Sagittal Plane Deformities in Children with SMA2 following Posterior Spinal Instrumentation

This is a retrospective radiographic review to assess post-operative sagittal plane deformities in patients with Spinal Muscular Atrophy type 2 that had been treated with posterior spinal instrumentation. Thirty-two patients with a history of either spinal fusion (N = 20) or growing rods (N = 12) were identified with an average of 7.6 (2.1–16.6) years post-operative follow-up. Forty percent (13/32) of the patients were identified as having obvious “tucked chin” (N = 4), “tipped trunk” (N = 9), or both (N = 3). Sacral incidence was the only parameter that was statistically significant change between pre-operative or immediate post-operative measurements (66.9° vs. 55.2° p = 0.03). However, at final follow-up, the post-operative thoracic kyphosis had decreased over time in those that developed a subsequent sagittal deformity (24.2°) whereas it increased in those that did not (44.7°, p = 0.008). This decrease in thoracic kyphosis throughout the instrumented levels, resulted in a greater lordotic imbalance (30.4° vs. 5.6°, p = 0.001) throughout the instrumented levels in the group that developed the subsequent cervical or pelvic sagittal deformities. In conclusion, sagittal plane deformities commonly develop outside the instrumented levels in children with SMA type 2 following posterior spinal instrumentation and may be the result of lordotic imbalance that occurs through continued anterior growth following posterior instrumentation.

Download Full-text

Effects of magnetically controlled growing rods surgery on pulmonary function in young subjects with spinal muscular atrophy type 2 and other neuromuscular scoliosis

Journal of Neurosurgical Sciences ◽

10.23736/s0390-5616.17.04052-8 ◽

2020 ◽

Vol 64 (3) ◽

Author(s):

Luca Colombo ◽

Carlotta Martini ◽

Chiara Bersanini ◽

Francesca Izzo ◽

Jorge H. Villafañe ◽

...

Keyword(s):

Pulmonary Function ◽

Spinal Muscular Atrophy ◽

Muscular Atrophy ◽

Neuromuscular Scoliosis ◽

Spinal Muscular Atrophy Type ◽

Growing Rods ◽

Young Subjects ◽

Magnetically Controlled Growing Rods

Download Full-text

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

Download Full-text

Spinal Fusion and Instrumentation for Paediatric Neuromuscular Scoliosis: Retrospective Review

Journal of Orthopaedic Surgery ◽

10.1177/230949900201000207 ◽

2002 ◽

Vol 10 (2) ◽

pp. 144-151 ◽

Cited By ~ 48

Author(s):

M Thacker ◽

JHP Hui ◽

HK Wong ◽

A Chatterjee ◽

EH Lee

Keyword(s):

Muscular Atrophy ◽

Posterior Instrumentation ◽

Spinal Instrumentation ◽

Forced Expiratory Volume ◽

Neuromuscular Scoliosis ◽

University Hospital ◽

Anterior Fusion ◽

Superficial Infection ◽

The Mean

Purpose. A retrospective study was conducted to review the surgical results among 24 patients with neuromuscular scoliosis, who were treated with spinal instrumentation and fusion at the Department of Orthopaedic Surgery, National University Hospital, Singapore between March 1993 and December 1998. Methods. We examined complete hospital records of patients who had scoliosis due to aetiologies such as spinal muscular atrophy, cerebral palsy, Duchenne muscular dystrophy, and congenital myopathies. The mean age of patients was 10.6 years (range, 6–14 years) and the mean follow-up duration was 5.5 years. Results. 18 patients had posterior surgery alone, whereas 4 had an anterior release with posterior instrumentation, and 2 had an anterior fusion with instrumentation. The mean length of stay in the intensive care unit was 2 days and the mean duration of hospital stay was 11 days. The mean correction in scoliosis angle ranged from 75.6° to 25.7°. All patients could at least sit without support postoperatively. The one-second forced expiratory volume and forced vital capacity were, in general, maintained throughout the follow-up. There were 2 major complications and 2 minor ones; these were pseudarthrosis with rod breakage requiring revision, deep infection necessitating hardware removal, superficial infection that responded to antibiotics, and urinary tract infection requiring 3 weeks of antibiotic treatment. There were no deaths or any neurological complications after instrumentation. Conclusion. Spinal stabilisation and fusion in children with neuromuscular scoliosis is a safe and effective treatment modality. The effect of surgery on long-term pulmonary function, however, remains controversial and needs to be addressed.

Download Full-text

Complications of dual growing rod with all-pedicle screw instrumentation in the treatment of early-onset scoliosis

Journal of Orthopaedic Surgery and Research ◽

10.1186/s13018-021-02267-y ◽

2021 ◽

Vol 16 (1) ◽

Cited By ~ 1

Author(s):

Mohammad Zarei ◽

Mehdi Tavakoli ◽

Ehsan Ghadimi ◽

Alireza Moharrami ◽

Ali Nili ◽

...

Keyword(s):

Pedicle Screw ◽

Early Onset ◽

Thoracic Kyphosis ◽

Spinal Instrumentation ◽

Early Onset Scoliosis ◽

Pedicle Screw Instrumentation ◽

Growing Rods ◽

Growing Rod ◽

Operative Notes

Abstract Background Treatment of early-onset scoliosis (EOS) is still a challenge to patients, families, and surgeons. Previous studies have indicated that EOS patients are at high risk for complications following growth-friendly surgery. This study was performed to evaluate the results and complications of all-pedicle screw dual growing rod instrumentation in the treatment of EOS. Methods In an IRB-approved retrospective study, we searched the electronic medical records of our institution for all patients who underwent posterior spinal instrumentation for scoliosis between March 2014 and March 2017. Patients under the age of 10 at the time of surgery who were treated with a growth-friendly technique were then selected. Patients with incomplete records and less than 2 years of follow-up were excluded. Charts, operative notes, clinic visits, and radiographs were extracted. Radiographs were reviewed, and the main curve Cobb angle, thoracic kyphosis, pelvic tilt, pelvic incidence, sacral slope, and proximal junctional angles were measured. We specifically looked for any intra-operative or post-operative complications. Statistical analysis was performed to determine the risk factors of complications. Results A total of 42 patients with a mean age of 4.8 ± 2.1 years (range, 1.5–8 years) were included in the final analysis. Patients were followed for a median of 34 months (range, 24–55). The major curve was corrected from a mean of 42.9° ± 10.7° to 28.8° ± 9.6° at the latest follow-up. Proximal junctional angles and thoracic kyphosis increased significantly during the follow-up period (both P values < 0.001). A total of 7 complications (17%) were observed. Four patients (10%) developed superficial surgical site infections, all of which resolved with antibiotics and one round of surgical debridement. Three cases (7%) of proximal junctional kyphosis (PJK) were encountered during the study period, none of which required revision surgery. Pre-operative thoracic kyphosis was the only significant risk factor for the development of PJK. Conclusions Our findings suggest that in settings without access to magnetically controlled growing rods, dual growing rods with all-pedicle screw instrumentation is still a viable treatment strategy with comparable results and complications. The most common complications are infection and PJK, with the latter being associated with a larger pre-operative thoracic kyphosis.

Download Full-text

Gene-Replacement Therapy (GRT) in Spinal Muscular Atrophy Type 1 (SMA1): Long-Term Follow-Up From the Onasemnogene Abeparvovec Phase 1/2 Clinical Trial

10.1055/s-0039-1698259 ◽

2019 ◽

Cited By ~ 1

Author(s):

Jerry R. Mendell ◽

Richard Shell ◽

Kelly J. Lehman ◽

Markus McColly ◽

Linda P. Lowes ◽

...

Keyword(s):

Clinical Trial ◽

Muscular Atrophy ◽

Phase 1 ◽

Gene Replacement ◽

Spinal Muscular Atrophy Type ◽

Gene Replacement Therapy ◽

Long Term Follow Up

Download Full-text

Adolescent idiopathic scoliosis: sagital plane and low density pedicle screws

Coluna/Columna ◽

10.1590/s1808-18512014130100210 ◽

2014 ◽

Vol 13 (1) ◽

pp. 13-15

Author(s):

Rodrigo Augusto do Amaral ◽

Robert Meves ◽

Maria Fernanda Silber Caffaro ◽

Ricardo Shigueaki Galhego Umeta ◽

Luciano Antônio Nassar Pelegrino ◽

...

Keyword(s):

Medical Records ◽

Lumbar Lordosis ◽

Thoracic Kyphosis ◽

Sagittal Plane ◽

Pedicle Screws ◽

Low Density ◽

Kyphosis Correction ◽

Pearson Coefficient ◽

Analysis Of Dispersion

OBJECTIVE: To examine the sagittal curves of patients treated with CD instrumentation using exclusively pedicle screws. METHODS: Image analysis of medical records of 27 patients (26 M and 1 F) with a minimum follow-up of 6 months, who underwent surgical treatment in our service between January 2005 and December 2010. The curves were evaluated on coronal and sagittal planes, taking into account the potential correction of the technique. RESULTS: In the coronal plan the following curves were evaluated: proximal thoracic (TPx), main thoracic (TPp), and thoracolumbar; lumbar (TL, L), and the average flexibility was 52%, 52%, and 92% and the capacity of correction was 51%, 72%, and 64%, respectively. In the sagittal plane there was a mean increase in thoracic kyphosis (CT) of 41% and an average reduction of lumbar lordosis (LL) of 17%. Correlation analysis between variables showed Pearson coefficient of correlation of 0.053 and analysis of dispersion of R2 = <0.001. CONCLUSION: The method has shown satisfactory results with maintenance of kyphosis correction in patients with normal and hyper kyphotic deformities.

Download Full-text

Improvement in Fine Manual Dexterity in Children with Spinal Muscular Atrophy Type 2 after Nusinersen Injection: A Case Series

Children ◽

10.3390/children8111039 ◽

2021 ◽

Vol 8 (11) ◽

pp. 1039

Author(s):

Minsu Gu ◽

Hyun-Ho Kong

Keyword(s):

Spinal Muscular Atrophy ◽

Motor Function ◽

Daily Living ◽

Muscular Atrophy ◽

Case Series ◽

Manual Dexterity ◽

Gross Motor ◽

Spinal Muscular Atrophy Type ◽

Gross Motor Function

Although nusinersen has been demonstrated to improve motor function in patients with spinal muscular atrophy (SMA), no studies have investigated its effect on fine manual dexterity. The present study aimed to investigate the ability of nusinersen to improve fine manual dexterity in patients with SMA type 2. A total of five patients with SMA type 2 were included. The Hammersmith Functional Motor Scale (expanded version) (HFMSE) and Purdue Pegboard (PP) tests were used to evaluate gross motor function and fine manual dexterity, respectively, until 18 months after nusinersen administration. HFMSE scores improved by 3–10 points (+13–53%) in all patients following nusinersen administration. PP scores also improved in all patients, from 4 to 9 points (+80–225%) in the preferred hand and from 3 to 7 points (+60–500%) in the non-preferred hand. These results suggest that nusinersen treatment improved both gross motor function and fine manual dexterity in children with SMA type 2. Addition of the PP test may aid in evaluating the fine manual dexterity essential for activities of daily living in these patients.

Download Full-text