Urinary Undiversion in Myelodysplasia: Criteria for Selection and Predictive Value of Urodynamic Evaluation

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Vol. 124, July Printed in U.S. A.

THE JOURNAL OF UROLOGY

Copyright© 1980 by The Williams & Wilkins Co.

URINARY UNDIVERSION IN MYELODYSPLASIA: CRITERIA FOR SELECTION AND PREDICifIVE VALUE OF URODYNAMIC EVALUATION STUART B. BAUER, ARNOLD H. COLODNY, MARK HALLET, SHAHRAM KHOSHBIN AND ALAN B. RETIK* From the Division of Urology and Department of Neurology, Children's Hospital Medical Center and Harvard Medical School, Boston, Massachusetts

ABSTRACT

Urinary undiversion was done in 15 children with myelodysplasia and ileal conduits. The process of evaluation and the criteria for selection are described. Neurologic and psychologic factors have an important role. Electromyography of the external urethral sphincter is helpful in predicting which children would be continent after undiversion and in managing the neurogenic bladder postoperatively. Over-all, a successful outcome has been achieved in two-thirds of the children chosen for undiversion. The urologic management of children with myelodysplasia has been altered by improvements in the method of urodynamic evaluation,'· 2 the acceptance of clean, intermittent catheterization as a way of emptying the bladder and achieving continence3-5 and in the recent advances in the development of an artificial urinary sphincter. 5 It is now uncommon to divert these children for any reason. After gaining experience in the techniques and management of undiversion in children without neurologic disease,6 a select group of children with myelodysplasia have undergone urinary undiversion since 1976 and are reported on herein. CLINICAL MATERIAL

From April 1976 to June 1978, 22 children with myelodysplasia and ileal conduits were evaluated completely for the possibility of reconstituting the urinary tract. Seven children were rejected because of social immaturity, significant upper urinary tract abnormalities or compromised renal function, although they had low neurologic lesions and minimal orthopedic deformities. The remaining 15 children who were undiverted subsequently included 10 boys and 5 girls. The characteristics of these 15 children are shown in table 1. Preoperative studies included excretory urography (IVP), simultaneous voiding cystourethrography and injection of contrast medium into the ileal conduit, and cystoscopy with retrograde ureterograms. In addition, creatinine clearances were obtained in all children. A suprapubic trocar cystotomy was placed at cystoscopy and for several days the bladder was filled physiologically (30 cc per hour) to assess continence, bladder capacity and the child's ability to empty the bladder voluntarily or by self-catheterization. In the 10 most recent children urodynamic evaluation was performed 24 to 48 hours after placement of the trocar cystotomy. Bladder and rectal pressure monitoring was conducted simultaneously with external urethral sphincter electromyography. The electrical activity of the striated muscle component of the external sphincter was observed on a TECA TE4 electromyograph with audio output and was measured in response to sacral reflexes, bladder filling, attempted voiding by the Crede and Valsalva maneuvers, and voluntary voiding. Urethral pressure measurements also were recorded at rest and in response to the aforementioned tests. Accepted for publication Septemb~r 28, 1979. . . . Read at annual meeting of Amencan Urological Assoc1at10n, New York, New York, May 13-17, 1979. * Requests for reprints: Children's Hospital Medical Center, 300 Longwood Ave., Boston, Massachusetts 02115.

All children underwent psychologic counseling to assess their ability to deal with the multiplicity of surgical procedures possibly required, the focus on the genital area, the probable need for self-catheterization, the emotional pressures surrounding renewed wetness and the chance of failure and rediversion. RESULTS

Every child had a normal or stable creatinine preoperatively with a clearance >40 ml. per minute. The appearance of the urinary tract on IVP was normal in 9, with evidence of stable calicectasis and mild hydronephrosis in 4 and 2 children, respectively. Voiding cystourethrography revealed vesicoureteral reflux in 15 unused ureteral stumps in 10 children (5 had bilateral and 5 had unilateral reflux) (fig. 1). Reflux was present in only 2 of 10 children after undiversion by ureteral reanastomosis, each of whom had bilateral reflux before the undiversion and the initial diversion. Thus, reflux disappeared in 8 children after the urinary tract was reconstituted. No child had reflux after undiversion who did not have it before the initial operation. The average bladder capacity at the time of evaluation for undiversion was 76 ml. and 70 per cent had a maximum volume of <80 ml. Postoperatively, the bladder capacity increased to an average volume of 290 ml. (fig. 2). An increase in volume was noted in every child. The neurologic level was at L5 in 6, 81 in 5 and 82 in 4 children. Everyone was ambulatory and could easily use a bathroom facility. Of the 10 children undergoing preoperative urodynamic evaluation a high intravesical filling pressure was noted in 5, a hyperreflexic bladder in 3 and a hyporeflexic bladder in 2. The bladders in those children with either hypertonicity or involuntary contractions on preoperative urodynamic evaluation reverted to normal or were managed easily with anticholinergic medication after undiversion. Despite the generally low level of the neurologic lesion 2 children had normal innervation of the sphincter with voluntary control (fig. 3). Following undiversion both are voiding spontaneously and are completely dry. Four patients had evidence of partial denervation of the external urethral sphincter. Of these children 2 had intact sacral reflexes, however, and both are continent on intermittent catheterization after urinary tract reconstruction. The other 2 children remained wet on a catheterization program (1 has undergone subsequently implantation of an artificial urinary sphincter). Four children had complete lower motor neuron lesions involving the external urethral sphincter with absence of electro89

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BAUER AND ASSOCIATES TABLE 1.

Characteristics of patients

Neurologic level (L5 or below): L5, 40% Sl, 33% S2, 27% Primary reason for diversion: Urinary tract infection with or without reflux, 60% Incontinence, 40% Av. age at diversion, 5.8 yrs. Av. time before undiversion, 7.6 yrs.

REFLUX IN CHILDREN DURING VARIOUS STAGES OF URINARY TRACT CONTINUITY

with low urethral resistance. Presently, he is dry after implantation of an artificial urinary sphincter. The last boy was started on intermittent catheterization but intensely disliked it and failed to continue the program on a regular basis. The bladder became chronically distended and he required rediversion because of progressive renal failure and recurrent urinary infection. Thus, 8 of the 14 children presently undiverted are dry on catheterization (6) or voiding (2). Of the remaining 6 patients 2 had continence after placement of an artificial urinary sphincter and 4 are either awaiting this or another procedure to achieve dryness (table 2). DISCUSSION

FIG. 1. Ureteral reanastomosis was done in 11 of 15 children. Reflux was present before diversion in 3 and during bladder defunctionalization in 7. Reflux was eliminated after undiversion in 7 plus 1 of initial 3 children.

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Previously, urinary diversion was considered appropriate for children with myelodysplasia who had recurrent urinary infection, vesicoureteral reflux and/or persistent urinary incontinence.7 We now know that this mode of therapy produces its own complications, especially in the long-term care of these children. 8 Far fewer patients are being diverted today for problems that would have been treated by diversion in the past. Therefore, a select group of children with myelodysplasia who might have been managed differently today and who wanted to eliminate the external urinary appliance were evaluated for possible undiversion. The selection process is of paramount importance in ensuring a successful outcome for this endeavor. 9 The criteria for selecting patients without neurogenic bladder dysfunction and the method of evaluating the upper and lower urinary tracts have been outlined by Richie and Sacks. 10 It is mandatory that the upper urinary tract appear normal or have only minor but stable architectural derangements. In addition, creatinine clearances should be adequate to withstand the stress of undiversion. Children with myelodysplasia add a new dimension to the magnitude of the problem. Ambulation and the ability to use a bathroom facility must be established initially. The neurogenic bladder dysfunction has to be defined carefully and its response to refunctionalization has to be determined before surgical reconstruction. Kogan and Levitt devised a simple method for testing the defunctionalized bladder in addition to performing

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myographic activity and 3 are incontinent on intermittent catheterization. They probably will require a secondary operation to control the incontinence. The fourth child is a boy who has been dry on self-catheterization. The urethral resistance was elevated significantly in the region of the external sphincter, presumably owing to atrophy and fibrosis of the skeletal muscle secondary to complete denervation. There were 5 patients early in the series who did not undergo preoperative urodynamic assessment. After urinary tract reconstitution 3 have been managed successfully with intermittent catheterization. One boy remained wet on catheterization and a subsequent postoperative urodynamic study revealed complete denervation of the bladder and external urethral sphincter

for incontinence

FIG. 3. Continence after undiversion generally is related to degree of denervation of external urethral sphincter. Incomplete lower motor neuron lesions (ILMN) are divided according to responsiveness to Crede or bulbocavernosus reflex stimulation. TABLE 2.

Results %

Excellent control, 10 pts.: Voiding, 2 Intermittent catheterization, 6 Artificial sphincter, 2 · Poor control-awaiting secondary·operation, 4 pts. Failures-rediversion, 1 pt.

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FIG. 4. A, urodynamic evaluation of 24-year-old woman who had partial denervation but adequate responsiveness of external sphincter. B, urethral pressure profile reveals near normal level of resistance. Patient is dry on intermittent catheterization.

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FIG. 5. A, urodynamic evaluation of IO-year-old girl who had complete sacral lesion with absent sphincter activity and no reaction to filling or coughing. B, urethral resistance is abnormally low especially in external sphincter (ES) region. Patient will require secondary operation to achieve continence after urinary undiversion.

routine voiding cystourethrography.n Placement of a suprapubic catheter at the time of cystoscopy can be used to fill the bladder physiologically repeatedly for several days. The bladder capacity generally increases during this time and then urodynamic assessment can be undertaken. The status of the external urethral sphincter appears to be the most important parameter in predicting subsequent continence in these children. 12 In most patients with myelodysplasia the bladder neck is incompetent1 3• 14 and the contribution from the smooth muscle component to urethral resistance 15 is diminished. If the sacral nerve roots are intact or only partially damaged the highest level of urethral resistance has been noted at the external sphincter region. 15 Electromyography of the striated muscle component of the external urethral sphincter reveals the degree of denervation and the response to bulbocavernosus reflex, the Valsalva and Crede maneuvers and attempts at voluntary voiding. 1 In general, sphincter activity correlates with the level of resistance in this region measured on urethral pressure profile. Near normal urethral resistance is found when the degree of external sphincter denervation is minimal (fig. 4). Rarely, there is adequate urethral resistance despite the severe or complete denervation presumably owing to atrophy and fibrosis of the striated muscle component of the sphincter. 12' 16 Occasionally, in children with complete sacral lesions the bladder neck may be competent but functional urethral resistance from the smooth muscle component is still inadequate to ensure continence because the striated muscle component of the external urethral sphincter is atrophic (fig.

FIG. 6. A, preoperative and B, postoperative cystograms in 11-yearold boy whose bladder capacity increased from 75 to 275 cc postoperatively,

5). 17 Thus, the reactivity of the sphincter is an important determinant of the degree of continence these children may achieve once the bladder has been refunctionalized. The response of the bladder before establishing continuity of the urinary tract is difficult to assess accurately by urodynamic

92

BAUER AND ASSOCIATES

FIG. 7. Voiding cystograms in 13-year-old girl show disappearance of bilateral vesicoureteral reflux (arrows) after re-establishing ureteral continuity.

evaluation. High bladder filling pressure noted in several children is probably related to the contracted state of the bladder, since this has not persisted postoperatively. Involuntary contractions are not infrequently seen even in these children with low neurologic lesions but are controlled easily with anticholinergic medication. The capacity of the unused bladder is not a useful parameter because it increases universally once the urinary tract has been reconstituted (fig. 6). The enlargement is based on the degree of outlet resistance, which allows for urine storage and expansion of the organ. The smallest increases in bladder volume in this series occurred in those patients with inadequate urethral resistance and severe denervation of the external sphincter. This response is irrespective of the length of time the bladder has remained dormant. One may get an idea of the potential postoperative bladder volume by noting its capacity before the initial diversion. Physiologic filling of the bladder preoperatively is helpful in that it may enlarge the organ but it never truly provides an accurate picture of the eventual bladder capacity after refunctionalization. 11 • 18 As a result of these studies the dynamics of the neurogenic bladder dysfunction can be defined and a reasonably accurate prediction can be made of the child's response to urinary undiversion, that is the ability to achieve continence by voiding voluntarily or with intermittent catheterization. Recently, in the child with adequate urethral resistance and a reactive external sphincter self-catheterization has been instituted during a period of suprapubic filling of the bladder before undiversion to evaluate its efficacy. If unsuccessful a secondary procedure to control continence, that is bladder neck reconstruction, may be attempted while the bladder is still in a partially defunctionalized state. When the external urethral sphincter is denervated severely or completely and urethral resistance is too low to ensure continence with the aforementioned techniques we do not recommend undiversion at the present time because we have found, retrospectively, that it is extremely difficult to achieve continence in these children. Therefore, urodynamic assessment has been helpful in selecting the child who may be continent immediately postoperatively or with minimal surgical or drug manipulation. Thus, the patient, his family and the surgeon can discuss intelligently the alternatives to management and make decisions based on facts rather than emotions. No one is encouraged to be undiverted unless he or she can achieve easily urinary control preoperatively. Early in this series 1 boy had progressive renal failure because he adamantly refused to catheterize himself after undiversion. This necessitated rediversion to reverse the renal deterioration. Since that time psychologic assessment has been an integral part of the evaluation process for undiversion. Most patients undergoing undiversion are adolescents. The intense interest that is suddenly directed toward the genital region along with the possible need to catheterize oneself or manipulate a device in the genital area must be accepted by the patient to ensure a

successful emotional as well as technical outcome. The preoperative use of intermittent catheterization during suprapubic bladder filling has helped determine if catheterization is acceptable psychologically. V esicoureteral reflux often is present in the unused ureters. However, it generally disappears after urinary tract reconstruction using the distal ureteral stumps (fig. 7). Teele and associates postulated that the absence of flow down the ureters may be responsible for this phenomenon. 19 Ureteral wall tension and fluid dynamics may have an active role in preventing reflux. They found no evidence to support the theory that the decompressed bladder allows reflux to occur. In this series reflux into the distal ureters ceased in every child when it had not been demonstrated on the pre-diversion cystogram. Reflux did not develop after undiversion in anyone. Therefore, it is imperative to evaJuate the pre-diversion cystograms before planning urinary tract reconstruction. Ureteral reimplantation or removal of a ureteral stump with reflux may be an unnecessary addition to the operation for undiversion20 and should not be undertaken unless it persists after refunctionalization. CONCLUSION

Undiversion in children with myelodysplasia places significant stress on the urinary tract and the emotional status of the individual. Complete radiologic and urodynamic evaluation of the urinary tract as well as psychologic assessment is mandatory when planning a surgical approach to re-establishing continuity and in ensuring a successful outcome. Alternatives to achieving urinary continence can be discussed and attempted during a trial period before a total commitment to the reconstruction is undertaken. The ability to achieve urinary continence is now considered a prerequisite to undiversion. REFERENCES 1.

2. 3. 4. 5. 6.

7.

Blaivas, J. G., Labib, K. B., Bauer, S. B. and Retik, A. B.: A new approach to electromyography of the external urethral sphincter. J. Urol., 117: 773, 1977. Raezer, D. M., Benson, G. S., Wein, A. J. and Duckett, J. W., Jr.: The functional approach to the management of the pediatric neuropathic bladder: a clinical study. J. Urol., 117: 649, 1977. Mulcahy, J. J., James, H. E. and McRoberts, J. W.: Oxybutynin chloride combined with intermittent clean catheterization in the treatment of myelomeningocele patients. J. Urol., 118: 95, 1977. Plunkett, J.M. and Braren, V.: Clean intermittent catheterization in children. J. Urol., 121: 469, 1979. Scott, F. B., Bradley, W. E., Timm, G. W. and Kothari, D.: Treatment of incontinence secondary to myelodysplasia by an implantable prosthetic urinary sphincter. South. Med. J., 66: 987, 1973. Colodny, A. H.: Reconstruction of the urinary system two to ten years following establishment of "permanent" ileal loop diversion. Read at annual meeting of American Urological Association, St. Louis, Missouri, May 19-23, 1974. Smith, E. D.: Urinary prognosis in spina bifida. J. Urol., 108: 815, 1972.

URINARY UNDIVERSION IN MYELODYSPLASIA

8. Shapiro, S. R., Lebowitz, R. and Colodny, A. H.: Fate of 90 children with ilea! conduit urinary diversion a decade later: analysis and complications, pyelography, renal function and bacteriology. J. Urol., 114: 289, 1975. 9. King, L. R.: Undiversion: when and how? J. Urol., 115: 296, 1976. 10. Richie, J.P. and Sacks, S. A.: Complications of urinary undiversion. J. Urol., 117: 362, 1977. 11. Kogan, S. J. and Levitt, S. B.: Bladder evaluation in pediatric patients before undiversion in previously diverted urinary tracts. J. Urol., 118: 443, 1977. 12. Bauer, S. B., Labib, K. B., Dieppa, R. A. and Retik, A. B.: Urodynamic evaluation of boy with myelodysplasia and incontinence. Urology, 10: 354, 1977. 13. McGuire, E. J.: Surgical treatment of neurogenic incontinence. Read at regional subject oriented seminar on controversies in clinical neurourology, Boston, Massachusetts, November 16-18, 1978. 14. Ericsson, N. 0., Hellstrom, B., Nergardh, A. and Rudhe, U.: Micturition urethrocystography in children with myelomeningocele. A radiologic and clinical investigation. Acta Rad. [Diag.] (Stockh.), 11: 321, 1971. 15. Awad, S. A. and Downie, J. W.: Relative contributions of smooth and striated muscles to the canine urethral pressure profile. Brit. J. Urol., 48: 347, 1976. 16. Mandell, J., Lebowitz, R. L., Hallet, M., Khoshbin, S. and Bauer, S. B.: Urethral narrowing in the region of the external sphinchter: radiologic-urodynamic correlations in boys with myelodysplasia. Amer. J. Roentgen., 134:731, 1980. 17. Koff, S. A.: Striated muscle determinants of intraurethral resistance. Invest. Urol., 15: 147, 1977. 18. Hendren, W. H.: Reconstruction of the long diverted urinary tract. Surg. Ann., 8: 335, 1976. 19. Teele, R. L., Lebowitz, R. L. and Colodny, A. H.: Reflux into the unused ureter. J. Urol., 115: 310, 1976.

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20. Hendren, W. H.: Urinary tract refunctionalization after prior diversion in children. Ann. Surg., 180: 494, 1974.

EDITORIAL COMMENT This article proves the feasibility of undiverting certain patients in whom the original cause for diversion was a neurogenic bladder. Furthermore, it emphasizes quite rightly that patients should be studied carefully before embarking on undiversion, perhaps even more than those in whom diversion was for obstructive uropathy or reflux in a non-neurogenic bladder. Although for several years undiversions were done without prior bladder "physiotherapy" or urodynamic testing, we currently use trocar cystostomy, hydrostatic bladder stretching and urodynamic evaluation. In some cases this is used for 2 to 3 weeks, with improved volume and control resulting as compared to initially. Reflux in a diverted bladder can, indeed, disappear after undiversion if it was not present originally and if the anatomy looks reasonably normal endoscopically. However, it probably will not subside if originally present and if "gopher holes" are seen endoscopically. The authors state that it is mandatory that the upper urinary tract appear normal or have only minor but stable architectural derangement. Although that is an ideal state of affairs relatively few of the 95 patients we have undiverted to date would meet those stringent requirements. Undiversion can be accomplished even in relatively poor urinary tracts if the bladder can empty and there is no reflux or obstruction after undiversion. Although the architecture may remain unchanged the patients can be much better off psychologically and also by getting rid of chronic bacilluria so common in diverted urinary tracts. W. Hardy Hendren Department of Urology Massachusetts General Hospital Boston, Massachusetts