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Jonathan G. Thomas, Jerome Boatey, Alison Brayton and Andrew Jea

Object

Outside of the patient population with achondroplasia, neurogenic claudication is rare in the pediatric age group. Neurogenic claudication associated with posterior vertebral rim fracture is even more uncommon but nonetheless causes pain and disability in affected children and adolescents. The purpose of this study was to describe the surgical results of 3 adolescents presenting with neurogenic claudication and posterior vertebral rim fracture when treated with laminectomy alone.

Methods

The medical and operative records of the 3 pediatric patients were retrospectively reviewed. Presenting signs and symptoms and CT findings, such as the interpedicular distances between T-12 and L-5, were obtained. Perioperative results were assessed, including operative time, blood loss, length of hospital stay, and complications. Findings at latest follow-up were also recorded, including a patient satisfaction survey.

Results

The 3 patients (1 girl and 2 boys) had a mean age of 14.7 years (range 14–15 years) and underwent follow-up for a mean of 11.3 months (range 5–18 months). Notable preoperative signs and symptoms included back pain (all patients), leg pain (all patients), leg numbness (1 patient), and leg weakness (1 patient). No patient presented with bowel and/or bladder dysfunction. The mean blood loss during laminectomy was 123 ml (range 20–300 ml), and the mean length of hospital stay was 4.3 days (range 3–6 days). On average, decompression was performed at 2.2 levels (range 2–2.5 levels). All 3 patients reported at most recent follow-up that they were “satisfied” with the surgery. There was 1 complication of instability from an iatrogenic pars fracture, which required reoperation and posterior instrumented fusion.

Conclusions

To the best of the authors' knowledge, this report represents the first surgical series of pediatric neurogenic claudication associated with posterior vertebral rim fractures. Pediatric neurosurgeons may infrequently encounter neurogenic claudication associated with a posterior vertebral rim fracture in children. To treat children with neurogenic claudication associated with posterior vertebral rim fractures, a simple laminectomy may be a safe and efficacious alternative to discectomy and removal of fracture fragments.

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Lourdes C. Eco, Alison Brayton, William E. Whitehead and Andrew Jea

Chordomas are histologically benign tumors with local aggressive behavior. They arise from embryological remnants of the notochord at the clivus, mobile spine, and sacrum. Chordomas are rare tumors in the pediatric age group. Their surgical management is difficult, given their propensity for inaccessible anatomical regions, and proximity to critical neurovascular structures. While en bloc resection with surgical margins has been advocated as the preferred approach for chordomas, tumor characteristics and violation of adjacent anatomical boundaries may not allow for safe en bloc resection of the tumor. Here, the authors present the case of a C1 chordoma in a 5-year-old boy with epidural and prevertebral extension. The patient’s treatment consisted of a far-lateral approach for resection of the tumor and C1 arch, followed by circumferential reconstruction of the craniocervical junction with an expandable cage spanning the skull base to C2, and posterior occipitocervical spinal instrumentation. At 42 months after surgery, the patient remains neurologically intact with stable oncological status, and no evidence of craniocervical junction instrumentation failure.

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Sohum K. Desai, Christina Sayama, Daniel Vener, Alison Brayton, Valentina Briceño, Thomas G. Luerssen and Andrew Jea

OBJECT

The authors have previously reported on their early experience with sublaminar polyester bands in spine surgery. In this paper, the authors describe the use of sublaminar polyester bands in long-segment posterior instrumented spinal fusions from the upper thoracic spine to the ilium in 21 children and transitional adults with progressive neuromuscular scoliosis. Transitional adults were patients older than 18 years of age with a spinal disorder of pediatric onset, such as spina bifida. This dedicated study represents the first reported use of polyester bands in spine surgery for neuromuscular scoliosis in this patient population in the US.

METHODS

The authors retrospectively reviewed the demographics and procedural data of patients who underwent posterior instrumented fusion using sublaminar polyester bands for neuromuscular scoliosis.

RESULTS

Twenty-one pediatric and adult transitional patients, ranging in age from 10 to 20 years (mean 14 years), underwent posterior instrumented fusion for progressive neuromuscular scoliosis. The average coronal Cobb angle measured 66° before surgery (range 37°–125°). Immediately after surgery, the mean coronal Cobb angle was 40° (range 13°–85°). At last follow-up, the average coronal Cobb angle was maintained at 42° (range 5°–112°). Regarding sagittal parameters, thoracic kyphosis was restored by 8%, and lumbar lordosis improved by 20% after surgery. Mean follow-up duration was 17 months (range 2–54 months). One patient with an aborted procedure due to loss of intraoperative evoked potentials was excluded from the analysis of radiographic outcomes. Mean surgical time was 7 hours 43 minutes (range 3 hours 59 minutes to 10 hours 23 minutes). All patients received either a 12- or 24-mg dose of recombinant human bone morphogenetic protein–2. Average estimated blood loss was 976 ml (range 300–2700 ml). Complications directly related to the use of sublaminar instrumentation included transient proprioceptive deficit (1 patient) and prolonged paraparesis (1 patient). Other complications noted in this series included disengagement of the rod from an iliac screw (1 patient), proximal junctional kyphosis (1 patient), noninfected wound drainage (2 patients), and perioperative death (1 patient). The lessons learned from these complications are discussed.

CONCLUSIONS

Pedicle screws, laminar/pedicle/transverse process hooks, and sublaminar metal wires have been incorporated into posterior spinal constructs and widely reported and used in the thoracic and lumbar spines and sacrum with varying success. This report demonstrates the satisfactory radiological outcomes of hybrid posterior spinal constructs in pediatric and adult neuromuscular scoliosis that include sublaminar polyester bands that promise the technical ease of passing sublaminar instrumentation with the immediate biomechanical rigidity of pedicle screws and hooks. However, the high neurological complication rate associated with this technique (2/21, or 10%) tempers the acceptable radiographic outcomes.

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Sohum K. Desai, Alison Brayton, Valerie B. Chua, Thomas G. Luerssen and Andrew Jea

Spinal arthrodesis was the first successful treatment for scoliosis, performed by Dr. Russell A. Hibbs in 1911 and later by Dr. Fred H. Albee for tuberculosis. In 1914, Dr. H.P.H. Galloway and Dr. Hibbs began using the method to treat neuromuscular scoliosis in patients with poliomyelitis. However, this treatment approach was plagued by loss of deformity correction over time and high pseudarthrosis rates. The turning point in the operative management of spinal deformities began in 1947 with Dr. Paul Randall Harrington when he started a decade-long process to revolutionize surgical treatment of spinal deformities culminating in the advent of the Harrington Rod, the first successful implantable spinal instrumentation system. During the epoch that he was in practice, Dr. Harrington's achievement influenced the technology and art of spine surgery for his contemporaries and the coming generations of spine surgeons. The purpose of this article is to review the life of Dr. Harrington, and how he has arguably come to be known as “Father of the Modern Treatment of Scoliosis.”

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Akash J. Patel, Ahilan Sivaganesan, Robert J. Bollo, Alison Brayton, Thomas G. Luerssen and Andrew Jea

Object

Recent attempts to control health care costs focus on reducing or eliminating payments for complications, hospital-acquired conditions, and provider preventable conditions, with payment restrictions applied uniformly. A patient's preexisting comorbidities likely influence the perioperative complication incidence. This relationship has not previously been examined in pediatric neurosurgery.

Methods

The authors conducted a retrospective assessment of prospectively collected relevant patient comorbidities and morbidity and mortality events at a large pediatric neurosurgical unit over a 5-year period. The authors examined the impact of specific comorbidities and the cumulative effect of multiple comorbidities on complication incidence.

Results

A total of 1990 patients underwent 3195 procedures at the authors' institution during the 5-year study period. Overall, 396 complications were analyzed; 298 patients (15.0%) experienced at least one complication. One or more comorbidities were present in 45.9% of patients. Renal comorbidities were clearly associated with the increased incidence of complications (p = 0.02), and they were specifically associated with infection (p = 0.006). Neurological comorbidities had a borderline association with complications (p = 0.05), and they were specifically associated with death (p = 0.037). A patient's having more comorbidities did not correlate with an increased risk of a perioperative complication (p = 0.8275).

Conclusions

The complication incidence in pediatric neurosurgery is variable and may be influenced by the type of neurosurgical procedure and patient-related factors. While patient-related factors beyond the control of the provider can significantly impact complications and hospital-acquired conditions in pediatric neurosurgery, an increasing number of comorbidities do not correlate with an increased risk of complications per patient.

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Sohum K. Desai, Sudhakar Vadivelu, Akash J. Patel, Alison Brayton and Andrew Jea

Object

Isolated cervical canal stenosis at the level of the atlas (C-1) is a rare cause of cervical myelopathy in the pediatric population. It has been associated with several genetic disorders including spondyloepiphysial dysplasia congenita, Down syndrome, and Klippel-Feil syndrome. The purpose of this study is to highlight the authors' experience with 4 additional pediatric cases, review the literature, and report a new association of this disease with Williams syndrome.

Methods

The medical records and radiological imaging studies of 4 patients treated at Texas Children's Hospital for symptomatic hypoplasia of the atlas were retrospectively reviewed. Pertinent patient demographic data, clinical presentation, imaging findings, and outcomes after surgery were recorded. A thorough literature review was performed, allowing the authors to compare and contrast their 4 cases to surgical cases already published in the literature.

Results

There were 11 boys and 1 girl in the aggregate series. The average age of the patients was 7 years (range 13 months–14 years), and the duration of symptoms prior to presentation was 6 months (range 0–36 months). The mean sagittal diameter of the spinal canal at the level of the atlas measured from the posterior aspect of the dens to the anterior aspect of the arch of C-1 was 11.9 mm (range 8.3–16 mm) in the aggregate series. In 2 new pediatric patients with hypoplasia of the atlas the disease was associated with Williams syndrome, which has not been previously described. Patients in the aggregate series were followed for an average of 18 months (range 3–50 months). Laminectomy of C-1 provided neurological improvement in all patients who presented.

Conclusions

Isolated cervical spinal canal stenosis at the level of the atlas is a rare cause of cervical myelopathy. The authors hope that this report will prompt clinicians to consider it when searching for the origin of signs and symptoms of cervical myelopathy, especially in children.

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Aditya Vedantam, Daniel Hansen, Valentina Briceño, Alison Brayton and Andrew Jea

OBJECTIVE

There is limited literature on patient-reported outcomes (PROs) and health-related quality of life (HRQOL) outcomes in pediatric patients undergoing surgery for craniovertebral junction pathology. The aim of the present study was to assess surgical and quality of life outcomes in children who had undergone occipitocervical or atlantoaxial fusion.

METHODS

The authors retrospectively reviewed the demographics, procedural data, and outcomes of 77 consecutive pediatric patients who underwent posterior occipitocervical or atlantoaxial fusion between 2008 and 2015 at Texas Children's Hospital. Outcome measures (collected at last follow-up) included mortality, neurological improvement, complications, Scoliosis Research Society Outcomes Measure–22 (SRS-22) score, SF-36 score, Neck Disability Index (NDI), and Pediatric Quality of Life Inventory (PedsQL). Multivariate linear regression analysis was performed to identify factors affecting PROs and HRQOL scores at follow-up.

RESULTS

The average age in this series was 10.6 ± 4.5 years. The median follow-up was 13.9 months (range 0.5–121.5 months). Sixty-three patients (81.8%) were treated with occipitocervical fusion, and 14 patients (18.1%) were treated with atlantoaxial fusion. The American Spinal Injury Association (ASIA) grade at discharge was unchanged in 73 patients (94.8%). The average PRO metrics at the time of last follow-up were as follows: SRS-22 score, 4.2 ± 0.8; NDI, 3.0 ± 2.6; the parent's PedsQL (ParentPedsQL) score, 69.6 ± 22.7, and child's PedsQL score, 75.5 ± 18.7. Multivariate linear regression analysis revealed that older age at surgery was significantly associated with lower SRS-22 scores at follow-up (B = −0.06, p = 0.03), and the presence of comorbidities was associated with poorer ParentPedsQL scores at follow-up (B = −19.68, p = 0.03).

CONCLUSIONS

This study indicates that occipitocervical and atlantoaxial fusions in children preserve neurological function and are associated with acceptable PROs and ParentPedsQL scores, considering the serious nature and potential for morbidity in this patient population. However, longer follow-up and disease-specific scales are necessary to fully elucidate the impact of occipitocervical and atlantoaxial fusions on children.

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Jonathan N. Sellin, Jeffrey S. Raskin, Kristen A. Staggers, Alison Brayton, Valentina Briceño, Amee J. Moreno and Andrew Jea

Thoracic and lumbar cortical bone trajectory pedicle screws have been described in adult spine surgery. They have likewise been described in pediatric CT-based morphometric studies; however, clinical experience in the pediatric age group is limited. The authors here describe the use of cortical bone trajectory pedicle screws in posterior instrumented spinal fusions from the upper thoracic to the lumbar spine in 12 children. This dedicated study represents the initial use of cortical screws in pediatric spine surgery.

The authors retrospectively reviewed the demographics and procedural data of patients who had undergone posterior instrumented fusion using thoracic, lumbar, and sacral cortical screws in children for the following indications: spondylolysis and/or spondylolisthesis (5 patients), unstable thoracolumbar spine trauma (3 patients), scoliosis (2 patients), and tumor (2 patients).

Twelve pediatric patients, ranging in age from 11 to 18 years (mean 15.4 years), underwent posterior instrumented fusion. Seventy-six cortical bone trajectory pedicle screws were placed. There were 33 thoracic screws and 43 lumbar screws. Patients underwent surgery between April 29, 2015, and February 1, 2016. Seven (70%) of 10 patients with available imaging achieved a solid fusion, as assessed by CT. Mean follow-up time was 16.8 months (range 13–22 months). There were no intraoperative complications directly related to the cortical bone trajectory screws. One patient required hardware revision for caudal instrumentation failure and screw-head fracture at 3 months after surgery.

Mean surgical time was 277 minutes (range 120–542 minutes). Nine of the 12 patients received either a 12- or 24-mg dose of recombinant human bone morphogenic protein 2. Average estimated blood loss was 283 ml (range 25–1100 ml).

In our preliminary experience, the cortical bone trajectory pedicle screw technique seems to be a reasonable alternative to the traditional trajectory pedicle screw placement in children. Cortical screws seem to offer satisfactory clinical and radiographic outcomes, with a low complication profile.

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Christina Sayama, Matthew Willsey, Murali Chintagumpala, Alison Brayton, Valentina Briceño, Sheila L. Ryan, Thomas G. Luerssen, Steven W. Hwang and Andrew Jea

OBJECT

The aim of this study was to determine the safety of recombinant human bone morphogenetic protein–2 (rhBMP-2) use in posterior instrumented fusions in the pediatric population, focusing on cancer risk. In a previous study, the authors reported the short-term (mean follow-up of 11 months) safety and efficacy of rhBMP-2 in the pediatric age group. The present study reports their results with a minimum of 24 months' follow-up.

METHODS

The authors retrospectively reviewed 57 consecutive cases involving pediatric patients who underwent posterior occiptocervical, cervical, thoracic, lumbar, or lumbosacral spine fusion from October 1, 2007, to June 30, 2011, at Texas Children's Hospital. Seven cases were excluded from further analysis because of loss to follow-up. Three patients died during the follow-up period and were placed in a separate cohort.

RESULTS

The patients' average age at the time of surgery was 11 years, 4 months (range 9 months to 20 years). The mean duration of follow-up was 48.4 months (range 24–70 months). Cancer status was determined at the most recent encounter with the patient and/or caretaker(s) in person, or in telephone follow-up. Twenty-four or more months after administration of rhBMP-2, there were no cases of new malignancy, degeneration, or metastasis of existing tumors. The cause of death of the patients who died during the study period was not related to BMP or to the development, degeneration, or metastasis of cancer.

CONCLUSIONS

Despite the large number of adult studies reporting increased cancer risk associated with BMP use, the authors' outcomes with rhBMP-2 in the pediatric population suggest that it is a safe adjunct to posterior spine fusions of the occipitocervical, cervical, thoracic, lumbar, and lumbosacral spine. There were no new cases of cancer, or degeneration or metastasis of existing malignancies in this series.

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Christina Sayama, Caroline Hadley, Gina N. Monaco, Anish Sen, Alison Brayton, Valentina Briceño, Brandon H. Tran, Sheila L. Ryan, Thomas G. Luerssen, Daniel Fulkerson and Andrew Jea

OBJECT

The purpose of this study focusing on fusion rate was to determine the efficacy of recombinant human bone morphogenetic protein–2 (rhBMP-2) use in posterior instrumented fusions of the craniocervical junction in the pediatric population. The authors previously reported the short-term (mean follow-up 11 months) safety and efficacy of rhBMP-2 use in the pediatric age group. The present study reports on their long-term results (minimum of 12 months' follow-up) and focuses on efficacy.

METHODS

The authors performed a retrospective review of 83 consecutive pediatric patients who had undergone posterior occipitocervical or atlantoaxial spine fusion at Texas Children's Hospital or Riley Children's Hospital during the period from October 2007 to October 2012. Forty-nine patients were excluded from further analysis because of death, loss to follow-up, or lack of CT evaluation of fusion at 12 or more months after surgery. Fusion was determined by postoperative CT scan at a minimum of 12 months after surgery. The fusion was graded and classified by a board-certified fellowship-trained pediatric neuroradiologist. Other factors, such as patient age, diagnosis, number of vertebral levels fused, use of allograft or autograft, dosage of bone morphogenetic protein (BMP), and use of postoperative orthosis, were recorded.

RESULTS

Thirty-four patients had a CT scan at least 12 months after surgery. The average age of the patients at surgery was 8 years, 1 month (range 10 months–17 years). The mean follow-up was 27.7 months (range 12–81 months). There were 37 fusion procedures in 34 patients. Solid fusion (CT Grade 4 or 4−) was achieved in 89.2% of attempts (33 of 37), while incomplete fusion or failure of fusion was seen in 10.8%. Based on logistic regression analysis, there was no significant association between solid fusion and age, sex, BMP dose, type of graft material, use of postoperative orthosis, or number of levels fused. Three of 34 patients (8.8%) required revision surgery.

CONCLUSIONS

Despite the large number of adult studies reporting positive effects of BMP on bone fusion, our long-term outcomes using rhBMP-2 in the pediatric population suggest that rates of fusion failure are higher than observed in contemporary adult and pediatric reports of occipitocervical and atlantoaxial spine fusions.