Faculty Bibliography

OBJECTIVE:Cervical spine disorders in children are relatively uncommon; therefore, paradigms for surgical and nonsurgical clinical management are not well established. The purpose of this study was to bring together an international, multidisciplinary group of pediatric cervical spine experts to build consensus via a modified Delphi approach regarding the clinical management of children with cervical spine disorders and those undergoing cervical spine stabilization surgery. METHODS:A modified Delphi method was used to identify consensus statements for the management of children with cervical spine disorders requiring stabilization. A survey of current practices, supplemented by a literature review, was electronically distributed to 17 neurosurgeons and orthopedic surgeons experienced with the clinical management of pediatric cervical spine disorders. Subsequently, 52 summary statements were formulated and distributed to the group. Statements that reached near consensus or that were of particular interest were then discussed during an in-person meeting to attain further consensus. Consensus was defined as ≥ 80% agreement on a 4-point Likert scale (strongly agree, agree, disagree, strongly disagree). RESULTS:Forty-five consensus-driven statements were identified, with all participants willing to incorporate them into their practice. For children with cervical spine disorders and/or stabilization, consensus statements were divided into the following categories: A) preoperative planning (12 statements); B) radiographic thresholds of instability (4); C) intraoperative/perioperative management (15); D) postoperative care (11); and E) nonoperative management (3). Several important statements reaching consensus included the following recommendations: 1) to obtain pre-positioning baseline signals with intraoperative neuromonitoring; 2) to use rigid instrumentation when technically feasible; 3) to provide postoperative external immobilization for 6-12 weeks with a rigid cervical collar rather than halo vest immobilization; and 4) to continue clinical postoperative follow-up at least until anatomical cervical spine maturity was reached. In addition, preoperative radiographic thresholds for instability that reached consensus included the following: 1) translational motion ≥ 5 mm at C1-2 (excluding patients with Down syndrome) or ≥ 4 mm in the subaxial spine; 2) dynamic angulation in the subaxial spine ≥ 10°; and 3) abnormal motion and T2 signal change on MRI seen at the same level. CONCLUSIONS:In this study, the authors have demonstrated that a multidisciplinary, international group of pediatric cervical spine experts was able to reach consensus on 45 statements regarding the management of pediatric cervical spine disorders and stabilization. Further study is required to determine if implementation of these practices can lead to reduced complications and improved outcomes for children.

BACKGROUND AND IMPORTANCE:Although rare, severe congenital cervical spine deformity can present with limited treatment options and potentially catastrophic outcomes. The use of halter traction for cervical deformity correction in children has been well described, but it has not been previously reported in the management of neonates. CLINICAL PRESENTATION:A baby girl born at full-term gestation presented with generalized hypotonia, bilateral club feet, and significant right upper extremity weakness. Imaging demonstrated a severe congenital swan-neck deformity with spinal cord compression. Halter traction was initiated in the neonatal intensive care unit with subsequent neurological and radiographic improvement. After 7 days, traction was discontinued and she was placed in a custom-fitted cervico-thoracic orthosis. At 2 years of follow-up, she remains neurologically stable with maintained cervical alignment. CONCLUSION:Halter traction followed by external bracing is technically possible in the neonatal period. For children with severe cervical congenital deformity, this technique can reduce spinal cord compression, provide significant deformity correction, and delay the need for definitive operative spinal stabilization.

PURPOSE/OBJECTIVE:In children with early onset scoliosis (EOS) who have tethered spinal cord (TSC), spinal cord detethering is commonly performed prior to spinal deformity correction (SDC). The purpose of this study was to investigate whether age or curve magnitude at the time of detethering is associated with curve progression at a follow-up of at least 2 years. It was hypothesized that patients who undergo detethering at a younger age, or those with a smaller curve magnitude, would experience a reduced rate of curve progression when compared with those who are older or with larger curves. METHODS:Patients with EOS who underwent detethering at least 2 years prior to SDC were identified in a multicenter international registry. Radiographs were assessed just prior to the detethering procedure (pre-detether) and at the most recent visit prior to SDC (most recent post-detether). The rate of curve progression > 10° was examined. Owing to unequal follow-up in individual patients, Cox regression was used to investigate associations between primary variables (age and magnitude of major coronal curve) and rate of curve progression. RESULTS:37 patients met inclusion criteria and 18 (mean age: 3.7 ± 2.9 years, 66.7% female, mean follow-up: 3.4 ± 1.3 years) had radiographic data available for analysis. Pre-detether and most recent post-detether major coronal curves were 44.8° ± 18.5° and 47.6° ± 23.9°, respectively. 5 (27.8%) patients had curve progression > 10° at a follow-up of 3.2 ± 1.2 years. Patients with progression > 10° were older at the time of detethering when compared with those without (5.6 ± 2.8 vs. 3 ± 2.7 years, p = 0.084). Regression analysis demonstrated that as age at detethering increased by 1 year, the rate of curve progression > 10° increased by 28.6% [95% confidence interval (CI) 0.899; 1.839, p = 0.169]. There was no evidence of an association between pre-detethering curve magnitude and rate of curve progression > 10° [HR: 1.027, 95% CI 0.977; 1.079, p = 0.297]. CONCLUSION/CONCLUSIONS:In a small multicenter cohort of EOS patients with TSC, younger age, but not curve size, at the time of detethering was associated with a lower rate of scoliosis progression. Although these results indicate a potential role for early spinal cord detethering in the EOS population, they require further prospective investigation with a larger number of patients. LEVEL OF EVIDENCE/METHODS:Level II.

Described in the seminal paper by Hans Chiari in 1891, the Chiari I malformation (CMI) is a radiographic diagnosis commonly encountered by neurosurgeons and is often treated surgically with generally positive clinic outcomes. Studies have documented that 1% to 4% of patients undergoing MRI of the brain or cervical spine will be diagnosed with CMI, characterized by greater than 5 mm tonsillar herniation below the foramen magnum. More recently CMI has been described as a spectrum of disease, which includes Chiari 0, Chiari 1.5, and the complex Chiari. Primarily through multicenter clinical outcomes research, our understanding of the pathology continues to evolve.

PURPOSE/OBJECTIVE:In patients with early onset scoliosis (EOS) and intraspinal anomalies, surgery may be necessary for both the tethered spinal cord (TSC) and spinal deformity. The purpose of this study was to determine if there is a difference in complications when TSC release and surgery for spinal deformity correction (SDC) are performed separately compared simultaneously. METHODS:EOS patients with TSC who underwent detethering and SDC surgeries were identified through a multicenter registry. Patients were stratified into two groups. The simultaneous cohort consisted of patients receiving both detethering and SDC surgeries in a single anesthetic event on the same day, and the staged cohort consisted of patients undergoing detethering and SDC on two separate occasions. Postoperative complications up to 180 days for either surgery were assessed. RESULTS:Twenty five (65.8%) patients were staged and 13 (34.2%) underwent a simultaneous approach. Percent curve correction following SDC surgery did not significantly differ between the groups (p = 0.36). Within 90 days postoperatively, 16 complications in 11 patients (44.0%) occurred in the staged group, whereas no complications occurred in the simultaneous cohort (p = 0.006). From 90-days to 180-days postoperatively, 4 additional complications in 3 patients (12.0%) occurred in the staged group, with no complications reported in the same timeframe for the simultaneous cohort. CONCLUSION/CONCLUSIONS:To our knowledge, this is the largest multicenter comparative study to date, and it suggests that a simultaneous approach can be performed safely for EOS patients undergoing detethering and SDC surgeries, with a potentially lower risk profile than the traditional staged approach to these pathologies. LEVEL OF EVIDENCE/METHODS:Level III.

OBJECTIVE:Institutions investigating value and quality emphasize utilization of two attending surgeons with different areas of technical expertise to treat complex surgical cases and to minimize complications. Here, the authors chronicle the 12-year experience of using a two-attending surgeon, two-specialty model to perform hemivertebra resection in the pediatric population. METHODS:Retrospective cohort data from 2008 to 2019 were obtained from the NewYork-Presbyterian Morgan Stanley Children's Hospital operative database. This database included all consecutive pediatric patients < 21 years old who underwent hemivertebra resection performed with the two-attending surgeon (neurosurgeon and orthopedic surgeon) model. Demographic information was extracted. Intraoperative complications, including durotomy and direct neurological injury, were queried from the clinical records. Intraoperative neuromonitoring data were evaluated. Postoperative complications were queried, and length of follow-up was determined from the clinical records. RESULTS:From 2008 to 2019, 22 patients with a median (range) age of 9.1 (2.0-19.3) years underwent hemivertebra resection with the two-attending surgeon, two-specialty model. The median (range) number of levels fused was 2 (0-16). The mean (range) operative time was 5 hours and 14 minutes (2 hours and 59 minutes to 8 hours and 30 minutes), and the median (range) estimated blood loss was 325 (80-2700) ml. Navigation was used in 14% (n = 3) of patients. Neither Gardner-Wells tongs nor halo traction was used in any operation. Neuromonitoring signals significantly decreased or were lost in 14% (n = 3) of patients. At a mean ± SD (range) follow-up of 4.6 ± 3.4 (1.0-11.6) years, 31% (n = 7) of patients had a postoperative complication, including 2 instances of proximal junctional kyphosis, 2 instances of distal junctional kyphosis, 2 wound complications, 1 instance of pseudoarthrosis with hardware failure, and 1 instance of screw pullout. The return to the operating room (OR) rate was 27% (n = 6), which included patients with the abovementioned wound complications, distal junctional kyphosis, pseudoarthrosis, and screw pullout, as well as a patient who required spinal fusion after loss of motor evoked potentials during index surgery. CONCLUSIONS:Twenty-two patients underwent hemivertebra resection with a two-attending surgeon, two-specialty model over a 12-year period at a specialized children's hospital, with a 14% rate of change in neuromonitoring, 32% rate of nonneurological complications, and a 27% rate of unplanned return to the OR.

Pediatric spinal trauma is a broad topic with nuances specific to each anatomic region of the spinal column. The purpose of this report is to provide a brief review highlighting the most important and common clinical issues regarding the diagnosis and management of pediatric spine trauma. Detailed descriptions of imaging findings along with specific operative and nonoperative management of each fracture and dislocation type are beyond the scope of this review.

BACKGROUND:Surgical site infections (SSIs) are one of the most common complications following pediatric complex tethered spinal cord release. This patient population is similar in some ways to the neuromuscular scoliosis population, in which higher-than-expected rates of gram-negative SSIs have been identified. METHODS:We conducted a single-center retrospective chart review of all patients who underwent complex tethered spinal cord release over a 10-year period between 2007 and 2017. RESULTS:A total of 69 patients were identified, with 10 documented SSIs (14%). 50% of the SSIs were polymicrobial or included at least 1 gram-negative organism. Among the organisms isolated, 3 were fully or -partially resistant to cefazolin, the most common antibiotic prophylaxis in this population. CONCLUSION/CONCLUSIONS:Among children undergoing complex tethered spinal cord release, gram-negative and polymicrobial infections are a significant cause of SSIs. Although further multicenter data are needed, these findings suggest that standard antibiotic prophylaxis with cefazolin may not be sufficient.

Adamantinomatous craniopharyngioma (ACP) is a biologically benign but clinically aggressive lesion that has a significant impact on quality of life. The incidence of the disease has a bimodal distribution, with peaks occurring in children and older adults. Our group previously published the results of a transcriptome analysis of pediatric ACPs that identified several genes that were consistently overexpressed relative to other pediatric brain tumors and normal tissue. We now present the results of a transcriptome analysis comparing pediatric to adult ACP to identify biological differences between these groups that may provide novel therapeutic insights or support the assertion that potential therapies identified through the study of pediatric ACP may also have a role in adult ACP. Using our compiled transcriptome dataset of 27 pediatric and 9 adult ACPs, obtained through the Advancing Treatment for Pediatric Craniopharyngioma Consortium, we interrogated potential age-related transcriptional differences using several rigorous mathematical analyses. These included: canonical differential expression analysis; divisive, agglomerative, and probabilistic based hierarchical clustering; information theory based characterizations; and the deep learning approach, HD Spot. Our work indicates that there is no therapeutically relevant difference in ACP gene expression based on age. As such, potential therapeutic targets identified in pediatric ACP are also likely to have relvance for adult patients.

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.