Update on Urological Management of Spina Bifida from Prenatal Diagnosis to Adulthood.

Snow-Lisy DC, Yerkes EB, Cheng EY. (2015) Update on Urological Management of Spina Bifida from Prenatal Diagnosis to Adulthood.

J Urol. 2015 Apr 1. pii: S0022-5347(15)03647-2. doi: 10.1016/j.juro.2015.03.107

Abstract

PURPOSE:

We review the current literature regarding urological management of spina bifida from prenatal diagnosis to adulthood.

MATERIALS AND METHODS:

We searched MEDLINE, EMBASE and PubMed for English articles published through December 2014 using search terms "spina bifida," "spinal dysraphism" and "bladder." Based on review of titles and abstracts, 437 of 1,869 articles were identified as addressing topics related to open spina bifida in pediatric patients, or long-term or quality of life outcomes in adults with spina bifida. We summarize this literature to inform clinical guidelines and create a framework for disease management.

RESULTS:

The birth prevalence of spina bifida in the United States has recently plateaued at approximately 30 per 100,000. With improved management more individuals are surviving to adulthood, with an economic impact of $319,000 during the lifetime of an individual with spina bifida. Recent advances in prenatal surgery have demonstrated that prenatal closure of spina bifida is possible. To assess safety and efficacy, the National Institutes of Health sponsored MOMS (Management of Myelomeningocele Study) was undertaken, in which subjects were randomized to prenatal or postnatal closure. Until the urological results of this trial are published, the impact of prenatal intervention on future bladder function remains unclear. Controversy continues regarding the optimal use and timing of urodynamic studies, and the indications for initiation of clean intermittent catheterization and anticholinergics in infants and children. Many favor expectant management, while others argue for a more proactive approach. Based on the current literature, both approaches appear to protect the child from renal injury, although delayed intervention may increase rates of bladder augmentation. The current literature regarding this topic is difficult to interpret and compare due to heterogeneity of patient populations, variable outcome measures and lack of reporting of quality of life outcomes. Surgical intervention is indicated for those at risk for renal deterioration and/or is considered for children who fail to achieve satisfactory continence with medical management. Traditionally surgery concentrates on 3 areas, ie bladder and bladder neck, and creation of catheterizable channels. For those with a hostile bladder enterocystoplasty remains the gold standard for bladder augmentation, although use of bowel for augmentation remains suboptimal due to secondary complications, including increased risk of infections, metabolic abnormalities, neoplastic transformation and risk of life threatening perforation. Recent advances in tissue engineering technology may provide an alternative to traditional augmentation. However, recent results from phase II trials using current techniques to augment the bladder with engineered bladder tissue are disappointing. Catheterizable channels to the bladder and ascending colon further facilitate continence measures and promote independent care. While surgical reconstruction is clearly successful in improving continence, recent outcome studies have questioned the true impact of this type of surgery on quality of life. With improved survival transitional care issues, including health related independence, sexual health needs and development of a support system, are increasingly important. Transitional care remains a significant issue for which few public health measures are being quantitatively evaluated.

CONCLUSIONS:

Despite consensus regarding early urological involvement in the care of patients with spina bifida, controversy remains regarding optimal management. Major reconstructive urological surgeries still have a major role in the management of these cases to protect the upper urinary tract and to achieve continence. However, future studies are needed to better clarify the true impact on quality of life that these interventions have on patients and their families. Transition of urological care to adulthood remains a major avenue for improvement in disease management.
Copyright © 2015 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.

KEYWORDS:

disease management; meningomyelocele; spinal dysraphism; urinary bladder

Urinary incontinence in spina bifida: Initial instrument validation.

Hubert KC, Sideridis G, Sherlock R, Rosoklija I, Kringle G, Johnson K, Bauer SB, Nelson CP.

(2015)  Urinary incontinence in spina bifida: Initial instrument validation.  Res Dev Disabil. 2015 May;40:42-50. doi: 10.1016/j.ridd.2015.01.008.

Abstract

The purpose of this study was to perform a psychometric assessment of the Incontinence Symptom Index-Pediatric (ISI-P) in a cohort of adolescents with spina bifida (SB) and neuropathic urinary incontinence (UI) to test its validity and reliability. The ISI-P, an 11-item instrument with domains for symptom severity and impairment, was self-administered by subjects 11-17 years old with SB and UI. Controls were 11-17 years old, with nephrolithiasis and no history of UI. Formal psychometric assessment included an evaluation of internal consistency, test re-test reliability and factor analysis. Of 78 study-eligible subjects we attempted to contact, 33 (66.7% female) with a median age of 13.1 years completed the ISI-P (42.3% response rate). 21 control patients also completed the ISI-P. Cronbach's alpha was 0.936 and 0.792 for the severity and bother factors respectively. The delta Chi-square test for the two-factor (vs. one-factor) model was significantly [χ(2)(89)=107.823, p<0 .05="" adolescents="" all="" analysis="" and="" associated="" being="" categories="" category="" comparative="" conclusion="" contributed="" descriptive="" desirable="" different="" e.g.="" each="" excellent="" favor="" fit="" for="" former="" furthermore="" has="" impairment="" in="" index="0.969)." indices="" information="" isi-p="" latent="" measurement="" model="" namely="" of="" p="" properties="" psychometric="" sb.="" severity="" showed="" symptom="" that="" the="" threshold="" trait.="" ui="" unique="" values="" were="" with="">Copyright © 2015 Elsevier Ltd. All rights reserved.

Prospective, longitudinal evaluation of health related quality of life in the pediatric spina bifida population undergoing reconstructive urological s

Parekh AD, Trusler LA, Pietsch JB, Byrne DW, DeMarco RT, Pope JC 4th, Adams MC, Deshpande JK, Brock JW 3rd. Prospective, longitudinal evaluation of health related quality of life in the pediatric spina bifida population undergoing reconstructive urological surgery. Journal of Urology. 2006 Oct;176(4 Pt 2):1878-82.

General Clinical Research Center, Vanderbilt Children's Hospital, 2200 Children's Way, Nashville, TN 37232, USA.

PURPOSE: Spina bifida, the most frequent permanently debilitating birth defect, results in major urological problems of voluntary bladder control and bowel function, which may impair quality of life. We prospectively assessed quality of life in patients with spina bifida using child and parent reports simultaneously. This study had 3 goals, that is to 1) document baseline health related quality of life in patients with spina bifida preoperatively, 2) study health related quality of life, reporting differences between parents and children, and 3) study changes in health related quality of life prospectively at preoperative and postoperative intervals.

MATERIALS AND METHODS: Patients with spina bifida who were 2 to 18 years old and required reconstructive urological surgery in 2004 were included in the study. Demographic survey and the validated PedsQL 4.0 health related quality of life questionnaire were used preoperatively and postoperatively. A clinical outcomes data set was completed after the clinician saw the patient. PedsQL 4.0 subscales were scored using the algorithms provided.

RESULTS: The response rate was 100%. Mean participant age was 10.3 years. Preoperatively child physical and psychosocial health and school functioning were significantly higher than parent reports (p <0.001). Overall health related quality of life in patients with spina bifida was lower than in healthy children (62.4 vs 85, p <0.001). Six weeks postoperatively significant differences in health related quality of life reporting between parents and children had lowered. Six months postoperatively child emotional and social functioning scores were higher than parent scores (p <0.001). No correlation was found between health related quality of life, and clinical and demographic factors due to insufficient sample size.

CONCLUSIONS: Children with spina bifida recorded higher health related quality of life scores than parents/guardians. This health related quality of life study addresses concerns that impact daily quality of life in patients with spina bifida. Future health related quality of life studies in patients with spina bifida should use child self-reports.

PMID: 16945679

Current approaches to the urologic care of children with spina bifida

Joseph DB. Current approaches to the urologic care of children with spina bifida. Current Urology Reports. 2008 Mar;9(2):151-7.

Most children born with spina bifida, the most common disabling congenital abnormality, have normal renal function. If left untreated, more than half of these children will have serious renal deterioration by age 5. This deterioration is secondary to hostile neurogenic changes of the bladder. Renal development should follow a normal course when close evaluation and intervention are undertaken during the newborn period and toddler years. As children age, attention is directed to quality-of-life issues, such as the establishment of urinary and bowel continence. Teenagers face the responsibility of understanding their medical condition and should begin to assume responsibility for their own care with eventual transition to the adult health care system. This article describes the foundations of management, beginning at birth, for caring for children with spina bifida.

PMID: 18420000

Estimation of Renal Function in Children and Adolescents With Spinal Dysraphism.

Abrahamsson K, Jodal U, Sixt R, Olsson I, Sillén U. Estimation of Renal Function in Children and Adolescents With Spinal Dysraphism. Journal Urology. 2008 Apr 22;179(6):2407-2409

PURPOSE: In children with spinal dysraphism such as myelomeningocele the relation between muscle mass and body composition varies considerably. Therefore, it is difficult to evaluate the relevance of renal function assessments done with serum creatinine. Since serum cystatin C has been suggested to be independent of body size and composition, this evaluation was compared to chromium(51) edetic acid clearance.

MATERIALS AND METHODS: Simultaneous measurements of cystatin C and chromium(51) edetic acid clearance were performed prospectively in 65 patients 2 to 19 years old with spinal dysraphism.

RESULTS: Cystatin C values were within the normal range in all patients, while chromium(51) edetic acid clearance was reduced in 10. A significant relation was seen.

CONCLUSIONS: Using chromium(51) edetic acid clearance as a gold standard, children with spinal dysraphism and slightly to moderately reduced renal function may remain undiagnosed if cystatin C is used for evaluation.

PMID: 18433781

An assessment of health related quality of life

Dodson JL, Diener-West M, Gerson AC, Kaskel FJ, Furth SL. An assessment of health related quality of life using the child health and illness profile-adolescent edition in adolescents with chronic kidney disease due to underlying urological disorders. Journal of Urology. 2007 Aug;178(2):660-5; discussion 665. Epub 2007 Jun 15.

PURPOSE: We examined health related quality of life in adolescents with congenital urological disease causing kidney disease using a generic health related quality of life instrument. We then compared the results to those in adolescents with medical kidney disease and to population based norms.

MATERIALS AND METHODS: The Child Health and Illness Profile-Adolescent Edition was administered to 113 patients 10 to 18 years old with chronic kidney disease. Mean domain and subdomain scores for adolescents with urological disease were compared to those of adolescents with medical kidney disease and to population based norms.

RESULTS: The cohort included adolescents with an underlying diagnosis of congenital urological anomaly (37 patients) or other causes of kidney disease (76). Compared to adolescents with kidney disease caused by other factors, those with congenital urological disease scored statistically significantly better in the Child Health and Illness Profile-Adolescent Edition subdomain of Limitations of Activity (mean 22.3 [SD 2.5] vs 20.4 [SD 5.0], p = 0.04). Compared to population norms, adolescents with congenital disorders scored lower in the Disorders domain (mean 16.5, 95% CI 14.2 to 18.9) but better in the Risks domain (mean 25.9, 95% CI 25.1 to 26.6) and in the Home Safety and Health subdomain (mean 25.2, 95% CI 23.7 to 26.6).

CONCLUSIONS: As assessed by the Child Health and Illness Profile-Adolescent Edition generic health status questionnaire, adolescents with kidney disease due to underlying congenital urological disease had fewer limitations of activity compared to those with underlying medical kidney disease. Except for low scores in the Disorders domain, children with underlying urological disease did not have significant impairments in any other domain compared to population based norms.

PMID: 17574620

Renal cortical deterioration in children with spinal dysraphism: analysis of risk factors

DeLair SM, Eandi J, White MJ, Nguyen T, Stone AR, Kurzrock EA. Renal cortical deterioration in children with spinal dysraphism: analysis of risk factors. Journal of Spinal Cord Medicine. 2007;30 Suppl 1:S30-4.

BACKGROUND/OBJECTIVE: Because hydronephrosis and reflux are reversible, we believe cortical loss represents true renal deterioration in children with spinal dysraphism. Our goal was to better define risk factors for cortical loss.

METHODS: After institutional review board approval, we reviewed the medical records of 272 children with spinal dysraphism. The following factors were evaluated: age, sex, renal and bladder imaging, urodynamic parameters, medications, catheterization program, continence, infections, and surgical history. Renal cortical loss was defined by scarring or a differential function greater than 15% using a nuclear scan. Univariate and multivariate logistic regression models were fitted to test the associations of specific variables with cortical loss.

RESULTS: Renal cortical loss was found in 41% of children with high-grade reflux vs. 2% of children without reflux. Univariate analysis showed only high-grade reflux and female sex to be independent risk factors. Controlling for age and sex, reflux and initiation of catheterization after 1 year of age are significant risk factors. High bladder pressure and hydronephrosis in the absence of reflux were not associated with cortical loss. Multivariate analysis showed that girls with reflux have a 55-fold increased risk of cortical loss.

CONCLUSION: By limiting the definition of renal deterioration to cortical loss, we identified relevant risk factors: reflux, female sex, and delayed initiation of clean intermittent catheterization. We have also discounted other suspected risk factors: hydronephrosis and elevated bladder pressure. Rather than continuing our focus on hydronephrosis and urodynamics, we believe more research and management debate should be afforded to females with reflux.

PMID: 17874684

Factors that influence outcomes of the Mitrofanoff and Malone antegrade continence enema reconstructive procedures in children

Clark T, Pope JC 4th, Adams C, Wells N, Brock JW 3rd. Factors that influence outcomes of the Mitrofanoff and Malone antegrade continence enema reconstructive procedures in children. Journal of Urology. 2002 Oct;168(4 Pt 1):1537-40; discussion 1540.

PURPOSE: Surgical techniques that provide adequate urinary and fecal continence in children with neurogenic bladder and bowel dysfunction are becoming increasingly used. We reviewed our experience and discuss factors that influence outcome.

MATERIALS AND METHODS: Between 1994 and 2000, 65 stomal procedures were performed in 47 patients. For the urinary continent catheterizable channel we used appendix in 60% of cases, a continent bladder tube in 20%, a Yang-Monti tube in 16% and ureter in 4%. For the antegrade continence enema continent catheterizable channel we used appendix in 85% of cases, a Yang-Monti tube in 5% and a cecal tube in 10%. In the 19 patients who underwent simultaneous Mitrofanoff and antegrade continence enema procedures the urinary continent catheterizable channel was appendix in 21%, a Yang-Monti tube in 32% and continent vesicostomy in 47%. Patients were divided into 2 groups based on compliance status. In addition, percentile body weight for age was evaluated.

RESULTS: Stomal continence was achieved in 63 of the 65 cases (97%). Of the patients who underwent the antegrade continence enema procedure 95% achieved continence via the rectum. Except for ureter stenosis rates according to continent catheterizable channel type did not differ greatly, namely 19% for appendix, 11% for the Yang-Monti tube, 22% for the bladder tube, 50% for ureter and 0% for the cecal tube. Infectious complications developed in 16 patients and 4 had stones. The rates of infection (p = 0.004), stomal stenosis (p = 0.001) and revision (p = 0.004) were statistically lower in the compliant group and the stone formation rate showed a trend favoring the compliant group (p = 0.11). No significant difference was noted for incontinence. Percentile weight predicted a higher rate of stomal stenosis with the highest rate of stomal stenosis overall in the greater than 100th percentile group.

CONCLUSIONS: The Mitrofanoff and antegrade continence enema procedures are reliable and effective. Proper patient selection and surgical technique with a tension-free anastomosis are essential. The choice of tissue for constructing the continent catheterizable channel is not as important as patient compliance, age and possibly body habitus. This report reinforces the importance of careful screening, and rigorous preoperative and postoperative teaching to achieve overall patient success.

PMID: 12352454

The effects of detethering on the urodynamics profile in children with a tethered cord.

Hsieh MH, Perry V, Gupta N, Pearson C, Nguyen HT. The effects of detethering on the urodynamics profile in children with a tethered cord. Journal of Neurosurgery. 2006 Nov;105(5 Suppl):391-5.

OBJECT: Tethering of the spinal cord is a pathological fixation of the cord in the vertebral column that can result in neurogenic bladder dysfunction and other neurological problems. It occurs in patients with closed spinal dysraphisms and those in whom postoperative scarring develops following spina bifida closure procedures. The authors of this study sought to determine the effects of detethering on the urodynamic profile of children with a tethered cord.

METHODS: The authors retrospectively reviewed the records of children who underwent surgical release of a tethered cord at a single institution between 2001 and 2003. They identified 17 children (nine girls and eight boys) who had undergone both preoperative and postoperative urodynamic evaluation. Preoperatively, 10 (59%) of the children with a tethered cord had abnormal urodynamic study (UDS) results. Only two (20%) of these patients had urological symptoms. All seven patients with normal preoperative UDS results had normal UDS results after detethering. In addition, in five (50%) of the 10 children with abnormal preoperative UDS results, the postoperative UDS demonstrated improved or normal urodynamics.

CONCLUSIONS: Because more than half of the children who underwent detethering were found to have abnormal preoperative UDS results, preoperative urodynamic evaluation should be performed in all cases in which detethering is considered. With regard to voiding function, detethering is relatively safe for children with normal preoperative UDS results. In children with abnormal preoperative UDS results, detethering may lead to improvement or even normalization of voiding, especially if the procedure is performed prior to 1 year of age. Finally, children with anorectal anomalies and a tethered cord may represent a subset of patients who are particularly likely to experience urodynamic improvement after detethering.

PMID: 17328264

Preservation of renal function in children with myelomeningocele managed with basic newborn evaluation and close followup.

Hopps CV, Kropp KA. Preservation of renal function in children with myelomeningocele managed with basic newborn evaluation and close followup. The Journal of Urology. 2003 Jan;169(1):305-8.

PURPOSE: Children with myelomeningocele who leak with high intravesical pressures are at risk for upper urinary tract deterioration. Urodynamic study shortly after birth and routinely thereafter has been advocated to predict which newborns are at risk for upper tract deterioration. We hypothesize an approach that excludes routine application of urodynamic evaluation in neonates, reserving this study for use only when clinically indicated, is a safe and effective management option.

MATERIALS AND METHODS: Of 188 patients seen in the myelomeningocele clinic 84 underwent initial evaluation at age 6 months or less and comprise the study group. Initial evaluation consisted of a history, physical examination, urine culture and renal ultrasound. Infants with hydronephrosis or evidence of retention were placed into a high risk group and all others were placed into a low risk group. High risk patients underwent prompt urodynamic evaluation. High and low risk patients were followed closely at 2 to 4-month intervals with serial physical examination, upper tract imaging and urine culture. Conversion from low to high risk occurred with new onset hydronephrosis, febrile urinary tract infection, urinary retention or incidental finding of vesicoureteral reflux at the time of evaluation for continence. These findings warranted urodynamic evaluation and appropriate intervention. Upper tract deterioration, defined as new onset hydronephrosis, was distinguished from renal deterioration, defined as cortical thinning or scarring, failure of renal growth or decreased renal function on renal scan.

RESULTS: A total of 18 infants were placed into the high risk group at initial evaluation, including 12 for retention and 6 for hydronephrosis. The majority of patients were treated with clean intermittent catheterization or vesicostomy. Of the 65 infants placed into the low risk group 29 were converted to high risk at a mean age of 3.1 years, most commonly for febrile urinary tract infection (45%), and appropriate intervention was instituted. Mean followup is 10.4 years (range 0.25 to 26.5). Despite the occurrence of upper tract deterioration, renal deterioration occurred in only 2 of the 162 total renal units (1.2%).

CONCLUSIONS: Basic evaluation of the newborn with myelomeningocele along with careful followup and intervention when indicated results in an excellent rate of renal preservation and represents a safe method of management.

PMID: 12478177

Early start to therapy preserves kidney function in spina bifida patients.

Dik P, Klijn AJ, van Gool JD, de Jong-de Vos van Steenwijk CC, de Jong TP. Early start to therapy preserves kidney function in spina bifida patients.
European Urology. 2006 May;49(5):908-13. Epub 2006 Jan 19.

OBJECTIVE: Renal scarring and renal failure remain life-threatening for children born with spinal dysraphism. We reviewed our data of spina bifida patients to evaluate whether optimal treatment of the neurogenic bladder from birth onwards can preserve kidney function.

METHODS: We reviewed data on all newborns with spinal dysraphism who were referred to our hospital between January 1988 and June 2001. We looked at their situations at referral and at follow-up: the type of treatment, antimuscarinic agents, clean intermittent catheterisation (CIC), antibiotic prophylaxis, and operations (sling procedures, bladder augmentations, antireflux procedures). Renal function (ultrasound, DMSA scan, serum creatinin, creatinin clearance) and bladder function (urodynamic studies) were evaluated over time.

RESULTS: Data of 144 children of 176 could be evaluated by the end of the study: 5 patients had pre-existing renal abnormalities, 69 had an overactive sphincter, 27 had reflux, and six had renal scarring. None are currently developing end-stage renal disease. All patients with spina bifida aperta started CIC and antimuscarinic therapy shortly after birth. Five of the six patients with renal scarring were started on therapy with intermittent catheterisation and antimuscarinic therapy several months after birth. Sixty-three of 82 children with spina bifida were dry at school age (age six), although 37 of these had not had an operation.

CONCLUSION: We show that an early start to therapy helps to safeguard renal function for children born with spina bifida. Our data support other recent reports that children born with spina bifida can probably use their own kidneys for a lifetime, if they are given adequate urological treatment. To protect the upper urinary tract, we need to ensure low intravesical pressure by starting children early on CIC (the preferred treatment); antimuscarinic agents to counteract detrusor instability are indispensable in most cases. Proactive treatment of risks for upper tract deterioration results in a negligible loss of renal function, even when early urinary continence is included in the treatment protocol.

PMID: 16458416

A multicenter evaluation of urinary incontinence management and outcome in spina bifida.

Lemelle JL, Guillemin F, Aubert D, Guys JM, Lottmann H, Lortat-Jacob S, Moscovici J, Mouriquand P, Ruffion A, Schmitt M. A multicenter evaluation of urinary incontinence management and outcome in spina bifida. J Urol. 2006 Jan;175(1):208-12.

PURPOSE: We describe urinary continence management and outcome in patients with spina bifida to identify the procedures that are most successful.

MATERIALS AND METHODS: In a multicenter retrospective cohort study medical charts were studied. At the same time in a cross-sectional survey sociodemographic characteristics, orthopedic features and urinary continence were described based on the frequency of leakage from the viewpoint of patients or close relatives using a Likert scale of 5 items, namely 1-leakage permanent to 5-leakage never.

RESULTS: A total of 421 patients were included, of whom 191 (45%) had been medically treated with a normal voiding pattern according to the patient viewpoint in 21%, clean intermittent catheterization in 61% and no specific bladder emptying method in 18%. The mean leakage score +/- SD was 2.74 +/- 1.55. On the other hand, 230 patients (55%) were surgically treated. Except for 23 patients who underwent noncontinent urinary diversion 207 were considered for treatment and continence description. The mean leakage score was 3.45 +/- 1.60. An artificial urinary sphincter in male and females, and a sling or Kropp technique in females were satisfactory when bladder enlargement was not required. In cases of bladder augmentation without continent diversion an artificial urinary sphincter in males and a bladder neck sling or cinch, Kropp and Young-Dees procedures in females have provided the best results. In cases of bladder enlargement with continent urinary diversion bladder neck closure or a wrap have provided the best results whatever the patient sex.

CONCLUSIONS: Many factors may influence the choice of a technique, such as patient sex, bladder characteristics or orthopedic conditions. However, since to our knowledge no randomized, controlled study has been yet performed, definitive conclusions on the best way to achieve urinary continence in patients with spina bifida cannot be established.

Bacteriuria management and urological evaluation of patients with spina bifida and neurogenic bladder: a multicenter survey.

Elliott SP, Villar R, Duncan B. Bacteriuria management and urological evaluation of patients with spina bifida and neurogenic bladder: a multicenter survey. J Urol. 2005 Jan;173(1):217-20.

PURPOSE: We assessed how groups at spina bifida clinics evaluate and manage the urinary tract in patients with spina bifida, neurogenic bladder and bacteriuria.

MATERIALS AND METHODS: A survey was mailed to all 169 clinics listed by the Spina Bifida Association of America. Survey items addressed baseline and surveillance evaluation, criteria used to assess urinary tract health and approaches to treatment in patients with spina bifida and neurogenic bladder.

RESULTS: Of the 169 clinics personnel at 59 (35%) responded to the survey. Almost half of the respondents had an established protocol or standard of care. At most clinics the use of ultrasound (93%), voiding cystourethrograms (85%) and urodynamic testing (76%) was supported but not renal isotopic studies (14%) or excretory urograms (2%) for baseline evaluation. At all clinics ultrasound was supported for routine surveillance but there was no consensus for other imaging modalities. Assessment of clinic approaches to the evaluation and management of bacteriuria demonstrated variable results, although at most clinics fever, flank pain, dysuria, and changes in urinary pattern were identified as being consistent with true infection. Groups at clinics following their protocol or standard of care showed no significant differences in their approach compared to those at clinics lacking a protocol or standard of care.

CONCLUSIONS: No consensus exists for the evaluation and management of bacteriuria in patients with spina bifida and neurogenic bladder at clinics specializing in the care of such patients, even at those with established standards of care. A clear need exists for an established, national set of evidence based guidelines to assist medical decision making in this high risk population and, thus, improve care.

Urological follow-up of adult spina bifida patients.

Ahmad I, Granitsiotis P. Urological follow-up of adult spina bifida patients. Neurourol Urodyn. 2007 Jul 11

AIMS: The vast majority of the current urological literature understandably has concentrated on the management of children with spina bifida, because in the past the majority did not survive into adulthood. With improvements in the understanding and multidisciplinary care of spina bifida patients it has become a disease of adults. Our aim was to evaluate the current literature to attempt to formulate evidence based guidelines for the management of this difficult group of patients.

METHODS: We reviewed the literature on adult urological management of spina bifida, all relevant articles which concentrated on adults and long-term management were studied in full.

RESULTS: Renal function may begin/continue to deteriorate into adulthood, becoming the leading cause of adult death. This is thought to occur because of changes in the adult bladder, with increases in storage pressure. Medical and surgical management should aim to preserve renal function as well as the maintenance of continence in the face of the growing and changing urinary tract. Follow-up should be regular and in the context of a specialist multidisciplinary clinic. Despite being unvalidated in the follow-up of adult spina bifida patient's annual serum creatinine, ultrasound and urodynamics are currently the best tools available.

CONCLUSIONS: There is no reason why the majority of spina bifida sufferers cannot use their own kidneys for the rest of their lives. This however relies on urological treatment being instigated soon after birth and continuing into adulthood. Neurourol. Urodynam. (c) 2007 Wiley-Liss, Inc.