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Kavelin Rumalla, Kyle A. Smith and Paul M. Arnold

Immunoglobulin G4–related disease (IgG4-RD) is a recently defined condition characterized by inflammatory tumefactive lesions in various organ systems. IgG4-RD is a clinical and radiological diagnosis of exclusion and requires the presence of specific histopathological criteria for diagnosis.

A 50-year-old man presented to an outside hospital with a 3-month history of progressively worsening back pain and symptoms of pleurisy, nasal crusting, and hematochezia. Radiological workup revealed an epidural-paraspinal mass with displacement of the spinal cord, destruction of the T5–6 vertebrae, and extension into the right lung. Biopsy sampling and subsequent histopathological analysis revealed dense lymphoplasmacytic infiltrate with an increased number of IgG4-positive plasma cells and a storiform pattern of fibrosis. With strong histopathological evidence of IgG4-RD, the patient was started on a regimen of prednisone. Further testing ruled out malignant neoplasm, infectious etiologies, and other autoimmune diseases. Two weeks later, the patient presented with acute-onset paraplegia due to spinal cord compression. The patient underwent decompression laminectomy of T5–6, posterior instrumented fusion of T2–8, and debulking of the epidural-paraspinal mass. After the continued administration of glucocorticosteroids, the patient improved remarkably to near-normal strength in the lower extremities and sensory function 6 months after surgery.

To the authors' knowledge, this is the first case of IgG4-related epidural inflammatory pseudotumor and spinal cord compression in the United States. This case highlights the importance of early administration of glucocorticosteroids, which were essential to preventing further progression and preventing relapse. IgG4-RD evaluation is important after other diseases in the differential diagnosis are ruled out.

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Kyle A. Smith and Morgan B. Glusman

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Sindhura Pisipati, Kyle A. Smith, Kushal Shah, Koji Ebersole, Roukoz B. Chamoun and Paul J. Camarata

OBJECTIVE

Laser interstitial thermal therapy (LITT) is used in numerous neurosurgical applications including lesions that are difficult to resect. Its rising popularity can be attributed to its minimally invasive approach, improved accuracy with real-time MRI guidance and thermography, and enhanced control of the laser. One of its drawbacks is the possible development of significant edema, which contributes to extended hospital stays and often necessitates hyperosmolar or steroid therapy. Here, the authors discuss the use of minimally invasive craniotomy to resect tissue ablated with LITT in attempt to minimize cerebral edema.

METHODS

Five patients with glioblastoma multiforme prospectively underwent LITT followed by resection. The LITT was performed with the aid of an MR-compatible skull-mounted frame in the MRI suite. Ablated tumor was then resected via small craniotomy by using the NICO Myriad system or cavitron ultrasonic surgical aspirator. Postoperative management involved dexamethasone administration slowly tapered over several weeks.

RESULTS

The use of resection following LITT, as compared with open resection or LITT alone, did not extend the hospital stay except in 1 patient who required 3-day inpatient management of edema with a trapped ventricle. No new neurological deficits were encountered, although 1 patient developed seizures postoperatively. No increase in infection rates was identified.

CONCLUSIONS

Resection of ablated tumor is a viable option to reduce the incidence of neurological deficits due to edema following LITT. This approach appears to mitigate cerebral edema by increasing available volume for mass effect and reducing the tissue burden that may promote an inflammatory response.

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Kyle A. Smith, Michael Salacz and Paul J. Camarata

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Anthony M. Alvarado, Kyle A. Smith and Roukoz B. Chamoun

Glioependymal cysts are rare congenital lesions of the central nervous system. Reported surgical treatments of these lesions have varied and yielded mixed results, and the optimal surgical strategy is still controversial. The authors here report the clinical and surgical outcomes for three adult patients successfully treated with neuroendoscopic fenestration into the ventricular system. The patients had presented with symptomatic glioependymal cysts in the period from 2013 to 2016 at the authors’ institution. All underwent minimally invasive neuroendoscopic fenestration of the glioependymal cyst into the lateral ventricle via a stereotactically guided burr hole. Presenting clinical and radiological findings, operative courses, and postintervention outcomes were evaluated.

All three patients initially presented with symptoms related to regional mass effect of the underlying glioependymal cyst, including headaches, visual disturbances, and hemiparesis. All patients were successfully treated with endoscopic fenestration of the cyst wall into the lateral ventricle, where the wall was thinnest. Postoperatively, all patients reported improvement in their presenting symptoms, and neuroimaging demonstrated decompression of the cyst. Clinical follow-up ranged from 4 months to 5 years without evidence of reexpansion of the cyst or shunt requirement.

Compared to open resection and shunting of the cyst contents, minimally invasive endoscopic fenestration of a glioependymal cyst into the ventricular system is a safe and effective surgical option. This approach is practical, is less invasive than open resection, and appears to provide a long-term solution.

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Kyle A. Smith, John D. Leever, Phillip D. Hylton, Paul J. Camarata and Roukoz B. Chamoun

OBJECTIVE

Meningioma consistency, firmness or softness as it relates to resectability, affects the difficulty of surgery and, to some degree, the extent of resection. Preoperative knowledge of tumor consistency would affect preoperative planning and instrumentation. Several methods of prediction have been proposed, but the majority lack objectivity and reproducibility or generalizability to other surgeons. In a previous pilot study of 20 patients the authors proposed a new method of prediction based on tumor/cerebellar peduncle T2-weighted imaging intensity (TCTI) ratios in comparison with objective intraoperative findings. In the present study they sought validation of this method.

METHODS

Magnetic resonance images from 100 consecutive patients undergoing craniotomy for meningioma resection were evaluated preoperatively. During surgery a consistency grade was prospectively applied to lesions by the operating surgeon, as determined by suction and/or cavitron ultrasonic surgical aspirator (CUSA) intensity. Consistency grades were A, soft; B, intermediate; and C, fibrous. Using T2-weighted MRI sequences, TCTI ratios were calculated. Analysis of the TCTI ratios and intraoperative tumor consistency was completed with ANOVA and receiver operating characteristic curves.

RESULTS

Of the 100 tumors evaluated, 50 were classified as soft, 29 as intermediate, and 21 as firm. The median TCTI ratio for firm tumors was 0.88; for intermediate tumors, 1.5; and for soft tumors, 1.84. One-way ANOVA comparing TCTI ratios for these groups was statistically significant (p < 0.0001). A single cutoff TCTI value of 1.41 for soft versus firm tumors was found to be 81.9% sensitive and 84.8% specific.

CONCLUSIONS

The authors propose this T2-based method of tumor consistency prediction with correlation to objective intraoperative consistency. This method is quantifiable and reproducible, which expands its usability. Additionally, it places tumor consistency on a graded continuum in a clinically meaningful way that could affect preoperative surgical planning.

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Kavelin Rumalla, Kyle A. Smith, Vijay Letchuman, Mrudula Gandham, Rachana Kombathula and Paul M. Arnold

OBJECTIVE

Posttraumatic seizures (PTSs) are the most common complication following a traumatic brain injury (TBI) and may lead to posttraumatic epilepsy. PTS is well described in the adult literature but has not been studied extensively in children. Here, the authors utilized the largest nationwide registry of pediatric hospitalizations to report the national incidence, risk factors, and outcomes associated with PTS in pediatric TBI.

METHODS

The authors queried the Kids’ Inpatient Database (KID) using ICD-9-CM codes to identify all patients (age < 21 years) who had a primary diagnosis of TBI (850.xx–854.xx) and a secondary diagnosis of PTS (780.33, 780.39). Parameters of interest included patient demographics, preexisting comorbidities, hospital characteristics, nature of injury (open/closed), injury type (concussion, laceration/contusion, subarachnoid hemorrhage, subdural hematoma, or epidural hematoma), loss of consciousness (LOC), surgical management (Clinical Classification Software code 1 or 2), discharge disposition, in-hospital complications, and in-hospital mortality. The authors utilized the IBM SPSS statistical package (version 24) for univariate comparisons, as well as the identification of independent risk factors for PTS in multivariable analysis (alpha set at < 0.05).

RESULTS

The rate of PTS was 6.9% among 124,444 unique patients hospitalized for TBI. The utilization rate of continuous electroencephalography (cEEG) was 0.3% and increased between 2003 (0.1%) and 2012 (0.7%). The most common etiologies of TBI were motor vehicle accident (n = 50,615), accidental fall (n = 30,847), and blunt trauma (n = 13,831). However, the groups with the highest rate of PTS were shaken infant syndrome (41.4%), accidental falls (8.1%), and cycling accidents (7.4%). In multivariable analysis, risk factors for PTS included age 0–5 years (compared with 6–10, 11–15, and 16–20 years), African American race (OR 1.4), ≥ 3 preexisting comorbidities (OR 4.0), shaken infant syndrome (OR 4.4), subdural hematoma (OR 1.6), closed-type injury (OR 2.3), brief LOC (OR 1.4), moderate LOC (OR 1.5), and prolonged LOC with baseline return (OR 1.8). Surgically managed patients were more likely to experience PTS (OR 1.5) unless they were treated within 24 hours of admission (OR 0.8). PTS was associated with an increased likelihood of in-hospital complications (OR 1.7) and adverse (nonroutine) discharge disposition (OR 1.2), but not in-hospital mortality (OR 0.5). The overall utilization rate of cEEG was 1.3% in PTS patients compared with 0.2% in patients without PTS. Continuous EEG monitoring was associated with higher rates of diagnosed PTS (35.4% vs 6.8%; OR 4.9, p < 0.001).

CONCLUSIONS

PTS is common in children with TBI and is associated with adverse outcomes. Independent risk factors for PTS include younger age (< 5 years), African American race, increased preexisting comorbidity, prolonged LOC, and injury pattern involving cortical exposure to blood products. However, patients who undergo urgent surgical evacuation are less likely to develop PTS.