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

Introduction: Spinal muscular atrophy (SMA) is defined as a neuromuscular disorder induced by progressive weakness of the skeletal muscle and is usually accompanied by progressive spinal deformity including scoliosis. The newly developed Nusinersen, which is the first approved drug worldwide for SMA...

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Main Authors: Shuhei Machida, Masayuki Miyagi, Wataru Saito, Ayano Matsui, Takayuki Imura, Gen Inoue, Toshiyuki Nakazawa, Eiki Shirasawa, Shinsuke Ikeda, Ayumu Kawakubo, Akiyoshi Kuroda, Yuji Yokozeki, Yusuke Mimura, Kentaro Uchida, Tsutomu Akazawa, Masashi Takaso
Format: Article
Language:English
Published: The Japanese Society for Spine Surgery and Related Research 2021-03-01
Series:Spine Surgery and Related Research
Subjects:
Online Access:https://www.jstage.jst.go.jp/article/ssrr/5/2/5_2020-0091/_pdf/-char/en
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author Shuhei Machida
Masayuki Miyagi
Wataru Saito
Ayano Matsui
Takayuki Imura
Gen Inoue
Toshiyuki Nakazawa
Eiki Shirasawa
Shinsuke Ikeda
Ayumu Kawakubo
Akiyoshi Kuroda
Yuji Yokozeki
Yusuke Mimura
Kentaro Uchida
Tsutomu Akazawa
Masashi Takaso
author_facet Shuhei Machida
Masayuki Miyagi
Wataru Saito
Ayano Matsui
Takayuki Imura
Gen Inoue
Toshiyuki Nakazawa
Eiki Shirasawa
Shinsuke Ikeda
Ayumu Kawakubo
Akiyoshi Kuroda
Yuji Yokozeki
Yusuke Mimura
Kentaro Uchida
Tsutomu Akazawa
Masashi Takaso
author_sort Shuhei Machida
collection DOAJ
description Introduction: Spinal muscular atrophy (SMA) is defined as a neuromuscular disorder induced by progressive weakness of the skeletal muscle and is usually accompanied by progressive spinal deformity including scoliosis. The newly developed Nusinersen, which is the first approved drug worldwide for SMA, requires accurate intrathecal injection, which is sometimes difficult in patients with severe spinal deformity. Technical Note: For an accurate intrathecal approach in patients who have spinal fusion surgery to treat neuromuscular scoliosis, we have combined an L3 laminectomy with spinal correction and fusion surgery. Here, we review four cases of SMA in patients who underwent the additional L3 laminectomy during surgery to treat spinal scoliosis. A successful intrathecal approach was made using fluoroscopic guidance in all four patients, who were then administered with Nusinersen. Conclusions: Our findings show that additional lumbar laminectomy during surgery for spinal scoliosis has effectively allowed for intrathecal injection of Nusinersen.
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spelling doaj.art-d4b748edc0e2459c9001b27126572b142022-12-21T22:30:19ZengThe Japanese Society for Spine Surgery and Related ResearchSpine Surgery and Related Research2432-261X2021-03-015210911310.22603/ssrr.2020-00912020-0091Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was PlannedShuhei Machida0Masayuki Miyagi1Wataru Saito2Ayano Matsui3Takayuki Imura4Gen Inoue5Toshiyuki Nakazawa6Eiki Shirasawa7Shinsuke Ikeda8Ayumu Kawakubo9Akiyoshi Kuroda10Yuji Yokozeki11Yusuke Mimura12Kentaro Uchida13Tsutomu Akazawa14Masashi Takaso15Department of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, National Center of Neurology and PsychiatryDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityDepartment of Orthopaedic Surgery, School of Medicine, Kitasato UniversityIntroduction: Spinal muscular atrophy (SMA) is defined as a neuromuscular disorder induced by progressive weakness of the skeletal muscle and is usually accompanied by progressive spinal deformity including scoliosis. The newly developed Nusinersen, which is the first approved drug worldwide for SMA, requires accurate intrathecal injection, which is sometimes difficult in patients with severe spinal deformity. Technical Note: For an accurate intrathecal approach in patients who have spinal fusion surgery to treat neuromuscular scoliosis, we have combined an L3 laminectomy with spinal correction and fusion surgery. Here, we review four cases of SMA in patients who underwent the additional L3 laminectomy during surgery to treat spinal scoliosis. A successful intrathecal approach was made using fluoroscopic guidance in all four patients, who were then administered with Nusinersen. Conclusions: Our findings show that additional lumbar laminectomy during surgery for spinal scoliosis has effectively allowed for intrathecal injection of Nusinersen.https://www.jstage.jst.go.jp/article/ssrr/5/2/5_2020-0091/_pdf/-char/enneuromuscular scoliosisspinal muscular atrophynusinersen
spellingShingle Shuhei Machida
Masayuki Miyagi
Wataru Saito
Ayano Matsui
Takayuki Imura
Gen Inoue
Toshiyuki Nakazawa
Eiki Shirasawa
Shinsuke Ikeda
Ayumu Kawakubo
Akiyoshi Kuroda
Yuji Yokozeki
Yusuke Mimura
Kentaro Uchida
Tsutomu Akazawa
Masashi Takaso
Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was Planned
Spine Surgery and Related Research
neuromuscular scoliosis
spinal muscular atrophy
nusinersen
title Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was Planned
title_full Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was Planned
title_fullStr Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was Planned
title_full_unstemmed Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was Planned
title_short Posterior Spinal Correction and Fusion Surgery in Patients with Spinal Muscular Atrophy-Associated Scoliosis for Whom Treatment with Nusinersen Was Planned
title_sort posterior spinal correction and fusion surgery in patients with spinal muscular atrophy associated scoliosis for whom treatment with nusinersen was planned
topic neuromuscular scoliosis
spinal muscular atrophy
nusinersen
url https://www.jstage.jst.go.jp/article/ssrr/5/2/5_2020-0091/_pdf/-char/en
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