A new look at myelomeningoceles: Functional level, vertebral level, shunting, and the implications for fetal intervention

A new look at myelomeningoceles: Functional level, vertebral level, shunting, and the implications for fetal intervention
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DOI:
10.1542/peds.109.3.409
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发表时间:
2002-03-01
期刊:
影响因子:
8
通讯作者:
Adzick, NS
Adzick, NS
中科院分区:
医学2区
文献类型:
--
作者:
Rintoul, NE;Sutton, LN;Adzick, NS

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客观的。先前的报告表明,80% 至 85% 患有脊髓脊膜膨出 (MMC) 并在出生后接受手术修复的患者会出现脑积水,需要放置脑室分流术。然而,分流率与脊柱水平的函数关系尚未明确。我们试图通过功能和放射学评估来确定出生后修复的 MMC 病变的分布,以及根据这两种方法对患者进行分类时的分流发生率。方法。对 297 名出生时患有开放性 MMC 并在费城儿童医院脊柱裂诊所进行随访的患者进行了回顾性图表审查。确定每个患者是否存在分流。功能性脊柱水平通过最佳记录的神经系统检查确定,椎体水平通过脊柱射线照片确定。结果。总体心室分流率为81%。病变的水平显着影响分流的发生率,头侧病变越多,分流率越高。对于功能分类和放射学分类都是如此。当按放射学标准而不是功能标准进行分类时,发现骶骨病变患者的分流率明显更高。 86% 的患者的功能水平等于或高于(更差)放射学水平。结论。本研究描述了 MMC 患者心室分流的自然史与放射学和功能标准的关系。一些中心正在进行胎儿 MMC 闭合术,试图减少分流的发生率并改善选定患者的腿部功能。目前的数据可以作为对照组,并有助于设计和分析前瞻性试验,以评估这一新程序的功效。
Objective. Previous reports have suggested that 80% to 85% of patients who have a myelomeningocele (MMC) and undergo surgical repair after birth develop hydrocephalus and require the placement of a ventricular shunt. However, the rate of shunting as a function of spinal level is not well established. We sought to determine the distribution of postnatally repaired MMC lesions as characterized by both functional and radiologic assessment, as well as the incidence of shunting when patients were categorized according to these 2 methods.Methods. A retrospective chart review of 297 patients who were born with open MMCs and followed in the spina bifida clinic at the Children's Hospital of Philadelphia was performed. The presence or absence of a shunt was determined for each patient. Functional spinal level was determined by the best-recorded neurologic examination and vertebral level by spine radiographs.Results. The overall rate of ventricular shunting was 81%. The level of the lesion significantly affected the incidence of shunting, with more cephalad lesions correlating with higher rates. This was true both for functional and radiologic categorizations. A significantly higher shunt rate was found among patients with sacral lesions when categorized by radiologic rather than functional criteria. In 86% of patients, the functional level was found to be equal to or higher (worse) than the radiologic level.Conclusions. This study describes the natural history of ventricular shunting in MMC patients with relation to both radiologic and functional criteria. Fetal MMC closure is being performed in some centers in an attempt to decrease the incidence of shunting and to improve leg function in selected patients. The present data may serve as a comparison group and aid in the design and analysis of a prospective trial to assess the efficacy of this new procedure.