, A. C. et d (1981). The significance of bacilluria in children on long term intermittent catheterisation. J. Urol., 126,223-235. Kock, N. G. (1971). Ileostomy without external appliances: a survey of 25 patients provided with intra-abdominal reservoir. Ann. Surg., 173,545-550. Koleilat, N., Sidi, A. A. and Gonzalez, R. (1989). Urethral false passage as a complication of intermittent catheterisation. J. Urol., 142, 1216-1217.
120 Leadbetter, G. W. (1985). Surgical reconstruction for complete urinary incontinence: a 10 to 22 year follow-up. J. Urol., 133, 205-206. Light, J. K. (1989). Long-term clinical results using the artificial sphincter around bowel. Br. J . Urol.,64,56-60. Lytton, B. and Green, D. F. (1989). Urodynamic studies in patients undergoing bladder replacement surgery. J . Urol., 141,13941397. Mitchell, M. E., Adams, M. C. and Rink, R. C. (1988). Urethral replacement with ureter. J . Urol.,139, 1282-1285. Mitchell, M. E. and Piser, J. A. (1987). Intestinocystoplasty and total bladder replacement in children and young adults : follow-up of 129 patients. J. Urol., 138, 579-584. Mitrofanoff, P. (1980). Cystostomie continente trans-appendiculaire dans le traitement des vessies neurologiques. Chir. Pediatr., 21,297-305. Mundy, A. R. (1988a). Cystogasty. In Current OperativeSurgeryUrology,ed: Mundy A. R. Chapter 11, pp. 140-159. London: Bailliere Tindall. Mundy, A. R. (1988b). A technique for total substitution of the lower urinary tract without the use of a prosthesis. Br. J . Urol., 62,334338. Mundy, A. R., Nurse, D. E., Dick, J. A. et al. (1986). Complex urinary undiversion. Br. J . Urol., 58,640-643. Nurse, D.E. and Mundy, A. R. (1989a). Metabolic complications of cystoplasty. Br. J. Urol., 63, 165-170. Nurse, D. E. and Mundy, A. R. (1989b). Assessment of the malignant potential of cystoplasty. Br. J . Urol., 64,489492. Parry, J. R. W., Nurse, D. E., Boucaut, H. A. P. et aL (1990). Surgical management of the congenital neuropathic bladder. Br. J.Urol., 65, 164-167.
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Rowland, R. G., Mitchell, M. E., Bhrle, R. et al. (1987). Indiana continent urinary reservoir. J . Urol.,137, 1136-1 139. Rushton, H. G., Woodward, J. R., Parrott, T.S. et al. (1988). Delayed bladder rupture after augmentation enteroplasty. J. Urol.,140, 344346. Wagstaff, K. E., Woodhouse, C. R. J., Duffy, P. G. et aL (1992). Delayed linear growth in children with enterocystoplasties. Br. J. Urol., 69, 314317. Wagstaff, K. E., Woodhouse, C. R. J., Rose, G. A. etaL (1991). Blood and urine analysis in patients with intestinal bladders. Br. J. Urol., 68, 311-316. Wang, S. C., McGuire, E. J. and Bloom, D. A. (1988). A bladder pressure management system for myelodysplasia-clinical outcome. J. Urol., 140, 1499-1502. Woodhouse, C. R. J. (1991). Undiversion and alternatives to a cutaneous urinary diversion. In Long Term Paediatric Urology. Pp. 97-1 15. Oxford: Blackwell. Woodhouse, C. R. J. (1992). Reconstruction of the lower urinary tract for neurogenic bladder: lessons from the adolescent age group. Br. J. Urol., 69,589-593.
The Author C. R. J. Woodhouse, MB, FRCS, Senior Lecturer, Institute of Urology; Consultant Urologist, St George’s Hospital and the Royal Marsden Hospital; Honorary Consultant Urologist, St Peter’s Hospitals and the Hospital for Sick Children, London. Requests for reprints to: C. R. J. Woodhouse, Institute of Urology and Nephrology, University College and Middlesex School of Medicine, Riding House Street, London W1P 7PN.
