Anterior cervical discectomy and fusion for the treatment of pediatric Hirayama disease.

PURPOSE: Hirayama disease, a rare cervical myelopathy in children and young adults, leads to progressive upper limb weakness and muscle loss. Non-invasive external cervical orthosis has been shown to prevent further neurologic decline; however, this treatment modality has not been successful at restoring neurologic and motor function, especially in long standing cases with significant weakness. The pathophysiology remains not entirely understood, complicating standardized operative guidelines; however, some studies report favorable outcomes with internal fixation. We report a successful surgically treated case of pediatric Hirayama disease, supplemented by a systematic review and collation of reported cases in the literature. METHODS: A review of the literature was performed by searching PubMed, Embase, and Web of Science. Full-length articles were included if they reported clinical data regarding the treatment of at least one patient with Hirayama disease and the neurologic outcome of that treatment. Articles were excluded if they did not provide information on treatment outcomes, were abstract-only publications, or were published in languages other than English. RESULTS: Of the fifteen articles reviewed, 63 patients were described, with 59 undergoing surgery. This encompassed both anterior and posterior spinal procedures and 1 hand tendon transfer. Fifty-five patients, including one from our institution, showed improvement post-treatment. Eleven of these patients were under 18 years old. CONCLUSION: Hirayama disease is an infrequent yet impactful cervical myelopathy with limited high-quality evidence available for optimal treatment. The current literature supports surgical decompression and stabilization as promising interventions. However, comprehensive research is crucial for evolving diagnosis and treatment paradigms.

Thoracolumbar Scoliosis in Pediatric Patients With Loeys-Dietz Syndrome: A Case Series.

Background Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder that predominantly affects cardiovascular, skeletal, and craniofacial structures. Associated thoracolumbar scoliosis in LDS can be challenging to manage, though other etiologies of pediatric scoliosis have better-defined management guidelines. We examined our institutional experience regarding the treatment of pediatric patients with LDS and scoliosis. Methodology In this retrospective study, all patients seen at our pediatric tertiary care center from 2004 through 2018 with a diagnosis of LDS were reviewed, and those with radiographic diagnoses of scoliosis (full-length scoliosis X-rays) were included. Demographic, clinical, and radiographic parameters were collected, and management strategies were reported. Results A total of 39 LDS patients whose ages ranged between seven and 13 years were identified. A total of nine patients were radiographically diagnosed with scoliosis, but three patients were excluded due to incomplete medical records, leaving six patients. The median age at scoliosis diagnosis was 11.5 years, with a median follow-up of 51 months. Two patients were successfully managed with observation (average initial Cobb angle (CA): 14°, average final CA: 20.5°). Two were braced, one successfully (initial CA: 15°, final CA: 30°) and one with a progressive disease requiring surgery (initial CA: 40°, final CA: 58°). Of the two who were offered surgical correction, one underwent surgery with a durable correction of spinal deformity (CA: 33° to 19°). One patient underwent a recent correction of aortic root dilatation and was not a candidate for scoliosis surgery. Conclusions Principles of adolescent idiopathic scoliosis management such as bracing for CA of 20-50° and surgery for CA of >50° can be applied to LDS patients with good outcomes. This augments our understanding of the treatment algorithm for pediatric patients with LDS.

Risk factors for sacral fracture following en bloc chordoma resection.

OBJECTIVE: The purpose of this study was to analyze risk factors for sacral fracture following noninstrumented partial sacral amputation for en bloc chordoma resection. METHODS: A multicenter retrospective chart review identified patients who underwent noninstrumented partial sacral amputation for en bloc chordoma resection with pre- and postoperative imaging. Hounsfield units (HU) were measured in the S1 level. Sacral amputation level nomenclature was based on the highest sacral level with bone removed (e.g., S1 foramen amputation at the S1-2 vestigial disc is an S2 sacral amputation). Variables collected included basic demographics, patient comorbidities, surgical approach, preoperative radiographic details, neoadjuvant and adjuvant radiation therapy, and postoperative sacral fracture data. RESULTS: A total of 101 patients (60 men, 41 women) were included; they had an average age of 69 years, BMI of 29 kg/m2, and follow-up of 60 months. The sacral amputation level was S1 (2%), S2 (37%), S3 (44%), S4 (9%), and S5 (9%). Patients had a posterior-only approach (77%) or a combined anterior-posterior approach (23%), with 10 patients (10%) having partial sacroiliac (SI) joint resection. Twenty-seven patients (27%) suffered a postoperative sacral fracture, all occurring between 1 and 7 months after the index surgery. Multivariable logistic regression analysis demonstrated S1 or S2 sacral amputation level (p = 0.001), combined anterior-posterior approach (p = 0.0064), and low superior S1 HU (p = 0.027) to be independent predictors of sacral fracture. The fracture rate for patients with superior S1 HU < 225, 225-300, and > 300 was 38%, 15%, and 9%, respectively. An optimal superior S1 HU cutoff of 300 was found to maximize sensitivity (89%) and specificity (42%) in predicting postamputation sacral fracture. In addition, the fracture rate for patients who underwent partial SI joint resection was 100%. CONCLUSIONS: Patients with S1 or S2 partial sacral amputations, a combined anterior-posterior surgical approach, low superior S1 HU, and partial SI joint resection are at higher risk for postoperative sacral fracture following en bloc chordoma resection and should be considered for spinopelvic instrumentation at the index procedure.

Primary spinal intramedullary anaplastic ganglioglioma in a pediatric patient.

Background: Gangliogliomas (GGs) are rare tumors of the central nervous system composed of neoplastic neural and glial cells and are typically low-grade. Intramedullary spinal anaplastic GGs (AGG) are rare, poorly understood, and often aggressive tumors that can result in widespread progression along the craniospinal axis. Due to the rarity of these tumors, data are lacking to guide clinical and pathologic diagnosis and standard of care treatment. Here, we present a case of pediatric spinal AGG to provide information on our institutional approach to work-up and to highlight unique molecular pathology. Case Description: A 13-year-old female presented with signs of spinal cord compression including right sided hyperreflexia, weakness, and enuresis. Magnetic resonance imaging (MRI) revealed a C3-C5 cystic and solid mass which was treated surgically with osteoplastic laminoplasty and tumor resection. Histopathologic diagnosis was consistent with AGG, and molecular testing identified mutations in H3F3A (K27M), TP53, and NF1. She received adjuvant radiation therapy and her neurological symptoms improved. However, at 6-month follow-up, she developed new symptoms. MRI revealed metastatic recurrence of tumor with leptomeningeal and intracranial spread. Conclusion: Primary spinal AGGs are rare tumors, but a growing body of literature shows some trends that may improve diagnosis and management. These tumors generally present in adolescence and early adulthood with motor/sensory impairment and other spinal cord symptoms. They are most commonly treated by surgical resection but frequently recur due to their aggressive nature. Further reports of these primary spinal AGGs along with characterization of their molecular profile will be important in developing more effective treatments.

Failures in Thoracic Spinal Fusions and Their Management.

Instrumented fixation and fusion of the thoracic spine present distinct challenges and complications including pseudarthrosis and junctional kyphosis. When complications arise, morbidity to the patient can be significant, involving neurologic injury, failure of instrumentation constructs, as well as iatrogenic spinal deformity. Causes of fusion failure are multifactorial, and incompletely understood. Most likely, a diverse set of biomechanical and biologic factors are at the heart of failures. Revision surgery for thoracic fusion failures is complex and often requires revision or extension of instrumentation, and frequently necessitates complex soft tissue manipulation to manage index level injury or to augment the changes of fusion.

Arteriovenous Malformations-Associated Epilepsy in Pediatrics.

PURPOSE: Seizures are the second most common presentation of cerebral arteriovenous malformations (AVMs); pediatric patients are more likely to develop AVM-associated epilepsy. We examined the role of multimodality AVM treatment in pediatric AVM-associated epilepsy to characterize long-term epilepsy outcomes. METHODS: A retrospective chart review identified pediatric patients with AVM-associated epilepsy seen at our institution from 2005 to 2018. Variables measured included demographic and descriptive data. Primary outcomes included seizure freedom, seizure control, and functional outcomes. RESULTS: Of 105 pediatric patients with AVMs, 18 had AVM-related epilepsy. Thirteen underwent surgical resection, of which 6 underwent preoperative embolization. Twelve (92.31%) had complete resection; one (7.69%) with residual underwent redo craniotomy with subsequent complete resection. All had radiographic cure at most recent follow-up, with no recurrence seen during length of follow-up (mean 2.17 years, SD 1.40, range 0.25-4.41). Eight (61.54%) experienced seizure freedom postoperatively; 12 (92.31%) were modified Engel Class I at last follow-up. Five patients underwent treatment without open surgical resection, with conservative management (3, 60%) or endovascular embolization (2, 40%). None in our cohort underwent radiosurgery. Of those embolized, one had complete AVM obliteration and two had partial obliteration. Four of the 5 patients (80%) treated without open surgery achieved seizure freedom. CONCLUSION: Long-term outcomes of AVM-related epilepsy are poorly characterized in children. We found that in addition to improved AVM outcomes regarding obliteration, treatment of residual, and recurrence, pediatric patients undergoing surgical AVM treatment had improved AVM-associated epilepsy outcomes, with 61.54% achieving seizure freedom and 92.31% classified as modified Engel Class I seizure control.

Congenital Fusion of Dens to T3 Vertebra in Klippel-Feil Syndrome.

BACKGROUND: Patients with Klippel-Feil syndrome may present with neurologic complaints such as neck pain, radiculopathy and gait instability. Here we describe surgical management of a patient with congenital fusion of the occipital-cervical region and also block circumferential fusion of dens to T3 with spinal cord compression. This report is the first of its kind with such extensive fusion. CASE DESCRIPTION: Our patient was a 56 year-old female, who presented with neck pain and tingling in all extremities. On exam, she had a short neck, prominent jaw with extremely limited range of motion in neck and features of myelopathy. CT showed fusion of the dens to T3 vertebrae. Patient underwent sub-occipital craniectomy, C1 laminectomy and Occiput to T5 posterior fixation and fusion with neurologic improvement. CONCLUSION: This is the first reported case of Klippel-Feil syndrome with fusion of all cervical vertebrae down to T3. We recommend surgery for advanced cases of myelopathy or radiculopathy due to stenosis and spinal instability.

Feasibility and safety of transradial access for pediatric neurointerventions.

BACKGROUND: Diagnostic cerebral angiograms are increasingly being performed by transradial access (TRA) in adults, following data from the coronary literature supporting fewer access-site complications. Despite this ongoing trend in neuroangiography, there has been no discussion of its use in the pediatric population. Pediatric TRA has scarcely been described even for coronary or other applications. This is the first dedicated large study of transradial access for neuroangiography in pediatric patients. METHODS: A multi-institutional series of consecutively performed pediatric transradial angiograms and interventions was collected. This included demographic, procedural, outcomes, and safety data. Data was prospectively recorded and retrospectively analyzed. RESULTS: Thirty-seven diagnostic angiograms and 24 interventions were performed in 47 pediatric patients. Mean age, height, and weight was 14.1 years, 158.6 cm, and 57.1 kg, respectively. The radial artery measured 2.09+/-0.54 mm distally, and 2.09+/-0.44 mm proximally. Proximal and distal angiography were performed for both diagnostic and interventional application (17 distal angiograms, two distal interventions). Clinically significant vasospasm occurred in eight patients (13.1%). Re-access was successfully performed 11 times in seven patients. Conversion to femoral access occurred in five cases (8.2%). The only access-related complication was a small asymptomatic wrist hematoma after TR band removal. CONCLUSIONS: Transradial access in pediatric patients is safe and feasible. It can be performed successfully in many cases but carries some unique challenges compared with the adult population. Despite the challenge of higher rates of vasospasm and conversion to femoral access, it is worth exploring further, given the potential benefits.

Accuracy and Workflow Improvements for Responsive Neurostimulation Hippocampal Depth Electrode Placement Using Robotic Stereotaxy.

Background: Robotic stereotaxy is increasingly common in epilepsy surgery for the implantation of stereo-electroencephalography (sEEG) electrodes for intracranial seizure monitoring. The use of robots is also gaining popularity for permanent stereotactic lead implantation applications such as in deep brain stimulation and responsive neurostimulation (RNS) procedures. Objective: We describe the evolution of our robotic stereotactic implantation technique for placement of occipital-approach hippocampal RNS depth leads. Methods: We performed a retrospective review of 10 consecutive patients who underwent robotic RNS hippocampal depth electrode implantation. Accuracy of depth lead implantation was measured by registering intraoperative post-implantation fluoroscopic CT images and post-operative CT scans with the stereotactic plan to measure implantation accuracy. Seizure data were also collected from the RNS devices and analyzed to obtain initial seizure control outcome estimates. Results: Ten patients underwent occipital-approach hippocampal RNS depth electrode placement for medically refractory epilepsy. A total of 18 depth electrodes were included in the analysis. Six patients (10 electrodes) were implanted in the supine position, with mean target radial error of 1.9 ± 0.9 mm (mean ± SD). Four patients (8 electrodes) were implanted in the prone position, with mean radial error of 0.8 ± 0.3 mm. The radial error was significantly smaller when electrodes were implanted in the prone position compared to the supine position (p = 0.002). Early results (median follow-up time 7.4 months) demonstrate mean seizure frequency reduction of 26% (n = 8), with 37.5% achieving ≥50% reduction in seizure frequency as measured by RNS long episode counts. Conclusion: Prone positioning for robotic implantation of occipital-approach hippocampal RNS depth electrodes led to lower radial target error compared to supine positioning. The robotic platform offers a number of workflow advantages over traditional frame-based approaches, including parallel rather than serial operation in a bilateral case, decreased concern regarding human error in setting frame coordinates, and surgeon comfort.

Gastrointestinal stromal tumor with intracranial metastasis: case presentation and systematic review of literature.

BACKGROUND: Intracranial metastasis of Gastrointestinal Stromal Tumors (GISTs) is rare but presents unique treatment challenges. We present a case of intracranial metastasis of GIST with a systematic review of the literature. A literature search using key terms "'gastrointestinal stromal tumor' AND brain AND metastasis"" was conducted through May 2019 via Embase and Pubmed according to PRISMA guidelines. Only cases describing intradural metastases rather than calvarial or intraorbital metastases were included. CASE PRESENTATION: A 57-year-old woman with history of GIST metastatic to the liver presented with a six-week history of left facial weakness, left hearing loss, and left facial numbness, and a one-week history of headaches, gait disturbance, and dizziness. MRI revealed a contrast-enhancing dural-based left middle cranial fossa mass measuring 2.9 cm × 3.1 cm × 3.4 cm with extension into the internal auditory canal and cerebral edema. A left temporal craniotomy was performed to excise the lesion, and the patient was discharged to a rehabilitation facility at her preoperative baseline. Intraoperative pathology revealed a spindle cell neoplasm, postoperative MRI demonstrated gross total resection of the lesion, and microscopic analysis demonstrated sheets of spindled tumor cells with short ovoid, irregular, hyperchromatic nuclei and scattered large atypical nuclei without extensive necrosis. Immunohistochemical staining was positive for KIT proto-oncogene (CD117, c-KIT), and the patient was put on imatinib (400 mg/day). CONCLUSIONS: Of the 18 cases analyzed and our present case, metastasis typically involved the cerebrum with only one in infratentorial elements. The tumors in seven of the cases involved the dura, and one case metastasized to the pituitary. Eight patients died following treatment. Surgery remains the mainstay of intracranial metastatic GIST, however there are many reports of good responses to radiation or chemotherapy alone. More investigation is required to determine the best treatment course for these patients.

An update on pediatric surgical epilepsy: Part I.

Epilepsy affects many children worldwide, with drug-resistant epilepsy affecting 20-40% of all children with epilepsy. This carries a significant burden for patients and their families and is strongly correlated with poor cognitive outcomes, depression, anxiety, developmental delay, and impaired activities of daily living. For this reason, we sought to explore the role of pediatric epilepsy surgery and provide an overview of the factors contributing to epilepsy surgery planning and execution. We review the necessary preoperative evaluations, surgical indications, planning considerations, and surgical options to provide a clear pathway in the evaluation and planning of pediatric epilepsy surgery.

Type 2 diabetes is an independent negative prognostic factor in patients undergoing surgical resection of a WHO grade I meningioma.

OBJECTIVES: In recent years, there has been increased recognition of the relationship between type 2 diabetes mellitus (DM) and poor outcomes following a variety of surgical procedures. We sought to study the role of type 2 DM as a prognostic factor affecting the long-term survival of patients undergoing surgical resection of a WHO Grade I meningioma. METHODS: We conducted a retrospective cohort study on 196 patients who had a WHO Grade I meningioma resected at our institution between 2001 and 2013. The medical record was reviewed to identify a pre-existing diagnosis of type 2 DM. Patient mortality was reviewed by medical record and Social Security Death Index (SSDI). Variables associated with survival in a univariate analysis were included in the multivariate Cox model if P<0.10. Variables with probability values >0.05 were then removed from the multivariate model in a step-wise fashion. RESULTS: 33 (17%) patients had pre-existing diagnoses of type 2 DM prior to clinical presentation. Mean survival time in diabetic patients was 52.1 months compared to 160.9 months in non-diabetics. The decreased survival rate and time in patients with type 2 DM were found to be statistically significant (p=0.008 and p<0.0001, respectively). In a multivariate Cox analysis, a pre-existing history of type 2 DM was independently associated with decreased survival following the resection of a WHO Grade I meningioma (HR=2.6, p=0.045). CONCLUSIONS: A pre-existing diagnosis of type 2 DM is an independent negative prognostic indicator following the resection of a WHO Grade I meningioma.

Short-term postoperative surveillance imaging may be unnecessary in elderly patients with resected WHO Grade I meningiomas.

The optimal timing and frequency of postoperative imaging surveillance after a meningioma resection are not well-established. The low recurrence rates and slow growth of World Health Organization (WHO) Grade I meningiomas in particular have raised doubts about the utility of postoperative imaging surveillance. We sought to analyze the cost and utility of asymptomatic surveillance imaging in elderly patients after the resection of a WHO Grade I meningioma. We conducted a retrospective cohort study on 45 patients who had a primary WHO Grade I meningioma resected at our institution between 2001-2013 at or above the age of 60 with a minimum of 2 years of follow-up. All postoperative clinic notes were reviewed alongside imaging results to verify that patients were asymptomatic during the surveillance period. MRI and CT scan costs (all $USD) were estimated at $599.61 and $334.31 respectively based on the Centers for Medicare and Medicaid national averages. During an average follow-up period of 4.5 years, the average number of total imaging studies performed per asymptomatic patient was 3.58 with an average total cost of $2086.30 per patient. Forty-two (93%) patients had no new abnormal findings on any of their imaging. Three (7%) patients demonstrated either a new meningioma or progressive growth of the postoperative residual tumor on imaging. No asymptomatic patient underwent a reoperation. Our data suggest that elderly patients with resected WHO Grade I meningiomas are at low risk for recurrence and may not need asymptomatic surveillance imaging for the first several postoperative years.

Surgical site infection in orthopedic trauma: A case-control study evaluating risk factors and cost.

BACKGROUND: With the shift of our healthcare system toward a value-based system of reimbursement, complications such as surgical site infections (SSI) may not be reimbursed. The purpose of our study was to investigate the costs and risk factors of SSI for orthopedic trauma patients. METHODS: Through retrospective analysis, 1819 patients with isolated fractures were identified. Of those, 78 patients who developed SSIs were compared to 78 uninfected control patients. Patients were matched by fracture location, type of fracture, duration of surgery, and as close as possible to age, year of surgery, and type of procedure. Costs for treatment during primary hospitalization and initial readmission were determined and potential risk factors were collected from patient charts. A Wilcoxon test was used to compare the overall costs of treatment for case and control patients. Costs were further broken down into professional fees and technical charges for analysis. Risk factors for SSIs were analyzed through a chi-squared analysis. RESULTS: Median cost for treatment for patients with SSIs was $108,782 compared to $57,418 for uninfected patients (p < 0.001). Professional fees and technical charges were found to be significantly higher for infected patients. No significant risk factors for SSIs were determined. CONCLUSIONS: Our findings indicate the potential for financial losses in our new healthcare system due to uncompensated care. SSIs nearly double the cost of treatment for orthopedic trauma patients. There is no single driver of these costs. Reducing postoperative stay may be one method for reducing the cost of treating SSIs, whereas quality management programs may decrease risk of infection.

Ankle Fractures and Modality of Hospital Transport at a Single Level 1 Trauma Center: Does Transport by Helicopter or Ground Ambulance Influence the Incidence of Complications?

In an era of concern over the rising cost of health care, cost-effectiveness of auxiliary services merits careful evaluation. We compared costs and benefits of Helicopter Emergency Medical Service (HEMS) with Ground Emergency Medical Service (GEMS) in patients with an isolated ankle fracture. A medical record review was conducted for patients with an isolated ankle fracture who had been transported to a level 1 trauma center by either HEMS or GEMS from January 1, 2000 to December 31, 2010. We abstracted demographic data, fracture grade, complications, and transportation mode. Transportation costs were obtained by examining medical center financial records. A total of 303 patients was included in the analysis. Of 87 (28.71%) HEMS patients, 53 (60.92%) had sustained closed injuries and 34 (39.08%) had open injuries. Of the 216 (71.29%) GEMS patients, 156 (72.22%) had closed injuries and 60 (27.78%) had open injuries. No significant difference was seen between the groups regarding the percentage of patients with open fractures or the grade of the open fracture (p = .07). No significant difference in the rate of complications was found between the 2 groups (p = 18). The mean baseline cost to transport a patient via HEMS was $10,220 + a $108/mile surcharge, whereas the mean transport cost using GEMS was $976 per patient + $16/mile. Because the HEMS mode of emergency transport did not significantly improve patient outcomes, health systems should reconsider the use of HEMS for patients with isolated ankle fractures.

Asking for the 22-modifier in isolated ankle fractures: does the operative note make the case?

We evaluated the operative notes for justification on the use of the 22-modifier in ankle fracture cases and compared the differences in physician billing and reimbursement. A total of 265 patients who had undergone operative management of isolated ankle fractures across a 10-year period were identified at a level I trauma center through a retrospective chart review. Of the 265 patients, 61 (23.0%) had been billed with the 22-modifier. The radiographs were reviewed by 3 surgeons to determine the complexity of the case. The amount of the professional fees and payments was obtained from the financial services department. Operative reports were reviewed for inclusion of eight 22-modifier criteria and word count. Mann-Whitney U tests of means were used to compare cases with and without the 22-modifier. From our analysis of preoperative radiographs, 37 (60%) showed evidence of a significantly complex fracture that justified the use of the 22-modifier. A review of the operative reports showed that 42 (68%) did not identify 2 or more reasons for requesting the 22-modifier in the report. Overall, the 22-modifier cases were not always reimbursed significantly greater amounts than the nonmodifier cases. No significant difference in the average word count of the operative notes was found. We have concluded that orthopedic trauma surgeons do not appropriately justify the use of the 22-modifier within their operative report. Further education on modifiers and the use of the operative report as billing documentation is required to ensure surgeons are adequately reimbursed for difficult trauma cases.