脊膜膨出和脊髓脊膜膨出30例分析

脊膜膨出和脊髓脊膜膨出是较常见的先天性畸形。其发病率为新生儿的1/1000～1/5000。病变部位有脊柱裂,硬脊膜经椎板缺损处向外膨出,在皮下形成囊肿,这种畸形可按囊内有无神经组织分为脊髓脊膜膨出和单纯脊膜膨出。我院自1976～1984年收治脊膜膨出(14例)和脊髓脊膜膨出(16例)共30例,均经手术治疗。现分析讨论如下。临床资料本组病例中,男14例,女16例。年龄最小者为出生后20天,最大者7岁,1岁以内22例(73%)。30例中病变部位位于颈段者3例,胸段5例,腰段12例,骶段10例。按膨出囊的形状,一般可分为有蒂型和广基型两种。本组有蒂型者5例,广基型者25例,有蒂型膨出的基底较小,成蒂状,表面的皮肤常属正常,皮下为一层脂肪和纤维组织,里面是膨出的硬脊膜囊,     

新生儿囊性脊柱裂诊断与治疗

囊性脊柱裂是小儿外科常见病。为中枢神经系统的发育畸形。包括以下两种:①脑(脊)膜膨出;②脑(脊)膜、脑(脊神经或脊髓)膨出。一、临床资料我院自1958～1979年共收治小儿囊性脊柱裂564例,其中新生儿33例。大多数是因囊肿较大欲破(13例),糜烂感染(15例)而急症入院手术治疗。其囊肿部位与内容情况如下。     

先天性椎管内畸形分类及手术处理

目的回顾性分析一组椎管内畸形病例的临床资料,探讨此类疾病的诊治策略和治疗效果。方法 2002年3月至2009年3月收治椎管内畸形患者80例,男36例,女44例,年龄1个月至71岁。脊柱裂70例,脊髓拴系综合征55例。脊髓纵裂11例,椎管内脂肪瘤19例。椎管内皮样囊肿5例。脊膜膨出14例,脊髓脊膜膨出20例,脂肪性脊髓脊膜膨出12例。合并有2种或2种以上畸形78例,仅2例为单纯椎管内畸形。所有病例分别按照脊膜膨出、椎管内脂肪瘤、脊髓纵裂的特殊方法进行手术。术前、术后随访均拍摄MRI作为研究资料。采用综合感觉、运动和排尿功能评估及手术操作是否达到目的作为疗效评估手段。结果结合MRI对80例患者进行病理性分析,大部分为脊柱裂合并的畸形。80%脊膜膨出和脊髓拴系病例获得功能改善。而椎管内脂肪瘤、二分脊髓近半数的病理未见明显效果。结论脊柱裂伴发椎管内畸形应早期发现,正确详尽诊断,综合评估,尽早医学介入,严格掌握手术适应证。     

儿童脂肪脊髓脊膜膨出外科治疗的探讨

目的 探讨脂肪脊髓脊膜膨出外科治疗的方法和技巧.方法 自2004年1月至2007年3月共收治脂肪脊髓脊膜膨出25例,年龄2个月～6岁,均行磁共振和肌电图检查,给予手术治疗.并采用Hoffman脂肪脊髓脊膜膨出功能分级进行疗效判定.结果 25例患者切口均一期愈合.随访1～4年,18例术前神经功能正常者,术后神经功能也正常.7例有神经症状者,术后神经症状改善,其中4例术后功能分级提高,另3例功能分级无变化而有症状改善.所有患者无再栓系的形成.结论 脂肪脊髓脊膜膨出采取切除大部脂肪组织,缝合脂肪表面软脊膜,切断终丝等方法和技巧,显著提高了疗效.     

神经外科修复的初始时间对脊髓脊膜膨出所致神经原性膀胱的预后有显著影响

为研究对开放性脊柱裂病患儿初始进行神经外科修复手术的时间与泌尿系预后的关系,作者对土耳其脊柱裂多学科临床中心1996年至2005年401例脊髓脊膜膨出患儿的随访记录进行了研究。脊髓脊膜膨出颈神经水平2例,胸神经3例,腰神经331例,骶神经13例,腰骶神经52例。其中能够获得3岁时     

新生儿先天性脊髓脊膜膨出的手术治疗体会

目的总结新生儿先天性脊髓脊膜膨出的手术治疗体会。方法回顾性分析14例新生儿先天性脊髓脊膜膨出的临床资料。结果 14例手术切口均I期愈合。未发生脑脊液漏等并发症。随访6个月～2年,1例患儿大小便失禁及双下肢运动、感觉障碍均无明显改善。大小便失禁及双下肢运动、感觉障碍较前加重1例,其余患儿恢复良好,未见膨出复发病例。结论早期诊断及手术可有效提高新生儿先天性脊髓脊膜膨出治愈率,减少术后并发症。     

人工体神经-内脏神经反射弧治疗脊髓脊膜膨出患者大小便功能障碍

目的 :建立“人工体神经 内脏神经反射弧”(简称人工反射弧 )治疗脊柱裂脊髓脊膜膨出患者大小便功能障碍。方法 :对 30例大小便功能障碍的脊髓脊膜膨出患者 ,手术建立人工反射弧。进行术前与术后 6～ 1 8个月的尿动力学比较。结果 :30例中 1 3例获得了至少 1年的随访。 7例无反射型患者中 4例获得控尿和自主排尿功能 ,尿失禁消失 ,排尿间隔期逐渐延长至 3h以上 ,逼尿肌压由 (1 .37± 0 .78)kPa(1kPa =0 .0 98cmH2 O)增至(3.1 4± 1 .6 7)kPa ;6例高反射型患者全部于术后 1年左右恢复可控排尿 ,剩余尿逐渐减至 (2 2± 1 5 )ml,充盈性尿失禁消失。 1 0例获得膀胱功能控制者 ,直肠功能转为基本正常。下肢功能损伤较小。结论 :人工反射弧能安全有效地治愈先天性脊髓脊膜膨出所致大小便失禁     

MR在脊柱裂脊膜膨出患者人工反射弧手术中的应用

目的：探讨MR在人工反射弧术前诊断、术后随访中的应用价值。方法：对67例脊柱裂脊膜膨出修补术后大小便功能障碍的患者建立人工体神经-内脏神经反射弧,以修复膀胱直肠功能。参照尿动力学检查结果,对比研究术前、术后MR的改变。结果：67例患者MR术前检查结果均经手术证实。20例患者术后随访2年以上,17例获得控尿和自主排尿功能,其中13例患者用MR检查随访,10例膀胱功能恢复的患者MR检查与术前无明显变化,而3例膀胱功能无改善者脊髓的走行、圆锥位置较术前改变不明显,圆锥末端与周围组织瘢痕增生、脂肪堆积较术前有加重趋势。结论：在应用人工体神经-内脏神经反射弧治疗先天性脊柱裂脊膜膨出所致大小便失禁时,MR对术前诊断、手术方案制定有较高的应用价值;在术后随访时,MR对治疗失败的患者有重要参考价值。     

先天性脊柱侧弯术前MRI检查的应用价值

[目的]探讨MRI在先天性脊柱侧弯术前检查的应用价值。[方法]分析49例先天性脊柱侧弯患者术前MRI影像特征。[结果]其中椎体发育不良25例，椎管发育不良9例，椎板及棘突发育不良11例，脊柱裂5例，椎管内骨嵴4例，脊髓纵裂7例，Chiari氏畸形伴脊髓空洞3例，脊髓栓系综合征7例，椎管内肿瘤3例，脊膜膨出4例。背部皮毛窦1例，[结论]MRI技术的应用，大大提高了对先天性脊柱侧弯诊断的直观性和准确性，对手术治疗有极大的指导作用。     

腰骶部脊髓脊膜膨出伴脂肪瘤的诊断与治疗体会

脊髓脊膜膨出(myelomeningocele,MMC)是胚胎期的神经管闭合发生障碍导致椎板融合不全,脊髓和/或神经根白骨裂处膨出的先天发育畸形,常伴有局部脂肪异常增生,也称脂肪瘤型脊髓脊膜膨出,其临床症状有不同程度的双下肢无力和大小便功能障碍,如不及时治疗或手术方式不当会严重影响患儿的生活质量.2007年1月-2008年5月我们收治18例脊髓脊膜膨出患儿,其中8例伴有脂肪瘤,总结报告如下.     

Spina bifida as an independent risk factor for sensitization to latex.

PURPOSE: Patients with spina bifida are at a high risk for having an immediate type allergy to latex products. The number of surgical interventions, atopy and catheterization are well known responsible factors, whereas the condition of spina bifida per se has not been established as an independent risk factor. MATERIALS AND METHODS: A total of 131 patients with a shunted hydrocephalus (48 with spina bifida and 83 of other origin) were investigated for sensitization to latex by skin prick tests and determination of specific IgE. We hypothesized that the diagnosis of spina bifida will increase the risk for latex sensitization while considering potential confounding factors. Thus, we performed a multiple logistic regression analysis to determine independent risk factors. RESULTS: Whereas 56.3% (27/48) of children with spina bifida proved sensitized against latex, this result was the case in only 16.9% (14/83) with another cause of hydrocephalus (p <0.001). The mean number of surgical interventions was 6.2 for patients with no latex sensitization and 9.3 for those with sensitization (p = 0.02). Of patient sensitized to latex 43.9% had a history of atopy compared to 15.5% of those not sensitized (p = 0.02). Sensitized and nonsensitized patients were comparable regarding gender and catheterization. In a multiple logistic regression analysis the cause of the hydrocephalus (odds ratio 6.76 for spina bifida), atopy (odds ratio 3.37) and the number of surgical interventions (odds ratio 1.14 per operation) were identified as independent risk factors. CONCLUSIONS: The increased risk of latex sensitization in patients with spina bifida seems to be disease associated. Possible explanations for this finding may be genetic, antigen mediated, early latex exposure and immunological reasons.     

Intraspinal epidermoid and dermoid cysts. Surgical results of seven cases

Seven cases of intraspinal inclusion cysts, surgically treated between 1970 and 1984, are reported in this paper. Four were epidermoid cysts and three dermoid. Two cysts were located in the thoracic spine (one dermoid and one epidermoid) and five in the lumbosacral region (three epidermoid and two dermoid). Associated spinal dysraphia was noted in five cases. Congenital dermal sinus was found in two cases, spina bifida cystica in one, spina bifida occulta in one, and diastematomyelia in one case. The average postoperative follow-up period was 5 years. No neurological symptoms were found in the five patients who received early surgical treatment, but for the two patients whose operations were late, there were some residual neurological deficits. Therefore, early and accurate diagnosis and radical surgical extirpation are emphasized.     

An artificial somatic-autonomic reflex pathway procedure for bladder control in children with spina bifida

PURPOSE: Neurogenic bladder is a major problem for children with spina bifida. Despite rigorous pharmacological and surgical treatment, incontinence, urinary tract infections and upper tract deterioration remain problematic. We have previously demonstrated the ability to establish surgically a skin-central nervous system-bladder reflex pathway in patients with spinal cord injury with restoration of bladder storage and emptying. We report our experience with this procedure in 20 children with spina bifida. MATERIALS AND METHODS: All children with spina bifida and neurogenic bladder underwent limited laminectomy and a lumbar ventral root (VR) to S3 VR microanastomosis. The L5 dorsal root was left intact as the afferent branch of the somatic-autonomic reflex pathway after axonal regeneration. All patients underwent urodynamic evaluation before and after surgery. RESULTS: Preoperative urodynamic studies revealed 2 types of bladder dysfunction- areflexic bladder (14 patients) and hyperreflexic bladder with detrusor external sphincter dyssynergia (6). All children were incontinent. Of the 20 patients 17 gained satisfactory bladder control and continence within 8 to 12 months after VR microanastomosis. Of the 14 patients with areflexic bladder 12 (86%) showed improvement. In these cases bladder capacity increased from 117.28 to 208.71 ml, and mean maximum detrusor pressure increased from 18.35 to 32.57 cm H2O. Five of the 6 patients with hyperreflexic bladder demonstrated improvement, with resolution of incontinence. Urodynamic studies in these cases revealed a change from detrusor hyperreflexia with detrusor external sphincter dyssynergia and high detrusor pressure to nearly normal storage and synergic voiding. In these cases mean bladder capacity increased from 94.33 to 177.83 ml, and post-void residual urine decreased from 70.17 to 23.67 ml. Overall, 3 patients failed to exhibit any improvement. CONCLUSIONS: The artificial somatic-autonomic reflex arc procedure is an effective and safe treatment to restore bladder continence and reverse bladder dysfunction for patients with spina bifida.     

Epilepsy in patients with spina bifida in the lumbosacral region

This study aimed to assess the relevance of epilepsy and spina bifida in the lumbosacral region. We evaluated 75 patients with spina bifida admitted to the Kyushu University Hospital from 1980 to 2004. Patients were classified as having meningocele (MC, 4 cases), myelomeningocele (MMC, 6), myeloschisis (MS, 45), and lumbosacral lipoma (LL, 20). Nine cases had epileptic disorders, and all showed MS. Meticulous neuroradiological investigations revealed cerebral abnormalities such as polymicrogyria or hypogenesis of the corpus callosum in all epileptic cases. Locations of cerebral abnormalities topographically correlated with areas of interictal EEG abnormalities. Although all epileptic cases had ventriculoperitoneal (VP) shunt for hydrocephalus before the onset of epilepsy, interictal EEG abnormalities could not be explained by location of the VP shunt. In all LL patients, neither history of epilepsy nor cerebral abnormalities were noted on magnetic resonance imaging (MRI). Epileptogenesis in spina bifida patients seemed to correlate with coexisting cerebral abnormalities in MS patients rather than with the VP shunt. However, not all spina bifida patients associated with cerebral abnormalities had epilepsy, and not all cerebral abnormalities were epileptogenic, suggesting that epilepsy in spina bifida patients was multifactorial.     

Early start to therapy preserves kidney function in spina bifida patients

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.     

Clean intermittent catheterization in the management of spina bifida: a review of 113 cases

One hundred and thirteen cases of spina bifida treated with clean intermittent catheterization (CIC) were reviewed. There were 42 males and 71 females, ranging from 4 months to 50 years. The major reasons for CIC from other voiding methods were vesicoureteral reflux (VUR) (48/113) old, and residual urine (36/113). The most common type of neurogenic bladder was hypoactive detrusor-active sphincter, in 56.6% of patients (64/113). The grade of paralytic disability of lower limb (Sharrard's classification) was low (group IV-VI) in 84 patients and high (group I-III) in 29 patients. Hydroureteronephrosis and VUR improved 52.1% (37/71) and 57.1% (36/63), urinary tract infection and urinary incontinence improved 70.9% (39/55) and 81.7% (72/87). Major complications were pyelonephritis (12 cases) and urethral pain (5 cases). We discussed the choice of voiding method in the management of spina bifida.     

Intraspinal epidermoid and dermoid cysts

Summary Seven cases of intraspinal inclusion cysts, surgically treated between 1970 and 1984, are reported in this paper. Four were epidermoid cysts and three dermoid. Two cysts were located in the thoracic spine (one dermoid and one epidermoid) and five in the lumbosacral region (three epidermoid and two dermoid). Associated spinal dysraphia was noted in five cases. Congenital dermal sinus was found in two cases, spina bifida cystica in one, spina bifida occulta in one, and diastematomyelia in one case. The average postoperative follow-up period was 5 years. No neurological symptoms were found in the five patients who received early surgical treatment, but for the two patients whose operations were late, there were some residual neurological deficits. Therefore, early and accurate diagnosis and radical surgical extirpation are emphasized.

---

Zusammenfassung In diesem Bericht wird eine Serie von sieben Fallen eingeschlossener Cysten in der Wirbelsaule beschrieben, die zwischen 1970 und 1984 behandelt wurden. Vier dieser Cysten waren epidermoid, drei dermoid. Zwei befanden sich im Brustwirbelbereich (eine Epidermoidcyste and eine Dermoid cyste) und fünf in der lumbosakralen Region (drei Epidermoid- und zwei Dermoidcysten). In fünf Fällen wurden hiermit verbundene Disraphismen in der Wirbelsäule festgestellt, ein angeborener Dermalsinus in zwei Fällen, spina bifida cystica, spina bifida occulta und diastematomyelia jeweils in den drei übrigen. Im Durchschnitt betrug die postoperative Nachbehandlung 5 Jahre. Die Rolle der frühen und

     

Intracranial anomalies and cloacal exstrophy — Is there a role for screening?

Background/Purpose

Cloacal exstrophy (CE) is a severe multi-system congenital defect. While spina bifida is a defining feature of cloacal exstrophy, patients are not routinely screened for intracranial anomalies (ICAs). We sought to better characterize this risk of ICA in the CE patient.

Methods

We retrospectively reviewed the medical records of 81 children with CE treated at our institution, identifying intracranial pathology, including hydrocephalus, Chiari malformation and craniosynostosis. Data points included ICA, neural tube defect, surgical procedures, and ambulatory status.

Results

Of the 39 patients with cranial imaging, 31% had an ICA: 6 hydrocephalus, 3 Chiari malformation, 1 craniosynostosis, 1 hydrocephalus and craniosynostosis, and 1 Chiari malformation and craniosynostosis. All patients with ICAs had spina bifida. Patients with ICAs underwent more neurosurgical procedures, including more spinal procedures. Patients with ICAs were much more likely to be wheelchair-bound or ambulate minimally when compared to patients without anomalies.

Conclusion

In our population of CE patients with available head imaging, 31% had an ICA, thus screening would seem prudent. As all patients with ICAs had spina bifida, it may be less important to screen those rare CE patients without spinal pathology. Patients with ICAs were more likely to be wheelchair-bound, suggesting worse neurologic outcomes.     

Association between Latex Sensitization and Repeated Latex Exposure in Children

Background: Children with spina bifida are at greater risk for latex and ethylene oxide sensitization. The authors' aim in this study was to evaluate the role of previous surgical procedures in the development of sensitization to latex and ethylene oxide.

Methods: The authors investigated 80 children 1-16 yr old, separated into 3 groups. Two groups had a history of 3 or more general anesthetics: 29 children had spina bifida (spina bifida group) and 31 had undergone multiple surgeries for another disease (multiple surgeries group). A control group of 20 children had undergone less than 1 anesthetic. Clinical manifestations with latex, perioperative anaphylactic reactions, and number of previous anesthetics were recorded. Skin prick tests with a commercial extract of latex, four common inhalant allergens, and radioallergosorbent test to latex and ethylene oxide were performed.

Results: The three groups did not differ significantly with respect to age, sex, and atopic status. Mean number of anesthetics was comparable in the spina bifida and the multiple surgeries group. Latex sensitization was common in the spina bifida group (59%) and in the multiple surgeries group (55%) but not in the control group (0%, P < 0.05). Ethylene oxide sensitization was significantly more frequent in the spina bifida group than in the multiple surgeries group (44% vs. 19%; P = 0.052) and strongly associated with latex sensitization. Mean number of previous anesthetics was greater in children sensitized to latex (8.4 vs. 3.9; P < 0.05).