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Keywords:

  • closed spinal dysraphism;
  • spina bifida occulta;
  • incontinence;
  • bladder function;
  • tethered cord;
  • neurogenic bladder

Abstract

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Conclusions
  8. Funding
  9. Conflict of Interest
  10. References
  11. Appendix 1 Search Strategy for the Present Review
  12. Supporting Information

Objectives

  • To describe the long-term upper (UUT) and lower urinary tract (LUT) outcomes in patients with closed spinal dysraphism (CSD).
  • CSD differs from open spinal dysraphism (OSD) by its long asymptomatic course and consequent later diagnosis. The outcome of UUT and LUT function in adults with CSD is relatively unknown.

Patients and Methods

  • A systematic review was performed following the standards of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. An extensive search was made of PubMed and EMBASE.
  • Included were papers on adults with any form of primary CSD that described bladder and/or kidney function. Only English and Dutch language papers were included. Excluded were papers on patients aged <18 years and patients with secondary tethered cord following childhood OSD repair. International Continence Society terminology was used to describe LUT outcomes.

Results

  • Eventually, only seven studies (90 patients) were included and none of these described renal outcomes. Five of the seven papers were on outcomes after untethering surgery during adulthood.
  • Urological complaints were present in 54/79 (68.4%) patients.
  • Urodynamic studies (48 patients available) revealed detrusor underactivity in 46.5% of the evaluated cases, detrusor overactivity in 32.6% and normal findings in 16.3% of the studied patients.
  • Symptomatic improvement after surgery for tethered cord occurred in 33.3–90.0%, depending on the subgroup studied. Urodynamic improvement rates ranged from 11.1% to 54.5% (but based on three studies with only 24 patients). Success of surgery depended on the time between onset of symptoms and operation, and (sometimes) type of lesion.

Conclusions

  • Few data are available on long-term urological outcomes in adult patients with CSD.
  • More extensive research on follow-up, including the functional status of the UUT, is recommended.
  • Based upon the little evidence available, we think life-long follow-up (from birth into adulthood) of those with CSD and neurogenic bladder is advisable.

Introduction

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Conclusions
  8. Funding
  9. Conflict of Interest
  10. References
  11. Appendix 1 Search Strategy for the Present Review
  12. Supporting Information

Closed spinal dysraphism (CSD; also [obsolete]: occult spina bifida) is a collective term for a group of heterogeneous congenital disorders of the spinal cord [1]. Dysraphia is derived from the Greek term ‘failure to fuse’ and refers to every fusion defect of the vertebral arch. Many classifications of CSD have been proposed, but one of the most usable is that of Tortori-Donati et al. [2] (Table 1).

Table 1. Radiological classification of CSD according to Tortori-Donati et al. [2]

With a subcutaneous mass:

  • Lumbosacral:
    • ● Lipoma with dural defect:
    • Lipomyelomeningocele
    • Lipomyeloschisis
    • ● Terminal myelocystocele
    • ● Meningocele
  • Cervical:
    • ● Cervical myelocystocele
    • ● Cervical myelomeningocele
    • ● Meningocele

Without a subcutaneous mass:

  • Simple dysraphic states:
    • ● Posterior spina bifida
    • ● Intradural and intramedullary lipoma
    • ● Filum terminale lipoma
    • ● Tight filum terminale
    • ● The abnormally long spinal cord
    • ● Persistent terminal ventricle
  • Complex dysraphic states:
    • ● Dorsal enteric fistula
    • ● Neurenteric cysts
    • ● Split cord malformation (diastematomyelia and diplomyelia)
    • ● Dermal sinus
    • ● Caudal regression syndrome
    • ● Segmental spinal dysgenesis

In contrast with open types of spina bifida (open spinal dysraphism, OSD), CSD may go unnoticed for years. In some cases CSD is accompanied by a subcutaneous mass-like lipomyelomeningocele and cervical myelocystocele. Clinical symptoms are not always manifest, and the fusion defect of the vertebral arch may be an incidental finding. In fact, unfused vertebral arches are very common in the open population, with a reported incidence of 17.3–23.0% in large series of patients undergoing plain abdominal X-rays [3, 4].

Symptoms may arise due to an associated tethered cord syndrome (TCS). This syndrome, caused by traction and subsequent oxygenation failure of the spinal cord, was first described in adults by Pang et al. [5] in 1982. If CSD becomes clinically manifest, complaints usually consist of pain (back and legs), gait difficulties, bowel and urinary tract problems. The exact number of patients developing a neurogenic bladder is unknown, but we can estimate from larger cohorts with both patients with CSD and OSD that 20.7% [6] to 24.3% [7]of all younger patients (aged 0–25 years) attending specialised SD-clinics have CSD.

It is widely assumed that CSD gives less urological problems (incontinence and renal damage) than OSD. Torre et al. [8] reported on the long-term consequences of caudal regression syndrome (CRS, a subset of CSD) on both bladder and kidney functioning. In a group of 398 patients (mainly children, few adults were included) they found that impaired renal function was more prevalent in the CRS group than among OSD patients; also, incontinence was more often present in the CRS group. In that study, most patients were aged ≤18 years (and those who were older were not separately analysable). In patients with OSD, bladder behaviour and renal function may alter during adolescence, influenced by factors such as secondary tethered cord. In patients with OSD, bladder behaviour and renal function may alter during adolescence, influenced by factors such as secondary tethered cord and possibly oestrogenisation of the urethra (in women) and prostate growth (in men), although controversy exists about this hypothesis [9, 10]. Far less is known about the long-term fate of the urinary tract in patients with CSD.

Surgical treatment of TCS in adulthood may influence bladder function. An extensive review by Aufschnaiter et al. [11] on outcome of adult TCS describes outcomes with regard to bladder functioning only very briefly. According to that review, ‘sphincter problems’ improved after untethering in 45.6% of the patients. A more focused review with respect to urological outcome is required.

CSD differs from open types of spina dysraphism in that it is mostly diagnosed at a later stage than OSD. One could assume on the one hand that the consequences (also for the urinary tract) are less severe than in OSD or, on the other, that they are more serious because of late diagnosis and late start of therapy. One study (children only) showed that CSD might not be as indolent as often assumed, with 7.5% of children with end-stage renal disease in a cohort of 65 children followed for 2–14 years [12], compared with a mere 1.3% in a recent review on OSD [13].

The aim of the present systematic review was to address the following questions: (i) What are the outcomes of neurogenic lower urinary tract (LUT) dysfunction for different types of CSD (i.e. primary tethered cord, lipomatous filum terminale, intradural lipoma, lipomyelomeningocele) for LUT and upper urinary tract (UUT) function in patients aged >18 years? (ii) What are the effects of surgery for primary tethering of the spinal cord in patients aged >18 years on bladder and kidney function?

Methods

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Conclusions
  8. Funding
  9. Conflict of Interest
  10. References
  11. Appendix 1 Search Strategy for the Present Review
  12. Supporting Information

Eligibility Criteria

For this review, studies were included when patients had CSD, were aged ≥18 years and when details on urological outcomes were presented in a standardised manner.

Data Sources

The literature search was performed on 17 July 2012 using PubMed/Medline and Embase; there was no restriction regarding the year of publication.

Search

Synonyms for various types of CSD (i.e. spina bifida occulta, spinal lipoma, caudal regression syndrome) were combined with synonyms for bladder function and kidney function, and also with terms for diagnostic methods to assess these conditions, i.e. urodynamic study (UDS), ultrasound and renal scintigraphy. Because no other limits were applied, the search was kept as sensitive as possible. Details on the search (including search terms) are shown in Appendix 1.

Study Selection

Results were exported to RefWorks® Version 2.0. Duplicates were deleted. Title screening was done by one author (P.V.), and two authors (independently of each other) screened the abstracts (P.V. and L.dK.). Screening results were compared and discrepancies were solved by discussion. Full-text screening (critical appraisal of validity and relevance) was subsequently performed in a comparable fashion by both P.V. and L.dK. Only English and Dutch language papers were included.

Exclusion criteria were spinal cord lesions other than CSD (i.e. traumatic spinal cord lesions, myelomeningocele, secondary spinal cord tethering after myelomeningocele), and patients aged <18 years (unless data were reported separately). Studies not describing either UUT or LUT function were excluded. Studies describing a mixed neurological population in which patients with CSD could not be properly separated were also excluded. Case reports describing fewer than five patients were excluded. Both studies with operated and non-surgically managed patients were considered. If patients underwent surgery during childhood but were adult during the time of study, they were included.

Data Collection Process and Data Items

For all studies the following data were extracted: number of patients, age (range), the treatment protocol followed, type/level of the lesion, method of bladder emptying, previous surgery of the kidneys and LUT, renal functioning, urodynamic parameters (if provided), VUR, and use of antimuscarinics/α-antagonists (if data on the latter were provided).

Dryness or ‘complete continence’ was defined as completely dry both day and night with no need to wear pads. For LUT outcomes (both LUTS and urodynamic outcomes), the 2002 ICS Standardisation of Terminology of the Lower Urinary Tract were used to standardise outcomes [14]. Parameters of filling cystometry (if presented in the study) that were analysed were bladder capacity (mL), detrusor overactivity (DO) or detrusor underactivity (DU), compliance and incontinence. Also, if possible, details on sensation during the filling phase of cystometry and the presence of detrusor-sphincter dyssynergia (DSD) were noted. Urodynamic improvement after surgery was defined as the disappearance of DSD, DO or any lowering of detrusor pressures. Improvement of compliance was also considered to be improvement. For renal functioning, papers were screened for reporting of GFR, ultrasonography of the UUT and nuclear studies of the kidneys.

Summary Measures and Synthesis of Results

Means were primarily used to report outcomes in the various groups (i.e. different neurological level, treatments, UDS parameters); differences in means (for treatment outcomes) were used. Harmonisation of results according to the above-mentioned standards (Good Urodynamic Practice) was done as much as possible to enable pooling of results. Means were calculated for the pooled study results.

Results

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Conclusions
  8. Funding
  9. Conflict of Interest
  10. References
  11. Appendix 1 Search Strategy for the Present Review
  12. Supporting Information

After applying all inclusion/exclusion criteria, seven studies were eligible for inclusion [15-21]: five studies on urological outcomes after untethering in adults with primary CSD [15, 17, 19-21], one on adult patients before surgery [18], and one long-term follow-up after surgery in childhood [16]. Figure 1 shows the selection process. All were retrospective cohorts and case series, and are discussed below. Outcomes are also shown in Table 2 [15-21].

figure

Figure 1. Search strategy and selection of studies for the present review (n = 7).

Download figure to PowerPoint

Table 2. Study outcomes
ReferenceNType of lesions (n)Mean (range) age at baseline*, yearsMean (range) postoperative follow-up, monthsLUTS, n/N (%)LUTS at baseline, (n)Postoperative urological symptomsPreoperative/baseline UDS, (n or n/N)Postoperative UDS, (n or n/N)
  1. DM, diastematomyelia; DS, dermal sinus; EL, extradural lipoma; FT, thickened/fatty filum terminale; IL, intradural lipoma; LMMC, lipomyelomeningocele; LSL, lumbosacral lipoma; MC, meningocele; MMC, myelomeningocele; N/A, not available; OS, open sphincter; PTC, primary tethered cord; PVR, post-void residual; SCM, split cord malformation; SM, syringomyelia; VS, voiding symptoms. *Baseline evaluation is equal to the preoperative situation in studies describing surgery (all but Sakikabara et al. [18]); in the case of Sakikabara et al., this should be read as ‘at time of study’, as these patients had been in follow-up longer but childhood characteristics are not given in this study. The age given of the study by Fukui et al. [16] is in fact the age at time of follow-up, as all children were operated during childhood. Follow-up in entire cohort. Only pre-surgical evaluation was given in this study; surgery was later performed in all patients but results were not described. §Although the entire study included 22 patients, of the 12 patients not operated the urological complaints of those with primary and secondary tethered cords could not be separated. Data from the study of Fukui et al. are included in this column, although they were operated in childhood. **Both nocturia and daytime frequency.

Kondo et al. (1986) [21]15

Own classification grades I–IV:

I. Tight filum terminale, not thickened (3)

II. FT, conus at normal level (6)

III. FT, conus at lower level (1)

IV. Conus trapped at bottom dural sac by lipoma or FT (6)

32 (16–66)20 (3–54)14/15

VS (6)

Daytime frequency (5)

Urinary incontinence (5)

Urgency (2)

PVR (5)

N/A

DU (11)

DO (1)

PVR (5)

Urgency (1)

DU and DO (2)

Normal UDS (1)

11 had surgery + UDS:

Improvement in 6/11:

DU [RIGHTWARDS ARROW] normal bladder (3)

DO [RIGHTWARDS ARROW] normal bladder (1)

Mixed [RIGHTWARDS ARROW] DU (1)

Resolution of urgency (1)

Satar et al. (1995) [19]6

MC (1)

LMMC (1)

Primary tethered cord (2)

Not specified/unclear (2)

30.3 (22–46)

(3–84)

mean N/A

3/6

Incontinence (2)

Urinary retention (1)

N/A

DU + DSD (2)

DU + open sphincter (1)

Normal UDS (2)

DO + normal sphincter (1)

4 had surgery + UDS:

Improved in 2/4:

DU + DSD [RIGHTWARDS ARROW] normal (1)

DU + DSD [RIGHTWARDS ARROW] DO + OS (1)

DU + OS [RIGHTWARDS ARROW] DU + improved sphincter (1)

Normal UDS [RIGHTWARDS ARROW] normal detrusor + DSD (1)

Giddens et al. (1999) [17]13

DM (4);

LMMC (1);

Epithelialised MMC (1)

PTC (2)

FT (2)

IL (2)

EL (2)

LSL (1)

44.0 (20–61)

N/A

(12.5)

N/AN/AN/A

UDS available in 12/13:

Normal UDS (2/12);

DO (7/12);

DO, [DOWNWARDS ARROW]sensation (1/12);

DO + crede (1/12);

DO + [DOWNWARDS ARROW]compliance (1/12)

Improvement (DO [RIGHTWARDS ARROW] normal UDS) in

1/9 evaluated patients.

Sakakibara et al. (2003) [18]5Not specified28.2 (19–41)N/A5/5

Nocturia (2)

Daytime frequency (3)

Urgency (2)

Incontinence (3)

VS (3)

Urgency and PVR (2)

N/A

Poor compliance (2)

DSD (1)

N/A
Haro et al. (2004) [20]10According to the Kondo classification (see above); cannot be determined for adult patients with CSD separately [21]43.9 (18–66)N/A10/10

Daytime frequency (3)

Hesistancy and slow stream (2)

Incontinence (2)

PVR (2)

Slow stream (1)

Improvement: 6/10

<5-year wait before surgery: 5/7

≥5-year wait before surgery: 1/3

Normal detrusor (2);

DU (6);

DO (2)

N/A
Duz et al. (2008) [15]8§

DM (1)

DS (3)

SCM (2)

SM (1)

LMMC (4)

20.8 (19–24)9.6 (6–144)4/8Urgency (4)

Improvement: 2/4

Urgency (2)

N/AN/A
Fukui et al. (2011) [16]33

FT (10)

CEAS (23)

35.7 (25–77)276 (240–348)18/33 (54.5)

TFT:

Incontinence (0)

VS (1)

Both (0)

CEAS:

Incontinence (7)

VS (3)

Both (7)

Improvement per group (compared with childhood baseline):

FT: 9/10

CEAS: 10/23 (43.5%)

N/AN/A
TOTAL90Cannot be pooled; different definitions of lesions used.35.8 (16–66)(3–348)

All:

54/79 (68.4)

Excluding Fukui et al.:

36/46 (78.3)

Incontinence: 26/79 (32.9%)

VS: 23/79 (29.1%)

Urgency: 10/79 (12.7%)

Frequency**: 5/79 (6.3%)

Urinary retention: 3/79 (3.8%)

Other: 18/79 (22.8%)

Improvement 27/47 (57.4%)

DO: 14/43 (32.6%)

DU: 20/43 (46.5%)

Normal UDS: 7/43 (16.3%)

DSD: 3/43 (6.9%)

Low compliance: 2/43 (4.6%)

Other: 8/43 (18.6%)

Improvement 9/24 (37.5%)

All studies together included 90 patients. Of the seven studies, five (with 52 patients) described surgery of CSD during adulthood with a mean age at surgery of 35.8 years. One study described long-term outcomes of surgery for CSD during childhood [16]; the mean age at surgery in this study was 12.7 years. Findings at physical (neurological) examination were only properly reported by one author [19]. Five studies described LUTS at baseline (before surgery), whereas five studies described baseline/preoperative urodynamic findings (Table 2).

One study briefly assessed the presence of VUR and impaired renal functioning, without specifying any further [21]. No GFR or outcomes of renal ultrasonography/nuclear studies were given by any paper. Bladder pressures were never provided. Methods of bladder emptying and urological management were only briefly mentioned in most studies, and described in 38 patients only (two studies) [16, 19]. Clean-intermittent catheterisation (CIC) was performed in 12/38 patients, 19/33 patients voided spontaneously, two of 33 used abdominal straining, and two of 33 did the Credé manoeuver.

Urological symptoms during adulthood in patients with CSD were reported in 54/79 (68.4%) patients, whereas abnormalities on UDS were found in 36/43 (83.7%). Urinary incontinence was the most frequent symptom (32.9%), whereas DU was the most frequently found abnormality with UDS (46.5%). When excluding the study of Fukui et al. [16] (a study that describes patients who were originally operated upon during childhood and had been followed into adulthood), symptoms were present in 78.3% of non-operated patients. Urinary incontinence was present in 26.1%, voiding difficulty in 26.1%, frequency in 10.9%, urgency in 21.7%, and other complaints in 39.1%.

The presence of UTIs was given by two studies [15, 16]. Of the 41 patients in these studies, UTIs at baseline were present in 13 (these were all patients from the study by Fukui et al. [16], so during childhood; Düz et al. [15] reported no UTIs). Of these 13, eight improved postoperatively, three remained unchanged and two deteriorated.

Five studies described in which patients a substantial improvement was to be expected after surgery [16, 17, 19-21]. Two studies reported that the longer the symptoms existed, the smaller the chance of improvement after surgery [20, 21]. A similar finding was presented by Satar et al. [19], showing worse outcomes after surgery in adult patients than in the children. Many factors played a role in success of surgery, including age and surgical techniques [17]. Both LUTS and urodynamic abnormalities were found to be irreversible in most patients [17, 19]. In one study, bladder function improved after surgery [21]. In a study where patients were operated upon during childhood but evaluated during adulthood, the type of lesion influenced outcome [16]. Higher improvement rates were found in patients with tight filum terminale (TFT) compared with those with cauda equina adhesion syndrome (CEAS) [16].

Discussion

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Conclusions
  8. Funding
  9. Conflict of Interest
  10. References
  11. Appendix 1 Search Strategy for the Present Review
  12. Supporting Information

There is a paucity of evidence on urological outcomes in adult patients with CSD. For the present review, most papers were excluded because they were written from a neurosurgical point of view, without addressing urological outcomes.

If CSD is diagnosed at birth, evaluation of the LUT and UUT should take place within 1 year [22]. This initial evaluation should consist of ultrasonography of bladder and kidneys. Findings at UDS will often not reveal any anomalies at this early age, and if these are found, these are often unrelated to the neurological function of the lower extremities [23, 24]. However, it seems that these lesions progress with advancing age; up to 25% will eventually develop DSD, which is possibly harmful to the kidneys [25]. In a group of 40 children (28 neonates and infants [mean age 8.7 months], 12 older children [mean age 11.7 years]) evaluated by Keating et al. [24] in 1988, it was shown that older children had more severe neurological deficits and complete denervation with acontractile bladder than the younger patients in the cohort. In patients with sacral agenesis, neurogenic bladder dysfunction will eventually develop in 90% of all patients (with DO/DSD arising in 50% and 50% suffering from complete denervation [26, 27]), although the more subtle forms of this condition are often missed at an early age due to the lack of neurological deficits. Close monitoring during the entire youth of all children with CSD is therefore mandatory. Treatment of the neurogenic bladder in CSD is the same as in OSD: antimuscarinics and self-catheterisation, with surgery if these cannot achieve low-pressure bladders with continence [22, 25].

CSD is not always visible by cutaneous stigmata [28], and many cases will go undiagnosed for years. This, in combination with initial normal evaluation during childhood, might lead to the assumption that follow-up is not necessary. This could lead to both patient's and doctor's delay. In the case of CSD one could say: the later the treatment, the worse the prognosis. It is known from children that successful untethering surgery is more likely when these patients are operated upon on an early age rather than an older age [19, 24, 29]. Hence, although no data exist on the consequences of undiagnosed SD, we can assume that the effects of unrecognised CSD can be severe.

In those children operated upon, the risk of re-tethering exists, especially during growth spurts [29, 30]. Therefore, during the entire childhood up until the age of 18 years, urological follow-up is required. However, although all these recommendations are clear for childhood, close monitoring often stops after the age of 18 years. It is not clear if loss to follow-up of those with CSD can be allowed, whereas the current consensus is that those with OSD should be followed for their entire lifetimes. Without follow-up, patients might discontinue their life-long regimens of CIC and antimuscarinics. One study showed that a 12-month adherence to CIC of 76.7% in adults with neurogenic bladder, implying that almost a quarter of all these patients had stopped catheterisation themselves. It is known that adherence to antimuscarinics are very poor, although this has not been studied extensively in the neurogenic bladder population. One may therefore not automatically assume good adherence to therapy in these adults.

Although children with CSD discovered during childhood do require follow-up, CSD can also manifest during adulthood (‘adult-onset TCS’). The patients in the present study who were operated upon during adulthood had a mean age of 35.8 years; they presented their symptoms only shortly before surgery. An important question is how a congenital condition can manifest itself for the first time at an adult age. One theory for this is that at a more subtle degree of tethering, CSD will remain asymptomatic during childhood. Cumulative damage due to repeated microtraumata throughout life (i.e. during spinal flexion) can cause adult onset TCS [5]. Other contributing factors could be age-related changes to the vertebral column, i.e. spinal cord stenosis [11]. Thus, a once asymptomatic, mild tethering can grow into a symptomatic one over the years.

In the relatively few papers found, 68.4% of adult patients with CSD appeared to have urological symptoms, mostly urgency, frequency, incontinence and voiding difficulty. Normal UDS were rare (16.3%), but this could be biased because most patients also presented with urological complaints. The most frequently found urodynamic outcomes were DU or DO, consistent with the symptomatology.

Improvement after untethering in case of TCS was seen in 11.1–54.5%. Because of the heterogeneity of results, it was not possible to pool data regarding success after untethering surgery. As far we can conclude from the retrieved data, untethering does not seem to harm bladder function. The question as to whether untethering should be performed with the aim to improve bladder function cannot not be answered based on the available data. The various authors present conflicting points of view regarding this matter: there is an ongoing debate whether neurogenic bladder dysfunction secondary to tethered cord is reversible or not. Most authors seem to agree that when time between onset of symptoms and surgery increases, outcomes of surgery are worse.

The urological outcome was worse in cases with a long interval between onset of symptoms and untethering surgery. Unfortunately, only two studies (with few patients) addressed this topic. Neurological damage, if present, may be irreversible [31]. For this reason, paediatric neurosurgeons tend to perform preventive surgery for CSD, e.g. before the onset of symptoms [32]. In adults, CSD will manifest with neurological or urological symptoms instead of a skin lesion [11], rendering preventive surgery impossible.

The different types of lesions are poorly described; the main problem being that each paper uses its own classification of CSD. Neurological impairment was poorly specified. When differentiating between TFT and CEAS (as done by Fukui et al. [16]), patients with CEAS showed markedly poorer outcomes than in those with TFT. In the study of Kondo et al. [21], outcomes were worse in high-grade tethering with a very low medullary cone, which has similarities with CEAS. This suggests that the lower the medullary cone and the more detethering required the worse prognosis is after surgery.

Surprisingly, renal outcomes were not addressed in the seven studies. Only Kondo et al. [21] mentioned ‘vesico-ureteral reflux and moderately impaired renal function’ in two of 15 patients, but without further clarification. It is known that certain urodynamic observations such as DSD, DO, poor compliance, high leak-point pressures and VUR are predictors of renal damage in patients with OSD [13]. In the studies of adult CSD, detrusor pressures were not described properly. DSD was described in two of the studies, and was found in two of 11 patients. DO was seen in 14/43 preoperatively available UDS. According to this, the LUT of patients with CSD may be harmful to the UUT. In these patients, results of ultrasonography of the UUT would be useful, but are currently lacking. In future studies, a combination of renal outcomes and proper reporting of urodynamic parameters is recommended.

Although case studies ( ≤5 patients) were excluded, they do present incidental cases of advanced renal problems in patients with CSD [33, 34]. However, as these are case reports they probably reflect the worse end of the spectrum and are exceptional. However, it should be stressed that in the presence of unfavorable urodynamic outcomes, the kidneys are at risk. Therefore, we suspect that there are more patients with CSD with renal damage than are currently reported.

This is the first systematic review to examine urological outcome in adult patients with CSD. Advantages of the study are that it was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines (Appendix S1) and used standardised outcome measures. However, there are a couple of limitations to acknowledge. First of all, the paucity of articles and the heterogeneity of data made pooling and statistical analysis of data impossible. For instance, the median age and interquartile ranges of the included patients could not even be calculated. Biases, inherent to retrospective cohort studies and case series, are omnipresent in the included studies. Although we chose to take 18 years as a threshold value for adulthood, some could argue that physical adulthood has been reached earlier than legal adulthood, and that we could have included patients aged >16 years, so that we would have had more results to present. However, as 18 years is generally the age from which patients visit adult outpatient clinics and stop paediatric follow-up, the inclusion criterion of >18 years is appropriate.

It is clear that outcome on bladder function in this patient group remains largely unknown. More striking is the lack of data on function of the UUT. Whereas in children (and to some extent in adults with OSD) renal function receives full attention, this is not the case in adults with CSD. We recommend that patients who have been followed from childhood onwards should be invited for a check of the LUT and especially UUT; (retrospective) cohort studies reporting on this should be conducted with priority. As we assume many of these individuals have been lost to follow-up, registers tracking every patient diagnosed with neurogenic bladder during childhood could be of great value. A large registry (the National Spina Bifida Patient Registry) is already present in the USA [35]; it also contains patients with lipomyelomeningocele and fatty filum terminale, both forms of CSD [36]. This database, which contained 2070 patients enrolled between 2009 and 2011, will, without a doubt, provide us with more information on the fate of the adult patient with CSD. This is the only way we will learn about the natural course of neurogenic LUT dysfunction in those with CSD beyond the age of 18 years, especially with regard to the kidneys.

Conclusions

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Conclusions
  8. Funding
  9. Conflict of Interest
  10. References
  11. Appendix 1 Search Strategy for the Present Review
  12. Supporting Information

There is a clear paucity of evidence on the long-term urological outcomes in adults with CSD. In adult patients with CSD, 68.4% reported urological symptoms and in 83.7% abnormal outcomes on UDS were found. Untethering of TCS in adults resulted in improvement of urological symptoms in 33.3–90.0%, depending on the subgroup characteristics. Urodynamic parameters improved in 11.1–54.5% of the patients. Practically no data are available on renal function of adults with CSD. More extensive urological follow-up of this patient group is recommended. Based upon the little evidence available, we think life-long follow-up (from birth into adulthood) of those with CSD and neurogenic bladder is advisable.

References

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Conclusions
  8. Funding
  9. Conflict of Interest
  10. References
  11. Appendix 1 Search Strategy for the Present Review
  12. Supporting Information
Abbreviations
CEAS

cauda equina adhesion syndrome

CIC

clean-intermittent catheterisation

(C)(O)SD

(closed) (open) spinal dysraphism

DO

detrusor overactiviy

DSD

detrusor sphincter dyssynergia

DU

detrusor underactivity

TCS

tethered cord syndrome

TFT

tight filum terminale

UDS

urodynamic studies

(L)(U)UT

(lower) (upper) urinary tract

Appendix 1 Search Strategy for the Present Review

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Conclusions
  8. Funding
  9. Conflict of Interest
  10. References
  11. Appendix 1 Search Strategy for the Present Review
  12. Supporting Information
  • Search spina bifida occulta d.d. 17 July 2012
  • PUBMED:
  • #1: (‘spina bifida’ [Title/Abstract] OR ‘spinal dysraphism’ [Title/Abstract] OR lipomyelomeningocele [Title/Abstract] OR lipomeningocele [Title/Abstract] OR lipomeningomyelocele [Title/Abstract] OR ‘lumbar lipoma’ [Title/Abstract] OR ‘lumbosacral lipoma’ [Title/Abstract] OR ‘intradural lipoma’ [Title/Abstract] OR ‘spinal lipoma’ [Title/Abstract] OR ‘tethered cord’ [Title/Abstract] OR ‘tethered spinal cord’ [Title/Abstract] OR ‘sacral lipoma’ [Title/Abstract] OR ‘occult spina bifida’ [Title/Abstract] OR ‘occulta’ [Title/Abstract] OR ‘filum terminale’ [Title/Abstract] OR ‘terminal filum’ [Title/Abstract] OR ‘split cord malformation’ [Title/Abstract] OR diplomyelia [Title/Abstract] OR diastematomyelia [Title/Abstract] OR ‘sacral agenesia’ [Title/Abstract] OR ‘caudal regression’ [Title/Abstract] OR ‘closed spina bifida’ [Title/Abstract] OR ‘dermal sinus’ [Title/Abstract] OR ‘Spina Bifida Occulta’ [MeSH] OR ‘Neural Tube Defects’ [MeSH])
  • AND
  • #2 (‘urinary incontinence’ [Title/Abstract] OR ‘incontinence’ [Title/Abstract] OR ‘enuresis’ [Title/Abstract] OR ‘urinary loss’ [Title/Abstract] OR ‘urine loss’ [Title/Abstract] continence [Title/Abstract] OR ‘urinary continence’ [Title/Abstract] OR ‘Urinary Incontinence, Urge’ [Mesh] OR ‘Urinary Incontinence’ [Mesh] OR ‘Urinary Incontinence, Stress’ [Mesh] OR ‘Diurnal Enuresis’ [Mesh] OR ‘Nocturnal Enuresis’ [Mesh] OR dry [Title/Abstract] OR continent [Title/Abstract] OR incontinent [Title/Abstract] OR dribbling [Title/Abstract] OR dribble [Title/Abstract] OR dryness [Title/Abstract] OR wet [Title/Abstract] OR wetting [Title/Abstract] OR bed-wetting [Title/Abstract])
  • OR
  • #3 (‘lower urinary tract’ [Title/Abstract] OR ‘bladder function’ [Title/Abstract] OR ‘bladder capacity’ [Title/Abstract] OR urodynamics [Title/Abstract] OR ‘urodynamic’ [Title/Abstract] OR ‘bladder pressure’ [Title/Abstract] OR ‘leak point pressure’ [Title/Abstract] OR ‘bladder pressure’ [Title/Abstract] OR ‘intravesical pressure’ [Title/Abstract] OR ‘intravesical’ [Title/Abstract] OR vesical [Title/Abstract] OR ‘detrusor sphincter dyssynergia’ [Title/Abstract] OR ‘dyssynergia’ [Title/Abstract] OR ‘dyssynergic’ [Title/Abstract] OR bladder [Title/Abstract] OR ‘urinary bladder’ [Title/Abstract] OR ‘pressure flow studies’ [Title/Abstract] OR ‘pressure flow study’ [Title/Abstract] OR PVR [Title/Abstract] OR ‘post void residual’ [Title/Abstract] OR ‘post void residue’ [Title/Abstract] OR ‘post-voiding residue’ [Title/Abstract] OR urodynamic [MeSH])
  • OR
  • #4 (‘Renal Insufficiency’ [MeSH] OR ‘Renal Insufficiency, Chronic’ [MeSH] OR ‘Kidney Failure, Chronic’ [MeSH] OR kidney [Title/Abstract] OR CKD [Title/Abstract] OR ESRD [Title/Abstract] OR dialysis [Title/Abstract] OR ‘renal replacement’ [Title/Abstract] OR ‘renal substitution’ [Title/Abstract] OR ‘chronic kidney disease’ [Title/Abstract] OR renal [Title/Abstract] OR kidneys [Title/Abstract] OR ‘renal function’ [Title/Abstract] OR ‘renal functioning’ [Title/Abstract] OR ‘renal disease’ [Title/Abstract] OR creatinin [Title/Abstract] OR creatinine [Title/Abstract] OR kreatinine [Title/Abstract] OR kreatinin [Title/Abstract] OR GFR [Title/Abstract] OR eGFR [Title/Abstract] OR ‘glomerular filtration rate’ [Title/Abstract] OR MDRD [Title/Abstract] OR Cockroft-Gault [Title/Abstract] OR DMSA [Title/Abstract] OR renography [Title/Abstract] OR renogram [Title/Abstract] OR MAG3 [Title/Abstract] OR hydronephrosis [Title/Abstract] OR renal scintigraphy [Title/Abstract] OR ‘nuclear studies’ [Title/Abstract]OR ‘renal scar’ [Title/Abstract] OR ‘renal scarring’ [Title/Abstract] OR ‘renal damage’ [Title/Abstract] OR ‘upper urinary tract status’ [Title/Abstract] OR ‘upper tract’ [Title/Abstract] OR ‘urinary tract’ [Title/Abstract] OR ‘upper urinary tract’ [Title/Abstract] OR ‘ultrasound’ [Title/Abstract] OR hydronephrosis [Title/Abstract])
  • OR
  • #5 (‘Vesico-ureteral Reflux’ [MeSH] OR VUR [Title/Abstract] OR reflux [Title/Abstract] OR ‘urinary reflux’ [Title/Abstract] OR ‘urine reflux’ [Title/Abstract] OR ‘renal reflux’ [Title/Abstract] OR ‘vesicoureteral reflux’ [Title/Abstract] OR ‘micturating cystogram’ [Title/Abstract] OR cystogram [Title/Abstract] OR cystography [Title/Abstract]))
  • #1 (Spina-synonyms), #2 (incontinence/continence-synonyms), #3 (bladder function), #4 (upper tract function), #5 (reflux-synonyms)
  • #1 AND (#2 OR #3 OR #4 OR #5) [RIGHTWARDS ARROW] 3124 results (PubMed) [RIGHTWARDS ARROW] imported into Refworks 2.0 on 17 july 2012
  • Embase:
  • #1: (‘spina bifida’:ti:ab OR ‘spinal dysraphism’:ti:ab OR lipomyelomeningocele:ti:ab OR lipomeningocele:ti:ab OR lipomeningomyelocele:ti:ab OR ‘lumbar lipoma’:ti:ab OR ‘lumbosacral lipoma’:ti:ab OR ‘intradural lipoma’:ti:ab OR ‘spinal lipoma’:ti:ab OR ‘tethered cord’:ti:ab OR ‘tethered spinal cord’:ti:ab OR ‘sacral lipoma’:ti:ab OR ‘occult spina bifida’:ti:ab OR ‘occulta’:ti:ab OR ‘filum terminale’:ti:ab OR ‘terminal filum’:ti:ab OR ‘split cord malformation’:ti:ab OR diplomyelia:ti:ab OR diastematomyelia:ti:ab OR ‘sacral agenesia’:ti:ab OR ‘caudal regression’:ti:ab OR ‘closed spina bifida’:ti:ab OR ‘dermal sinus’:ti:ab OR ‘spina bifida occulta’:ti:ab OR ‘neural tube defects’:ti:ab)
  • AND
  • #2 (‘urinary incontinence’:ti:ab OR ‘incontinence’:ti:ab OR ‘enuresis’:ti:ab OR ‘urinary loss’:ti:ab OR ‘urine loss’:ti:ab continence:ti:ab OR ‘urinary continence’:ti:ab OR dry:ti:ab OR continent:ti:ab OR incontinent:ti:ab OR dribbling:ti:ab OR dribble:ti:ab OR dryness:ti:ab OR wet:ti:ab OR wetting:ti:ab OR bed-wetting:ti:ab)
  • OR
  • #3 (‘lower urinary tract’:ti:ab OR ‘bladder function’:ti:ab OR ‘bladder capacity’:ti:ab OR urodynamics:ti:ab OR ‘urodynamic’:ti:ab OR ‘bladder pressure’:ti:ab OR ‘leak point pressure’:ti:ab OR ‘bladder pressure’:ti:ab OR ‘intravesical pressure’:ti:ab OR ‘intravesical’:ti:ab OR vesical:ti:ab OR ‘detrusor sphincter dyssynergia’:ti:ab OR ‘dyssynergia’:ti:ab OR ‘dyssynergic’:ti:ab OR bladder:ti:ab OR ‘urinary bladder’:ti:ab OR ‘pressure flow studies’:ti:ab OR ‘pressure flow study’:ti:ab OR PVR:ti:ab OR ‘post void residual’:ti:ab OR ‘post void residue’:ti:ab OR ‘post-voiding residue’:ti:ab)
  • OR
  • #4 (kidney:ti:ab OR CKD:ti:ab OR ESRD:ti:ab OR dialysis:ti:ab OR ‘renal replacement’:ti:ab OR ‘renal substitution’:ti:ab OR ‘chronic kidney disease’:ti:ab OR renal:ti:ab OR kidneys:ti:ab OR ‘renal function’:ti:ab OR ‘renal functioning’:ti:ab OR ‘renal disease’:ti:ab OR creatinin:ti:ab OR creatinine:ti:ab OR kreatinine:ti:ab OR kreatinin:ti:ab OR GFR:ti:ab OR eGFR:ti:ab OR ‘glomerular filtration rate’:ti:ab OR MDRD:ti:ab OR Cockroft-Gault:ti:ab OR DMSA:ti:ab OR renography:ti:ab OR renogram:ti:ab OR MAG3:ti:ab OR hydronephrosis:ti:ab OR renal scintigraphy:ti:ab OR ‘nuclear studies’:ti:ab OR ‘renal scar’:ti:ab OR ‘renal scarring’:ti:ab OR ‘renal damage’:ti:ab OR ‘upper urinary tract status’:ti:ab OR ‘upper tract’:ti:ab OR ‘urinary tract’:ti:ab OR ‘upper urinary tract’:ti:ab OR ‘ultrasound’:ti:ab OR hydronephrosis:ti:ab)
  • OR
  • #5 (VUR:ti:ab OR reflux:ti:ab OR ‘urinary reflux’:ti:ab OR ‘urine reflux’:ti:ab OR ‘renal reflux’:ti:ab OR ‘vesicoureteral reflux’:ti:ab OR ‘micturating cystogram’:ti:ab OR cystogram:ti:ab OR cystography:ti:ab)
  • #1 And (#2 OR #3 OR #4 OR #5) [RIGHTWARDS ARROW] 1975 results, imported in RefWorks on 17 July 2012

Supporting Information

  1. Top of page
  2. Abstract
  3. Introduction
  4. Methods
  5. Results
  6. Discussion
  7. Conclusions
  8. Funding
  9. Conflict of Interest
  10. References
  11. Appendix 1 Search Strategy for the Present Review
  12. Supporting Information
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bju12289-sup-0001-appendix s1.pdf43K

Appendix S1 PRISMA 2009 Checklist.

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