The history of spina bifida, hydrocephalus, paraplegia, and incontinence

This paper covers the history since ancient times of spina bifida (SB) and its main associated conditions, viz., hydrocephalus, paraplegia and incontinence. Particular stress has been placed on the ancient authors who recognised these conditions. The article concludes with the history of some general aspects of SB and the dilemmas in its treatment. Accepted: 28 April 2000     

The prevalence of hypertension in children with spina bifida

Aim: To determine whether children with spina bifida (SB) have a higher prevalence of hypertension (HTN) than population‐based controls. Methods: Charts of all patients (n = 123) seen in the Spina Bifida Clinic at Shriners Hospital in Houston, Texas, were reviewed for age, gender, ethnicity, type and level of neural tube defect, height, weight and blood pressure (BP). HTN was defined as a systolic blood pressure or diastolic blood pressure ≥95th percentile for age, gender and height on ≥3 occasions. Data from the National Health and Nutrition Examination Survey and from a Houston‐based study were used for comparison. Results: Fifty‐one (41.5%) patients were hypertensive. This was significantly higher than the 3% prevalence in the national controls, p < 0.001 and the 4.5% prevalence in the Houston controls, p < 0.001. Maximum body mass index increased the risk for HTN [odds ratio, 1.018; 95% confidence interval (1.005, 1.031); and p = 0.005]. Conclusion: Children with SB have a significantly higher prevalence of HTN than children in the general population. Age‐, gender‐, and height‐based norms are important for the early identification and treatment of HTN in children.     

Urinary continence across the life course

Spina bifida is the most common defect of the central nervous system. It is a congenital malformation of the spine with abnormal neural tube closure occurring between the third and fourth weeks of gestation, and most frequently affecting the lumbar and sacral regions. Most children with spina bifida have a normal urinary tract at birth, although renal damage and renal failure are among the most severe complications of spina bifida. Before ventricular shunting, survival rates for children with spina bifida were low, but most patients can now be expected to live into adulthood, thus prevention of urologic complications and promotion of continence have become critical. This article reviews the literature regarding urinary continence, and discusses issues across the lifespan, and implications for clinical practice and the pediatrician's role in the urologic care of children with spina bifida.     

显性脊柱裂术后脊髓再栓系的诊断和治疗

目的 囊性脊柱裂手术后,脊髓因纤维瘢痕粘连而造成脊髓栓系,使得神经损害症状加重或产生新的症状。本文就该病的诊断及治疗进行分析。方法 对１４例脊髓脊膜膨出或脂肪脊髓脊膜膨出修补术后病例资料进行分析。再手术时年龄２～１２岁,术后随访３～１２个月,对大小便失禁分别采用Ｋｅｌｌｙ评分法及Ｂｒｕｓｋｅｗｉｚｅ评分法。结果 第一次术后１０例原有的神经损害症状进一步加重,４例无神经损害症状者术后产生新的症状。Ｍ     

脊髓拴系松解术后脊柱裂患者的肛管直肠功能研究

目的：与临床对于脊柱裂致膀胱功能障碍的大量研究报道相比,关于脊柱裂患者的肠道功能障碍研究资料非常有限。该文拟研究脊柱裂患者行脊髓拴系松解术后的肛管直肠功能。方法：采用多通道肛管直肠测压技术对因排尿功能障碍而来就诊的21例脊柱裂患者进行肛管直肠功能检测,入选患者皆已于至少2年前行脊髓拴系松解术。结果：脊柱裂患者的最大肛管静息压低于对照组,但二者没有统计学差异(P=0.372)。在嘱脊柱裂患者行最大限度收缩肛门动作时,绝大多数患者肛管压力没有任何升高。在行模拟排便动作时,19例(90.5%)患者表现为盆底功能紊乱型肛管压力变化。直肠肛管抑制反射在所有受检者均存在,诱发该反射所需最小直肠气囊容量在脊柱裂患者组和对照组间差异无显著性(P=0.725);诱发持续性直肠肛管抑制反射所需直肠气囊容量在脊柱裂患者组显著性高于对照组(P<0.001)。直肠感觉阈值在脊柱裂患者显著高于对照组(P<0.0001)。结论：大多数脊柱裂患者不能自主收缩肛门外括约肌,排便时表现为盆底功能紊乱型直肠肛管压力曲线,同时直肠感觉功能也受到严重损害。直肠肛管抑制反射在所有脊柱裂患者均存在,该反射可能受到中枢神经系统的调控。     

Using the spina bifida life course model in clinical practice: an interdisciplinary approach

The Life Course Model for patients, families, caregivers, teachers, and clinicians was developed with support by the National Spina Bifida Program, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, for individuals with spina bifida. The Life Course Model facilitates a developmental approach to assessment and intervention along life's trajectory. This Life Course Model provides information about key developmental milestones for particular age groups, validated assessments that can be performed by clinicians or teachers to determine if milestones have been reached, useful suggestions for intervening in creative ways at each step, and evidence-based references. In this article, the authors introduce the viewpoints of several key clinicians who are involved in the care of individuals with spina bifida and how the Life Course Model can assist them, their patients, and their families in the process of assessment, intervention, collaboration with other clinicians, and follow-up. A case study is used to demonstrate the experience of comprehensive and collaborative management in transitioning a child and his family from infancy to adulthood.     

伴排尿障碍的隐性脊柱裂患儿的尿动力学特点

目的 研究伴有排尿功能障碍的隐性脊柱裂患儿的尿动力学特征.方法 对113例有排尿障碍的患儿进行尿动力学检查,其中48例经X线确诊为隐性脊柱裂者为观察组,无脊柱裂65例为对照组.检测项目包括:尿流率测定、充盈期膀胱压力容积测定、压力流率测定、同步括约肌肌电测定、静态尿道压力测定.比较两组间主要尿动力参数异常的发生率.观察组按主要临床症状分为尿失禁、尿频、单纯夜间遗尿和排尿困难4组,应用统计学研究临床症状与尿动力学主要参数的相关性.结果 在检测中发现观察组48例中有46例有不同程度的异常.其中逼尿肌过度活动22例,排尿期逼尿肌活动低下和无收缩21例,最大尿流率降低18例,膀胱容积缩小15例,残余尿量增多12例,低顺应性膀胱7例,逼尿肌外括约肌协同失调4例,最大尿道压降低4例.观察组中逼尿肌过度活动、逼尿肌活动低下、残余尿量增多及低顺应性膀胱发生率更高.按临床症状来看,隐性脊柱裂伴有尿失禁的患儿更多的表现为逼尿肌活动低下及最大尿流率降低,尿频的患儿在尿动力检查中多表现为逼尿肌过度活动及残余尿增多,排尿困难的患儿逼尿肌活动低下的发生率更高,而遗尿的患儿更易检出逼尿肌过度活动.结论 小儿隐性脊柱裂伴有排尿功能障碍的患儿具有多种尿动力学改变,且相同的症状可表现为不同类型的尿动力学异常,临床症状与尿动力学参数有一定的相关性,尿动力检查为其临床诊断和治疗方案制定提供重要客观依据.     

隐性脊椎裂胎鼠支配肛提肌脊髓运动神经元定量研究

目的采用神经解剖学常用的荧光金(FG)标记逆向神经示踪法,结合胎儿外科显微手术和显微注射技术,探讨脊椎裂胎鼠支配肛提肌的脊髓运动神经元的发育特点。方法在大鼠孕20d用10%水合氯醛麻醉,应用胎儿外科显微手术和显微注射技术,用纤细玻璃针将5%FG注入雄性胎鼠直肠两侧肛提肌;胎鼠子宫内存活24h后,4%多聚甲醛进行灌注固定;取脊柱标本阿尔新蓝软骨染色后实体显微镜下数码相机采集脊柱软骨染色图像,经计算机软件测量出每个椎体椎弓两个软骨端距离并判定隐性脊椎裂;采集图像后取腰骶段脊髓标本置入20%蔗糖溶液内,24h后连续冰冻横切片,脊髓冰冻切片荧光显微镜下观察并计数FG标记的运动神经元数目。结果正常组脊髓前角FG标记的运动神经元呈团簇状分布,左右两侧对称,主要位于脊髓前角的背内侧群和背外侧群;畸形组脊髓前角FG标记的运动神经元数目少,而且在脊髓前角的背内侧群和背外侧群之间也有少量散在分布,分布团簇状、对称性差,畸形组FG标记的运动神经元数量(244±41)个,与正常组(426±36)个和用药无畸形组(397±20)个差异显著,P<0.05。结论隐性脊椎裂胎鼠支配肛提肌的脊髓运动神经元发育异常,运动神经元数目明显减少,分布异常。     

隐性脊柱裂流行病学及诊疗研究进展

隐性脊柱裂(spina bifida occulta,SBO)是指有一个或数个椎骨的椎板闭合不全,脊柱背侧皮肤完整,椎管内的脊髓及神经组织不会直接突出于皮肤表面的脊柱裂.SBO可发生于脊柱任何部位,但常发生于腰骶部,可伴有脊髓神经发育畸形,产生神经系统、泌尿系统、消化系统以及运动系统等一系列临床症状和体征.SBO若伴有脊髓神经损伤会影响脊髓的正常解剖,使其受到异常牵拉,局部缺血、缺氧,可造成神经功能障碍而产生一系列临床症状,称脊髓栓系综合征(tethered spinal cord syndrome,TCS).国内外目前对SBO特别是合并TCS时的诊治存在一定争议,故本文就SBO的病因、发病率、及诊断治疗研究进展进行综述.     

脊柱裂动物模型制作与胚胎发生机制探讨

目的 建立大鼠隐性脊柱裂和显性脊柱裂畸形动物模型，观察丙戊酸钠和维甲酸诱导大鼠脊柱裂畸形的特点，初步探讨脊柱裂畸形的发生机制。方法 雌性Wistar孕鼠随机分成组：对照组，实验组分成丙戊酸钠三个不同剂量组和维甲酸组。对照组孕鼠不予任何处置，20d胎鼠脊柱染色标本采集图像经计算机软件显微图像分析，测量出每个椎体椎弓两个软骨端距离，目的确定软骨端距离参考值。雌性Wistar大鼠，在确定怀孕的第9d9：00、16：00，按体重计算剂量后于后肢皮下注射20％丙戊酸钠溶液。妊娠第20d剖宫，检查胚胎外观，判定是否有外观可见畸形，然后取脊柱进行骨和软骨双重染色，染色后标本采集图像测量出每个椎体椎弓两个软骨端距离。参照正常胎鼠确定的参考值判定隐性脊柱裂。Wistar大鼠确定怀孕的第10d9：00，胃管灌入维甲酸矿物油混悬液135mg／kg(40mg／m1)，妊娠第20d剖宫，检查胚胎外观判定显性脊柱裂畸形发生情况并记录。结果 丙戊酸钠诱导隐性脊柱裂畸形随药物剂量加大，畸形发生率升高，丙戊酸钠剂量400mg／kg、450mg／kg、500rng／kg~导畸形发生率分别是80％、93％、100％，腰椎对丙戊酸钠的致畸作用最敏感；丙戊酸钠还可以导致头端神经管畸形，未观察到显性脊柱裂的发生。维甲酸135rng／kg诱导胎鼠显性脊柱裂的发生率52．3％，畸形都发生在腰骶段，其他畸形的总发生率高达90．7％。结论 丙戊酸钠抑制软骨细胞增生，影响椎体的软骨发生使椎弓不能闭合导致胎鼠隐性脊柱裂的发生。腰椎对丙戊酸钠的致畸作用最敏感，隐性脊柱裂多发生在腰骶段。过量的维甲酸直接影响神经上皮细胞、神经嵴细胞、原基器官间质细胞的生化过程和尾端未分阶段的中胚层，导致胚胎尾端发育不良综合症复杂畸形出现。丙戊酸钠和维甲酸通过不同机制导致畸形的发生。     

Natural rubber latex allergy: prevalence and risk factors in patients with spina bifida compared with atopic children and controls

Type 1 allergy against natural rubber latex is an increasing problem in health care workers and children with spina bifida or urogenital malformations. The aim of our study was to evaluate the prevalence of latex IgE antibodies and cross-reacting fruit antibodies in patients with spina bifida compared with atopic and non-atopic controls. Risk factors for sensitization should be determined. Sera of 148 patients with spina bifida and 98 controls (44 with atopy) were screened for IgE antibodies against latex, banana and kiwi by fluorescence enzyme immunoassay (CAP system). Atopies, allergic symptoms after latex contacts and the number of operations were compiled by a questionnaire. Patients with spina bifida developed latex IgE antibodies (≥0.7 kU/l) more frequently (40.5%) than atopic children (11.4%) or healthy controls (1.9%). All 18 symptomatic patients belonged to the spina bifida group and had high values of latex antibodies. The risk for developing latex antibodies increases with the number of operations. There was no difference in the history of atopic diseases and in a screening test of IgE antibodies against inhalative allergens between latex sensitized and not sensitized children with spina bifida. Antibodies against banana were more frequent in the latex sensitized children with spina bifida. (18.3% vs 3.4%, P = 0.002). Conclusion The high prevalence of latex antibodies in children with spina bifida justifies a primary prophylaxis by avoiding latex contacts, especially during anaesthesia and surgery, a correlation between the number of operations and the development of latex antibodies exists. Received: 30 March 1996 and in revised form: 19 March 1997 / Accepted: 20 March 1997