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Background Context: Perioperative visual loss (POVL) after spinal deformity surgery is an uncommon but severe complication. Data on the incidence and risk factors of this complication after corrective surgery in the pediatric population are limited., Purpose: The present study aimed to investigate nationwide estimates of POVL after corrective surgery for pediatric scoliosis., Study Design: This is a retrospective study that uses a nationwide database., Patient Sample: The sample includes 42,339 patients under the age of 18 who underwent surgery for idiopathic scoliosis., Outcome Measures: The outcome measures were incidence of POVL and risk factors., Methods: Patients under the age of 18 who underwent elective surgery for idiopathic scoliosis between 2002 and 2011 were identified using the Nationwide Inpatient Sample database. The incidence of POVL (ischemic optic neuropathy, central retinal artery occlusion, or cortical blindness) was estimated after application of discharge weights. Demographics, comorbidities, and operative parameters were compared between patients with and without visual loss. A multivariate logistic regression was performed to identify significant risk factors for POVL development. No funds were received in support of this work., Results: The incidence of POVL was 1.6 per 1,000 procedures (0.16%). Patients with visual loss were significantly more likely to be younger and male, have Medicaid as insurance, and undergo fusion of eight or more spinal levels compared with patients without visual loss. Following multivariate analysis, older patients (odds ratio [OR]: 0.84; 95% confidence interval [CI]: 0.77-0.91) and female patients (OR: 0.08; 95% CI: 0.04-0.14) were significantly less likely to develop POVL compared with younger and male patients. On the other hand, having Medicaid as insurance (OR: 2.13;95% CI: 1.32-3.45), history of deficiency anemia (OR: 8.64; 95% CI: 5.46-14.31), and fusion of eight or more spinal levels (OR: 2.40; 95% CI: 1.34-4.30) were all independently associated with POVL., Conclusions: In this nationwide study, the incidence of POVL after scoliosis surgery in patients under the age of 18 was estimated at 0.16%, similar to the rate reported in adult patients. Cortical blindness accounted for all cases of POVL in the present study. Younger patients, patients with history of deficiency anemia, and patients undergoing long-segment fusions may be at increased risk of POVL after corrective surgery for pediatric scoliosis., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Stenosis and kyphosis are common in achondroplasia, often requiring lumbar fusion, sometimes to the sacrum. The purpose of this study was to determine the functional effect of lumbar fusion to the sacrum in patients with achondroplasia., Methods: Functional and SF-36 questionnaires were sent to the 66 patients with achondroplasia who underwent instrumented lumbar fusion at our institution from 1991 to 2006. Of the 35 who returned questionnaires, 13 had fusions to the sacrum and 22 did not. Chi-square analysis and a t test were used to evaluate outcomes. Significance was set at P<0.05 or a 95% confidence interval that did not cross 1., Results: There were no statistical differences in SF-36 scores. There was a trend toward more pain in the group fused to the sacrum (P=0.1). There were no statistically significant differences in difficulty with activities, but there was a trend toward increased difficulty with hygiene after toileting in the group with fusion to the sacrum (odds ratio: 3.76, confidence interval: 0.53-26.87, P=0.19)., Conclusions: Fusion to the sacrum did not significantly affect function in patients with achondroplasia, although there was a trend toward increased pain and greater difficulty with hygiene after toileting.

Study Design: Retrospective prognostic study., Objective: To evaluate the safety and efficacy of cervical arthrodesis for cervical instability in patients with skeletal dysplasia., Summary of Background Data: Individuals with certain skeletal dysplasias have a high incidence of cervical instability, which can lead to compression of the spinal cord and subsequent severe spinal cord symptoms, progressive neurologic decline, quadriplegia, and death., Materials and Methods: The charts of 25 patients with skeletal dysplasia (spondyloepiphyseal dysplasia, spondyloepimetaphyseal dysplasia, pseudoachondroplasia, Morquio, or Kniest) who had undergone cervical arthrodesis to treat instability were reviewed for evidence of fusion, neurologic improvement, and complications., Results: Of the 25 patients, 23 (92%) achieved a solid bony fusion, and 5 (20%) experienced surgery-related complications. One of the two patients who did not achieve fusion had a stable pseudarthrosis without neurologic complications, and additional surgical intervention was unnecessary; the second patient is contemplating revision surgery. Of 16 patients with preoperative neurologic manifestations, 14 (88%) experienced improvement., Conclusion: Cervical arthrodesis can be a safe and effective treatment for patients with skeletal dysplasia and cervical instability, despite the inherent complications associated with a dysplastic skeleton. The procedure can preserve and/or improve neurologic function while minimizing the risk of neurologic injury from spinal cord compression.

Study Design: Retrospective radiograph and chart review., Objectives: To assess the safety, efficacy, and complications after posterior spinal arthrodesis with instrumentation for thoracolumbar kyphosis in the pediatric achondroplast., Summary of Background Data: The conventional approach of anterior and posterior arthrodesis has achieved minimal correction, avoided instrumentation, and had a high risk of neurologic deterioration. To the current authors' knowledge, there are only two reports of successful instrumentation with pedicle screw fixation for kyphosis in pediatric achondroplasts; the outcome of such procedures remains largely unknown., Methods: The current authors evaluated the results of posterior spinal arthrodeses with instrumentation performed between 1998 and 2001 on 12 consecutive patients (mean age, 12 years). Indications for arthrodesis were concomitant laminectomy for neurologic symptoms or progressive deformity. Arthrodesis methods included anterior and posterior arthrodesis (5 patients) and posterior arthrodesis only (7 patients). Intraoperative somatosensory-evoked potentials, pedicle screw stimulation, and/or a wake-up test were used to confirm neurologic status in all patients., Results: Successful fusion was obtained in all patients. No intraoperative or postoperative neurologic deterioration was encountered. Mean improvement in kyphotic deformity was 50%. Complications included three instrumentation fractures (2 patients) and one dural leak. Parents rated outcome as excellent (4), good (6), and fair (2). No patients have subsequently experienced deformity progression., Conclusions: Spinal arthrodesis with vertebral body and/or pedicle screw instrumentation was a reliable technique for treating thoracolumbar kyphosis in achondroplasia and did not precipitate any of the neuromonitoring difficulties or neurologic deficits that have been reported in previous studies.

Persistent thoracolumbar kyphosis in patients with achondroplasia is typically prevented with sitting modifications and bracing. When the kyphosis persists and progresses despite bracing, spinal fusion is indicated to prevent further progression and neurologic complications. Previous reports have suggested that instrumentation in such patients carries a high risk of neurologic injury. The purpose of this study was to evaluate the safety and efficacy of a two-stage procedure to control progressive kyphosis in the patient with achondroplasia. The authors treated four such patients (4-8 years old) surgically. The first stage involved an anterior spinal fusion with instrumentation and a posterior spinal fusion, and the second stage involved an additional posterior fusion. There were no neurologic complications. Correction ranged from 23.0% to 31.25%. All patients achieved a solid fusion. These results suggest that when nonoperative treatments fail, this procedure for thoracolumbar kyphosis in the achondroplastic patient can be done safely and effectively.