J Korean Neurosurg Soc.  2020 Nov;63(6):738-746. 10.3340/jkns.2019.0218.

Surgical Outcomes and Complications Following All Posterior Approach for Spinal Deformity Associated with Neurofibromatosis Type-1

• Park BJ
• Hyun SJ
• Wui SH
• Jung JM
• Kim KJ
• Jahng TA

• KMID: 2508604
• DOI: http://doi.org/10.3340/jkns.2019.0218

Abstract

Objectives
: The purpose of this study was to evaluate surgical outcomes and complications of spinal deformity associated with neurofibromatosis type-1 (NF-1).
Methods
: From 2012 to 2018, patients suffering from spinal deformity associated with NF-1 who underwent surgical correction were identified. Demographic data and radiographic measures were retrospectively reviewed. Pre- and postoperative whole spine radiograph images were used to determine both coronal and sagittal Cobb angles. All of patients underwent 3-dimentional computed tomographic scan and magnetic resonance imaging scan to confirm dystrophic features. For evaluation of clinical outcomes, we surveyed the pre- and postoperative scoliosis research society-22r (SRS-22r) score.
Results
: Seven patients with spinal deformity associated with NF-1 were enrolled in this study. The mean age of patients was 29.5±1.2 years old. The mean follow-up period was 2.8±1.4 years. The apex of the deformity was located in cervicothoracic (n=1), thoracic (n=4), and lumbar region (n=2). Most patients have poor bone quality and decreased bone mineral density with average T-score of -3.5±1.0. All patients underwent surgical correction via posterior approach. The pre- and postoperative mean coronal and sagittal Cobb angle was 61.6±22.6° and 34.6±38.1°, 56.8±18.5° and 40.2±9.1°, respectively. Mean correction rate of coronal and sagittal angle was 44.7% and 23.1%. Ultimate follow-up SRS-22r score (average score, 3.9±0.4) improved comparing to preoperative score (average score, 3.3±0.9). Only one patient received revision surgery due to rod fracture. No serious complication occurred, such as neurological deficit, and viscerovascular injury.
Conclusion
: The surgical correction of patients having spinal deformity associated with NF-1 is challenging, however the radiographic and clinical outcomes are satisfactory. The all posterior approach can be a safe and effective surgical option for patients having dystrophic curves associated with NF-1.

Keyword

Neurofibromatosis 1; Scoliosis; Kyphosis; Surgical procedures; Complication; Instrumentation

Figure

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  Fig. 1. Pre- and postoperative mean SRS-22r-score of all patients. Preop. : preoperative, Postop. : postoperative, SRS-22r : scoliosis research society-22r.

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  Fig. 2. Preoperative (A) and postoperative (B) plain radiographs of a 17-year old female patient (case No. 7).

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  Fig. 3. A and B : Follow-up plain radiographs after initial surgery showing rod fracture (red arrow) above side connector of iliac screw on the right side. C : Postoperative plain radiograph after revision surgery using multiple rod construct by 4-Cobalt Chrome rods. D : Two-year follow-up plain radiograph of a 24-year old male patient (case No. 5).

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  Fig. 4. A : Preoperative whole spine radiograph showing trunk shift to the left side. B : Postoperative plain radiograph revealing improvement of the coronal imbalance. C : Two-year follow-up plain radiograph of a 54-year old female patient (case No. 1). A horizontal line is drawn through the apex of thoracic curve. The bisecting perpendicular line represents the vertical trunk reference line (dashed). The central sacral vertical line (line), and the C7 plumb line (dotted).

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