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Surgical challenges and functional outcomes in dystrophic cervical kyphosis in Neurofibromatosis -1: an institutional experience

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Abstract

Objective

Dystrophic cervical kyphosis secondary to neurofibromatosis 1 (NF1) is rarely reported. The primary objective is to highlight the clinical presentation and surgical outcomes based on clinical and biomechanical parameters. The secondary objective is to highlight the early and late complications of these surgeries.

Methods

The hospital records of six patients operated between 2008 and 2018 were retrospectively reviewed with a minimum follow-up of 24 months. Besides demographics information, radiological findings and operative details, the outcome measures reported were neurological (MJOA score, Nurick scale), pain (VAS score) and operative complications.

Results

The mean age of patients was 15.1 years (8–32 years). The average kyphotic vertebra involved—3.6 bodies (range 2–5 bodies) with four patients showing intraspinal anomaly—neurofibromas, dural ectasia. Clinically, patients improved postoperatively with—VAS (pre vs. post-: 6.6 vs. 2.6, p − < 0.05), MJOA score (pre vs. post: 10.3 vs. 13.3, p − < 0.05), Nurick grade (pre vs. post: 3.5 vs. 2.1, p − < 0.05). There was significant deformity correction from 66.8° to 20.7° (p value < 0.031), mean T1 Slope (pre vs. post − 1.8 ± 20.4 to 0.6 ± 12.8, p value − < 0.43). Mean blood loss encountered was 1800 ± 434.6 cc; however, patients with paraspinal neurofibromas reported greater blood loss. Late complications included pseudoarthrosis (1), C5 palsy (1) and junctional kyphosis (1). Vertebral dysplasia and erosions continued in all patients post-operatively.

Conclusions

Antero-posterior approach provides circumferential decompression and better sagittal balance correction. Cervical spine must be screened in all NF-1 patients and followed up regularly to assess for dystrophic changes.

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Availability of data and material

Available on request.

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Abbreviations

CT:

Computed tomography

MJOA:

Modified Japanese Orthopaedic Association

NDI:

Neck Disability Index

NF-1:

Neurofibromatosis Type 1

AP:

Antero-posterior approach

AO:

Anterior only

PO:

Posterior only

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Funding

There is no financial support pertinent to the paper. The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article.

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Author notes

  1. Both Shrijith Murlidharan and Pankaj Kumar Singh have contributed equally and are first authors.

Authors and Affiliations

  1. Department of Neurosurgery and Gamma Knife Center, All India Institute of Medical Sciences, New Delhi, Room No 720, 7th Floor, C.N. Tower, New Delhi, 110029, India

    Shrijith Murlidharan, Pankaj Kumar Singh, P. Sarat Chandra, Deepak Agarwal & Shashank Sharad Kale

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  1. Shrijith Murlidharan
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  2. Pankaj Kumar Singh
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Contributions

Made substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data; or the creation of new software used in the work: SM, PKS, PSC, DA, SSK. Drafted the work or revised it critically for important intellectual content: SM, PKS, PSC, DA, SSK. Approved the version to be published: SM, PKS, PSC, DA, SSK. Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved: SM, PKS, PSC, DA, SSK.

Corresponding author

Correspondence to Shashank Sharad Kale.

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Conflict of interest

Dr. Shrijith Murlidharan (SM) certifies that I have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript. Dr. Pankaj Kumar Singh (PKS) certify that I have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript. Dr. Sarat Chandra (PSC) certifies that I have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript. Dr. Deepak Agarwal (DA) certifies that I have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript. Dr. Shashank Sharad Kale (SSK) certifies that I have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript.

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Murlidharan, S., Singh, P.K., Chandra, P.S. et al. Surgical challenges and functional outcomes in dystrophic cervical kyphosis in Neurofibromatosis -1: an institutional experience. Spine Deform 10, 697–707 (2022). https://doi.org/10.1007/s43390-021-00465-6

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