Pediatric cervical spine instability: evolving concepts.

The pediatric cervical spine is structurally and biomechanically unique in comparison to adults. Guidelines to assess for cervical spine instability and standard of care treatments in the pediatric population have yet to be delineated. This is due to the rarity of the condition and the lack of multicenter data published on the topic. Our review explores the biomechanics of the pediatric cervical spine and highlights evolving concepts/research over the last several decades, with special attention to the Down syndrome and complex Chiari malformation cohorts.

Costs of abdominal aortic aneurysm care at a regional Veterans Affairs medical center with the implementation of an abdominal aortic aneurysm screening program.

BACKGROUND: Abdominal aortic aneurysm (AAA) screening has demonstrated to be cost-effective in reducing AAA-related morbidity and all-cause mortality. However, the downstream care costs of an implemented AAA screening in clinical practice have not been reported. The purpose of this study is to determine direct regional Department of Veterans Affairs (VA) costs in implementing and sustaining an AAA screening program over a 10-year period. METHODS: A cost data analysis (adjusted to 2021 U.S. dollars) of an AAA screening program was conducted from 2007 to 2016, where 19,649 veteran patients aged 65-75 with a smoking history were screened at a regional VA medical center. A decision support system tracked direct and indirect encounter costs from Medicare billing codes associated with AAA care. Costs from a patient's initial screening, follow-up imaging, to AAA repair or at the end of the analysis period, March 31, 2021, were recorded. Costs for AAA repairs outside the VA system were also tracked. RESULTS: A total of 1,183 patients screened were identified with an AAA ≥3.0 cm without history of repair. Estimated screening costs were $2.8 million or $280,000 annually ($143/screening) in the care of 19,649 screened patients. There were 221 patients who required repair (143 repairs in VA, 78 repairs outside VA). The average cost of elective endovascular repair was $43,021 and that of open repair was $49,871. The total costs for all elective repairs were $9,692,591. Screening, implementation, maintenance, and surgical repair cost involved in the management of patients with AAA disease was $13.7 million, with $10,686 per life-year lived after repair (5.8 ± 3.5 mean life-years) and $490 per life-year lived after screening (6.9 ± 3.5 mean life-years) for all patients screened. There were 13 deaths of unknown causes and one patient with a ruptured AAA that required emergency repair at a cost of $124,392. CONCLUSIONS: Despite known limitations, the implementation of an AAA ultrasound screening program is feasible, cost-effective, and a worthwhile endeavor.

Management of sagittal synostosis in the Synostosis Research Group: baseline data and early outcomes.

OBJECTIVE: Sagittal synostosis is the most common form of isolated craniosynostosis. Although some centers have reported extensive experience with this condition, most reports have focused on a single center. In 2017, the Synostosis Research Group (SynRG), a multicenter collaborative network, was formed to study craniosynostosis. Here, the authors report their early experience with treating sagittal synostosis in the network. The goals were to describe practice patterns, identify variations, and generate hypotheses for future research. METHODS: All patients with a clinical diagnosis of isolated sagittal synostosis who presented to a SynRG center between March 1, 2017, and October 31, 2019, were included. Follow-up information through October 31, 2020, was included. Data extracted from the prospectively maintained SynRG registry included baseline parameters, surgical adjuncts and techniques, complications prior to discharge, and indications for reoperation. Data analysis was descriptive, using frequencies for categorical variables and means and medians for continuous variables. RESULTS: Two hundred five patients had treatment for sagittal synostosis at 5 different sites. One hundred twenty-six patients were treated with strip craniectomy and 79 patients with total cranial vault remodeling. The most common strip craniectomy was wide craniectomy with parietal wedge osteotomies (44%), and the most common cranial vault remodeling procedure was total vault remodeling without forehead remodeling (63%). Preoperative mean cephalic indices (CIs) were similar between treatment groups: 0.69 for strip craniectomy and 0.68 for cranial vault remodeling. Thirteen percent of patients had other health problems. In the cranial vault cohort, 81% of patients who received tranexamic acid required a transfusion compared with 94% of patients who did not receive tranexamic acid. The rates of complication were low in all treatment groups. Five patients (2%) had an unintended reoperation. The mean change in CI was 0.09 for strip craniectomy and 0.06 for cranial vault remodeling; wide craniectomy resulted in a greater change in CI in the strip craniectomy group. CONCLUSIONS: The baseline severity of scaphocephaly was similar across procedures and sites. Treatment methods varied, but cranial vault remodeling and strip craniectomy both resulted in satisfactory postoperative CIs. Use of tranexamic acid may reduce the need for transfusion in cranial vault cases. The wide craniectomy technique for strip craniectomy seemed to be associated with change in CI. Both findings seem amenable to testing in a randomized controlled trial.

Risk of developing an abdominal aortic aneurysm after ectatic aorta detection from initial screening.

OBJECTIVE: Current abdominal aortic aneurysm (AAA) surveillance guidelines lack any follow-up recommendations after initial abdominal aortic screening diameter of less than 3.0 cm. Some reports have demonstrated patients with late AAA formation and late ruptures after initial ultrasound screening detection of patients with an aortic diameter of 2.5 to 2.9 cm (ectatic aorta). The purpose of this study was to determine ectatic aorta prevalence, AAA development, rupture risk, and risk factor profile in patients with detected ectatic aortas in a AAA screening program. METHODS: A retrospective chart review of all patients screened for AAA from January 1, 2007, to December 31, 2016, within a regional health care system was conducted. Screening criteria were men 65 to 75 years of age that smoked a minimum of 100 cigarettes in their lifetime. An ectatic aorta was defined as a maximum aortic diameter from 2.5 to 2.9 cm. An AAA was defined as an aortic diameter of 3 cm or greater. Patients screened with ectatic aortas who had subsequent follow-up imaging of the aorta with a minimum of 1-year follow-up were analyzed for associated clinical and cardiovascular risk factors. All data were collected through December 3,/2018. A logistic regression of statistically significant variables from univariate and χ2 analyses were performed to identify risks associated with the development of AAA from an initially diagnosed ectatic aorta. A Cox proportional hazard model was used to assess survival data. A P value of less than .05 was considered statistically significant. RESULTS: From a screening pool of 19,649 patients, 3205 (16.3%) with a mean age of 72.1 ± 5.3 years were identified to have an ectatic aorta from January 1, 2007, to December 31, 2016. The average screening ectatic aortic diameter was 2.6 ± 0.1 cm. There were 672 patients (21.0%) with a mean age of 73.0 ± 5.7 years who received subsequent imaging for other clinical indications and 193 of these patients (28.7%) with ectatic aortas developed an AAA from the last follow-up scan (4.2 ± 2.5 years). The average observation length of all patients was 6.4 ± 2.9 years. No ruptures were reported, but 27.8% of deaths were of unknown cause. One patient had aortic growth to 5.5 cm or greater (0.15%). Larger initial screening diameter (P < .01), presence of chronic obstructive pulmonary disease (P < .01), and active smoking (P = .01) were associated with AAA development. CONCLUSIONS: Patients with diagnosed ectatic aortas from screening who are active smokers or have chronic obstructive pulmonary disease are likely to develop an AAA.

High Prevalence of Gram-Negative Rod and Multi-Organism Surgical Site Infections after Pediatric Complex Tethered Spinal Cord Surgery: Preliminary Report from a Single-Center Study.

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: 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.

Evaluation of the Patient-Practitioner Consultation on Surgical Treatment Options for Patients With Craniosynostosis.

INTRODUCTION: Endoscope-assisted craniectomy and spring-assisted cranioplasty with post-surgical helmet molding are minimally invasive alternatives to the traditional craniosynostosis treatment of open cranial vault remodeling. Families are often faced with deciding between techniques. This study aimed to understand providers' practice patterns in consulting families about surgical options. METHODS: An online survey was developed and distributed to 31 providers. The response rate was 84% (26/31). RESULTS: Twenty-six (100%) respondents offer a minimally invasive surgical option for sagittal craniosynostosis, 21 (81%) for coronal, 20 (77%) for metopic, 18 (69%) for lambdoid, and 12 (46%) for multi-suture. Social issues considered in determining whether to offer a minimally invasive option include anticipated likelihood of compliance (23 = 88%), distance traveled for care (16 = 62%) and financial considerations (6 = 23%). Common tools to explain options include verbal discussion (25 = 96%), 3D reconstructed CT scans (17 = 65%), handouts (13 = 50%), 3D models (12 = 46%), hand drawings (11 = 42%) and slides (10 = 38%). Some respondents strongly (7 = 27%) or somewhat (3 = 12%) encourage a minimally invasive option over open repair. Others indicate they remain neutral (7 = 27%) or tailor their approach to meet perceived needs (8 = 31%). One (4%) somewhat encourages open repair. Despite this variation, all completely (17 = 65%), strongly (5 = 19%) or somewhat agree (4 = 15%) they use shared decision making in presenting surgical options. CONCLUSION: This survey highlights the range of practice patterns in presenting surgical options to families and reveals possible discrepancies in the extent providers believe they use shared decision making and the extent it is actually used.

The 10-year outcomes of a regional abdominal aortic aneurysm screening program.

OBJECTIVE: In 2007, Medicare established ultrasound screening guidelines to identify patients at risk for abdominal aortic aneurysm (AAA). The purpose of this study was to evaluate AAA diagnosis rates and compliance with screening during 10 years (2007-2016) of the Screen for Abdominal Aortic Aneurysms Very Efficiently Act implementation within a regional health care system. METHODS: A retrospective chart review of all patients screened for AAA from 2007 to 2016 within a regional Veterans Affairs health care system was conducted. Screening criteria were men 65 to 75 years of age who smoked a minimum of 100 cigarettes in their lifetime. An AAA was defined as a maximum aortic diameter ≥3 cm. A comparison was made of the AAA diagnosis rate and clinical adherence rate of screening criteria between the first 5 years and total years evaluated. AAA-related mortality was identified by using terminal diagnosis notes or autopsy reports. All data were recorded by August 31, 2017. RESULTS: A total of 19,649 patients (70.7 ± 4.8 years of age, mean ± standard deviation) were screened from January 1, 2007, to December 31, 2016. There were 9916 new patients screened from 2012 to 2016. A total of 1232 aneurysms (6.3% total patients) were identified during the 10-year period. The overall AAA diagnosis rate has declined from 7.2% in the first 5 years to 6.3% in 10 years (13.5% decrease; P < .01). There were 66 patients found with AAA ≥5.5 cm (5.3% of AAAs), and 54 of these patients received successful elective repair. A total of 2321 patients died (11.8%) and 6 deaths were suspected AAA ruptures (0.03%) within the analysis period. A total of 3680 patients screened (18.7%) did not meet screening criteria: 593 patients were <65 years of age, 3087 patients were >75 years of age, and 59 patients were women. This rate has declined from 28.2% within the first 5 years to 18.7% overall in 10 years (33.7% decrease; P < .01). The compliance of screened patients using screening criteria improved significantly from 61.7% in 2007 to 92.4% in 2016 (P < .01). The overall compliance rate since implementation of the screening program during the past 10 years is 81.3%. CONCLUSIONS: The overall 10-year rate of AAA diagnosis is 6.3%. There are more smaller aneurysms (3.0-4.4 cm) detected and fewer large AAAs ≥5.5 cm in the last 5 years compared with the first 5 years of the screening program. The overall AAA-related mortality rate of all screened patients is 0.03%. There were 54 patients with AAA ≥5.5 cm who underwent successful elective repair resulting from the AAA screening program. The overall compliance of screened patients using screening criteria improved significantly from 61.7% in 2007 to 81.3% since implementation of the screening program during the past 10 years.

Increased complications without neurological benefit are associated with prophylactic spinal cord untethering prior to scoliosis surgery in children with myelomeningocele.

PURPOSE: Children with myelomeningocele (MMC) are at increased risk of developing neuromuscular scoliosis and spinal cord re-tethering (Childs Nerv Syst 12:748-754, 1996; Neurosurg Focus 16:2, 2004; Neurosurg Focus 29:1, 2010). Some centers perform prophylactic untethering on asymptomatic MMC patients prior to scoliosis surgery because of concern that additional traction on the cord may place the patient at greater risk of neurologic deterioration peri-operatively. However, prophylactic untethering may not be justified if it carries increased surgical risks. The purpose of this study was to determine if prophylactic untethering is necessary in asymptomatic children with MMC undergoing scoliosis surgery. METHODS: A multidisciplinary, retrospective cohort study from seven children's hospitals was performed including asymptomatic children with MMC < 21 years old, managed with or without prophylactic untethering prior to scoliosis surgery. Patients were divided into three groups for analysis: (1) untethering at the time of scoliosis surgery (concomitant untethering), (2) untethering within 3 months of scoliosis surgery (prior untethering), and (3) no prophylactic untethering. Baseline data, intra-operative reports, and 90-day post-operative outcomes were analyzed to assess for differences in neurologic outcomes, surgical complications, and overall length of stay. RESULTS: A total of 208 patients were included for analysis (mean age 9.4 years, 52% girls). No patient in any of the groups exhibited worsened motor or sensory function at 90 days post-operatively. However, comparing the prophylactic untethering groups with the group that was not untethered, there was an increased risk of surgical site infection (SSI) (31.3% concomitant, 28.6% prior untethering vs. 12.3% no untethering; p = 0.0104), return to the OR (43.8% concomitant, 23.8% prior untethering vs. 17.4% no untethering; p = 0.0047), need for blood transfusion (51.6% concomitant, 57.1% prior untethering vs. 33.8% no untethering; p = 0.04), and increased mean length of stay (LOS) (13.4 days concomitant, 10.6 days prior untethering vs. 6.8 days no untethering; p < 0.0001). In multivariable logistic regression analysis, prophylactic untethering was independently associated with increased adjusted relative risks of surgical site infection (aRR = 2.65, 95% CI 1.17-5.02), unplanned re-operation (aRR = 2.17, 95% CI 1.02-4.65), and any complication (aRR = 2.25, 95% CI 1.07-4.74). CONCLUSION: In this study, asymptomatic children with myelomeningocele who underwent scoliosis surgery developed no neurologic injuries regardless of prophylactic untethering. However, those who underwent prophylactic untethering were more likely to experience SSIs, return to the OR, need a blood transfusion, and have increased LOS than children not undergoing untethering. Based on these data, prophylactic untethering in asymptomatic MMC patients prior to scoliosis surgery does not provide any neurological benefit and is associated with increased surgical risks.

Traumatic Occipitocervical Distraction Injuries in Children: A Systematic Review.

BACKGROUND: Occipitocervical distraction injuries (OCDI) in children occur on a wide spectrum of severity, and decisions about treatment suffer from a lack of rigorous guidelines and significant inter-institutional variability. While clear cases of frank atlanto-occipital dislocation (AOD) are treated with surgical stabilization, the approach for less severe cases of OCDI is not standardized. These patients require a careful assessment of both radiographic and clinical criteria, as part of a complex risk-benefit analysis, to establish whether occipitocervical fusion (OCF) is indicated. Here, we performed a systematic review of the literature that describes traumatic OCDI in children < 18 years of age. SUMMARY: We performed a systematic review, according to PRISMA guidelines, of children < 18 years of age presenting with traumatic etiologies of OCDI. We searched PubMed to identify papers congruent with these criteria. Exclusion criteria included (1) reports on atraumatic causes of OCDI and (2) studies with insufficient clinical and radiographic details on individual patients. We identified 16 reports describing a total of 144 patients treated for pediatric traumatic OCDI. Based on the synthesis of these findings and the collective experience of the authors, we present the demographic, clinical, and radiographic factors that underlie OC instability, which we hope will serve as components of a grading system in the future. We considered various clinical and radiographic findings including: (1) the mechanism of injury, (2) the patient's age, (3) CT/CT angiography of head and neck findings and parameters, (4) MRI findings, and (5) neurological exam, for the purpose of determining the severity of the OCDI and offering treatment guidelines based on the summative risk of underlying OC instability. Key Messages: OCDI is a potentially devastating injury, especially in children. Although missing the diagnosis can have potentially catastrophic consequences, reverting to surgical fixation in less severe cases can subject children to unnecessary operative risk and permanently reduce their range of motion. After reviewing all the available reports of pediatric traumatic OCDI in the neurosurgical literature, we propose an outline of clinical and radiographic factors influencing underlying OC instability that could be incorporated into a grading scale to guide treatment. We hope this study stimulates discussion on the standardization of treatment for pediatric OCDI.

Cerebral Vasospasm after Open Fenestration of an Arachnoid Cyst in a 4-Year-Old Boy: Case Report and Review of the Literature.

Cerebral vasospasm is associated with significant morbidity, and most commonly occurs following subarachnoid hemorrhage. Rarely, vasospasm can follow tumor resection and traumatic brain injury. We present the first reported case of a young child who developed diffuse vasospasm following open fenestration of an arachnoid cyst and was promptly treated, with full recovery of neurologic function. Although vasopasm after arachnoid cyst fenestration is rare, it can be included in the differential for a new focal neurologic deficit.

Treatment of Synchronous Traumatic Perforations of the Trachea and the Esophagus With Two Inferiorly Based Strap Muscle Interposition Flaps: An Innovative Approach.

: Traumatic perforations of the esophagus and trachea are devastating injuries that necessitate prompt treatment. Large defects benefit from coverage with well vascularized tissue. Injuries at the level of the thoracic inlet are more challenging, as the options for local tissue coverage are limited.This report describes the case of a 24-year-old male who suffered gunshot wounds to his neck resulting in right posterolateral tracheal perforation as well as esophageal perforation at the level of the thoracic inlet. Bronchoscopy and esophagogastroduodenoscopy showed injury of the trachea at 19 cm from the incisors and 2 large defects of the anterior and posterior esophagus at 26 cm. The esophageal defects were temporized with a stent at a first stage. Plastic surgery team was then consulted for flap coverage of the defects.The thoracic team exposed the tracheal and esophageal perforations with a j-type incision of the neck, extending to the sternal notch, and the esophageal stent was removed. The 2 muscles, sternohyoid (SH) and sternothyroid (ST), were dissected free and were inferiorly rotated after they were disinserted superiorly. The SH was placed between the trachea and the esophagus, and the ST between the esophagus and the spine.Postoperative, the patient was receiving nutrition via a gastrostomy tube. An esophagogram was performed on postoperative day (POD) # 7, which showed no esophageal leak. Postoperative diet was started and the patient was discharged on POD# 10 in a good condition. Twelve months postoperative, his wounds were found to be intact, and had no trouble either with breathing or swallowing. LEVEL OF EVIDENCE:: V.

Temporary occipital fixation in young children with severe cervical-thoracic spinal deformity.

OBJECTIVE The long-term effects of instrumentation and fusion of the occipital-cervical-thoracic spine on spinal growth in young children are poorly understood. To mitigate the effects of this surgery on the growing pediatric spine, the authors report a novel technique used in 4 children with severe cervical-thoracic instability. These patients underwent instrumentation from the occiput to the upper thoracic region for stabilization, but without bone graft at the craniovertebral junction (CVJ). Subsequent surgery was then performed to remove the occipital instrumentation, thereby allowing further growth and increased motion across the CVJ. METHODS Three very young children (15, 30, and 30 months old) underwent occipital to thoracic posterior segmental instrumentation due to cervical or upper thoracic dislocation, progressive kyphosis, and myelopathy. The fourth child (10 years old) underwent similar instrumentation for progressive cervical-thoracic scoliosis. Bone graft was placed at and distal to C-2 only. After follow-up CT scans demonstrated posterior arthrodesis without unintended fusion from the occiput to C-2, 3 patients underwent removal of the occipital instrumentation. RESULTS Follow-up cervical spine flexion/extension radiographs demonstrated partial restoration of motion at the CVJ. One patient has not had the occipital instrumentation removed yet, because only 4 months have elapsed since her operation. CONCLUSIONS Temporary fixation to the occiput provides increased biomechanical stability for spinal stabilization in young children, without permanently eliminating motion and growth at the CVJ. This technique can be considered in children who require longer instrumentation constructs for temporary stabilization, but who only need fusion in more limited areas where spinal instability exists.

Isolated thoracic syrinx in children with Chiari I malformation.

INTRODUCTION: Syrinx has been reported in 25-85 % of children with Chiari malformation type I (CMI), and it is most commonly cervical in location. As a result, cervical MRI is routinely included in an evaluation for CMI. Isolated thoracic syrinx without involvement of the cervical cord in this population is uncommon but clinically important because its presence may influence the decision to operate, surgical techniques employed, or interpretation of follow-up imaging. The purpose of this study was to determine the incidence of isolated thoracic syrinx in a large group of children evaluated for CMI. METHODS: We retrospectively reviewed all patients under 21 years of age who were evaluated for CMI at Columbia University/Morgan Stanley Children's Hospital of New York from 1998 to 2013. All patients underwent MRI of the entire spine as part of the CMI evaluation, regardless of whether surgery was planned. The proportion of patients exhibiting isolated thoracic syrinx was determined. Presenting signs, symptoms, and imaging findings were then studied in an attempt to identify any clinical features associated with isolated thoracic syrinx. RESULTS: We identified 266 patients evaluated over the study period. One-hundred thirty-two patients (50 %) presented with a syrinx, and 12 patients (4.5 % of all patients evaluated and 9.1 % of all patients with a syrinx) had an isolated thoracic syrinx. Demographic variables, clinical presentation, and extent of tonsillar ectopia showed great heterogeneity in this group, and no factor was consistently associated with isolated thoracic syrinx. CONCLUSIONS: Isolated thoracic syrinx is an uncommon but clinically significant finding in children with CMI. Our data demonstrate that the presence of a CMI-related thoracic syrinx cannot be reliably predicted clinically and is therefore likely to be missed in patients who do not undergo complete spinal cord imaging. MRI of the entire spinal cord should be considered for all children undergoing initial evaluation for CMI.

Bilateral occipital endoscopic choroid plexus cauterization for persistent hydrocephalus following frontal endoscopic third ventriculostomy and choroid plexus cauterization--the "bowling ball" technique.

Endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) as a primary treatment for hydrocephalus is gaining popularity in North America, particularly among the infant population. Unfortunately, despite considerable experience with ETV/CPC at several centers, treatment failures still exist. Early reports have suggested that greater than 90 % cauterization of the choroid plexus is associated with improved clinical outcomes. However, individual patient anatomy and smaller overall ventricular size can limit the amount of choroid plexus cauterization that is technically possible through a single frontal burr hole. Furthermore, the degree of cauterization achieved by surgeons using this technique is difficult to quantify objectively. In this report, we describe the case of an infant who failed initial ETV/CPC but then had successful resolution of hydrocephalus after additional choroid plexus cauterization performed through bilateral occipital burr holes. The child remains shunt-free over a year after treatment, suggesting that this three-pronged CPC approach (the "bowling ball" technique) may be successful in some young children with persistent hydrocephalus after ETV/CPC from a single frontal burr hole.

Pial synangiosis for moyamoya syndrome in children with sickle cell anemia: a comprehensive review of reported cases.

OBJECT: Pediatric patients with sickle cell anemia (SCA) carry a significant risk of developing moyamoya syndrome (MMS) and brain ischemia. The authors sought to review the safety and efficacy of pial synangiosis in the treatment of MMS in children with SCA by performing a comprehensive review of all previously reported cases in the literature. METHODS: The authors retrospectively reviewed the clinical and radiographic records in 17 pediatric patients with SCA treated at the Morgan Stanley Children's Hospital of New York (MSCHONY) who developed radiological evidence of MMS and underwent pial synangiosis between 1996 and 2012. The authors then added any additional reported cases of pial synangiosis for this population in the literature for a combined analysis of clinical and radiographic outcomes. RESULTS: The combined data consisted of 48 pial synangiosis procedures performed in 30 patients. Of these, 27 patients (90%) presented with seizure, stroke, or transient ischemic attack, whereas 3 (10%) were referred after transcranial Doppler screening. At the time of surgery, the median age was 12 years. Thirteen patients (43%) suffered an ischemic stroke while on chronic transfusion therapy. Long-term follow-up imaging (MR angiography or catheter angiography) at a mean of 25 months postoperatively was available in 39 (81%) treated hemispheres. In 34 (87%) of those hemispheres there were demonstrable collateral vessels on imaging. There were 4 neurological events in 1590 cumulative months of follow-up, or 1 event per 33 patient-years. In the patients in whom complete data were available (MSCHONY series, n = 17), the postoperative stroke rate was reduced more than 6-fold from the preoperative rate (p = 0.0003). CONCLUSIONS: Pial synangiosis in patients with SCA, MMS, and brain ischemia appears to be a safe and effective treatment option. Transcranial Doppler and/or MRI screening in asymptomatic patients with SCA is recommended for the diagnosis of MMS.

Challenges and Insights: Cervical Spine Injuries in Children with Traumatic Brain Injury.

Cervical spine injuries (CSIs) in pediatric patients with traumatic brain injury (TBI) pose unique diagnostic and management challenges. Current studies on the intricate overlap between pediatric TBI and CSI are limited. This paper explores the existing literature as well as the epidemiology, mechanisms of injury, diagnostic criteria, treatment strategies, and outcomes associated with CSI in pediatric TBI patients.

Association between structural rib autograft and the rate of arthrodesis in children undergoing occiput-C2 instrumentation and fusion.

OBJECTIVE: The purpose of this study was to identify factors associated with fusion success among pediatric patients undergoing occiput-C2 rigid instrumentation and fusion. METHODS: The Pediatric Spine Study Group registry was queried to identify patients ≤ 21 years of age who underwent occiput-C2 posterior spinal rigid instrumentation and fusion and had a 2-year minimum clinical and radiographic (postoperative lateral cervical radiograph or CT scan) follow-up. Fusion failure was defined clinically if a patient underwent hardware revision surgery > 30 days after the index procedure or radiographically by the presence of hardware failure or screw haloing on the most recent follow-up imaging study. Univariate comparisons and multivariable logistic regression analyses were subsequently performed. RESULTS: Seventy-six patients met inclusion criteria. The median age at surgery was 9 years (range 1.5-17.2 years), and 51% of the cohort was male. Overall, 75% of patients had syndromic (n = 41) or congenital (n = 15) etiologies, with the most frequent diagnoses of Down syndrome (28%), Chiari malformation (13%), and Klippel-Feil syndrome (12%). Data were available to determine if there was a fusion failure in 97% (74/76) of patients. Overall, 38% (28/74) of patients had fusion failure (95% CI 27%-50%). Univariate analysis demonstrated that use of a rigid cervical collar postoperatively (p = 0.04) and structural rib autograft (p = 0.02) were associated with successful fusion. Multivariable logistic regression analysis determined that patients who had rib autograft used in surgery had a 73% decrease in the odds of fusion failure (OR 0.27, 95% CI 0.09-0.82; p = 0.02). Age, etiology including Down syndrome, instrumentation type, unilateral instrumentation, use of recombinant human bone morphogenetic protein, and other variables did not influence the risk for fusion failure. CONCLUSIONS: In this multicenter, multidisciplinary, international registry of children undergoing occiput-C2 instrumentation and fusion, fusion failure was seen in 38% of patients, a higher rate than previously reported in the literature. The authors' data suggest that postoperative immobilization in a rigid cervical collar may be beneficial, and the use of structural rib autograft should be considered, as rib autograft was associated with a 75% higher chance of successful fusion.

Timing of intraoperative neurophysiological monitoring (IONM) recovery and clinical recovery after termination of pediatric spinal deformity surgery due to loss of IONM signals.

BACKGROUND CONTEXT: Intraoperative neurophysiological monitoring (IONM) is used to reduce the risk of spinal cord injury during pediatric spinal deformity surgery. Significant reduction and/or loss of IONM signals without immediate recovery may lead the surgeon to acutely abort the case. The timing of when monitorable signals return remains largely unknown. PURPOSE: The goal of this study was to investigate the correlation between IONM signal loss, clinical examination, and subsequent normalization of IONM signals after aborted pediatric spinal deformity surgery to help determine when it is safe to return to the operating room. STUDY DESIGN/SETTING: This is a multicenter, multidisciplinary, retrospective study of pediatric patients (<18 years old) undergoing spinal deformity surgery whose surgery was aborted due to a significant reduction or loss of IONM potentials. PATIENT SAMPLE: Sixty-six patients less than 18 years old who underwent spinal deformity surgery that was aborted due to IONM signal loss were enrolled into the study. OUTCOME MEASURES: IONM data, operative reports, and clinical examinations were investigated to determine the relationship between IONM loss, clinical examination, recovery of IONM signals, and clinical outcome. METHODS: Information regarding patient demographics, deformity type, clinical history, neurologic and ambulation status, operative details, IONM information (eg, quality of loss [SSEPs, MEPs], laterality, any recovery of signals, etc.), intraoperative wake-up test, postoperative neurologic exam, postoperative imaging, and time to return to the operating were all collected. All factors were analyzed and compared with univariate and multivariate analysis using appropriate statistical analysis. RESULTS: Sixty-six patients were enrolled with a median age of 13 years [IQR 11-14], and the most common sex was female (42/66, 63.6%). Most patients had idiopathic scoliosis (33/66, 50%). The most common causes of IONM loss were screw placement (27/66, 40.9%) followed by rod correction (19/66, 28.8%). All patients had either complete bilateral (39/66, 59.0%), partial bilateral (10/66, 15.2%) or unilateral (17/66, 25.8%) MEP loss leading to termination of the case. Overall, when patients were returned to the operating room 2 weeks postoperatively, nearly 75% (40/55) had monitorable IONM signals. Univariate analysis demonstrated that bilateral SSEP loss (p=.019), bilateral SSEP and MEP loss (p=.022) and delayed clinical neurologic recovery (p=.008) were significantly associated with having unmonitorable IONM signals at repeat surgery. Multivariate regression analysis demonstrated that delayed clinical neurologic recovery (> 72 hours) was significantly associated with unmonitorable IONM signals when returned to the operating room (p=.006). All patients ultimately made a full neurologic recovery. CONCLUSIONS: In children whose spinal deformity surgery was aborted due to intraoperative IONM loss, there was a strong correlation between combined intraoperative SSEP/MEP loss, the magnitude of IONM loss, the timing of clinical recovery, and the time of electrophysiological IONM recovery. The highest likelihood of having a prolonged postoperative neurological deficit and undetectable IONM signals upon return to the OR occurs with bilateral complete loss of SSEPs and MEPs.

The role of occipital condyle and atlas anomalies on occipital cervical fusion outcomes in Chiari malformation type I with syringomyelia: a study from the Park-Reeves Syringomyelia Research Consortium.

OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia. The authors also investigated the predictive contribution of these anomalies to the occurrence of OCF following PFD (PFD+OCF). METHODS: The authors analyzed the prevalence of condylar hypoplasia and atlas arch anomalies for patients in the Park-Reeves Syringomyelia Research Consortium database who underwent PFD+OCF. Condylar hypoplasia was defined by an atlanto-occipital joint axis angle (AOJAA) ≥ 130°. Atlas assimilation and arch anomalies were identified on presurgical radiographic imaging. This PFD+OCF cohort was compared with a control cohort of patients who underwent PFD alone. The control group was matched to the PFD+OCF cohort according to age, sex, and duration of symptoms at a 2:1 ratio. RESULTS: Clinical features and radiographic atlanto-occipital joint parameters were compared between 19 patients in the PFD+OCF cohort and 38 patients in the PFD-only cohort. Demographic data were not significantly different between cohorts (p > 0.05). The mean AOJAA was significantly higher in the PFD+OCF group than in the PFD group (144° ± 12° vs 127° ± 6°, p < 0.0001). In the PFD+OCF group, atlas assimilation and atlas arch anomalies were identified in 10 (53%) and 5 (26%) patients, respectively. These anomalies were absent (n = 0) in the PFD group (p < 0.001). Multivariate regression analysis identified the following 3 CVJ radiographic variables that were predictive of OCF occurrence after PFD: AOJAA ≥ 130° (p = 0.01), clivoaxial angle < 125° (p = 0.02), and occipital condyle-C2 sagittal vertical alignment (C-C2SVA) ≥ 5 mm (p = 0.01). A predictive model based on these 3 factors accurately predicted OCF following PFD (C-statistic 0.95). CONCLUSIONS: The authors' results indicate that the occipital condyle-atlas joint complex might affect the biomechanical integrity of the CVJ in children with CM-I and syringomyelia. They describe the role of the AOJAA metric as an independent predictive factor for occurrence of OCF following PFD. Preoperative identification of these skeletal abnormalities may be used to guide surgical planning and treatment of patients with complex CM-I and coexistent osseous pathology.

Transnasal endoscopic approach to the pediatric craniovertebral junction and rostral cervical spine: case series and literature review.

The endoscopic transnasal approach to the rostral pediatric spine and craniovertebral junction is a relatively new technique that provides an alternative to the traditional transoral approach to the anterior pediatric spine. In this case series, the authors provide 2 additional examples of patients undergoing endoscopic transnasal odontoidectomies for ventral decompression of the spinal cord. Both patients would have required transection of the palate to undergo an effective transoral operation, which can be a cause of significant morbidity. In one case, transnasal decompression was initially incomplete, and decompression was successfully achieved via a second endoscopic transnasal operation. Both cases resulted in significant neurological recovery and stable long-term spinal alignment. The transnasal approach benefits from entering into the posterior pharynx at an angle that often reduces the length of postoperative intubation and may speed a patient's return to oral intake. Higher reoperation rates are a concern for many endoscopic approaches, but there are insufficient data to conclude if this is the case for this procedure. Further experience with this technique will provide a better understanding of the indications for which it is most effective. Transcervical and transoral endoscopic approaches have also been reported and provide additional options for pediatric anterior cervical spine surgery.