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脊髓脊膜膨出的产前修复与学龄期的功能结局

Prenatal Repair of Myelomeningocele and School-age Functional Outcomes.

机构信息

Department of Physical Medicine and Rehabilitation, University of Pittsburgh, Pittsburgh, Pennsylvania.

Biostatistics Center, Milken Institute School of Public Health, The George Washington University, Washington, District of Columbia;

出版信息

Pediatrics. 2020 Feb;145(2). doi: 10.1542/peds.2019-1544.

Abstract

BACKGROUND AND OBJECTIVES

The Management of Myelomeningocele Study (MOMS), a randomized trial of prenatal versus postnatal repair for myelomeningocele, found that prenatal surgery resulted in reduced hindbrain herniation and need for shunt diversion at 12 months of age and better motor function at 30 months. In this study, we compared adaptive behavior and other outcomes at school age (5.9-10.3 years) between prenatal versus postnatal surgery groups.

METHODS

Follow-up cohort study of 161 children enrolled in MOMS. Assessments included neuropsychological and physical evaluations. Children were evaluated at a MOMS center or at a home visit by trained blinded examiners.

RESULTS

The Vineland composite score was not different between surgery groups (89.0 ± 9.6 in the prenatal group versus 87.5 ± 12.0 in the postnatal group; = .35). Children in the prenatal group walked without orthotics or assistive devices more often (29% vs 11%; = .06), had higher mean percentage scores on the Functional Rehabilitation Evaluation of Sensori-Neurologic Outcomes (92 ± 9 vs 85 ± 18; < .001), lower rates of hindbrain herniation (60% vs 87%; < .001), had fewer shunts placed for hydrocephalus (49% vs 85%; < .001) and, among those with shunts, fewer shunt revisions (47% vs 70%; = .02) than those in the postnatal group. Parents of children repaired prenatally reported higher mean quality of life scores (0.15 ± 0.67 vs 0.11 ± 0.73; = .008) and lower mean family impact scores (32.5 ± 7.8 vs 37.0 ± 8.9; = .002).

CONCLUSIONS

There was no significant difference between surgery groups in overall adaptive behavior. Long-term benefits of prenatal surgery included improved mobility and independent functioning and fewer surgeries for shunt placement and revision, with no strong evidence of improved cognitive functioning.

摘要

背景与目的

《脊髓脊膜膨出管理研究》(MOMS)是一项比较胎儿期与出生后修复脊髓脊膜膨出的随机临床试验,该研究发现胎儿期手术可减少小脑蚓部疝出和 12 月龄时分流管改道的需求,并改善 30 月龄时的运动功能。在这项研究中,我们比较了胎儿期与出生后手术组之间的学龄期(5.9-10.3 岁)适应性行为和其他结局。

方法

对 MOMS 中入组的 161 名儿童进行随访队列研究。评估包括神经心理学和身体评估。由经过培训的盲法检查者在 MOMS 中心或家庭访视中对儿童进行评估。

结果

手术组之间的维兰尼综合评分无差异(胎儿组为 89.0±9.6,出生后组为 87.5±12.0; =.35)。胎儿组中更经常无需矫形器或辅助装置行走(29% vs 11%; =.06),功能性康复评估的感觉-运动结果的平均百分比评分更高(92±9 比 85±18; <.001),小脑蚓部疝出发生率更低(60% vs 87%; <.001),放置用于治疗脑积水的分流管更少(49% vs 85%; <.001),且在需要分流管的儿童中,分流管修订术更少(47% vs 70%; =.02)。接受胎儿期手术的儿童的父母报告更高的平均生活质量评分(0.15±0.67 比 0.11±0.73; =.008)和更低的家庭影响评分(32.5±7.8 比 37.0±8.9; =.002)。

结论

手术组之间总体适应性行为无显著差异。胎儿期手术的长期益处包括提高活动能力和独立功能,减少分流管放置和修订术,而认知功能无明显改善的有力证据。

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本文引用的文献

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The Management of Myelomeningocele Study: full cohort 30-month pediatric outcomes.脊髓脊膜膨出管理研究:全队列 30 个月儿科结局。
Am J Obstet Gynecol. 2018 Feb;218(2):256.e1-256.e13. doi: 10.1016/j.ajog.2017.12.001. Epub 2017 Dec 12.
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Spina bifida.脊柱裂。
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Fetal surgery for myelomeningocele: progress and perspectives.胎儿手术治疗脊髓脊膜膨出:进展与展望。
Dev Med Child Neurol. 2012 Jan;54(1):8-14. doi: 10.1111/j.1469-8749.2011.04049.x. Epub 2011 Jul 11.
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A randomized trial of prenatal versus postnatal repair of myelomeningocele.随机试验:产前与产后修复脊髓脊膜膨出。
N Engl J Med. 2011 Mar 17;364(11):993-1004. doi: 10.1056/NEJMoa1014379. Epub 2011 Feb 9.

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