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Early and Midterm Outcomes of Surgical Correction for Severe Dystrophic Cervical Kyphosis in Patients with Neurofibromatosis Type 1: A Retrospective Multicenter Study.

Abstract：

OBJECTIVE:To evaluate the early and midterm outcomes of surgical correction for severe dystrophic cervical kyphosis in patients with neurofibromatosis type 1 (NF-1) and analyze the pathomechanics and the influence on surgical efficacy of related systemic skeletal dystrophy. METHODS:Ten patients who underwent surgical correction for NF-1-related severe dystrophic cervical kyphosis were reviewed. Radiographic parameters, including local and global Cobb angle, sagittal vertical axis, and T-1 slope, were measured. The visual analog scale score, Japanese Orthopaedic Association score, Neck Disability Index, Patient Satisfaction Index, and complications were evaluated. RESULTS:The average follow-up was 50.6 months. The local and global Cobb angle improved from the preoperative average of 82.0° and 54.9° to an average of 35.6° and 29.8°, respectively, at the time of final follow-up. The C2-7 sagittal vertical axis averaged 5.8 mm before surgery and 8.9 mm at the final follow-up. The average T1 slope was -12.3° before surgery and -1.6° at the final follow-up. The visual analog scale score, Japanese Orthopaedic Association score, and Neck Disability Index improved significantly, and the overall satisfaction rate was 90.0%. One death and 4 instrumentation failures occurred, 3 patients showed progression of the kyphosis, and 2 fusion failures were observed. CONCLUSIONS:Surgical correction, specifically the combined anteroposterior procedure, is essential and effective for management of NF-1-related severe dystrophic cervical kyphosis. However, high incidences of instrumentation failure, kyphosis progression, and fusion failure were observed. NF-1-related continuous skeletal dystrophy caused by multiple metabolic factors remarkably affected the midterm outcomes. Early prevention and targeted pharmacotherapy may be necessary.

journal_name

World Neurosurg

journal_title

World neurosurgery

authors

Wang J,Liu C,Wang C,Li J,Lv G,A J,Deng Y,Wang W

doi

10.1016/j.wneu.2019.04.096

subject

Has Abstract

pub_date

2019-07-01 00:00:00

pages

e1190-e1200

eissn

1878-8750

issn

1878-8769

pii

S1878-8750(19)31088-5

journal_volume

127

pub_type

杂志文章,多中心研究

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