Surgical Treatment of Severe Spine Deformity in Patients with Spinal Muscular Atrophy

Two-step surgical treatment was conducted in a 17 year old woman with extremely severe kyphoscoliotic deformity on the background of spinal muscular atrophy. At first step a ring of haloapparatus was assembled and gradual halotraction was performed in an armchair within 21 days. Second step included dorsal correction and stabilization of scoliosis by hybrid fixation system at Th3-L4 level with pelvis fixation. As a result of surgical intervention a proper trunk balance was formed, pelvic deformity was diminished, selfservice and use of wheelchair in a sitting position was improved.

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Surgical Treatment for Severe Decompensated Neuromuscular Scoliosis in Patient with Spinal Muscular Atrophy type II

N N Priorov Journal of Traumatology and Orthopedics ◽

10.32414/0869-8678-2017-1-73-76 ◽

2017 ◽

pp. 73-76

Author(s):

A. N. Baklanov ◽

A. N. Shaboldin ◽

B. Yu. Barchenko

Keyword(s):

Surgical Treatment ◽

Spinal Muscular Atrophy ◽

Surgical Intervention ◽

Muscular Atrophy ◽

Type Ii ◽

Dorsal Approach ◽

Spinal Muscular Atrophy Type ◽

One Step ◽

Trunk Balance

Treatment results for 16 years old patient with grade 4 thoracolumbar scoliosis on the background of Type II spinal muscular atrophy are presented. One step surgical treatment via dorsal approach was performed: posterior corrective two-rodtranspedicular corporal screw C7 - S1 fusion with fixationof iliac bones by transpedicular system with bone autoplasty + osteomatrix. Surgical intervention resulted in the formation of satisfactory trunk balance, reduction of pelvic distortion and improvement of the patient’s quality of life.

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Surgical Treatment for Severe Decompensated Neuromuscular Scoliosis in Patient with Spinal Muscular Atrophy type II

N.N. Priorov Journal of Traumatology and Orthopedics ◽

10.17816/vto201724173-76 ◽

2017 ◽

Vol 24 (1) ◽

pp. 73-76

Author(s):

A. N Baklanov ◽

A. N Shaboldin ◽

B. Yu Barchenko

Keyword(s):

Surgical Treatment ◽

Spinal Muscular Atrophy ◽

Surgical Intervention ◽

Muscular Atrophy ◽

Type Ii ◽

Dorsal Approach ◽

Spinal Muscular Atrophy Type ◽

One Step ◽

Trunk Balance

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Are Magnetically Controlled Growing Rods a Good Option for Collapsing Spine Deformity in Spinal Muscular Atrophy Type-2 Associated Early Onset Scoliosis?

Spine Deformity ◽

10.1016/j.jspd.2018.09.045 ◽

2018 ◽

Vol 6 (6) ◽

pp. 811

Author(s):

Senol Bekmez ◽

Alper Yataganbaba ◽

Z. Deniz Olgun ◽

H. Gokhan Demirkiran ◽

Muharrem Yazici

Keyword(s):

Spinal Muscular Atrophy ◽

Early Onset ◽

Muscular Atrophy ◽

Good Option ◽

Early Onset Scoliosis ◽

Spine Deformity ◽

Spinal Muscular Atrophy Type ◽

Growing Rods ◽

Magnetically Controlled Growing Rods

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Children With Spinal Muscular Atrophy With Prior Growth-Friendly Spinal Implants Have Better Results After Definite Spinal Fusion in Comparison to Untreated Patients

Neurosurgery ◽

10.1093/neuros/nyaa053 ◽

2020 ◽

Vol 87 (5) ◽

pp. 910-917 ◽

Cited By ~ 2

Author(s):

Anna K Hell ◽

Lena Braunschweig ◽

Konstantinos Tsaknakis ◽

Urs von Deimling ◽

Katja A Lüders ◽

...

Keyword(s):

Surgical Treatment ◽

Spinal Muscular Atrophy ◽

Spinal Fusion ◽

Muscular Atrophy ◽

Pelvic Obliquity ◽

Prior Treatment ◽

Childhood And Adolescence ◽

Scoliotic Curve ◽

Spinal Implants ◽

Dorsal Spondylodesis

Abstract BACKGROUND Almost all children with spinal muscular atrophy (SMA) develop a scoliosis during childhood and adolescence. In the last decades, growth-friendly spinal implants have been established as an interim solution for these patients until definite spinal fusion can be performed. The effect of those implants on the final outcome has yet to be described. OBJECTIVE To assess the effect of prior growth-friendly spinal surgical treatment on the outcome after spinal fusion in SMA children in comparison to untreated SMA patients through the prospective study. METHODS A total of 28 SMA patients with (n = 14) and without (n = 14) prior surgical treatment with growth-friendly implants were included. Average surgical treatment prior to definite spinal fusion was 4.9 yr. Scoliotic curve angle, pelvic obliquity, spinal length, kyphosis, and lordosis were evaluated for children with prior treatment and before and after dorsal spondylodesis for all children. RESULTS The curve angle before definite spinal fusion averaged at 104° for SMA patients without prior treatment and 71° for patients with prior treatment. Spondylodesis reduced the scoliotic curve to 50° and 33°, respectively, which equals a correction of 52% vs 54%. Pelvic obliquity could be improved by spinal fusion in all patients with better results in the pretreated group. Results for spinal length, kyphosis, and lordosis were similar in both groups. CONCLUSION These data show the positive effect of prior growth-friendly surgical treatment on radiographic results of spinal fusion in children with SMA. Both scoliotic curve angles and pelvic obliquity showed significantly better values when patients had growth-friendly implants before definite spinal fusion.

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Spine Deformity in Spinal Muscular Atrophy

The Journal of Bone and Joint Surgery (American) ◽

10.2106/jbjs.f.00293 ◽

2007 ◽

Vol 89 (suppl 1) ◽

pp. 148-154 ◽

Cited By ~ 21

Author(s):

Daniel J. Sucato

Keyword(s):

Spinal Muscular Atrophy ◽

Muscular Atrophy ◽

Spine Deformity

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Surgical Correction of Spine Deformity in Patients with Spinal Muscular Atrophy: Multicenter Analysis of Domestic Experience and Literature Review

N.N. Priorov Journal of Traumatology and Orthopedics ◽

10.17816/vto20172436-13 ◽

2017 ◽

Vol 24 (3) ◽

pp. 6-13

Author(s):

Sergey O. Ryabykh ◽

E. Yu Filatov ◽

D. M Savin ◽

P. V Ochirova ◽

T. V Ryabykh ◽

...

Keyword(s):

Spinal Muscular Atrophy ◽

Muscular Atrophy ◽

Life Quality ◽

Frontal Plane ◽

Axial Skeleton ◽

Postoperative Management ◽

High Risk Group ◽

Surgical Correction ◽

Spine Deformity ◽

Multicenter Analysis

Purpose: to analyze the domestic experience in surgical correction of spine deformity in patients with spinal muscular atrophy (SMA). Patients and methods. Retrospective multicenter nonrandomized study (evidentiary level III) was performed. Treatment results were analyzed for 26 patients aged 6 - 25 years who were operated on at four RF centers. Inclusion criteria: patients with neurogenic scoliosis on a background of SMA, genetically confirmed diagnosis of type II and III SMA, frontal-plane Cobb angle over 40°, availability of radiologic archives. Results. Preoperative scoliotic curve ranged from 40° to 135° (mean 92°), postoperatively - from 10° to 92° (mean 52°). Correction index varied within 13-75% (mean 40%). Frontal balance correction made up 23%, frontal pelvic tilt - 17%. Indices of external expiration functions showed a slight increase. Functional status by GMFCS was determined preoperatively as class 4 in 2 (8.3%) and class 5 in 24 (91.7%) patients; after intervention as class 10 (38.5%) and 16 (61.5%) patients, respectively. Conclusion. Patients with axial skeleton deformity on a background of SMA are at high risk group and require multidisciplinary examination and peri/postoperative management. Surgical correction is indicated in decompensated deformity over 40°. Surgical rehabilitation improves the self-care of patients as well as the life quality of both the patients and their surroundings.

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