Neurofibromatosis: Etiology, Commonly Encountered Spinal Deformities, Common Complications and Pitfalls of Surgical Treatment

Crawford, Alvin H.; Lykissas, Marios G.; Schorry, Elizabeth K.; Gaines, Sean; Jain, Viral V.; Greggi, T.; Viskochil, David

doi:10.1016/j.jspd.2012.04.004

Cited by 14 publications

(7 citation statements)

References 22 publications

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“…In our population, dystrophic scoliosis occurred in approximately 39% of patients. This percentage was quite higher than reported in the available literature (ranging from 10 to 30%) [ 15 , 16 ]. A possible explanation is that it may be related to the high percentage of patients who underwent to MRI in our population.…”

Section: Discussioncontrasting

confidence: 65%

Natural History of Scoliosis in Children with NF1: An Observation Study

et al. 2021

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(1) Background. Scoliosis is the most common musculoskeletal manifestation of Neurofibromatosis type 1 (NF1), and it might be dystrophic (D) or non-dystrophic (ND) depending on the presence of dysplastic changes of the spine. The aim of our study was to describe the characteristics and natural history of patients with NF1 and scoliosis. (2) Methods. We retrospectively reviewed records from patients with NF1 and scoliosis. Scoliosis was classified as D if at least two dystrophic changes were documented at imaging. (3) Results. Of the 438 patients reviewed, 43 fulfilled inclusion criteria; 17 were classified in D group and 26 in ND. The groups did not differ in age and localization of scoliosis curvature. Surgery was needed more often in D group, but the between-group difference was not significant. Male-to-female ratios of 3:1 and 4:1 were reported in surgically treated NF1 patients with ND and D scoliosis, respectively. (4) Conclusions. Our data suggests independently by the presence of dysplastic changes affecting the spine that males with NF1 are more often affected by scoliosis that requires surgery.

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Section: Discussioncontrasting

confidence: 65%

Natural History of Scoliosis in Children with NF1: An Observation Study

et al. 2021

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“…The mean follow-up period was 5.15±1.78 years (range, [3][4][5][6][7][8]. The mean operative time was 195±20.12 minutes (range, 160-240) and the mean amount of blood loss was 895.…”

Section: Resultsmentioning

confidence: 99%

“…7 Scoliosis is the most common osseous presentation of NF-1 21 occurring in 10-30% of NF-1 patients. 1,7,14 Two distinct types of scoliosis curves occur in NF-1: the nondystrophic type which is similar to the idiopathic scoliosis in its natural history and treatment and the dystrophic type which is characterized by being sharp, angular, short segment curve and usually accompanied by kyphosis and associated with dystrophic changes of the spine. The dystrophic type is the most likely to progress into severe deformity and instability even in the presence of solid bony fusion.…”

Section: Introductionmentioning

confidence: 99%

Evaluation of Efficacy and Safety of All Pedicle Screw Posterior-Only Surgery in Patients with Dystrophic Neurofibromatosis Scoliosis

Abdel-Latif

2019

Egyptian Spine Journal

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Background Data: Neurofibromatosis type 1 (NF-1) patients with dystrophic changes of the spine develop severe spinal deformity and/or instability and always need early surgical treatment. Combined anterior-posterior fusion was the treatment of choice of such curves because of the risk of progression and pseudoarthrosis even in the presence of solid posterior fusion mass. Recent 3rd-generation posterior stabilization systems using pedicle screw fixation allow better 3-column purchase and 3D control and correction of deformity and stable posterior fixation of the spine preventing progression of the curves. Posterior-only surgery has been recently used in dystrophic NF-1 scoliosis due to use of pedicle screws fixation and development of different types of posterior release/osteotomies which lead to successful and sustained correction of these curves. Study Design: A retrospective clinical cohort study. Purpose: To study the results of posterior stabilization alone using all pedicle screws fixation in NF-1 patients with dystrophic scoliosis. Patients and Methods: 16 patients with NF-1 having dystrophic scoliosis were treated by posterior instrumentation using all pedicle screws. There were 5 female and 11 male patients and the average age was 13 years (range, 9-16 years). The study was done in the period between 2009 and 2016. Preoperative clinical examination included examination for neurofibromatosis, assessment of curve flexibility, and thorough neurological examination. Radiological examination included standard plain X-rays, anteriorposterior (A-P) and lateral and flexibility views, and MRI. Screw density of the whole construct and of the apical region (two vertebrae above and below the apex) was calculated. Functional outcomes were assessed by the ODI and SRS-30 scores at the last follow-up. Results: The mean follow-up was 5.15±1.78 years (range, 3-8). The mean Cobb angle improved in the coronal plane from 67.13±11.88 (range, 45-90 •) to 20±8.9 (range, 6-30 •) showing 70.1±11.2%. The mean preoperative sagittal plane deformity significantly improved from 61.82±20.28 (range, 35-90 •) to 25.91±7.68 (range, 10-35 •) postoperatively yielding 53.15±21.43 (range, 25-83.33%) mean correction with insignificant loss of correction at last follow-up. Loss of correction was 2±1.8% and 1.8±2.1% in the coronal and sagittal planes, respectively. At last follow-up the average SRS-30 score was 121±17.

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“…Posterior surgery alone was reported to be associated with higher risk of pseudoarthrosis 6 , 7 , 8 . A combined anterior/posterior approach was recommended to reduce the risk of non-union 9 , 11 . However, acute correction with combined anterior/posterior surgeries or posterior only approaches utilising posterior column osteotomies were often associated with higher perioperative morbidities.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, early and aggressive surgical intervention is usually required to halt further progression of the deformity 5 . However, correction of the dystrophic spinal deformity in neurofibromatosis patients is not only difficult, but carries much higher risks of neurologic complications, bleeding, pseudoarthrosis, and implant failure necessitating a revision surgery 6 - 9 .…”

Section: Introductionmentioning

confidence: 99%

Pre-operative Halo-Pelvic Traction for Neurofibromatosis Patients with Severe Proximal Thoracic Spinal Deformity: Indications and Early Treatment Outcome

WH¹,

Mihara²,

SS³

et al. 2021

Malays Orthop J

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Introduction: To report the indications and early treatment outcomes of pre-operative halo-pelvic traction in patients with neurofibromatosis associated with severe proximal thoracic (PT) spinal deformity. Materials and methods: We reviewed four patients with neurofibromatosis with severe PT spinal deformity. Case 1, a 16-year-old male presented with severe PT kyphoscoliosis (scoliosis: 89°, kyphosis: 124°) and thoracic myelopathy. Case 2 was a 14-year-old, skeletally immature male who presented with a PT lordoscoliosis (scoliosis: 85°). Case 3, a 13-year-old male, presented with severe PT kyphoscoliosis (scoliosis: 100°, kyphosis: 95°). Case 4, a 35-year-old gentleman, presented with severe PT kyphoscoliosis (scoliosis: 113°, kyphosis: 103°) and thoracic myelopathy. All patients underwent pre-operative halo-pelvic traction. After a period of traction, all patients underwent posterior spinal fusion (PSF) with autologous bone grafts (local and fibula bone grafts) and recombinant human bone morphogenetic protein-2 (rhBMP-2). Results: Both patients with thoracic myelopathy regained near normal neurological status after halo-pelvic traction. Following traction, the scoliosis correction rate (CR) ranged from 18.0% to 38.9%, while the kyphosis CR ranged from 14.6% to 37.1%. Following PSF, the scoliosis CR ranged from 24.0% to 58.8%, while the kyphosis CR ranged from 29.1% to 47.4%. The total distraction ranged from 50-70mm. Duration of distraction ranged from 26-95 days. The most common complication encountered during halo-pelvic traction was pin-related e.g. pin tract infection, pin loosening and migration, osteomyelitis, and halo-pelvic strut breakage. No patients had cranial nerve palsies or neurological worsening. Conclusion: Pre-operative correction of severe PT spinal deformities could be performed safely and effectively with the halo-pelvic device prior to definitive surgery.

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Neurofibromatosis: Etiology, Commonly Encountered Spinal Deformities, Common Complications and Pitfalls of Surgical Treatment

Cited by 14 publications

References 22 publications

Natural History of Scoliosis in Children with NF1: An Observation Study

Natural History of Scoliosis in Children with NF1: An Observation Study

Evaluation of Efficacy and Safety of All Pedicle Screw Posterior-Only Surgery in Patients with Dystrophic Neurofibromatosis Scoliosis

Pre-operative Halo-Pelvic Traction for Neurofibromatosis Patients with Severe Proximal Thoracic Spinal Deformity: Indications and Early Treatment Outcome

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