Uroneurological assessment of spina bifida cystica and occulta

AIMS: Spina bifida is a rare but well-documented congenital spinal anomaly comprising a cystic form, which appears in infancy, and an occult form, which appears in late childhood and in adulthood. Herein, we report the results of uroneurological assessment in patients with spina bifida cystica and occulta, with respect to diagnosis and management of the disorders. METHODS: We performed a neurological examination, urinary questionnaire, and urodynamic studies in 28 consecutive patients with urinary symptoms, including 16 with the cystic form (nine male and seven female patients, ages 2 to 25 years), all of whom underwent neonatal surgical management, and 12 with occult form (six men and six women, ages 7 to 32 years) diagnosed by myelography and spinal magnetic resonance imaging who did not undergo surgery. RESULTS: Neurological examination revealed a combination of flaccid and spastic paresis, with dominant lower motoneuron signs in the cystic form, whereas there were dominant upper motoneuron signs in the occult form. Four patients with occult spina bifida presented with urinary symptoms as the sole initial complaint. There was no correlation between neurological findings and urodynamic abnormalities. Urinary incontinence and enuresis were common at all ages, and large post-micturition residuals and vesicoureteral reflux were not uncommon, particularly in the cystic form. Bladder abnormalities in the cystic and occult forms included detrusor hyperreflexia during filling in 38% and 42%, low compliance detrusor in 81% and 67%, supersensitivity to bethanechol in two (100%) patients with the cystic form and in three of four (75%) with the occult form, and impaired bladder sensation in 25% and 8% in each form, respectively. Urethral abnormalities in cystic and occult forms included detrusor/sphincter dyssynergia in 50% and 27%, low Pure max in 56% and 17%, silent sphincter electromyographic activity in 25% and 0%, absent bulbocavernosus reflex in 87% and 56%, absent anal reflex in 100% and 57%, neurogenic motor unit potentials in one patient with cystic form studied and in two of three patients with occult form, respectively. CONCLUSIONS: Spina bifida cystica and occulta present with a wide spectrum of urodynamic abnormalities including upper and lower motoneuron types of bladder and urethral dysfunction. Careful uroneurological assessment and spinal magnetic resonance imaging are important for diagnosing young adult patients with spina bifida occulta because they may present with urinary symptoms as the sole initial complaint and have no other obvious neurological abnormalities.     

Study of Botulinum Toxin A in Neurogenic Bladder Due to Spina Bifida in Children

Background: We report results of a pilot study investigating the safety and efficacy of Botulinum A toxin on urinary incontinence and bladder function in children with neurogenic bladder. Methods: This was a prospective, non‐randomized clinical trial. Seven children with median age of 16 years with spina bifida who had high storage pressures, poor bladder compliance and had failed treatment with anticholinergic medications were offered a single intra‐detrusor injection of Botulinum A toxin. All subjects were on clean intermittent catheterization before and during the study. Follow‐up videourodynamic studies were performed at 1 month, between 3 and 6 months, and at 9 months. Data were collected on safety and on subjective outcomes through validated questionnaires filled out by patients at each visit. Results: In majority of the patients (5/7), the injection produced an increase in bladder compliance (P < 0.05) and an improvement in incontinence (P < 0.05) at 1‐month follow‐up. However, in two patients whose baseline bladder capacity was markedly reduced (<200 mL), the improvement was very minimal. The beneficial effects in bladder compliance and incontinence dissipated by 9 months. The changes in subjective outcomes (incontinence and satisfaction scores) did not parallel the changes in urodynamics through the study period. No side effects of Botulinum toxin were seen. Conclusion: Botulinum A toxin injection produces beneficial urodynamics and clinical effects. These beneficial effects last for approximately 9 months. There is a poor correlation between improvement in the urodynamics and the subjective outcomes. Botulinum A toxin injection is a safe alternative treatment for patients with spina bifida and a neurogenic bladder.     

Sacral nerve modulation for defaecation and micturition disorders in patients with spina bifida

Aim Sacral nerve modulation is a well accepted method for the treatment of defaecation disorders and voiding dysfunction. Results of sacral nerve modulation in patients with spinal cord lesions are not well assessed, but preliminary results look poor. Therefore, the purpose of this study was to assess the effectiveness of sacral nerve modulation for defaecation disorders and voiding dysfunction in patients with spina bifida. Method Consecutive patients with spina bifida suffering from a myelomeningocele and combined faecal and urinary functional disorders that were eligible for peripheral nerve evaluation (PNE) were studied. A permanent sacral nerve modulation implantation was performed after successful PNE. Results Ten patients (four female) were included in this study with a median age of 26.4 (range 11.1–41.0) years. In two the PNE was not possible. The median faecal incontinence days (6.0 vs 3.5) and episodes (8.5 vs 3.5) per 21 days decreased significantly during the 3‐week period of PNE (P = 0.033). Only 3/10 (30%) patients had a more than 50% improvement and proceeded to a permanent sacral nerve modulation implantation. In one patient it was not possible to perform the permanent implant. Conclusion Preliminary results of sacral nerve modulation in a subgroup of spina bifida patients with combined faecal and urinary functional disorders look promising, but long‐term results in larger patient groups need to be studied.     

伴有神经源性膀胱和前列腺憩室的前列腺巨大结石1例报告并文献复习

目的:探讨和总结伴有神经源性膀胱和前列腺憩室的前列腺巨大结石的病因、临床表现、诊断与治疗。方法:结合相关文献复习并回顾性分析1例伴有神经源性膀胱和前列腺憩室的前列腺巨大结石患者的临床资料。患者男,37岁,尿失禁22年,间断排尿困难伴尿频9年,加重3个月。既往有脊柱裂及耻骨上膀胱切开取石术病史。术前尿常规:WBC 17～20/HPF,RBC 12～15/HPF。腹部平片(KUB)+静脉尿路造影(IVU)及盆腔CT:隐性脊柱裂,神经源性膀胱,前列腺巨大结石。结果:患者行经尿道前列腺电切(TURP)+钬激光碎石术,结石成分为碳酸磷灰石。术后2周复查影像尿动力学:最大尿流率及残余尿量均明显好转。术后至今随访17个月,尿失禁较术前明显减轻,尿线粗。结论:前列腺憩室合并前列腺巨大结石非常罕见,而神经源性膀胱可能为其发病的一个因素。膀胱镜检查是准确的检查方法。对于年轻和需要保留性功能者可采取TURP联合钬激光碎石术,术中结合直肠指检尽量彻底清除结石,术后密切随访。     

Management of difficult cases in female urology and neurourology at the Reed M. Nesbit society meeting festschrift in honor of Edward J. McGuire,MD

This is a panel discussion of seven complex urologic cases in female urology and neurourology. Differences in diagnosis and management are discussed by this international panel of experts. Neurourol. Urodynam. 29:S2–S12, 2010. © 2010 Wiley‐Liss, Inc.