INCIDENCE OF URINARY TRACT COMPLICATIONS WITH MYELOMENINGOCELE* A. S. CASS, M.B.B.S. M. LUXENBERG C. F. JOHNSON, P. GLEICH,
M.D.
M.D.
From the Urology Service, Gillette Children’s Hospital, St. Paul, Minnesota
ABSTRACT-Children born with myelomeningocele face the morbidity of urinary tract complications after they have survived the neurologic complications of the first years of life. The incidence of urinary tract complications was evaluated in 258 children before any operation or intermittent catheter management was performed. In 119 children under one year of age the incidence of urina y tract infection of more than 105 colonies was 23 per cent, reflux was 22 per cent, and upper tract dilatation was 6 per cent. In children of all ages the incidence of urinary tract infection of more than lo5 colonies was 26 per cent, reflux 34 per cent, upper tract dilatation 18 per cent, and urinary incontinence 87 per cent. In 11 children with complete denervation of the pelvic floor muscles reflux developed in 2 (18 70) and upper tract dilatation in l(9 TO). In 13 children with slight or moderate denervation of the pelvic floor muscles reflux developed in 5 (38%) and upper tract dilatation in 7 (54 %). The development of upper tract changes with myelomeningocele was mainly related to some innervation of the pelvic floor muscles (including periurethral striated muscle) with incoordinate detrusor/‘hincter activity. These changes mainly occurred in the first two to four years of life.
Urinary complications of infection, reflux, upper tract dilatation, and incontinence cause morbidity to children born with myelomeningocele who have survived the neurologic complications in their first years of life. The incidence of these urinary tract complications was determined in 258 children comprising 219 children managed by observation alone and 39 children managed by Crede expression of the bladder alone. Regular urologic evaluation included urine culture, voiding cystourethrogram, and excretion urography (IVP). Cystometrography and electromyography of the
pelvic floor muscles including periurethral striated muscle were performed in 33 incontinent children.’ The incidence of the urinary tract complications, the age at which they appeared, and the urodynamic factors associated with upper tract deterioration were determined. Material and Methods
*Supported by Medical Education and Research Association, Gillette Children’s Hospital, St. Paul, Minnesota.
From 1951 to 1982, 341 children presented with spina bifida cystica. Eighteen children who died or were lost to follow-up before urologic evaluation were excluded. Of the remaining 323 children, 12 were lost to follow-up, 31 were followed by letter/questionnaires from outside doctors or hospitals, 264 were followed
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TABLE I.
1951-59
1970-82 (13 Yr)
Total
(Yr)
(9 Yr)
1960-69 (10 Yr)
0 7 12 1
29 36 11 10
90 61 35 31
119 104 58 42
-20
86
217
323
Age
Ureteral reflur with observation and Cred&
TABLE III.
Age at initial urologic evaluation (%) (37) (32) (18) (13)
Ureteral Reflux
Initial Evaluation No. (%)
Children having cystograms Unilateral reflux Bilateral reflux Transient reflux
205 . . 30 (15) IO (5) . . . .
During Follow-Up No. (%) 243
. .
22 (9) IO (4) 52 (21)
TABLE II.
Urinary tract infection with observation and Credi
Urinary Tract Infection
Initial Evaluation No. (%)
Children having cultures No. of cultures > lo5 col/ml 10-105 Sterile
222 222
. . . .
60 (27) 69 (31) 93 (42)
During Follow-Up No. (%) 192 962
. . . .
253 (26) 258 (27) 451 (47)
at this hospital, and 16 died during follow-up. Of the 264 followed at our hospital, 219 children were managed by observation alone for an average length of follow-up of 2.8 years, and 39 children were managed by Crede expression of the bladder alone for an average length of follow-up of 4.7 years before management changed to an invasive type, such as intermittent catheterization or urinary diversion. The urologic complications were recorded before any operation or intermittent catheter management was performed. The skeletal sites of the spinal lesions were cervical/cranial in 4 (1.5 % ), thoracic in 8 (3%), thoracolumbar 16 (6%), lumbar 140 (55%), lumbosacral 70 (27%), sacral 19 (7.5 % ), and a double lesion in 1. The lesion was repaired in 242 cases (94%). There were 138 girls and 120 boys. During the earlier years the urologic evaluation consisted of urinalysis, urine culture, excretory urogram (IVP), and blood urea nitrogen (BUN) at yearly intervals. Since 1968 aggressive urologic evaluation has been undertaken consisting of measurement of residual urine, urinalysis, urine culture, voiding cystourethrogram, IVP BUN, and serum creatinine. These determinations were done at six monthly intervals up to the age of five or six years, and then each one to two years thereafter.
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TABLE IV.
Upper tract dilatation with observation and Credk Initial Evaluation No. (%)
Upper Tract Dilatation Children with IVP
230
Unilateral dilatation Bilateral dilatation Transient dilatation
. .
12 (5) 8 (3.5) . . . .
During Follow-Up No. (%) 247
. .
8 (3) 8 (3) 30 (12)
Cystometrography and electromyography of the pelvic floor muscles (levator ani, anal sphincter, periurethral striated muscles) were performed in 33 incontinent children. l Results Age at initial evaluation During the first nine years of the study, 35 per cent of the children were aged under five and 65 per cent were aged five years or more when first seen at the hospital; during the last thirteen years, 70 per cent were aged under five and 30 per cent were aged five years or more (Table I). The children who were five years or more when first seen at the hospital represent a select group, having survived neurologic and early urologic complications of myelomeningocele and were referred to this hospital for orthopedic management in most cases. Urinary tract infection Urinary tract infection with more than lo5 colonies per milliliter was present in 27 per cent of the children on initial evaluation and in 26 per cent of the children during follow-up (Table II). Urine specimens for culture were obtained by sterile urethral catheterization. Antibiotics were not used during this follow-up period except for those children in whom ureteral reflux occurred.
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TABLE V.
myelomeningocele
Evaluation
Initial evaluation in 119 children aged under one year
Number
Urine culture Sterile 10-105 2 105 Not done Reflux Not present Unilateral Bilateral CGM not done U. T. dilatation Not present Unilateral IVP not done
Per cent
47 39 25 8
42 35 23
81 15 7 16
78.5 14.5 7
104 6 9
94.5
5.5
cent of the cultures in children with reflux on initial evaluation and in 38 per cent of the cultures in children with reflux during follow-up. Upper tract dilatation Dilatation of the upper tracts occurred in 8.5 per cent of the children on initial evaluation and in 18 per cent during follow-up comprising 6 per cent with persistent dilatation and 12 per cent with transient dilatation (Table IV). Zncontinence Incontinence was not diagnosed until the child was five years old. If control was not present by this age it did not develop subsequently. Of the 153 children who were five years or more 133 (87%) were incontinent and only 20 (13 % ) were continent.
Ureteral reflux Reflux was present in 20 per cent of the children on initial evaluation and in 34 per cent during follow-up comprising 13 per cent with persistent reflux and 21 per cent with transient reflux (Table III). Urine cultures yielded infection with more than lo5 colonies/ml in 47 per
TABLE VI.
in children
Urinary complications year of age
under one
The urinary complications found on initial evaluation in children under one year of age are listed in Table V. Urinary tract infection with more than lo5 colonies/ml was present in 23 per
Urinary tract complications
with myelomeningocele
Pellman 1965 Gulp 1970 Graf 1964 Present iStudy
376
6/104
69 22/103
22%
25/111
L
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23%
cent of the children, ureteral reflux in 21.5 per cent, and upper tract dilatation in 5.5 per cent. In those children who have been followed since they were less than one year old the majority of upper tract changes occurred in the first two to four years of life and ureteral reflux developed earlier than upper tract dilatation (Table VI).
reflux developed in 1 (25 % ) and upper tract dilatation in 3 (75%). Comment While some authors believe that upper tract damage with myelomeningocele is present at birth from primary congenital ureterovesical abnormality,2%3most believe that the upper tract damage is secondary to progressive bladder dysfunction and/or urinary tract infection.4-6.8 Our study showed that the majority of upper tract changes occurred in the first two to four years of life. Pompino et aZ.’reviewed the case histories of children with neurogenic bladders due to myelomeningocele and had follow-up data on 91 children to five years and on 40 children to ten years. During the first five years of life there were increasing pathologic findings in the upper urinary tract, namely scarring and dilatation of the upper urinary tracts. Few children had development of scarring between five and ten years of age.7 A review of published studies of the urinary complications of myelomeningocele shows the overall incidence of upper tract dilatation is from 18 to 45 per cent, reflux 34 to 61 per cent,
Urinary complications correlated with urodynamic findings Of 11 children with hyperreflexic detrusor function reflux developed in 4 (36 % ) and upper tract dilatation in 3 (27 %). Of 21 children with areflexic detrusor function (progressive increase in pressure with increasing volume) reflux developed in 4 (19%) and upper tract dilatation in 8 (38%). Complete denervation of the pelvic floor muscles was present in 11 children of whom reflux developed in 2 (18 %) and upper tract dilatation in 1 (9%). Severe denervation was present in 9, and reflux developed in 3 (33 %) and upper tract dilatation in 2 (22 %). Moderate denervation was present in 9: reflux developed in 4 (44 % ) and upper tract dilatation in 4 (44 % ). Slight denervation was present in 4: TABLE VI.
Continued
I
490 33%
599
459
619
180
349
639
269
87% ,
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377
urinary tract infection 26 to 60 per cent, and incontinence 63 to 95 per cent (Table VI), Detrusor hypertonia has been found to be associated with upper urinary tract deterioration comprising dilatation and/or reflux, and this can occur in older childrenzl as well as younger childrenz2 McGuire et aLz2 followed the clinical progress and urodynamic evaluations of 42 myelodysplastic patients for a mean of 7.1 years. The 20 children with a low urethral closure pressure showed incontinence and a low incidence of reflux and upper tract dilatation, The 22 children with a high urethral closure pressure showed a high incidence of large residual urine, reflux, and upper tract dilatation. Diokno, Kass, and Lapides evaluated 21 myelomeningocele patients urodynamically and found no patient with absent sphincter activity or coordinated detrusor/sphincter activity had urinary tract infection or upper tract changes, while patients who lacked coordinated detrusor/sphincter activity demonstrated infection and/or renal damage. Our study confirmed that children with complete denervation of the pelvic floor muscles, including periurethral striated muscle, had a low incidence of reflux and/or upper tract dilatation while the children with some innervation of the pelvic floor muscles had a high incidence of reflux and/or upper tract dilatation. However, there was no significant difference in the upper tract deterioration in children with hyperreflexic.detrusor function or areflexic detrusor function. The incidence of the urinary complications of infection, reflux, upper tract dilatation, and incontinence was determined in 258 children with myelomeningocele before any operation or intermittent catheter management was performed. Urinaryftract infection with more than lo5 colonies occurred in 26 per cent, reflux in 34 per cent, upper tract dilatation in 18 per cent, and urinary incontinence in 87 per cent. The upper tract changes mainly occurred in the first two to four years of life and were mainly associated with some innervation of the pelvic floor muscles (including periurethral striated muscle) with incoordinate detrusor/sphincter activity.
1. Godec CJ, and Cass AS: Electrical stimulation for incontinence in myelomeningocele, J Ural 120: 729 (1978). 2. Strafford SJ, et al: Hydronephrosis in the asymptomatic neonate with myelodysplasia, ibid 129: 340 (1983). 3. Smith ED: Spina Bifida and the Total Care of Spinal Myelomeningocele, Springfield, Illinois, Charles C Thomas, 1965. 4. Magnus R: Vesicoureteral reflux in babies with myelomeningocele, J Urol 114: 122 (1975). 5. Levitt SB, and Sandler HJ: The absence of vesicoureteral reflux in the neonate with myelodysplasia, ibid 114: 118 (1975). 6. Gaum LD, et al: Radiologic investigation of the urinary tract in the neonate with myelomeningocele, ibid 127: 510 (1982). 7. Pompino HJ, Devens K, Kubler U, and Morales W: Longterm results of obstructive neurogenic bladder in children with myelomeningocele, Develop Med Child Nemo1 18: 89 (1976). 8. Madersbacher H: Development of the upper urinary tract in myelomeningocele and consequent urological care, Int Urol Nephro17: 13 (1975). 9. Carlson HE: Urologic problems in meningomyelocele, J Urol 95: 245 (1966). 10. Mebust WK, Foret JC, and Valk WL: Fifteen years of experience with urinary diversion in myelomeningocele patients, ibid 101: 177 (1969). 11. Cooper DG: Urinary tract infection in children with myelomenmgocele, Arch Dis Child 42: 521 (1967). 12. Tohennine PW. DeVault WD Ir. and Paauin AT Ir: The urinary tract and spina bifida cystica: Virginia Med M&hly 91: 189 (1964). 13. Spellman RM, and Kickham CJE: Management of the neurogenic bladder in spina bifida, J Urol 88: 243 (1962). 14. Rose JF: What happens to the child with myelomeningocele? ibid 97: 625 (1962). 15. Pellman C: The neurogenic bladder in children with congenital malformations of the spine: a study of 61 patients, ibid 93: 472 (1965). 16. Culp DA, Bekhrad A, and Flocks RH: Urological management of the meningomyelocele patient, JAMA 213: 753 (1970). 17. Graf RA, Smith JH, Flocks RH, and Van Epps EF: Urinary tract changes associated with spina bifida and myelomeningocele, AJR 92: 255 (1964). 18. Shulman K, and Ames MD: Intensive treatment of fifty children born with myelomeningocele, NY State J Med 68: 2656 (1968). 19. E&stein HB: Urinary control in children with myelomeningocele, Br J Urol40: 191 (1968). 20. Stark G: The pathophysiology of the bladder in myelomeningocele and its correlation with-the neurological picture; Develop Med Child Neurol (SUPP~) 16: 76 (1968). 2i. McGuire EJ, WoodsideJR, and Bdrden’TA: Upper urinary tract deterioration in patients with myelodysplasia and detrusor hypertonia: a follow-up study, J Urol 129: 823 (1983). 22. McGuire EJ, Woodside JR, Borden TA, and Weiss RM: Prognostic value of urodynamic testing in myelodysplastic patients, ibid 126: 205 (1981). 23. Diokno AC, Kass E, and Lapides J: New approach to myelodysplasia, ibid 116: 771 (1976).
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UROLOGY
Department of Urology St. Paul-Ramsey Medical Center St. Paul,
Minnesota 55101 (DR. CASS)
References
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