scholarly journals Mid-long-term surgical outcomes of posterior only instrumented spinal fusion procedure for early-onset scoliosis in type 1 neurofibromatosis

2019 ◽  
Author(s):  
Jianguo zhang ◽  
Siyi Cai ◽  
zhenyao Li ◽  
Guixing Qiu ◽  
Jianxiong shen ◽  
...  
Keyword(s):  
Spinal Fusion ◽  
Surgical Outcomes ◽  
Early Onset ◽  
Neurofibromatosis Type ◽  
Early Onset Scoliosis ◽  
Major Curve ◽  
Fusion Procedure

Abstract Background The pediatric neurofibromatosis type 1 (NF-1) patients’ mid-long-term surgical outcomes of posterior spinal fusion is rarely reported, and the orthopedic maintenance effect is not clear. This study aims to evaluate the mid-long-term surgical outcomes of posterior only instrumented spinal fusion for early-onset scoliosis (EOS) in NF-1 patients.Methods A retrospective review was performed on a cohort of 10 patients diagnosed as NF-1 with EOS from 2008 to 2014 in our hospital. This study included four male and six female NF-1 patients with a mean age of 7.8 years old when they underwent posterior only instrumented spinal fusion for their EOS. General clinical data, operation-related data were reviewed, and the dystrophic progression of EOS was evaluated during the follow-up.Results The mean follow-up duration was 54 months (24 to 88 months). All patients underwent posterior only instrumented spinal fusion at 1 stage. The primary curves of EOS were thoracic in 9 cases, and only one patient had lumbar scoliosis. Preoperative major curve was significantly corrected (from 66.1 to 31.1 degrees). However, the average major curve deteriorated significantly to 40.1 degrees on average at the end of follow-up. The T1-S1 distance increased 2.77cm on average and increasing at 0.6cm/year during the follow-up.Conclusions For the surgical treatment on EOS in NF-1 patients, although with relatively short segments involvements,posterior only fusion was not good way to treat such patients, especially the maintenance of orthopedic effect was not satisfactory.

Long-term Surgical Outcomes After Early Definitive Spinal Fusion for Early-onset Scoliosis With Neurofibromatosis Type 1 at Mean Follow-up of 14 Years

2020 ◽  
Vol 40 (1) ◽  
pp. 42-47 ◽  
Author(s):  
Ryoji Tauchi ◽  
Noriaki Kawakami ◽  
Michael Anton Castro ◽  
Tetsuya Ohara ◽  
Toshiki Saito ◽  
...  
Keyword(s):  
Spinal Fusion ◽  
Neurofibromatosis Type 1 ◽  
Surgical Outcomes ◽  
Early Onset ◽  
Neurofibromatosis Type ◽  
Early Onset Scoliosis

scholarly journals Posterior only instrumented fusion provides incomplete curve control for early-onset scoliosis in type 1 neurofibromatosis

2019 ◽  
Author(s):  
Jianguo zhang ◽  
Siyi Cai ◽  
zhenyao Li ◽  
Guixing Qiu ◽  
Jianxiong shen ◽  
...  
Keyword(s):  
Spinal Fusion ◽  
Early Onset ◽  
Neurofibromatosis Type ◽  
Posterior Spinal Fusion ◽  
Early Onset Scoliosis ◽  
Fusion Surgery ◽  
Major Curve

Abstract Background: The mid-long term outcomes of posterior spinal fusion in pediatric neurofibromatosis type 1 (NF-1) patients are rarely reported, so does the effectiveness of itsorthopeidc maintenance function. This study aims to evaluate the mid-long term surgical outcomes of posterior only instrumented spinal fusion for early-onset scoliosis (EOS) in NF-1 patients. Methods: A retrospective review was performed on a cohort of 10 NF-1 patients having EOS from 2008 to 2014 in our hospital, including four male and six female patients with an age averaged at 7.8 years old when they underwent posterior only instrumented spinal fusion for their EOS. Both general clinical data and surgical specific data of the patients were collected and reviewed, and the dystrophic progression of EOS was evaluated during the follow-up. Results: The average duration of follow-up was 54 months (24 to 88 months). All patients underwent posterior only instrumented spinal fusion at 1 stage. The primary curves of EOS were thoracic in 9 cases and 1 patient had lumbar scoliosis. Preoperative major curve was significantly corrected (from 66.1 to 31.1 degrees). However, the major curve deteriorated significantly to 40.1 degrees on average at the end of the follow-up. The T1-S1 distance increased 2.8 cm on average and kept increasing at a rate of 0.6cm/year during the follow-up. Conclusions: Posterior only fusion surgery was not a good option to treat the EOS in NF-1 patients despite the relatively short segments involvement in the disease. The maintenance of orthopedic effect after treatment was not satisfactory.

scholarly journals Posterior only instrumented fusion provides incomplete curve control for early-onset scoliosis in type 1 neurofibromatosis

2020 ◽  
Author(s):  
Siyi Cai ◽  
Zhenyao Li ◽  
Guixing Qiu ◽  
Jianxiong shen ◽  
Hong Zhao ◽  
...  
Keyword(s):  
Spinal Fusion ◽  
Early Onset ◽  
Neurofibromatosis Type ◽  
Posterior Spinal Fusion ◽  
Early Onset Scoliosis ◽  
Fusion Surgery ◽  
Major Curve

Abstract Background: The mid-long term outcomes of posterior spinal fusion in pediatric neurofibromatosis type 1 (NF-1) patients are rarely reported, so does the effectiveness of itsorthopeidc maintenance function. This study aims to evaluate the mid-long term surgical outcomes of posterior only instrumented spinal fusion for early-onset scoliosis (EOS) in NF-1 patients. Methods: A retrospective review was performed on a cohort of 10 NF-1 patients having EOS from 2008 to 2014 in our hospital, including four male and six female patients with an age averaged at 7.8 years old when they underwent posterior only instrumented spinal fusion for their EOS. Both general clinical data and surgical specific data of the patients were collected and reviewed, and the dystrophic progression of EOS was evaluated during the follow-up. Results: The average duration of follow-up was 54 months (24 to 88 months). All patients underwent posterior only instrumented spinal fusion at 1 stage. The primary curves of EOS were thoracic in 9 cases and 1 patient had lumbar scoliosis. Preoperative major curve was significantly corrected (from 66.1 to 31.1 degrees). However, the major curve deteriorated significantly to 40.1 degrees on average at the end of the follow-up. The T1-S1 distance increased 2.8 cm on average and kept increasing at a rate of 0.6cm/year during the follow-up. Conclusions: Posterior only fusion surgery was not a good option to treat the EOS in NF-1 patients despite the relatively short segments involvement in the disease. The maintenance of orthopedic effect after treatment was not satisfactory.

scholarly journals Posterior only instrumented fusion provides incomplete curve control for early-onset scoliosis in type 1 neurofibromatosis

2020 ◽  
Author(s):  
Siyi Cai ◽  
zhenyao Li ◽  
Guixing Qiu ◽  
Jianxiong shen ◽  
hong zhao ◽  
...  
Keyword(s):  
Spinal Fusion ◽  
Early Onset ◽  
Neurofibromatosis Type ◽  
Posterior Spinal Fusion ◽  
Early Onset Scoliosis ◽  
Fusion Surgery ◽  
Major Curve

Abstract Background: The mid-long term outcomes of posterior spinal fusion in pediatric neurofibromatosis type 1 (NF-1) patients are rarely reported, so does the effectiveness of itsorthopeidc maintenance function. This study aims to evaluate the mid-long term surgical outcomes of posterior only instrumented spinal fusion for early-onset scoliosis (EOS) in NF-1 patients. Methods: A retrospective review was performed on a cohort of 10 NF-1 patients having EOS from 2008 to 2014 in our hospital, including four male and six female patients with an age averaged at 7.8 years old when they underwent posterior only instrumented spinal fusion for their EOS. Both general clinical data and surgical specific data of the patients were collected and reviewed, and the dystrophic progression of EOS was evaluated during the follow-up. Results: The average duration of follow-up was 54 months (24 to 88 months). All patients underwent posterior only instrumented spinal fusion at 1 stage. The primary curves of EOS were thoracic in 9 cases and 1 patient had lumbar scoliosis. Preoperative major curve was significantly corrected (from 66.1 to 31.1 degrees). However, the major curve deteriorated significantly to 40.1 degrees on average at the end of the follow-up. The T1-S1 distance increased 2.8 cm on average and kept increasing at a rate of 0.6cm/year during the follow-up. Conclusions: Posterior only fusion surgery was not a good option to treat the EOS in NF-1 patients despite the relatively short segments involvement in the disease. The maintenance of orthopedic effect after treatment was not satisfactory.

scholarly journals Posterior only instrumented fusion provides incomplete curve control for early-onset scoliosis in type 1 neurofibromatosis

2020 ◽  
Author(s):  
Siyi Cai ◽  
zhenyao Li ◽  
Guixing Qiu ◽  
Jianxiong shen ◽  
Hong Zhao ◽  
...  
Keyword(s):  
Spinal Fusion ◽  
Early Onset ◽  
Neurofibromatosis Type ◽  
Posterior Spinal Fusion ◽  
Early Onset Scoliosis ◽  
Fusion Surgery ◽  
Major Curve

Abstract Background: The mid-long term outcomes of posterior spinal fusion in pediatric neurofibromatosis type 1 (NF-1) patients are rarely reported, so does the effectiveness of itsorthopeidc maintenance function. This study aims to evaluate the mid-long term surgical outcomes of posterior only instrumented spinal fusion for early-onset scoliosis (EOS) in NF-1 patients. Methods: A retrospective review was performed on a cohort of 10 NF-1 patients having EOS from 2008 to 2014 in our hospital, including four male and six female patients with an age averaged at 7.8 years old when they underwent posterior only instrumented spinal fusion for their EOS. Both general clinical data and surgical specific data of the patients were collected and reviewed, and the dystrophic progression of EOS was evaluated during the follow-up. Results: The average duration of follow-up was 54 months (24 to 88 months). All patients underwent posterior only instrumented spinal fusion at 1 stage. The primary curves of EOS were thoracic in 9 cases and 1 patient had lumbar scoliosis. Preoperative major curve was significantly corrected (from 66.1 to 31.1 degrees). However, the major curve deteriorated significantly to 40.1 degrees on average at the end of the follow-up. The T1-S1 distance increased 2.8 cm on average and kept increasing at a rate of 0.6cm/year during the follow-up. Conclusions: Posterior only fusion surgery was not a good option to treat the EOS in NF-1 patients despite the relatively short segments involvement in the disease. The maintenance of orthopedic effect after treatment was not satisfactory.

scholarly journals Outcomes of growing rods in a series of early-onset scoliosis patients with neurofibromatosis type 1

2020 ◽  
Vol 33 (3) ◽  
pp. 373-380
Author(s):  
Charlie Bouthors ◽  
Ruben Dukan ◽  
Christophe Glorion ◽  
Lotfi Miladi
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Early Onset ◽  
Neurofibromatosis Type ◽  
Posterior Spinal Fusion ◽  
Early Onset Scoliosis ◽  
Mechanical Complication ◽  
Growing Rods ◽  
The Mean

OBJECTIVEEarly-onset scoliosis (EOS) is not uncommon in patients with neurofibromatosis type 1 (NF1). Despite conservative treatment, spinal deformities progress and require early surgical intervention. To avoid potential interference with chest and trunk growth, growing rods (GRs) have been used effectively in EOS of various etiologies. In this study the authors sought to analyze the outcomes of GRs in EOS patients with NF1.METHODSThis was a retrospective single-center cohort study that included consecutive EOS patients with NF1 who were treated with GRs and were followed up for a minimum of 2 years. Clinical and radiological analyses were performed preoperatively and until the last follow-up.RESULTSFrom to 2008 to 2017, 18 patients (6 male, 12 female) underwent GR surgery (14 single GRs, 4 dual GRs) at a mean age of 8 ± 2.1 years. Mean follow-up was 5 ± 2.4 years. Fifty-five lengthenings were performed at a mean rate of 3 lengthenings per patient (range 0–7). Ten of 14 single GRs (71%) were converted into dual GRs during treatment. No patient underwent definitive posterior spinal fusion (PSF) at GR treatment completion. The mean initial and last follow-up major curves were 57° and 36°, respectively (p < 0.001, 37% correction). The average T1–S1 increase was 13 mm/yr. Six of 9 hyperkyphotic patients had normal kyphosis at last follow-up. There were 26 complications involving 13 patients (72%), with 1 patient who required unplanned revision. The primary complications were instrumentation related, consisting of 17 proximal hook dislodgments, 6 distal pedicle screw pullouts, and 2 rod fractures. Only 1 patient experienced a mechanical complication after dual GR implantation. There were no wound infections.CONCLUSIONSThe GR technique provided satisfactory spinal deformity control in EOS patients with NF1 while allowing substantial spinal growth. Adequately contoured dual GRs with proximal hooks placed in nondystrophic regions should be used to minimize implant-related complications. Surgeons should not attempt to correct kyphosis at GR implantation.

scholarly journals NIMG-66. LONG-TERM FOLLOW-UP OF NEUROFIBROMATOSIS TYPE 1 PATIENTS USING WHOLE-BODY MRI DEMONSTRATES DYNAMIC CHANGES IN INTERNAL NEUROFIBROMA SIZE

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi176-vi176
Author(s):  
Ina Ly ◽  
Raquel Thalheimer ◽  
Wenli Cai ◽  
Miriam Bredella ◽  
Vanessa Merker ◽  
...  
Keyword(s):  
Tumor Growth ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Whole Body ◽  
Childhood And Adolescence ◽  
Entire Study ◽  
Whole Body Mri

Abstract BACKGROUND Neurofibromas affect 40–50% of neurofibromatosis type 1 (NF1) patients and can cause significant morbidity and mortality. They grow more rapidly during childhood and adolescence but studies in adults are limited by their retrospective nature and follow-up time < 3 years. The long-term natural history of neurofibromas remains unknown. No guidelines exist on the need and frequency of surveillance imaging for patients. Whole-body MRI (WBMRI) can detect whole-body tumor burden, including internal neurofibromas. METHODS 17 adult NF1 patients who underwent WBMRI between 2007–2010 (Scan 1) underwent repeat WBMRI between 2018–2019 (Scan 2). Internal neurofibromas were segmented on short tau inversion recovery (STIR) sequences and tumor volume was calculated using a computerized volumetry and three-dimensional segmentation software. Circumscribed tumors were defined as discrete; invasive tumors or those involving multiple nerves were defined as plexiform. Tumor growth and shrinkage were defined as volume change ≥ 20% over the entire study period. RESULTS Median patient age was 43 years during Scan 1 and 53 years during Scan 2. Median time between Scan 1 and 2 was 9 years. A total of 140 neurofibromas were assessed. 24% of tumors grew by a median 63% (6.8% per year). 54% of tumors spontaneously decreased in volume by a median 60% (7% per year) without treatment. On a per-patient basis, 18% of patients had overall tumor growth and 41% overall tumor shrinkage. 8 new tumors developed in 7 patients. 16 tumors resolved entirely without medical or surgical intervention. Growth behavior did not correlate with discrete or plexiform morphology. CONCLUSION A subset of internal neurofibromas in adult NF1 patients grow significantly over a long-term period, suggesting that continued monitoring of these patients may be warranted. Surprisingly, more than half of neurofibromas shrink spontaneously without intervention. Continued patient enrollment and correlation of imaging findings with functional outcomes are underway.

Growing Rods Are an Effective Fusionless Method of Controlling Early-Onset Scoliosis Associated With Neurofibromatosis Type 1 (NF1)

2017 ◽  
Vol 37 (8) ◽  
pp. e612-e618 ◽  
Author(s):  
Viral V. Jain ◽  
Chirag A. Berry ◽  
Alvin H. Crawford ◽  
John B. Emans ◽  
Paul D. Sponseller
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Early Onset ◽  
Neurofibromatosis Type ◽  
Early Onset Scoliosis ◽  
Growing Rods

scholarly journals NFM-13. LONG TERM FOLLOW-UP OF OPTIC PATHWAY GLIOMAS IN CHILDREN WITH NEUROFIBROMATOSIS TYPE 1 – AN ONCOLOGY HOSPITAL EXPERIENCE

2018 ◽  
Vol 20 (suppl_2) ◽  
pp. i145-i145
Author(s):  
Mariana Fernandes ◽  
João Passos ◽  
Daniela Garcez ◽  
Manuela Mafra ◽  
Maria Fátima Campos ◽  
...  
Keyword(s):  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Optic Pathway ◽  
Hospital Experience ◽  
Long Term Follow Up ◽  
Optic Pathway Gliomas
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