Tips and Pitfalls of Surgical Techniques for Scoliotic Deformities in Neurofibromatosis Type 1

<b>Background</b>: Neurofibromatosis 1 is an autosomal dominant disorder accompanied by extensive early-onset spinal manifestations, with or without dystrophic scoliotic features. While non-dystrophic subtypes can often be treated similarly to idiopathic scoliosis, dystrophic scoliosis typically requires more aggressive intervention, often involving instrumentation in severely compromised pedicles or vertebrae. <b>Purpose</b>: This review aims to present recent advances in the surgical treatment of Neurofibromatosis 1-associated scoliosis, including surgical techniques and emerging guidance methods. <b>Methods</b>: An electronic literature search was conducted in Web of Science and PubMed to identify surgical techniques for scoliosis in patients with Neurofibromatosis 1. <b>Results</b>: Forty-one studies on the operative treatment of dystrophic scoliosis or both subtypes were retrieved. Although aggressive treatment with combined anterior and posterior fusion are widely used, posterior-only methods, which avoid plexiform tumours, present encouraging results. Recent studies highlight the effectiveness of growing rod systems in early-onset cases, enabling delayed fusion while preserving T1-S1 growth. Promising results from sectional or segmented correction techniques demonstrate better sagittal balance and Cobb angle correction, respectively. Preoperative use of halo-gravity traction, which has been extensively studied, is associated with reduced neurological impairment and encourages better correction results, avoiding autofusion. Various studies have also reported more precise pedicle screw placement with guidance of O-arm and triggered electromyography (t-EMG). <b>Conclusions</b>: The correction of spinal scoliotic deformities presents a significant challenge. However, recent advances in surgical techniques and intraoperative guidance offer promising strategies for more effective management.

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Publication Details

Journal
Journal of Clinical Medicine
Published
2025-12-23
DOI
https://doi.org/10.3390/jcm15010104
Primary Topic
Neurofibromatosis and Schwannoma Cases
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article
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article

Tips and Pitfalls of Surgical Techniques for Scoliotic Deformities in Neurofibromatosis Type 1

Ioanna Lianou, Evangelos Sakellariou, Angelos Kaspiris, Panagiotis Karampinas et al.
Journal of Clinical Medicine
Neurofibromatosis and Schwannoma Cases
article

Tips and Pitfalls of Surgical Techniques for Scoliotic Deformities in Neurofibromatosis Type 1

Ioanna Lianou, Evangelos Sakellariou, Angelos Kaspiris, Panagiotis Karampinas, Spiros G. Pneumaticos, Andreas Morakis, Elias S. Vasilisadis, Roberta-Spyridoula Afrati, Vasileios Marouglianis
article en

Abstract

<b>Background</b>: Neurofibromatosis 1 is an autosomal dominant disorder accompanied by extensive early-onset spinal manifestations, with or without dystrophic scoliotic features. While non-dystrophic subtypes can often be treated similarly to idiopathic scoliosis, dystrophic scoliosis typically requires more aggressive intervention, often involving instrumentation in severely compromised pedicles or vertebrae. <b>Purpose</b>: This review aims to present recent advances in the surgical treatment of Neurofibromatosis 1-associated scoliosis, including surgical techniques and emerging guidance methods. <b>Methods</b>: An electronic literature search was conducted in Web of Science and PubMed to identify surgical techniques for scoliosis in patients with Neurofibromatosis 1. <b>Results</b>: Forty-one studies on the operative treatment of dystrophic scoliosis or both subtypes were retrieved. Although aggressive treatment with combined anterior and posterior fusion are widely used, posterior-only methods, which avoid plexiform tumours, present encouraging results. Recent studies highlight the effectiveness of growing rod systems in early-onset cases, enabling delayed fusion while preserving T1-S1 growth. Promising results from sectional or segmented correction techniques demonstrate better sagittal balance and Cobb angle correction, respectively. Preoperative use of halo-gravity traction, which has been extensively studied, is associated with reduced neurological impairment and encourages better correction results, avoiding autofusion. Various studies have also reported more precise pedicle screw placement with guidance of O-arm and triggered electromyography (t-EMG). <b>Conclusions</b>: The correction of spinal scoliotic deformities presents a significant challenge. However, recent advances in surgical techniques and intraoperative guidance offer promising strategies for more effective management.

Journal of Clinical MedicineVol. 15(1)
National and Kapodistrian University of Athens (GR), Agios Andreas Hospital (GR), KAT General Hospital of Attica (GR)
Openalex Percentile: Top 32%
Neurofibromatosis and Schwannoma Cases
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