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孕期脊柱闭合不全的儿童脊髓发育不良的尿动力学特征

The urodynamic profile of myelodysplasia in childhood with spinal closure during gestation.

作者信息

Holzbeierlein J, Pope JC I V, Adams M C, Bruner J, Tulipan N, Brock J W

机构信息

Divisions of Pediatric Urology, Pediatric Neurosurgery and Maternal-Fetal Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

出版信息

J Urol. 2000 Oct;164(4):1336-9.

PMID:10992409
Abstract

PURPOSE

Spinal dysraphism is the most common cause of neurogenic bladder dysfunction in newborns. Urodynamic findings in these patients include uninhibited bladder contractions, bladder areflexia, decreased compliance and detrusor-sphincter dyssynergia. Early urodynamic studies are recommended for spina bifida to help identify bladder characteristics that may cause a risk of upper tract deterioration. We recently evaluated a new early type of intervention involving closure of the neural tube defect during gestation in 25 patients at our institution. We hypothesize that this procedure results in decreased exposure of the spinal cord to amniotic fluid, which may improve neurological function. To date we have evaluated 16 of the 25 patients with video urodynamics. We compared the results to those in the literature on patients with myelomeningocele and without prenatal intervention.

MATERIALS AND METHODS

We performed urodynamic testing in 16 patients with a mean age of 6.5 months, including cystometrography, fluoroscopic evaluation of filling and voiding, pelvic floor electromyography and post-void residual urine measurement. In addition, we retrospectively reviewed renal ultrasound, voiding cystourethrography, catheterization need, number of urinary tract infections and medication in these cases.

RESULTS

Uninhibited detrusor contractions and an areflexic bladder were identified in 6% and 43% of patients, respectively, while 19% had decreased compliance and 75% had leak point pressure greater than 40 cm. water. Mean bladder capacity was 40 cc and 31% of patients had much lower capacity than expected for age. Previous renal ultrasound and voiding cystourethrography showed evidence of upper tract dilatation and reflux in 2 cases, respectively. Intermittent catheterization and anticholinergic therapy were required by 1 patient each and 1 had a significant urinary tract infection.

CONCLUSIONS

Urodynamic findings in this population are comparable to those previously reported in the literature in patients with spina bifida without prenatal closure of the spinal defect. The lower incidence of urinary tract infection and reflux in our study probably represents more aggressive early urological management rather than neurological improvement. These urodynamic studies were performed early in life and future evaluation may ultimately reveal improved bladder function compared with that in others with myelodysplasia. However, at this time we recommend that patients who undergo spinal cord defect closure during gestation be evaluated and treated in the same manner as those with myelomeningocele but without fetal intervention.

摘要

目的

脊髓发育不良是新生儿神经源性膀胱功能障碍最常见的原因。这些患者的尿动力学检查结果包括膀胱无抑制性收缩、膀胱无反射、顺应性降低以及逼尿肌-括约肌协同失调。建议对脊柱裂患者尽早进行尿动力学检查,以帮助确定可能导致上尿路恶化风险的膀胱特征。我们最近在本机构对25例患者评估了一种新的早期干预措施,即在孕期闭合神经管缺陷。我们推测该手术可减少脊髓与羊水的接触,这可能改善神经功能。迄今为止,我们已对25例患者中的16例进行了影像尿动力学检查。我们将结果与文献中关于脊髓脊膜膨出且未进行产前干预患者的结果进行了比较。

材料与方法

我们对16例平均年龄为6.5个月的患者进行了尿动力学检查,包括膀胱容量测定、充盈和排尿的荧光透视评估、盆底肌电图检查以及排尿后残余尿量测量。此外,我们回顾性分析了这些病例的肾脏超声、排尿性膀胱尿道造影、导尿需求、尿路感染次数及用药情况。

结果

分别有6%和43%的患者出现膀胱逼尿肌无抑制性收缩和无反射性膀胱,19%的患者顺应性降低,75%的患者漏尿点压力大于40 cm水柱。平均膀胱容量为40 cc,31%的患者容量远低于预期年龄。之前的肾脏超声和排尿性膀胱尿道造影分别显示2例存在上尿路扩张和反流。分别有1例患者需要间歇性导尿和抗胆碱能治疗,1例发生严重尿路感染。

结论

该人群的尿动力学检查结果与文献中先前报道的未进行产前脊柱缺陷闭合的脊柱裂患者的结果相当。我们研究中尿路感染和反流的发生率较低,这可能更多地代表了更积极的早期泌尿外科管理,而非神经功能改善。这些尿动力学检查在患者生命早期进行,与其他脊髓发育异常患者相比,未来的评估可能最终显示膀胱功能有所改善。然而,目前我们建议对孕期进行脊髓缺陷闭合的患者,应与未进行胎儿干预的脊髓脊膜膨出患者一样进行评估和治疗。

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Fetal Surgery for Myelomeningocele: Neurosurgical Perspectives.脊髓脊膜膨出的胎儿手术:神经外科视角
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Urological Outcome after Fetal Spina Bifida Repair: Data from the Zurich Cohort.胎儿脑脊膜膨出修复术后的泌尿系统结局:苏黎世队列研究数据。
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