Literature DB >> 31435537

Safety and Efficacy of Treatment for Scolios Is Secondary to Spinal Muscular Atrophy Fused to Lumbar 5 Level.

Shoichiro Takei1, Masayuki Miyagi1, Wataru Saito1, Takayuki Imura1, Gen Inoue1, Toshiyuki Nakazawa1, Eiki Shirasawa1, Kentaro Uchida1, Tsutomu Akazawa2, Naonobu Takahira1, Masashi Takaso1.   

Abstract

INTRODUCTION: Patients with spinal muscular atrophy (SMA) usually have progressive scoliosis. Although fusion of the sacrum or pelvis has been recommended for correcting pelvic obliquity (PO), the procedure is invasive. This study determined as to whether performing instrumentation to the fifth lumbar vertebra (L5) is safe and effective for scoliosis in patients with SMA.
METHODS: Twelve patients with SMA underwent posterior spinal fusion and stopping instrumentation at the L5 level. We evaluated age at surgery, the duration of surgery, blood loss, complications, preoperative and postoperative Cobb angles, and PO.
RESULTS: The mean age at surgery was 11.4 years; the mean duration of surgery was 319 minutes, and the mean blood loss was 1170 mL. The Cobb angle improved from 97.3° to 39.1° at 1 month postoperatively (correction rate, 60.9%) and to 42.3° at the final follow-up. PO was corrected from 27.8° to 13.1° at 1 month postoperatively (correction rate, 51.7%) and to 19.8° at the final follow-up. No complications were reported. All patients showed improvement in low back pain, with reduced difficulty while sitting. However, >10% correction loss of PO was observed in 6 patients with high preoperative PO.
CONCLUSIONS: The correction rate of scoliosis in SMA patients with posterior spinal fusion and instrumentation to the L5 level was acceptable, and no complications occurred. Scoliosis associated with SMA was more rigid and severer than scoliosis associated with Duchenne muscular dystrophy. Correction rates of the Cobb angle and PO in SMA patients with instrumentation to L5 were similar to those in SMA patients with instrumentation to the sacrum or pelvis. Correction loss of PO was greater in patients with high preoperative PO than in those with low preoperative PO. Instrumentation and fusion to L5 for scoliosis in patients with SMA seems safe and effective, except in cases of high preoperative PO.

Entities:  

Keywords:  Cobb angle; Correction loss; Neuromuscular scoliosis; Pelvic obliquity; Posterior spinal fusion surgery; Spinal muscular atrophy

Year:  2018        PMID: 31435537      PMCID: PMC6690103          DOI: 10.22603/ssrr.2017-0093

Source DB:  PubMed          Journal:  Spine Surg Relat Res        ISSN: 2432-261X


  22 in total

1.  Two-year results for scoliosis secondary to Duchenne muscular dystrophy fused to lumbar 5 with segmental pedicle screw instrumentation.

Authors:  Masashi Takaso; Toshiyuki Nakazawa; Takayuki Imura; Takamitsu Okada; Masahiro Toyama; Masaki Ueno; Kensuke Fukushima; Wataru Saito; Atsushi Minatani; Gennyo Miyajima; Michinari Fukuda; Naonobu Takahira; Kazuhisa Takahashi; Masashi Yamazaki; Seiji Ohtori; Hirotsugu Okamoto; Toshiyuki Okutomi; Makito Okamoto; Takashi Masaki
Journal:  J Orthop Sci       Date:  2010-04-01       Impact factor: 1.601

2.  The treatment of scoliosis in muscular dystrophy using modified Luque and Harrington-Luque instrumentation.

Authors:  G Bentley; F Haddad; T M Bull; D Seingry
Journal:  J Bone Joint Surg Br       Date:  2001-01

3.  Operative treatment of scoliosis in proximal spinal muscular atrophy: results of 41 patients.

Authors:  Albert Fujak; Wolfgang Raab; Alexander Schuh; Alexander Kreß; Raimund Forst; Jürgen Forst
Journal:  Arch Orthop Trauma Surg       Date:  2012-10-04       Impact factor: 3.067

4.  Comparison between Operated Muscular Dystrophy and Spinal Muscular Atrophy Patients in terms of Radiological, Pulmonary and Functional Outcomes.

Authors:  Hyon Su Chong; Eun Su Moon; Hak Sun Kim; Nanda Ankur; Jin Oh Park; Jin Young Kim; Phillip Anthony B Kho; Seong Hwan Moon; Hwan Mo Lee; Nam Hun Seul
Journal:  Asian Spine J       Date:  2010-11-24

5.  Posterior spinal fusion for scoliosis in duchenne muscular dystrophy diminishes the rate of respiratory decline.

Authors:  Maria V Velasco; Andrew A Colin; David Zurakowski; Basil T Darras; Frederic Shapiro
Journal:  Spine (Phila Pa 1976)       Date:  2007-02-15       Impact factor: 3.468

Review 6.  Spinal muscular atrophy.

Authors:  Mitchell R Lunn; Ching H Wang
Journal:  Lancet       Date:  2008-06-21       Impact factor: 79.321

7.  Pelvic or lumbar fixation for the surgical management of scoliosis in duchenne muscular dystrophy.

Authors:  Dilip K Sengupta; S H Mehdian; Jeffrey R McConnell; Stephen M Eisenstein; John K Webb
Journal:  Spine (Phila Pa 1976)       Date:  2002-09-15       Impact factor: 3.468

8.  The effect of posterior spinal fusion on respiratory function in Duchenne muscular dystrophy.

Authors:  W M Alexander; M Smith; B J C Freeman; L M Sutherland; J D Kennedy; P J Cundy
Journal:  Eur Spine J       Date:  2012-11-20       Impact factor: 3.134

9.  Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III.

Authors:  S Y Chng; Y Q Wong; J H Hui; H K Wong; H T Ong; D Y Goh
Journal:  J Paediatr Child Health       Date:  2003-12       Impact factor: 1.954

10.  Premature termination mutations in exon 3 of the SMN1 gene are associated with exon skipping and a relatively mild SMA phenotype.

Authors:  V Sossi; A Giuli; T Vitali; F Tiziano; M Mirabella; A Antonelli; G Neri; C Brahe
Journal:  Eur J Hum Genet       Date:  2001-02       Impact factor: 4.246

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  2 in total

1.  Quality of life of children with spinal muscular atrophy and their caregivers from the perspective of caregivers: a Chinese cross-sectional study.

Authors:  Mei Yao; Ying Ma; Ruiying Qian; Yu Xia; Changzheng Yuan; Guannan Bai; Shanshan Mao
Journal:  Orphanet J Rare Dis       Date:  2021-01-06       Impact factor: 4.123

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

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
Journal:  Spine Surg Relat Res       Date:  2020-08-31
  2 in total

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