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Renal function and urinary/faecal incontinence - Long-term follow-up in spinal dysraphism

Bettina Jørgensen¹, Lars Henning Olsen², Troels Munch Jørgensen²,
¹Institute of Clinical Medicine, Aarhus University Hospital - Skejby, Denmark
²Department of Urology, Section of Pediatric Urology, Aarhus University Hospital - Skejby, Denmark

 

Correspondence

Bettina Jørgensen
Department of Nephrology,
Institute of Clinical Medicine
Aarhus University Hospital, Skejby, Denmark
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

 

Abstract

Objective: A cohort of adolescents with congenital spinal malformations followed at our department were evaluated in terms of renal and functional outcome. All patients went through same follow up and algorithm of intervention during childhood. None of the patients were not treated profylactically besides from antibiotics.

Material and Methods: Sixty children (31 males, 29 females), born 1989-1995, were evaluated from June 2006 to may 2007. Ultrasound of the bladder and the upper urinary tract was performed in all patients. Surgical and pharmacological intervention, bowel function, faecal and urinary continence were registered. Renal function was assessed by 51Cr-EDTA and/or MAG-3 scintigraphy in patients who earlier experienced renal affection or were presenting with deteriorated bladder function.

Results: Two children died during follow up. A total of 47 urological procedures were performed in 23 patients. Most frequently conducted were appendicovesicostomy (n=11) and autoaugmentation (n=11). Fourteen patients underwent untethering procedures, resulting in improved bladder function in 5 patients, 9 were unaltered. Five patients had a moderate and 2 a severe decrease in GFR. Eighty-one percent were performing CIC, starting at median age 3.6 years. Anticholinergics were used in 50%. Sixty-two % were totally urine continent, 22% used incontinence pads in case of leakage and 16% were urine incontinent. Two patients had Malone procedures, 29% were using trans-anal-irrigation. Eighty-three percent were totally faecal continent.

Conclusion: Close follow up and appropriate intervention has improved renal function in children with congenital spinal malformation but renal deterioration remains at risk. Total faecal and urinary continence could be achieved in 81% and 62%, respectively.

K ey words: neurogenic bladder, renal function, continence

 

 

Introduction

In the recent years prophylactic treatment has replaced watchful waiting in managing the neurogenic bladder in children with congenital spinal malformations. Several studies show that early prophylactic treatment with clean intermittent catheterization (CIC) and anticholinergic medication may prevent the need for bladder augmentation, improve bladder function, and preserve kidney function [1-4]. Contrary to this, other studies demonstrate that the selective use of urodynamics in myelodysplastic children equals the outcome of renal function compared to a more prophylactic intervention regime [5,6]. As a third approach has also been suggested: extensive observation with ultrasound (US) of the kidneys and the urinary tract and prompt intervention in case of signs of upper urinary tract deterioration (UUTD) [7]. Although very different approaches these varying strategies do not seem to exhibit great differences, with the reservation that outcome, side effects and expenses are difficult to compare.

Since McGuire et al in 1981 demonstrated a correlation between urethral leakpoint pressure, vesicourethral reflux, and ureteral dilatation, urodynamics has been utilised with varying frequencies among different centres. Still there is only little consensus according to indications, methodology, interpretation, and the reliability of the urodynamic investigation.

Changes in the neurogenic bladder dysfunction secondary to tethering of the spinal cord make it difficult, and probably impossible, to predict long term kidney function based on a few cystometries. Special attention to bladder function is required during the first 6 years of life and during puberty as changes are most pronounced in these periods [9,10].

Later in life, another important goal and crucial for the child’s self-esteem, is to achieve faecal and urinary continence, Special efforts should be made prior to starting school, and no later than puberty if continence is not attained otherwise.

The purpose of this study was to follow up a group of patients at the age of 12-17 years after they have been subjected to a conservative treatment algorithm, where intervention was carried out only in case of risk of renal deterioration. In 1989 we changed the paradigm in managing children with congenital spinal malformations. Extensive evaluation was carried out at 6-9 month of age to ensure the phase with spinal shock after closuring procedure had ceased. The population followed and managed from infancy was evaluated at their latest follow up for continence and renal function. Furthermore we reviewed patient chart for interventions, including performed surgery, during the follow up period.

Material and methods

Population: Sixty-five infants, with spinal dysraphia born between 1989 and 1995 were followed and managed at the department from infancy. At the age of 6-9 months evaluation including ultrasound (US), voiding cystourethrography (VCUG), conventional artificial fill cystometry (CMG), natural fill urodynamics (NFU), chromiumethylenediaminetetraacetic acid (51Cr-EDTA) clearance and renography, were performed. All examinations were summarized in a risk score and an algorithm of decided follow up and intervention (fig. 1). Subsequently the patients were followed with urine culture monthly, annual or biannual US of the kidneys and urinary tract, and cystometry. In case of dilatation of the upper urinary tract (UUT) VCUG was performed. Treatment strategy was determined by repeated investigations with cystometry at appropriate intervals. Prophylactic CIC and anticholinergic medication were not initiated routinely, but all new patients received prophylactic antibiotics. When signs of impaired bladder emptying, bladder overactivity or increased detrusor pressure appeared, non-surgical treatment (CIC, anticholinergics, antibiotics) was instituted. Surgical reconstruction was indicated when non-surgical treatment had failed. At the age of 7-8 years children with faecal or urine incontinence were examined with uro- or colonic dynamic examinations in the attempt to release the child from diapers.

fig 1 flowcharts

Figure 1: Flowchart of risk scoring and follow-up/treatment algorithm

From June 2006 to May 2007 we evaluated all patients followed at the Outpatient Clinic and information about CIC, pharmacological (anticholinergic) treatment, and urinary continence were registered. Continence were categorized as “total continence” (no use of incontinence pads), “occasional incontinent” (patients needing of incontinence pads), and “incontinent” (wetting regular diapers). Patients were interviewed about bowel function, use of enemas, trans-anal irrigation or laxatives, and faecal continence which was categorized as urinary continence.

Deteriorated renal function was defined as a decrease in renal functional share and/or occurrence of renal scarring at renal scintigraphy. In case of deteriorated bladder function, earlier affected renal function, or newly diagnosed UUT dilatation, total renal function was assessed by 51Cr-EDTA clearance and from a MAG-3 or dimercaptosuccinic acid (DMSA) renogram.

Review of the patient charts, risk factors of potential UUTD revealed: reflux, numbers of febrile urinary tract infections (UTI), and urinary tract dilatation. CIC, pharmacological and surgical intervention (urological and untethering procedures) were registered. Data were not normal distributed and all data are presented as median (range). Mann-Whitney Rank Sum test was used to compare groups. A value of P < 0.05 was considered statistically significant.

2 JPSS 8 1 2010-2-1

Figure 2: Surgical procedures performed during the follow up period

 

Results

Sixty-five patients born, 1989-1995, were managed by the new regimen. Five patients were lost for follow up: 2 moved abroad, 2 for unknown reasons and 1 declined further follow up. Of the 60 patients included, 55 patients were born with open myelomeningocele, 3 sacral agenesis (2 with imperforate anuses), 2 having caudal regressions syndrome. Two females died during the follow up period: One at the age of 3 years after a rapidly progressing renal failure. Attempts to treat were desisted, since the child was severely retarded and had no motor skills. A 5 year old girl died from peritonitis after spontaneous rupture of the sigmoid, presumably due to massive constipation.

The remaining 58 patients had a median follow up of 16.3 years (12.0-17.8). Twenty-nine were girls, 31 were boys. At early evaluation (6-9 months), 42% (n=25) were categorized as high risk. The label low risk were categorized 37 % (n=22). Normal (non-neurogenic) bladder function was found in 21% (n=13).

CIC and pharmacologic treatment

Nine of the patients were already performing CIC at 6-9 months, and 16 started CIC after the examinations. At follow up, 81 % (n=47) were performing CIC. Median age at start was 3.6 years (0.1-12.2). Anticholinergic treatment was used in 50% (n=29 (12 intra-vesical; 17 per oral)), starting at a median age of 8.6 years (1.4-15.6). Botulinium-toxin-A injections replaced oxybutynin in 3 patients: 2 males with low patient compliance and 1 female with severe side-effects from oxybutynin. There was no gender difference in the number of treated patients (CIC: n=22(♀); n=25(♂), anticholinergic treatment: n=14(♀); n=15(♂)) or age at start of treatment (start age CIC: 3.5 yrs.(♀); 3.6 yrs.(♂), (p=0.69), start age for anticholinergic treatment: 8.6 yrs. (♀); 9.6 yrs.(♂), (p=0.59)).

Urine continence and bowel function

Table 1 presents the distribution of urinary and faecal continence. In the non-pharmacologically treated group 4 of the 18 patients were continent after autoaugmentation and/or sling procedure. All patients experienced problems with constipation during childhood. At follow up, 67% (n=39) were using some kind of purgative. Forty-seven surgical procedures were performed in 23 patients. Median age for surgery was 8.2 years (range 1.2-16.8). Out of 17 patients (10 females, 7 males) with reflux found at VCUG, 4 patients had reflux surgery performed. The remainder resolved spontaneously on CIC and anticholoinergics. Two patients living for longer periods in third world countries without any possibility of performing CIC had temporary vesicocutaneostomy. Both were closed on return to follow up at our clinic.

Number of patients

Urine Continence

Treatment

Complete

Pharmacological

Incontinent

Total

No Treatment

14

2

5

21

Pharmacological

9

8

2

19

Surgical

4

2

1

7

Pharmacological and Surgical

9

1

1

11

Total

36

13

9

58

Fecal Continence

No Treatment

17

1

1

19

Irrigation

17

3

1

21

Enemas

7

3

0

10

Laxatives

5

1

0

6

Malone

2

0

0

2

Total

48

8

2

58

Table 1: Urinary and faecal continence


Renal function

Fifty-eight patients had 115 renal units; one male had right sided renal agenesis. Five patients had decreased single kidney function at the first scintigraphy (30%, 32%, 25%, 24%, and 9%). All 5 had reflux during infancy. Three underwent reflux surgery, 2 of them improved their single kidney function (30%→36% and 24%→34%). Three patients developed decreased single kidney function during follow up: 2 with reflux and 1 after multiple UTI´s. Seven patients experienced a decrease in total renal function. Two patients, both females, had a significantly decreased GFR (38 and 55 ml/min/1.73 m2, respectively): one female with poor family compliance had decreased renal function at the age of 13.

Discussion

We found, that renal function was well preserved in the vast majority of the patients. None of the patients had end stage renal disease at adolescence, and significantly decreased renal function was only detected in 2 patients. Urinary- and faecal continence was achievable in the majority of the patients when ensuring, that the patients maintained a steady purgative regimen. Since the introduction of CIC in the 1970´s renal morbidity and mortality decreased significantly [11]. Development of surgical techniques and introduction of anticholinergics during the last 2 decades has further reduced the risk of UUT deterioration, and has also improved renal outcome [12,13]. Throughout the 1980´s and 90´s several studies have concluded, that close follow up and early intervention diminish UUT deterioration and improve renal outcome [1-3,5]. The previous expectant approach, in which repeated urodynamics was the mainstay, has in many centres been replaced by a more proactive intervention, where CIC and anticholinergics are initiated soon after birth [4]. Instead of prophylactic CIC and anticholinergics we followed the bladder function closely with frequently repeated cystometries and US. Patients were not assigned to CIC, unless they were unable to empty the bladder in one or two attempts. In our material 81% of the population ended up performing CIC, which is equivalent to the 85% described in other studies [14,15]. CIC was started immediately in case of UUT impairment (hydronephrosis, renal pelvis dilatation, reflux). It could be argued that the need for augmentation could be increased, since we did not treat prophylactically with CIC and anticholinergics, as suggested by others [1,3,4]. In this population, 19% had augmentation performed which is fully comparable to the group treated profylactically by Kaefer et al [1].

In a recent study it has been described how approximately half of the children with myelomeningocele achieved continence spontaneously during puberty [16]. This may favour a more conservative approach to surgery in childhood, but also induce a substantial delay in obtaining continence, since it is unpredictable which patients will develop continence spontaneously. Urinary incontinence is easier to conceal than faecal incontinence and social acceptance and discomfort by being faecal incontinent should not be underrated. Improved purgative actions (laxatives, transanal irrigation, Malone antegrade continence enema) have improved quality of life and self-esteem for these children [17]. It is our experience that urinary continence is improved significantly when constipation has been treated thoroughly. This circumstance is probably the main reason for the low percentage having urine incontinence. Specialized nurses are educating patients and parents in the importance of purgative actions in the settings of our clinic. It is evident, that patients who are not treated and are not completely continent should receive treatment for faecal incontinence. Despite this, some patients refuse all efforts to initiate treatment. Seven of 38 (18%) patients receiving anti-constipating treatment experience “occasional faecal incontinence”. This result is satisfying compared to other recent studies describing incomplete bowel control in 89, 35 and 34%, respectively [14,15,18].

Conclusion

Renal deterioration remains a risk, but with close follow up and pharmacologic and surgical intervention it is possible to reduce renal deterioration. Total faecal and urinary continence can be achieved in 81% and 62%, respective.

 

 

 

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