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对于不再进行延长的非行走型脊髓性肌萎缩症患儿且使用生长棒的情况,是否有必要进行预防性的正式融合并翻修植入物?

Is prophylactic formal fusion with implant revision necessary in non-ambulatory children with spinal muscular atrophy and growing rods who are no longer lengthened?

作者信息

Hanna Rewais, Sharafinski Mark, Patterson Karen, Noonan Kenneth J, Sund Sarah, Schultz Meredith, Schroth Mary K, Hetzel Scott, Halanski Matthew A

机构信息

School of Medicine and Public Health, University of Wisconsin-Madison, Madison, WI, USA.

AveXis, Inc., Bannockburn, IL, USA.

出版信息

Spine Deform. 2020 Jun;8(3):547-552. doi: 10.1007/s43390-020-00077-6. Epub 2020 Feb 24.

Abstract

STUDY DESIGN

Single center, retrospective chart review.

OBJECTIVES

To determine if routine posterior spinal fusion (PSF) is unnecessary in non-ambulatory growing rod graduates with SMA. Most non-ambulatory children with SMA develop early-onset scoliosis (EOS). Posterior growing rods (GR) have been shown safe and effective in managing spinal deformities in these children. The best management of these children, once graduated from their GR, is currently unknown. In this study, we report the clinical results of managing these children without routine definitive fusion following a course of GR treatment.

METHODS

A single-center, retrospective chart and radiographic review was performed on children with SMA treated with posterior distraction GR, with a two-year minimum follow-up since final lengthening. Electronic medical records and radiographs were reviewed for demographic variables, Cobb measurements, implant revisions, occult radiographic implant failure, symptomatic failure, and/or conversion to PSF.

RESULT

12 patients (2 type 1, 9 type 2, 1 type 1/2) met inclusion criteria. Mean age at growing rod insertion was 6.2 years of age (range 4.1-8.2) and age at final lengthening 10.3 years of age (range 9.3-11.9). The mean time between last lengthening and latest clinical or radiographic review was 5.5 (range 2.1-9.0) years. Average mean pre, post, final Cobb angles were 71°, 27° (p < 0.001), 25°. Following final lengthening, only one patient required hardware revision and conversion to definitive fusion in attempts to alleviate chronic hip pain, which was unsuccessful. One additional patient was found to have an occult rod failure that has not required treatment.

CONCLUSION

While limited by sample size, this single-center cohort of non-ambulatory SMA patients with EOS treated with similar constructs suggests that routine, definitive fusion in SMA GR graduates may be unnecessary.

LEVEL OF EVIDENCE

Level IV.

摘要

研究设计

单中心回顾性病历审查。

目的

确定对于非行走型生长棒治疗后毕业的脊髓性肌萎缩症(SMA)患者,常规后路脊柱融合术(PSF)是否不必要。大多数非行走型SMA儿童会发展为早发性脊柱侧弯(EOS)。后路生长棒(GR)已被证明在治疗这些儿童的脊柱畸形方面是安全有效的。目前尚不清楚这些儿童在生长棒治疗毕业之后的最佳治疗方案。在本研究中,我们报告了在GR治疗疗程后不进行常规确定性融合来治疗这些儿童的临床结果。

方法

对接受后路撑开GR治疗的SMA儿童进行单中心回顾性病历和影像学审查,自最后一次延长手术起至少随访两年。查阅电子病历和X光片,了解人口统计学变量、Cobb角测量、植入物翻修、隐匿性影像学植入物失败、症状性失败和/或转为PSF的情况。

结果

12例患者(2例1型,9例2型,1例1/2型)符合纳入标准。生长棒植入时的平均年龄为6.2岁(范围4.1 - 8.2岁),最后一次延长手术时的年龄为10.3岁(范围9.3 - 11.9岁)。最后一次延长手术与最新临床或影像学审查之间的平均时间为5.5年(范围2.1 - 9.0年)。术前、术后、最终的平均Cobb角分别为71°、27°(p < 0.001)、25°。最后一次延长手术后,只有1例患者因试图缓解慢性髋部疼痛而需要进行硬件翻修并转为确定性融合,但未成功。另外1例患者被发现有隐匿性棒材失败,但无需治疗。

结论

虽然受样本量限制,但这个单中心队列中接受类似结构治疗的非行走型EOS - SMA患者表明,对于SMA生长棒治疗毕业的患者,常规确定性融合可能不必要。

证据级别

四级。

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