Prevalence and risk factors for scoliosis in adults with closed and open spina bifida: A large, cross-sectional study.

BACKGROUND: Scoliosis develops in a proportion of children with myelomeningocele; however, little is known about scoliosis in adulthood and in other forms of spina bifida (SB). OBJECTIVES: The aims of this study were to describe the prevalence of scoliosis and identify risk factors for its development in a large cohort of adults with open and closed SB. METHODS: This was a cross-sectional study of data from 580 adults with SB attending their first consultation at a French multidisciplinary referral centre for SB. Sex, anatomical location and type of SB (open or closed), neurological level, back pain and ambulatory status (new Functional Ambulation Classification [new FAC]) were compared in adults with and without scoliosis. These characteristics were used to determine scoliosis risk factors. RESULTS: In total, 331 adults fulfilled the inclusion criteria: 221 had open and 110 had closed SB. Of these, 176 (53%) had scoliosis: 57% open and 45% closed SB. As compared with individuals without scoliosis, those with scoliosis more frequently had open SB (p=0.03), more cranially located SB (p<0.0001), more severe neurological deficits (p≤0.02) and poorer walking ability (mean new FAC score 3.5 [SD 3.3] vs 6.1 [2.6], [p<0.0001]). In total, 69% had chronic back pain, with no difference in frequency between those with and without scoliosis. The odds of scoliosis was associated with asymmetrical motor level and a new FAC score <4 (odds ratio 0.46, p<0.006, and 0.75, p<0.0001, respectively). CONCLUSION: About half of adults with open and closed SB had scoliosis. Back pain was frequent in those both with and without scoliosis. Individuals with low walking ability and an asymmetrical motor level should be monitored early and continuously to limit the consequences of scoliosis during their lifetime. A major issue is to determine how scoliosis evolves and to determine appropriate monitoring and treatment strategies for individuals at risk.

Fecal incontinence in patients with spina bifida: The target is the rectum.

AIMS: The prevalence rate of severe fecal incontinence (FI) in adults with spina bifida (SB) is high. The physiological basis of FI in SB has not been clearly established, which contributes to inadequate care. The aim was to better characterize a large cohort of adults with special consideration of anorectal physiology. METHODS: A multidisciplinary team from a French referral centre for SB prospectively collected data on patients who had an anorectal manometry. Factors associated with severe FI (Cleveland clinical incontinence score ≥ 9) were assessed in a multivariate analysis model. RESULTS: A total of 132 adults with SB (sex ratio M/F: 55 [41.7%]/77 [58.3%]; mean age of 38.2 [11.6] years old) were assessed. Among these patients, 83/132 (62.9%) suffered from severe FI. Rectal perception was not evaluable among 17 patients who had a latex allergy. Overall, 29/115 (25.2%) had maximal tolerable volume (MTV) > 330 mL or no sensation. The absence of anal canal sensitivity, MVT > 330 mL and the amplitude of the recto-anal inhibitory reflex (RAIR) >75% after a rectal isovolumic inflation of 50 mL were significantly associated with severe FI in the multivariate analysis model. Neither neurological level nor other neurological features were associated with severe FI. CONCLUSIONS: This study showed that FI in patients with SB is mainly associated with rectal abnormalities. This should be taken into consideration to improve incontinence management of patients with SB.

Artificial Urinary Sphincter in Male Patients with Spina Bifida: Comparison of Perioperative and Functional Outcomes between Bulbar Urethra and Bladder Neck Cuff Placement.

PURPOSE: We evaluated the perioperative and long-term functional outcomes of bladder neck and peribulbar cuff placement of an artificial urinary sphincter in a population of adult male patients with spinal dysraphism. MATERIALS AND METHODS: We retrospectively analyzed the French spina bifida network database. Patients who underwent implantation of an artificial urinary sphincter from January 1985 to November 2015 were selected and stratified into 2 groups according to cuff location, that is bladder neck vs bulbar urethra. Explantation-free and revision-free device survival was estimated by the Kaplan-Meier method and compared with the log rank test. Cox regression models were created to assess prognostic factors of artificial urinary sphincter device failure. RESULTS: A total of 65 patients were included in study. Most patients were not wheelchair bound. The cuff was implanted around the bulbar urethra at 46 procedures (59%) and around the bladder neck in 32 (41%). In the peribulbar and bladder neck groups median revision-free device survival was 11.7 and 14.3 years, respectively (p = 0.73). Median explantation-free device survival was 18.5 and 24.5 years, respectively (p = 0.08). On multivariate analysis clean intermittent catheterization was the only predictor of artificial urinary sphincter device failure. Cuff location had no influence. At the last followup satisfactory continence was similar in the 2 groups (83% vs 75%, p = 0.75). CONCLUSIONS: In male patients with spinal dysraphism morbidity and functional outcomes were similar for bladder neck and bulbar urethra cuff placement but with a trend toward longer survival without explantation in the bladder neck group. Clean intermittent catheterization was the only predictor of shorter device survival on multivariate analysis.

Bowel Dysfunction Related to Spina Bifida: Keep It Simple.

BACKGROUND: Although care of urological disorders in spina bifida is well established, there is yet no agreement on a standardized approach to bowel dysfunction in this population. OBJECTIVE: The purpose of this study was to assess bowel dysfunction using validated instruments and the risk factors in adults with spina bifida. DESIGN: A multidisciplinary team prospectively collected patient data, focusing on anorectal and urological symptoms. SETTINGS: The study was conducted with data from a French referral center for spina bifida. PATIENTS: A total of 228 adults with spina bifida (sex ratio men:women, 92 (40%):136 (60%)) with a median age of 34.7 years (range, 26.8-44.7 y) were assessed. MAIN OUTCOMES MEASURES: Factors associated with severe fecal incontinence (Cleveland Clinic Incontinence Score ≥9) and severe bowel dysfunction (Neurogenic Bowel Dysfunction score ≥14) were assessed in a multivariate analysis model. RESULTS: The prevalence rates of severe fecal incontinence and severe bowel dysfunction were 60% (130/217) and 42% (71/168). Bowel dysfunction was the second most common major concern of patients after lower urinary tract dysfunction. Male sex, obesity, urinary incontinence, and a Knowles-Eccersley-Scott symptom constipation score ≥10 were independently associated with severe fecal incontinence. Patients with soft stools had significantly less severe bowel dysfunction. Neither neurologic level nor other neurologic features of spina bifida were associated with severe fecal incontinence or severe bowel dysfunction. LIMITATIONS: The recruitment of patients with spina bifida through a national referral center might have resulted in selection bias, and some data were missing especially regarding BMI and Neurogenic Bowel Dysfunction score (21% and 26% of missing data). CONCLUSIONS: The prevalence rates of severe fecal incontinence and severe bowel dysfunction in adults with spina bifida were high and were adequately perceived by the patients. The present study emphasized the association of bowel dysfunction and fecal incontinence with obesity, urologic disorders, and stool consistency rather than neurologic features. See Video Abstract at http://links.lww.com/DCR/A394.

Outcomes of intra-detrusor injections of botulinum toxin in patients with spina bifida: A systematic review.

CONTEXT: Bladder management in spina bifida patients relies on clean intermittent catheterization and oral antimuscarinics with a significant failure rate. The efficacy of intradetrusor injections of botulinum toxin has been confirmed in patients with spinal cord injury or multiple sclerosis but not in patients with myelomeningocele. OBJECTIVE: To conduct a systematic review of current evidence regarding the efficacy of intra-detrusor injections of Botulinum Toxin A (BTX-A) in spina bifida patients with neurogenic detrusor overactivity (NDO) refractory to antimuscarinics. METHODS: A research has been conducted on Medline and Embase using the keywords: ("spina bifida" OR "myelomeningocele" OR "dysraphism") AND "toxin." The search strategy and studies selection were performed using the PICOS method according to the PRISMA statement. RESULT: Twelve published series were included (n = 293 patients). All patients were <18 years old. There was no randomized study comparing BTX-A versus placebo and most studies had no control group. Most studies reported a clinical and urodynamic improvement with resolution of incontinence in 32-100% of patients, a decrease in maximum detrusor pressure from 32 to 54%, an increase of maximum cystometric capacity from 27 to 162%, and an improvement in bladder compliance of 28-176%. Two studies suggested lower efficacy in patients with low compliance bladder compared to those with isolated detrusor overactivity. CONCLUSION: Intradetrusor injections of BTX-A could be effective in children with spina bifida but this assumption is not supported by high level of evidence studies. There is no data available in adult patients. Neurourol. Urodynam. 36:557-564, 2017. © 2016 Wiley Periodicals, Inc.