National Trends in Spinal Fusion Surgery for Neurofibromatosis (original) (raw)

Spinal Deformity in Neurofibromatosis: Classification and Management

Journal of Spine, 2014

The effects of treatment of scoliosis in neurofibromatosis type 1 are less satisfactory than other scoliotic types due to the particular pathogenesis and clinical characteristics. Surgical treatment usually included 360 degrees fusion with instrumentation, but optimal strategies are different according to the degree of deformity. High incidence of pseudarthrosis, extensive bleeding and curve progression even after solid spinal fusion are also major concerns of neurofibromatosis type 1 associated spinal deformity surgery. With advance of technology, cobalt-chrome rod with dural rod technique, tranexamic acid, recombinant human bone morphogenetic protein-2 and electrophysiologic monitoring were used in neurofibromatosis deformity surgery to get better outcome. Searching from PubMed, we reviewed overall about neurofibromatosis spine deformity surgery.

Operative treatment of spinal deformities in patients with neurofibromatosis

2012

Introduction. Neurofibromatosis is a hereditary disorder, in which scoliosis is the most common orthopedic problem. The goal of this study is a retrospective analysis of results of scoliosis surgery in patients with neurofibromatosis, depending on the type of spinal deformity, and with assessment of spinal balance both in coronal and sagittal planes. Material consists of 28 consecutive patients with von Recklinghausen disease, treated surgically due to scoliosis between 1986 and 2008. Dystrophic scoliosis was observed in 21 cases, “normal” scoliosis – in 7. At the surgery, average patients’ age was 13.5 yrs. The mean follow up period was 5.4yrs. Methods. Radiologic data before and after treatment were analyzed as well as at last control examination. Type of surgery and intra- and postoperative complications were noted. Posterior fusion was performed in 11 patients, anterior fusion (with or without instrumentation) and posterior fusion with implants in second stage – 8 cases, anterio...

Evaluation surgical treatment results of scoliosis in patients with neurofibromatosis

Introduction: Surgical treatment of spinal deformity resulting from neurofibromatosis (NF) is a major challenge for orthopedic spine surgeons. There are several problems and complications including pseudoarthrosis and cure progress despite treatment. Progressive kyphoscoliosis is the most important spinal deformity. The present study aims to evaluate surgical treatment results in severe spinal involvement cases. Methods: This analytical a-descriptive study evaluated 20 patients with NF, severe scoliosis and kyphosis (up to 50°) hospitalized and treated at our center during the past 10 years. The treatment failure rate and complications were studied. Results: In this study, 20 cases with NF and kyphoscoliosis with the mean age of 13.00 ± 7.18 years were studied. These case series were consisted of 13 (65%) males and 7 (35%) females. Overall treatment failure was 45%. However, it was 55% of failure happened in posterior fusion alone. Failure rate was reported 36% in the combined anterior and posterior fusion and mainly seen in younger than 8-years children. Surgical complications were found in 20% with pseudoarthrosis as the most common one. There were no infections and neurological complications. Statistically, there was a significant negative relation between age and curve progression in scoliosis and kyphosis during the 2 years follow-up period. There was not any significant difference between genders considering curvature progress. Conclusion: The combined anterior and posterior fusion is probably more effective treatment, especially at early ages when more aggressive treatment is required since it reduces the treatment failure possibility.

The Number of Surgical Interventions and Specialists Involved in the Management of Patients with Neurofibromatosis Type I: A 25-Year Analysis

Journal of Personalized Medicine

Objective: In this study, we aim to present a single institution’s 25-year experience of employing a comprehensive multidisciplinary team-based surgical approach for treating patients with NF-1. Summary Background Data: All patients (n = 106) with a confirmed diagnosis of NF-1 who were treated using a multidisciplinary surgical treatment algorithm at Chang Gung Memorial Hospital between 1994 and 2019 were retrospectively enrolled. Patients were categorized into groups according to the anatomy involved (craniofacial and noncraniofacial groups) and the type of clinical presentation (plexiform and cutaneous neurofibromas groups) for comparative analysis. Methods: The number of surgical interventions and number of specialists involved in surgical care were assessed. Results: Most of the patients exhibited craniofacial involvement (69.8%) and a plexiform type of NF-1 (58.5%), as confirmed through histology. A total of 332 surgical interventions (3.1 ± 3.1 procedures per patient) were per...

Spinal Manifestations of Neurofibromatosis: An Update

Negah Institute for Social Research & Scientific Communication, 2020

Background and Aim: Neurofibromatosis-1 (NF1) is a common genetic condition in children. It is becoming increasingly recognized that in neurofibromatosis, there might be anomalous development of bone with or without any local anomaly of neuroectodermal basis. This review was conducted to highlight the different features of spinal disorders in this congenital disease. Methods and Materials/Patients: Different search engines were used in this research. After going through the results and discarding the repeated ones, 2 reviewers began to inspect the studies independent of one another. In case of any disagreements between the reviewers over the inclusion of certain research material into the study, the final decision was made by the senior author. In the end, accepted research materials were used as needed in categorizing different types of spinal injuries. Results: Skeletal defects are among the most effective complications on the quality of life of patients. Bone deformities, osteoporosis, osteopenia, reduction of bone mass density, and spinal osteopathy such as scoliosis among such defects. Although the relative prevalence of spinal defects in neurofibromatosis type 1 is not clear, scoliosis can be considered the most common type of spinal deformity in this illness to the point it is present in 10 to 71% of cases. Thus, spine surgeons may face numerous common challenging conditions in NF1 patients like scoliosis, spondylolisthesis, and dural ectasia. Conclusion: As NF1 distresses several organ systems, radiologic spine screening is important in patients with NF1 and patients are likely to profit most from a multidisciplinary treatment policy.

Management and surgical outcomes of dystrophic scoliosis in neurofibromatosis type 1: a systematic review

Neurosurgical Focus

OBJECTIVE Neurofibromatosis type 1 (NF1) dystrophic scoliosis is an early-onset, rapidly progressive multiplanar deformity. There are few studies on the surgical management of this patient population. Specifically, perioperative morbidity, instrument-related complications, and quality-of-life outcomes associated with surgical management have not been systematically evaluated. In this study, the authors aimed to perform a systematic review on the natural history, management options, and surgical outcomes in patients who underwent NF1 dystrophic scoliosis surgery. METHODS A PubMed search for articles with “neurofibromatosis” and either “dystrophic” or “scoliosis” in the title or abstract was performed. Articles with 10 or more patients undergoing surgery for NF1 dystrophic scoliosis were included. Data regarding indications, treatment details, morbidity, and outcomes were summarized and analyzed with descriptive statistics. RESULTS A total of 310 articles were identified, 48 of which ...

Corpectomy and circumferential spinal fusion in dystrophic neurofibromatous curves

Journal of Children's Orthopaedics, 2010

Purpose Spinal deformity in neurofibromatosis, when associated with dystrophic change, is a major treatment challenge. Early anterior and posterior spinal fusion is the accepted treatment plan for severe progressive kyphoscoliosis. Resection of the vertebral body as an additional step in correction and fusion has not been previously investigated. Information on the functional status of the neurofibromatous curves is also scarce in the literature. The purpose of this study is to report an experience with corpectomy and circumferential fusion in dystrophic spines of neurofibromatosis. Methods Among 16 patients with dystrophic spinal curves, nine cases who had undergone anterior and posterior fusion with 6.7 years (range 2–11.9) average follow-up were evaluated clinically, radiographically, and functionally (SRS-22). Results Nine cases with a mean age of 11.8 years (range 7.8–17) at surgery consisted of seven kyphoscoliotic patients, who had received one or two levels of cord decompres...