Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was Planned

Machida, Shuhei; Miyagi, Masayuki; Saito, Wataru; Matsui, Ayano; Imura, Takayuki; Inoue, Gen; Nakazawa, Takahiro; Shirasawa, Eiki; Ikeda, Shinsuke; Kawakubo, Ayumu; Kuroda, Akiyoshi; Yokozeki, Yuji; Mimura, Yusuke; Uchida, Katsuhisa; Akazawa, Tsutomu; Takaso, Masashi

doi:10.22603/ssrr.2020-0091

Cited by 6 publications

(3 citation statements)

References 14 publications

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“…When surgical intervention for spinal deformity in patients is required, it is advisable to create a safe and reliable access point for intrathecal infiltration. This can be effectively achieved by performing a lumbar laminotomy or by creating an interlaminar lumbar fenestration at the convex side of the spine [ 61 , 62 , 63 ]. A study by Konigsberg et al recommends using ‘skip’ constructs when considering traditional PSF.…”

Section: Discussionmentioning

confidence: 99%

Spinal Muscular Atrophy Scoliosis in the Era of Background Therapies—A Review of the Literature

Ruythooren,

Moens

2024

JCM

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Spinal deformities are considered an important complication of neuromuscular disorders such as spinal muscular atrophy (SMA). SMA patients typically develop progressive early-onset scoliosis, which is associated with increased functional decline, discomfort, and respiratory dysfunction. Over the second decade of the twenty-first century, a lot has changed in terms of the therapeutic options available to people with SMA. Specifically, the use of pharmaceutical agents such as nusinersen (Spinraza), onasemnogene abeparvovec (Zolgensma), and risdiplam (Evrysdi) has dramatically changed the landscape for SMA patients. These medications significantly alter motor- and respiratory functioning, as well as the natural progression of spinal deformities. When evaluating these agents and their impact on the development of scoliosis and motor functioning, it is important to consider the timing of treatment initiation. In patients treated after they had already developed symptoms, a shift of phenotype to a less severe subtype has been observed. This results in a delay in the onset of scoliosis for the less severe SMA types and an increase in early-onset scoliosis for the severe types in patients who would typically not live to develop scoliosis. Patients who receive treatment before they develop symptoms achieve almost normal motor functioning and will likely have a significant decrease in scoliosis prevalence or at least delay its onset.

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Section: Discussionmentioning

confidence: 99%

Spinal Muscular Atrophy Scoliosis in the Era of Background Therapies—A Review of the Literature

Ruythooren,

Moens

2024

JCM

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“…12 Methods for performing injections through transforaminal approaches [13][14][15] or catheters inserted through drill holes placed in the fusion mass have also been described in patients who had previously undergone fusion. 16 Machida et al 17 combined L3 laminectomy with spinal fusion in the same session to allow access to the intrathecal space. Cervical puncture was also suggested as a feasible method to deliver nusinersen intrathecally in patients with complete spinal fusion.…”

Section: Discussionmentioning

confidence: 99%

Impact of Spinal Surgery on Intrathecal Nusinersen Injections in Pediatric Spinal Muscular Atrophy

Aksoy,

Ramazanov,

Öz Yildiz

et al. 2024

Journal of Pediatric Orthopaedics

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Background: Complex spinal deformities necessitate surgical interventions that may intervene with intrathecal injections in patients with spinal muscular atrophy (SMA). This study aimed to determine the effect of spinal deformity correction surgery on nusinersen administration. Methods: Pediatric patients with SMA, operated by a single surgeon, either via magnetically controlled growing rod (MCGR) or definitive fusion (DF) with skip instrumentation, were evaluated retrospectively in terms of safety and feasibility of intrathecal injections. Patients’ and their parents’ perspectives were evaluated through a questionnaire regarding any shift in the setting of injections. Results: Fourteen patients with 15 spinal surgeries (10 MCGR and 5 DF) were included. Eleven patients received intrathecal treatment both before and after the surgery. Preoperative (n=3) and postoperative (n=9) fluoroscopic guidance was required leading to a shift in the application settings in 6 patients. Of 106 preoperative injections, 15% required fluoroscopy and 2% required anesthesia. Postoperatively, of 88 injections, 73% required fluoroscopy and 26% required anesthesia. No patients discontinued intrathecal injections due to technical difficulties associated with the spinal surgery. Conclusions: This study demonstrates that spinal surgery does not prevent safe and successful intrathecal nusinersen injections. In the DF group, the skip instrumentation technique provided access to interlaminal space for intrathecal injections. In either surgical group, no further auxillary approach was required. Modifications in the injection technique require an institutional approach, and concerns of patients and their families should be addressed accordingly. Level of Evidence: IV

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“…Besides, hemoclips was used as radiographic references to localize the window. Machida and colleagues [ 26 ] combined L3 laminectomy with PSF to create an access for nusinersen administration. A transverse connector was placed at the level of L3 laminectomy as a radiographic marker.…”

Section: Discussionmentioning

confidence: 99%

Unilateral interlaminar fenestration on the convex side provides a reliable access for intrathecal administration of nusinersen in spinal muscular atrophy: a retrospective study

Wang,

Feng,

Jiao

et al. 2023

Orphanet J Rare Dis

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Background As the first gene therapy for spinal muscular atrophy (SMA), nusinersen is supposed to be administrated via intrathecal injection regularly for a lifetime. However, for SMA patients with severe spinal deformities, bony fusion following posterior spinal instrumentation sets great obstacles for the application of nusinersen. Therefore, efforts have been devoted to the exploration of appropriate approach for nusinersen administration. This study aims to evaluate the safety and reliability of unilateral interlaminar fenestration on the convex side during spinal fusion surgery for intrathecal nusinersen injection in SMA. Results SMA patients receiving posterior spinal fusion and interlaminar fenestration in Peking Union Medical College Hospital from January 2020 to October 2021 were retrospectively analyzed. 13 patients were included. Of the 13 patients, 10 were classified into SMA type II and 3 into SMA type III. Distal fusion to pelvis was undertaken in 11 patients; while L5 was selected as the lowest instrumented vertebra in the other 2 patients. All patients received interlaminar fenestration on the convex side only with an area of about 15 mm × 20 mm. Fenestration at L2–L3 level was performed in 6 patients; while L3–L4 level was selected for windowing in the remaining 7 patients. 9 of the 13 patients received lumbar puncture and intrathecal nusinersen administration during the 1-year follow-up, with an accumulative total of 50 times. All injections were performed successfully under ultrasound guidance, with no one transferred to radiographic assistance. No severe complications occurred after injection. Conclusions In SMA with severe scoliosis planning to receive posterior spinal fusion, unilateral lumbar interlaminar fenestration on the convex side provides a feasible and reliable access for intrathecal nusinersen administration after surgery.

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Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was Planned

Cited by 6 publications

References 14 publications

Spinal Muscular Atrophy Scoliosis in the Era of Background Therapies—A Review of the Literature

Spinal Muscular Atrophy Scoliosis in the Era of Background Therapies—A Review of the Literature

Impact of Spinal Surgery on Intrathecal Nusinersen Injections in Pediatric Spinal Muscular Atrophy

Unilateral interlaminar fenestration on the convex side provides a reliable access for intrathecal administration of nusinersen in spinal muscular atrophy: a retrospective study

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