Arch Dis Child: first published as 10.1136/adc.43.232.672 on 1 December 1968. Downloaded from
Arch. Dis. Childh., 1968, 43, 672.
Operative Management of Neurogenic Bladder Dysfunction in Children: Ureterostomy JAMES LISTER, R. C. M. COOK,* and R. B. ZACHARY From the Congenital Anomalies Research Unit, Department of Child Health, University of Sheffield, and the Children's Hospital, Western Bank, Sheffield In the child with a neurogenic bladder, diversion Smith (1966) found such a high incidence ofurinary of the urinary stream may be indicated for the infection after the age of 2 years that he considered attainment of confident dryness and social accept- persistence beyond that age with efforts at achieving ance in the incontinent child, or for the preservation bladder training and continence to be unjustifiable. ofrenal function. All too often we have found persistent infection in For the first few years of life lack of bladder children over the age of 2, and this is frequently control is acceptable and life with nappies is associated with severe upper urinary dilatation and expected (Smith, 1965; Nash, 1957; Carlson, 1966). renal insufficiency, so that urinary diversion has In the child with anaesthetic buttocks and perineum, been a salvage operation which has perhaps slowed however, special care is necessary during these down the progressive destruction of renal tissue years for the avoidance of trophic ulceration, and but has been too late either to allow reversal of the steps must be taken to avoid urinary stagnation dilatation, or control ofthe chronic urinary infection. and to recognize and treat any urinary infection Like others, therefore, we have felt increasingly as soon as it occurs. conscious of the need for early assessment with a copyright. Regular timed bladder expression is generally view to possible earlier operative procedures. recommended to avoid stagnation (Williams, 1956; Diversion on purely social grounds can presumably Nash, 1957; Smith, 1965; Carlson, 1966), and be undertaken at any time; but if one accepts that some workers (e.g. Williams, 1960; Carlson, 1966) the renal insufficiency in children with spina bifida have been prepared to continue with this regimen is due to progressive renal destruction as a result until the age of 8 or 9 years or even older in the of infection of inadequately draining urine, then hope of achieving reasonable bladder control. The there is considerable urgency in achieving adequate use of antibiotics or chemotherapy as prophylactic drainage of the renal tract. Such drainage may
agents may be of some value in reducing the degree sometimes be achieved by procedures to widen http://adc.bmj.com/ of renal damage due to recurrent infection (Lorber, the bladder neck, but often it can only be Menneer, and Allott, 1968). achieved by diverting the urinary stream above the Provided the upper renal tract remains normal, bladder. bladder incontinence is not necessarily an indica- Early assessment of the urinary tract in these tion for diversion of the urinary stream. children may involve undertaking radical procedures A boy with dribbling incontinence can readily at a very early age, and we thought it of value to be fitted with an appliance which will keep him review our own experiences in the Paediatric dry, though even without urinary infection he may Surgical Unit at the Sheffield Children's Hospital on October 3, 2021 by guest. Protected develop such severe ulcerative dermatitis in his over four years, in order to assess the results of anaesthetic scrotum and perineum that a diversion various procedures. has to be made. A girl with dribbling incontinence cannot be fitted with a satisfactory appliance, but Material some girls (and boys too) can remain dry for two hours or more after bladder expression and we During the period January 1963 to December 1966, have taken this as an acceptable period of dryness, 132 patients had surgical procedures designed to im- a prove urinary drainage; ureterostomies are considered and thus such a child is not candidate for diversion here, diversions through ileal conduits are described on the grounds of social acceptance. elsewhere (Cook, Lister, and Zachary, 1968). Received June 13, 1968. In that period 52 ureterostomies were made in 31 *Supported by a Grant from 'Action for the Crippled Child'. patients with neurogenic bladder disease resulting 672 Arch Dis Child: first published as 10.1136/adc.43.232.672 on 1 December 1968. Downloaded from
Operative Management of Neurogenic Bladder Dysfunction in Children: Ureterostomy 673 from myelomeningocele or lipoma of the cauda equina. Their age and sex distribution is shown; most of the 10- IS a Male ureterostomies were made before the age of 5 years, 9- I--A and a significantly large proportion (9 out of 52-17%) 'C' Female at less than 1 year (Fig. 1). 8- F-II CIIL 7- F!--I I C I Indications 6 - F--n1 r-n 0 ICCI I I C I 5 - The general indications for supravesical urinary 0 F--.1 F--I I--i diversion have already been mentioned. Once it has i B i I B X C RT.i_J zz 4- been decided that the bladder must be abandoned, I BF-II I B-iI then we to 3 - bS/Bl have usually chosen undertake the simplest c procedure and thus aimed, ifpossible, to make a uretero- 2- cutaneous spout, interposing a conduit of ileum or A _B Bi B B colon only if the ureter itself is either too short or too i small in calibre to allow the placing of the spout in a 0 A B AI B' F C I situation where an effective collecting apparatus can
but the cases fall into fairly clearly defined groups, (Fig. 2). on October 3, 2021 by guest. Protected *(b) (c)
FIG. 2.-Diagrammatic representation of types of operation (a) Loop ureterostomy; (b) single terminal ureterostomy; (c) double-barrelled terminal ureterostomy: and (d) uretero-ureterostomy. Arch Dis Child: first published as 10.1136/adc.43.232.672 on 1 December 1968. Downloaded from
674 Lister, Cook, and Zachary copyright.
FIG. 3a.-Pre-operative pyelogram showing left hydronephrosis and no function on right side. b.-Intravenous pyelogram after right loop ureterostomy. Some function shown on right; considerable improvement on left. Left ureter draining retrogradefrom bladder to right ureterostomy. Note distal right ureter outlined by opaque medium refluxingfrom bladder.
(1) Loop ureterostomy. By an extraperitoneal its end to the side of a dilated one that was then used http://adc.bmj.com/ approach, the ureter is exteriorized over a skin bridge to form the stoma. in the iliac fossa and an opening made in it. Where there is a vesico-ureteric reflux on the side operated Complications. Though technically this has usually on, the resulting double stoma may drain the urine been a fairly simple and short operation, the general from both kidneys, none being passed per urethram. condition of the child has often been poor-this of Such a unilateral procedure may result in considerable course being one of the indications for the operation. reduction of bilateral hydronephrosis (Fig. 3). In four cases renal failure was exacerbated. In 2
other children uretero-ureterostomy anastomoses have on October 3, 2021 by guest. Protected (2) Terminal ureterostomy. A single ureter is leaked causing cellulitis (or in one instance peri- brought out at a site of election with respect to the tonitis). The terminal exteriorized portion of the ureter appliance and the end 'fliped'* to form a short (1 cm.) has undergone ischaemic necrosis in two instances spout. If the degree of dilatation and tortuosity of and in two others the kidney has failed to drain because the ureters allow, both are brought out in the midline kinks had been left in the ureter, demanding revision. (following an extraperitoneal route) as a double- Infection has often been exacerbated, or superinfection barrelled stoma. with resistant Gram-negative organisms has followed antibiotic treatment. Wounds have been slow to heal. (3) Uretero-ureterostomy was used in a number of The effective fitting of appliances has been difficult instances, the smaller ureter being anastomosed at except in the midline double-barrelled stomata.
*For the benefit of the uninitiated Sassenach, 'flipe' is what the Scot Results has to do to his sock before he can put it on. Ed. Mortality. 7 out of 31 children have since Arch Dis Child: first published as 10.1136/adc.43.232.672 on 1 December 1968. Downloaded from
Operative Management of Neurogenic Bladder Dysfunction in Children: Ureterostomy 675 died (23%). A 6-week-old girl (in Group A) with 40 gross hydronephrosis of a solitary kidney and a raised blood urea had a loop ureterostomy made, 36 but died on the 4th post-operative day. A 6-week-old girl (in Group C) was found to 32 have bilateral hydronephrosis and a raised blood 28 urea and the urine was already infected. Bilateral Satisfactory .4 loop ureterostomies were made. She remained 0L. 24 Deterioration fairly well for 7 months but then died of renal Ureter in child 20 i who had died failure. A 5-week-old girl (Group A) had bilateral termi- 0 16 nal ureterostomies because of bilateral hydro- z nephrosis and a raised blood urea. The urine was 12* uninfected. She died of ventriculitis in an un- treated hydrocephalus at the age of 7 weeks. 8 A 3-month-old girl with bilateral double kidneys and ureters had moderate hydronephrosis in three 4. renal elements, and gross hydronephrosis in the 0 fourth. The blood urea was within normal limits, Nrmal Moderately Grossly but the urine was infected (Group B). The urine dilated dilated was diverted through a series of uretero-ureter- FIG. 4.-The results of ureterostomy in relation to the ostomies but after operation there was urine leakage, pre-operative calibre of the ureter. wound dehiscence, and renal failure from which the child died. Fig. 5 shows the results in relation to the 4 A 7-month-old boy was found to have a persist- pre-operative groups (Groups S, A, B, and C); ently raised blood urea. A pyelogram showed one patient appears twice in the 'satisfactory' bilateral hydronephrosis and hydroureters. The column in Fig. 5, since ureterostomies were made copyright. urine was uninfected (Group A). Bilateral terminal at separate times-one in Group A and one in ureterostomies were made and clinical improvement Group B. secured. He died at home one year later and the Both the children (Group S) with no upper cause of death was not determined. A girl of 13 months had bilateral terminal ureterostomies made for bilateral hydronephrosis, urinary infection, and a high blood urea. Irreversible 14, renal failure occurred after operation. Satisfactory A 41-year-old girl had a double-barrelled 12 Deterioration http://adc.bmj.com/ terminal ureterostomy made for hydronephrosis, Dead infection, and a raised blood urea (Group C). She recovered well but defaulted in the follow-up. a 8 She died of uraemia 7 months after operation, the u stoma of the ureterostomies being very stenosed. 0U The majority of these cases had ureterostomies z made because of failing renal function, and those 4. H on October 3, 2021 by guest. Protected under 1 year, particularly those of 3 months and under, had diversions performed very largely as a 2 salvage procedure. Of the 7 children who died, only 1 was over 13 months at the time of operation. It 0 L_ seems likely that this was the only death that could S A B C have been avoided: but post-operative follow-up A-Hydronephrosis with raised blood urea but no infection had failed and she clearly required a further pro- B-Hydronephrosis with infection; normal blood urea cedure to maintain her urinary drainage. C-Hydronephrosis with infection; raised blood urea S-Normal ureters Survivors. Fig. 4 shows the present state of FIG. 5.-Results of individual children in relation to the kidneys and ureters in relation to their pre- pre-operative grouping (one case appears twice, having operative calibre. a left ureterostomy at 8years and a right at 10years). Arch Dis Child: first published as 10.1136/adc.43.232.672 on 1 December 1968. Downloaded from
676 Lister, Cook, and Zachary renal tract pathology (as defined in the gross terms this 'safety valve' by allowing reflux to continue used here) required further operative procedures up the more dilated ureter. The parents of the and could only be maintained with their ureter- child whose x-rays are shown in Fig. 3 were con- ostomies for a very limited period. vinced that a complete urinary diversion had been Only 3 of the 6 children in Group A survived, made, because after a loop ureterostomy had been 2 of them being accepted as satisfactory, the third made on one side only the child stopped passing requiring revision because of stenosis at the skin any urine per urethram, both kidneys draining level. This group of children in fact forms a by the loop ureterostomy. special group of very young children most of whom In older children the indications for diversion already have considerable renal damage at the time will be combinations of incontinence, obstruction, of birth. In Groups B and C the mortality is lower and infection. Incontinence alone will demand and the proportion of successful cases rises to the interposition of a loop of ileum or colon between 15 out of 24; understandably the mortality in ureter and skin, because in our experience the Group C is higher than that in Group B, but in ureter of normal length and calibre cannot be only 1 of the survivors of Group C did the uretero- brought to the surface at a site suitable for the stomy prove unsatisfactory, whereas it was un- wearing of an effective collecting apparatus, and satisfactory in 4 of the Group B survivors. This is all the kidneys drained by ureterostomies of normal largely explained by the fact that the proportion calibre ureters showed deterioration at follow-up. of grossly dilated ureters was lower in Group B Obstruction is an indication for diversion in many than in Group C, and Fig. 4 shows that, in terms conditions other than neurogenic bladder disorders of calibre, grossly dilated ureters have a greater (Rickham, 1964), but in neurological disorders success rate, but that the price of this success is is in danger of being overlooked when it brings the amount of renal damage acquired due to back apparent 'control' to the incontinent (Smith, 1966), pressure and infection while the ureter was as the child may remain dry for lengthening becoming dilated. periods. There is general agreement that obstruc- tion is an indication for diversion at any age, but Discussion most writers are not specific about what they copyright. The indications for supravesical diversion of the consider constitutes obstruction, other than the urinary stream are so varied that differences of grosser manifestations of hydroureter and hydro- opinion in the literature as to the age of operation nephrosis. have arisen largely because of lack of clear definition The amount of residual urine is taken as one ofthese indications. criterion of obstruction, but the permissible It has been well shown by others (Eckstein, maximum allowed is not defined. Smith (1966) 1965) that upper renal dilatation may already be takes persistent or recurrent infection as evidence present at birth in the child with myelomeningo- of obstruction, and unhesitatingly recommends cele, and it has been our routine for several years diversion in these cases unless the infection is http://adc.bmj.com/ to include an intravenous pyelogram in the investi- readily controlled by drugs. gations carried out on these children during their There seems little doubt that where there is first admission, usually before they are 4 weeks obstruction as evidenced by hydronephrosis and old. Those cases shown to have grossly dilated hydroureters, with or without infection, operative upper urinary tracts at this very early age usually intervention is essential. Y-V Plasty or trans- fall into our Group A category, with evidence of urethral resection offers help in the mild cases. The renal failure but usually without severe infection grosser degrees of hydroureter are best managed by as yet. These children require urgent drainage ureterostomy, not only because such dilated ureters on October 3, 2021 by guest. Protected of the urinary tract, and as in other neonatal form excellent stomata and will drain readily, but urinary outflow obstructions, bladder drainage also because the clinical condition will preclude the will not always satisfactorily drain the tortuous more major operative procedures of intestinal dilated ureters. Loop ureterostomy in these conduit construction. In the past the presence of children is likely to be the simplest, safest, and one normal or only mildly dilated ureter was taken most effective form of treatment; not infrequently to indicate the need for diversion through an the gross hydronephrosis is unilateral, and the intestinal conduit, but it is now well established contralateral relatively undilated ureter and renal that interureteric anastomoses can function well, pelvis seems to be protected by the 'safety valve' and the smaller ureter can be implanted into the bladder pressure release of the dilated side. A loop larger, which itself forms the stoma (Madden, ureterostomy on the dilated side only preserves Tan, and McCann, 1967). Arch Dis Child: first published as 10.1136/adc.43.232.672 on 1 December 1968. Downloaded from
Operative Management of Neurogenic Bladder Dysfunction in Children: Ureterostomy 677
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FIG. 6. a-Bilateral hydronephrosis. b-Recovery of both renalpelvesfollowin double-barrelled suprapubic ureterostomy.
Provided there has not been prolonged severe be an intestinal conduit; but nevertheless ureteros- recurrent infection, and hydronephrosis is not tomy is a valuable second choice operation. gross, then the dilated kidney will usually recover http://adc.bmj.com/ once adequate drainage is achieved by ureterostomy (Fig. 6). However, the follow-up time in this Summary series has been only from 1 to 5 years, and time Experience in 52 ureterostomies carried out in may show further deterioration. One cannot deli- 31 patients suffering from myelomeningocele is berately delay operation and allow upper urinary reviewed. 7 of these children died mainly from tract dilatation in order to have a ureter of a suitable persistence of renal insufficiency but in one case calibre to form a spout, since this undoubtedly there was progression of that insufficiency. Of 24 will involve further renal damage. The aim of survivors, 7 have required further procedures, on October 3, 2021 by guest. Protected treatment in the neurogenic bladder must be the but 17 (52% of total) have not deteriorated or protection of the upper urinary tract; and thus have actually improved. urinary diversion should ideally be undertaken While it does not appear to have a place in before there is marked upper urinary tract dilatation, urinary diversion when both ureters are of normal at a stage when the calibre and length of the ureter calibre, ureterostomy is particularly indicated is insufficient to allow the formation of a satisfactory in three situations in the child with a neurogenic cutaneous ureterostomy. Ureterostomy for neuro- bladder: (1) As a definitive procedure in the genic bladder, therefore, is indicated mainly in child with at least one dilated ureter in whom those children whose kidneys are already damaged supravesical urinary diversion is required. (2) As an and if the need for diversion is recognized at the emergency measure, possibly temporary, a single proper time, then the procedure of choice will loop ureterostomy in a unilateral hydroureter with Arch Dis Child: first published as 10.1136/adc.43.232.672 on 1 December 1968. Downloaded from
678 Lister, Cook, and Zachary protecting the con- Lurber, J., Menneer, P. C., and Allott, D. C. (1968). An investiga- bladder outflow obstruction; tion into prophylactic treatment of urinary tract infections in tralateral kidney by allowing reflux up and out of infants born with spina bifida cystica. Develop. Med. Child the dilated ureter, at the same time as preserving Neurol., Suppl. 5,30. Madden, J. L., Tan, P. Y., and McCann, W. J. (1967). An experi- the dilated kidney. (3) As a temporary method mental and clinical study of cross ureteroureterostomy. Surg. of assessing function in the seriously deficient Gynec. Obstet., 124,483. Nash, D. F. E. (1957). Congenital spinal palsy. The management hydronephrotic kidney. of incontinence. Ann. roy. Coll. Surg. Engl., 20, 349. Rickham, P. P. (1964). Permanent urinary diversion in childhood. ibid., 35, 84. REFERENCES Smith, E. D. (1965). Spina Bifida and the Total Care of Myelomen- ingocek. C. C. Thomas, Springfield, Illinois. Carlson, H. E. (1966). Urologic problems in meningornyelocele. - (1966). Management of the urinary tract in spinal myelo- J. Urol. (Baltimore), 95, 245. meningocele: the case for early surgical diversion of urine. Cook, R. C. M., Lister, J., and Zachary, R. B. (1968). Operative Aust. pediat.3J., 2, 27. management of the neurogenic bladder in children: diversion Williams, D. I. (1956). The congenital neurogenic bladder. Proc. through intestinal conduits. Surgery, 63, 825. roy. Soc. Med., 49, 896. Eckstein, H. B. (1965). Urinary diversion in children. Develop. Med. - (1960). Management of the bladder in congenital spinal Child Neurol., 7, 167. cord lesions. ibid., 53, 272. copyright. http://adc.bmj.com/ on October 3, 2021 by guest. Protected