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Andrew C. Vivas, Steven W. Hwang, and Joshua M. Pahys

Phrenic stimulators offer an alternative to standard mechanical ventilation as well as the potential for ventilator independence in select patients with chronic respiratory failure. Young patients (< 10 years old) with high cervical spinal cord injuries often develop paralytic scoliosis due to loss of muscle tone caudal to their spinal cord lesion. Growing rod systems allow for stabilization of spinal deformity while permitting continued growth of the spine and thoracic cavity. Magnetically controlled growing rods (MCGRs) offer the advantage of noninvasive expansion, as opposed to the operative expansion required in traditional growing rod systems. To the authors’ knowledge, this is the first reported case of MCGRs in a patient with a diaphragmatic pacemaker (DP). A 7-year-old boy with ventilator dependence after a high cervical spinal cord injury presented to the authors’ institution with paralytic scoliosis that progressed to > 120°. The patient had previously undergone insertion of phrenic nerve stimulators for diaphragmatic pacing. The decision was made to insert MCGRs bilaterally to stabilize his deformity, because the planned lengthening surgeries that are necessary with traditional growing rods would be poorly tolerated in this patient. The patient’s surgery and postoperative course were uneventful. The DP remained functional after insertion and lengthening of the MCGRs by using the external magnet. The DP had no effect on the expansion capability of the MCGRs. In conclusion, the MCGRs appear to be compatible with the DP. Further studies are needed to validate the long-term safety and compatibility of these 2 devices.

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Maria Zuccaro, James Zuccaro, Amer F. Samdani, Joshua M. Pahys, and Steven W. Hwang

OBJECTIVE

Intraoperative neuromonitoring (IONM) involves the use of somatosensory evoked potentials (SSEPs) and transcranial electric motor evoked potentials (TceMEPs). In this retrospective study the authors examined the sensitivity and specificity of both SSEPs and TceMEPs during pediatric spinal deformity surgeries.

METHODS

The authors performed a retrospective quantitative analysis of data obtained in 806 patients (197 males and 609 females) treated from December 2011 until October 2015. All patients were diagnosed with scoliosis that was classified as one of the following: adolescent idiopathic scoliosis (AIS) (38%), congenital scoliosis (22%), or syndromic scoliosis (40%). Also, 53 patients underwent vertebral column resection (VCR). All surgeries were monitored by high-level neuromonitoring specialists and were performed with total intravenous anesthesia. Alerts were described as a decrease in amplitude by 50% or greater (bilateral or unilateral) in SSEPs, TceMEPs, or both.

RESULTS

True-positive alerts for TceMEPs were observed in 60 of the 806 patients (7.4%). True-positive alerts for SSEPs were observed in 7 of the 806 patients (0.9%). In contrast, there were no false-positive or false-negative outcomes. Only 1 case (0.1%) was reported with a permanent postoperative deficit. No reported false negatives or false positives were observed, and thus sensitivity was 100% and specificity was 93%–100% for TceMEPs. The rate of sensitivity was 13.2% and the rate of specificity was 100% for SSEPs. The breakdown of total alert was as follows: 6.6% in AIS cases, 24.5% in congenital scoliosis cases, and 10.2% in syndromic scoliosis cases. Neurological injury rates were significantly lower than in previous studies, as there were 0% for AIS cases (p = 0.12), 0.6% for congenital scoliosis cases (p = 0.17), and 0% for syndromic scoliosis cases (p = 0.07). One injury in a patient with congenital scoliosis occurred during a VCR procedure, which brought the injury rate to 1.9% (p < 0.005). IONM alerts occurred during 34% of rod/correction cases, 25% of thoracic screw placements, 20% of the osteotomies, 17% of the resections, 3% of the cage insertions, and 2% of the sublaminar wiring procedures.

CONCLUSIONS

The authors hypothesize that the results of this study will support the necessity, as a standard of care, of multimodality neuromonitoring during high-risk pediatric spinal deformity surgery because of the decrease in postoperative deficits. Their data suggest that the TceMEPs are more sensitive than SSEPs, but when used in combination, they offer the patient a level of safety that would otherwise not exist. Last, these findings support the notion that better outcomes are achieved with high-level IONM professionals.

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Brandon J. Toll, Amer F. Samdani, M. Burhan Janjua, Shashank Gandhi, Joshua M. Pahys, and Steven W. Hwang

OBJECTIVE

High rates of perioperative complications are associated with deformity correction in neuromuscular scoliosis. The current study aimed to evaluate complications associated with surgical correction of neuromuscular scoliosis and to characterize potential risk factors.

METHODS

Data were retrospectively collected from a single-center cohort of 102 consecutive patients who underwent spinal fusions for neuromuscular scoliosis between January 2008 and December 2016 and who had a minimum of 6 months of follow-up. A subgroup analysis was performed on data from patients who had at least 2 years of follow-up. Univariate and multivariate regression analyses, as well as binary correlational models and Student t-tests, were employed for further statistical analysis.

RESULTS

The present cohort had 53 boys and 49 girls with a mean age at surgery of 14.0 years (± 2.7 SD, range 7.5–19.5 years). The most prevalent diagnoses were cerebral palsy (26.5%), spinal cord injury (24.5%), and neurofibromatosis (10.8%). Analysis reflected an overall perioperative complication rate of 27% (37 complications in 27 patients), 81.1% of which constituted major complications (n = 30) compared to a rate of 18.9% for minor complications (n = 7). Complications were predicted by nonambulatory status (p = 0.037), increased intraoperative blood loss (p = 0.012), increased intraoperative time (p = 0.046), greater pelvic obliquity at follow-up (p = 0.028), and greater magnitude of sagittal profile at follow-up (p = 0.048). Pulmonary comorbidity (p = 0.001), previous operations (p = 0.013), history of seizures (p = 0.046), diagnosis of myelomeningocele (p = 0.046), increase in weight postoperatively (p < 0.005), and increased lumbar lordosis at follow-up (p = 0.015) were identified as risk factors for perioperative infection.

CONCLUSIONS

These results suggest that in neuromuscular scoliosis, patients with preexisting pulmonary compromise and greater intraoperative blood loss have the greatest risk of experiencing a major perioperative complication following surgical deformity correction.

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Daniel J. Cognetti, Amer F. Samdani, Joshua M. Pahys, Mari L. Groves, and Steven W. Hwang

Growing rod surgery for skeletally immature patients helps correct severe scoliosis while allowing continued spinal column growth. Previous reports have studied vertebral body changes following growing rod surgery, but there are currently no published reports on alterations in pedicle morphology. Given the potential need for definitive spinal fusion with pedicle screw instrumentation, an awareness of changes in pedicle morphology is critical. A morphometric analysis of pedicles was performed using 3D reconstructions of 3 CT scans (preoperative and at 3 and 6 years) obtained in a young girl with infantile idiopathic scoliosis (T7 apex) who underwent unilateral rib-to-spine growing rod (2nd–4th ribs to L1) implantation with lengthening every 6 months for 6 years. The pedicle widths on the growing rod side from T5 to T9 (apex ± 2) were all smaller at 6 years postoperatively than preoperatively, while the same-level pedicles opposite the device significantly increased in width. These findings support anecdotal intraoperative reports by surgeons and provide evidence of pedicle stress shielding due to growing rod distraction and force deprivation.

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Daniel Cognetti, Heather M. Keeny, Amer F. Samdani, Joshua M. Pahys, Darrell S. Hanson, Kathy Blanke, and Steven W. Hwang

OBJECTIVE

Postoperative complications are one of the most significant concerns in surgeries of the spine, especially in higher-risk cases such as neuromuscular scoliosis. Neuromuscular scoliosis is a classification of multiple diseases affecting the neuromotor system or musculature of patients leading to severe degrees of spinal deformation, disability, and comorbidity, all likely contributing to higher rates of postoperative complications. The objective of this study was to evaluate deformity correction of patients with neuromuscular scoliosis over a 12-year period (2004–2015) by looking at changes in postsurgical complications and management.

METHODS

The authors queried the Scoliosis Research Society (SRS) Morbidity and Mortality (M&M) database for neuromuscular scoliosis cases from 2004 to 2015. The SRS M&M database is an international database with thousands of self-reported cases by fellowship-trained surgeons. The database has previously been validated, but reorganization in 2008 created less-robust data sets from 2008 to 2011. Consequently, the majority of analysis in this report was performed using cohorts that bookend the 12-year period (2004–2007 and 2012–2015). Of the 312 individual fields recorded per patient, demographic analysis was completed for age, sex, diagnosis, and preoperative curvature. Analysis of complications included infection, bleeding, mortality, respiratory, neurological deficit, and management practices.

RESULTS

From 2004 to 2015, a total of 29,019 cases of neuromuscular scoliosis were reported with 1385 complications, equating to a 6.3% complication rate when excluding the less-robust data from 2008 to 2011. This study shows a 3.5-fold decrease in overall complication rates from 2004 to 2015. A closer look at complications shows a significant decrease in wound infections (superficial and deep), respiratory complications, and implant-associated complications. The overall complication rate decreased by approximately 10% from 2004–2007 to 2012–2015.

CONCLUSIONS

This study demonstrates a substantial decrease in complication rates from 2004 to 2015 for patients with neuromuscular scoliosis undergoing spine surgery. Decreases in specific complications, such as surgical site infection, allow us to gauge our progress while observing how trends in management affect outcomes. Further study is needed to validate this report, but these results are encouraging, helping to reinforce efforts toward continual improvement in patient care.

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Jason R. Smith, Amer F. Samdani, Joshua Pahys, Ashish Ranade, Jahangir Asghar, Patrick Cahill, and Randal R. Betz

Object

There are few data on treatment results for patients with idiopathic infantile scoliosis (IIS). Thus, the authors have performed a retrospective review of their experience with treating these patients, particularly as newer technologies, such as the vertical expandable prosthetic titanium rib (VEPTR), emerge.

Methods

This retrospective study was conducted to evaluate the methods of treatment used to manage IIS at a single institution. The authors reviewed 31 consecutive patients with a primary diagnosis of IIS. Patients were screened to ensure that there were no confounding congenital anomalies or comorbidities that may have contributed to the spinal deformity. The average age at the time of initial treatment was 25 months. Treatment modalities included bracing, serial body casting, and VEPTR. Pretreatment, posttreatment, and most recent Cobb angles were compared to assess the overall curve correction, and patient charts were reviewed for the occurrence of complications.

Results

Of the 31 patients, 17 were treated with a brace, 9 of whom had curve progression and went on to other forms of treatment. Of the 8 who did respond, there was an overall improvement of 51.2%. The 10 patients who received body casts, who had a mean preoperative Cobb angle of 50.4°, demonstrated an average correction of 59.0%, with only a few skin irritations reported. The 10 patients treated with VEPTR devices demonstrated a mean preoperative Cobb angle of 90.0°, and an average correction of 33.8% was attained. Three of the VEPTR-treated patients (33%) experienced minor problems.

Conclusions

The authors' results suggest that body casting has utility for appropriately selected patients; that is, those with smaller, flexible spinal curves. Bracing had limited utility, with high levels of progression and the need for secondary treatments. The VEPTR device appears to be a viable alternative for large-magnitude curves.

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James T. Bennett, Jane S. Hoashi, Robert J. Ames, Jeff S. Kimball, Joshua M. Pahys, and Amer F. Samdani

Object

Several studies of the outcomes of patients with adolescent idiopathic scoliosis (AIS) with thoracolumbar and lumbar curves after treatment with posterior pedicle screws have been reported, but most of these studies reported only 2-year follow-up. The authors analyzed the radiographic and clinical outcomes of patients with thoracolumbar and lumbar curves treated with posterior pedicle screws after 5 years of follow-up.

Methods

A multicenter database was retrospectively queried to identify patients with AIS who underwent spinal fusion for Lenke 3C, 5C, and 6C curves. Radiographs from the following times were compared: preoperative, first follow-up visit, 1-year follow-up visit, 2-year follow-up visit, and 5-year follow-up visit. Chart review included scoliometer measurements, Scoliosis Research Society (SRS)–22 questionnaires, and complications requiring return to the operating room.

Results

Among 26 patients with Lenke 3C, 5C, and 6C curves, the mean (± SD) age was 14.6 ± 2.1 years. From the time of the preoperative radiographs to the 5-year follow-up radiographs, there was a statistically significant improvement in the mean coronal lumbar Cobb angles (p < 0.0001), and from the time of the first postoperative radiographs to the 5-year follow-up radiographs, the lumbar curve remained stable (p = 0.14). From the time of the preoperative radiographs to the 5-year follow-up radiographs, there was a statistically significant improvement in the mean coronal thoracic Cobb angles (p < 0.0001), and from the time of the first postoperative radiographs to the 5-year follow-up radiographs, the thoracic curve remained stable (p = 0.10). From the first postoperative visit to the 5-year follow-up visit, the thoracic kyphosis (T5–12) remained stable (p = 0.10), and from the time of the preoperative radiographs to the 5-year follow-up radiographs, the lumbar lordosis (T-12 to top of sacrum) remained stable (p = 0.44). From the preoperative visit to the 5-year follow-up visit, the coronal balance improved significantly (p < 0.05) and remained stable from the first postoperative visit to the 5-year follow-up visit (p = 0.20). The SRS-22 total scores improved significantly from before surgery to 5 years after surgery (p < 0.0001). No patients required reoperation because of complications.

Conclusions

Correction of the coronal, sagittal, and axial planes in this cohort of patients was maintained from the first follow-up measurements to 5 years after surgery. In addition, at 5 years after surgery total SRS-22 scores and inclinometer readings were improved from preoperative scores and measurements.

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Jonathan E. Martin, Brandon G. Rocque, Andrew Jea, Richard C. E. Anderson, Joshua Pahys, and Douglas Brockmeyer

OBJECTIVE

Hypermobility of the craniocervical junction (CCJ) in patients with Down syndrome (DS) is common. Whereas atlantoaxial (C1–2) hypermobility is well characterized, occipitoatlantal (Oc–C1) laxity is recognized but poorly defined. A clear understanding of the risks associated with DS-related hypermobility is lacking. Research efforts to address the topic of axial cervical spine instability in the patient with DS require a reliable and reproducible means of assessing CCJ mobility. The authors conducted a pilot study comparing two methods of quantifying motion of the CCJ on dynamic (flexion/extension) plain radiographs: the delta–condyle-axial interval (ΔCAI) and the delta–basion-axial interval (ΔBAI) methods.

METHODS

Dynamic radiographs from a cohort of 10 patients with DS were evaluated according to prescribed standards. Independent movement of Oc–C1, C1–2, and Oc–C2 was calculated. Interrater and intrarater reliability for CCJ mobility was then calculated for both techniques.

RESULTS

Measurement using the ΔCAI technique had excellent fidelity with intraclass correlation coefficients (ICCs) of 0.77, 0.71, and 0.80 for Oc–C1, C1–2, and Oc–C2, respectively. The ΔBAI technique had lower fidelity, yielding respective ICCs of 0.61, 0.65, and 0.50.

CONCLUSIONS

This pilot study suggests that ΔCAI is a superior measurement technique compared to ΔBAI and may provide reliable assessment of the mobility of the CCJ on dynamic radiographs in the pediatric patient with DS. The use of reliable and reproducible measurement techniques strengthens the validity of research derived from pooled database efforts.

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Charles E. Mackel, Patrick J. Cahill, Marie Roguski, Amer F. Samdani, Patrick A. Sugrue, Noriaki Kawakami, Peter F. Sturm, Joshua M. Pahys, Randal R. Betz, Ron El-Hawary, and Steven W. Hwang

OBJECTIVE

The authors performed a study to identify clinical characteristics of pediatric patients diagnosed with Chiari I malformation and scoliosis associated with a need for spinal fusion after posterior fossa decompression when managing the scoliotic curve.

METHODS

The authors conducted a multicenter retrospective review of 44 patients, aged 18 years or younger, diagnosed with Chiari I malformation and scoliosis who underwent posterior fossa decompression from 2000 to 2010. The outcome of interest was the need for spinal fusion after decompression.

RESULTS

Overall, 18 patients (40%) underwent posterior fossa decompression alone, and 26 patients (60%) required a spinal fusion after the decompression. The mean Cobb angle at presentation and the proportion of patients with curves > 35° differed between the decompression-only and fusion cohorts (30.7° ± 11.8° vs 52.1° ± 26.3°, p = 0.002; 5 of 18 vs 17 of 26, p = 0.031). An odds ratio of 1.0625 favoring a need for fusion was established for each 1° of increase in Cobb angle (p = 0.012, OR 1.0625, 95% CI 1.0135–1.1138). Among the 14 patients older than 10 years of age with a primary Cobb angle exceeding 35°, 13 (93%) ultimately required fusion. Patients with at least 1 year of follow-up whose curves progressed more 10° after decompression were younger than those without curve progression (6.1 ± 3.0 years vs 13.7 ± 3.2 years, p = 0.001, Mann-Whitney U-test). Left apical thoracic curves constituted a higher proportion of curves in the decompression-only group (8 of 16 vs 1 of 21, p = 0.002).

CONCLUSIONS

The need for fusion after posterior fossa decompression reflected the curve severity at clinical presentation. Patients presenting with curves measuring > 35°, as well as those greater than 10 years of age, may be at greater risk for requiring fusion after posterior fossa decompression, while patients less than 10 years of age may require routine monitoring for curve progression. Left apical thoracic curves may have a better response to Chiari malformation decompression.

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M. Omar Iqbal, Amer F. Samdani, Joshua M. Pahys, Peter O. Newton, Suken A. Shah, Tracey P. Bastrom, Paul D. Sponseller, Firoz Miyanji, and Steven W. Hwang

OBJECTIVE

Spontaneous lumbar curve correction after selective thoracic fusion in surgery for adolescent idiopathic scoliosis (AIS) is well described. However, only a few articles have described the course of the uninstrumented upper thoracic (UT) curve after fusion, and the majority involve a hybrid construct. In this study, the authors sought to determine the outcomes and associated factors of uninstrumented UT curves in patients with AIS.

METHODS

The authors retrospectively reviewed a prospectively collected multicenter AIS registry for all consecutive patients with Lenke type 1–4 curves with a 2-year minimum follow-up. UT curves were considered uninstrumented if the upper instrumented vertebra (UIV) did not extend above 1 level from the lower end vertebra of the UT curve. The authors defined progression as > 5°, and divided patients into two cohorts: those with improvement in the UT curve (IMP) and those without improvement in the UT curve (NO IMP). Radiographic, demographic, and Scoliosis Research Society (SRS)–22 survey outcome measures were compared using univariate analysis, and significant factors were compared using a multivariate regression model.

RESULTS

The study included 450 patients (370 females and 80 males). The UT curve self-corrected in 86% of patients (n = 385), there was no change in 14% (n = 65), and no patients worsened. Preoperatively, patients were similar with respect to Lenke classification (p = 0.44), age (p = 0.31), sex (p = 0.85), and Risser score (p = 0.14). The UT curves in the IMP group self-corrected from 24.7° ± 6.5° to 12.6° ± 5.9°, whereas in the NO IMP group UT curves remained the same, from 20.3° ± 5.8° to 18.5° ± 5.7°. In a multivariate analysis, preoperative main thoracic (MT) curve size (p = 0.004) and MT curve correction (p = 0.001) remained significant predictors of UT curve improvement. Greater correction of the MT curve and larger initial MT curve size were associated with greater likelihood of UT curve improvement.

CONCLUSIONS

Spontaneous UT curve correction occurred in the majority (86%) of unfused UT curves after MT curve correction in Lenke 1–4 curve types. The magnitude of preoperative MT curve size and postoperative MT curve correction were independent predictors of spontaneous UT curve correction.