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 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.

Use of Rib-Based Distraction in the Treatment of Early-Onset Scoliosis Associated With Neurofibromatosis Type 1 in the Young Child

2015 ◽  
Vol 3 (3) ◽  
pp. 239-245 ◽  
Author(s):  
John A. Heflin ◽  
Andrew Cleveland ◽  
Scott D. Ford ◽  
Jessica V. Morgan ◽  
John T. Smith
Keyword(s):  
Young Child ◽  
Neurofibromatosis Type 1 ◽  
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.

Germline mutations in NF1 and BRCA1 in a family with neurofibromatosis type 1 and early-onset breast cancer

2013 ◽  
Vol 139 (2) ◽  
pp. 597-602 ◽  
Author(s):  
Berta Campos ◽  
Judith Balmaña ◽  
Josep Gardenyes ◽  
Irene Valenzuela ◽  
Oscar Abad ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Neurofibromatosis Type 1 ◽  
Early Onset ◽  
Neurofibromatosis Type ◽  
Germline Mutations ◽  
Early Onset Breast Cancer
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