Rapidly progressive diffuse leptomeningeal glioneuronal tumor in an adult female: illustrative case.

BACKGROUND: Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare brain tumor only recently classified by the World Health Organization in 2016 and has few reports on its incidence in adults. OBSERVATIONS: The authors describe a case of DLGNT presenting in a 47-year-old female with seizures, cranial neuropathies, and communicating hydrocephalus with rapid clinical progression. Workup demonstrated progressive leptomeningeal enhancement of the skull base, cranial nerves, and spine, and communicating hydrocephalus. Elevated serum rheumatological markers and early response to systemic corticosteroids and immunosuppressant therapy complicated the diagnosis. Multiple biopsy attempts were required to obtain diagnostic tissue. Pathology demonstrated hypercellularity surrounding leptomeningeal vessels with nuclear atypia, staining positive for GFAP, Olig2, S100, and synaptophysin. Molecular pathology demonstrated loss of chromosome 1p, BRAF overexpression but no rearrangement, and H3K27 mutation. Repeat cerebrospinal fluid (CSF) diversion procedures were required for hydrocephalus management due to high CSF protein content. LESSONS: This report describes a rare, aggressive, adult presentation of DLGNT. Leptomeningeal enhancement and communicating hydrocephalus should raise suspicion for this disease process. Biopsy at early stages of disease progression is essential for early diagnosis and prompt treatment. Further study into the variable clinical presentation, histological and molecular pathology, and optimal means of diagnosis and management is needed.

Radiographic risk factors for intracranial hemorrhage in patients with left ventricular assist devices.

OBJECTIVE: Intracranial hemorrhage (ICH) in patients with left ventricular assist devices (LVAD) is a devastating complication. Demographic risk factors for ICH in LVAD patients are defined, however anatomic predispositions to ICH are unknown. We sought to interrogate intracranial radiographic risk factors for ICH in LVAD patients. METHODS: We reviewed 440 patients who received an LVAD from 2008-2021. We selected patients with CT scans of the head either before or after LVAD placement, but typically within 5 years. 288 patients (21 ICH, 267 Control) with imaging were included. A detailed chart review was performed on demographics, radiographic features, and management. RESULTS: The incidence of ICH in our total cohort was 8.6% (38/440). The presence of pump thrombosis (p=0.001), driveline infection (p=0.034), other hemorrhage (p=0.001), or previous placement of a cardio-defibrillator (p=.003) was associated with increased risk for ICH. An analysis of imaging revealed that the presence of a mass (p=0.006), vascular pathology (p=0.001), and microangiopathy (p=0.04) was significantly associated with ICH in LVAD patients. These radiographic features were validated with a multivariate logistic regression which confirmed presence of a mass (aOR 332.1, 95% CI: 14.7-7485.1, p<0.001), vascular pathology (aOR 69.7, 95% CI: 1.8-2658.8, p=0.022), and microangiopathy (aOR 6.5, 95% CI: 1.1-37.6, p=0.035) were independently associated with ICH. CONCLUSION: Radiographic evidence of microangiopathy, intracranial mass, and vascular pathology are independent risk factors for ICH which are readily identified by imaging. We advocate that CT imaging be used to further stratify patients at highest risk of ICH during treatment with an LVAD.

Modern Appraisal of Patency and Complications in Cerebral Bypass Surgery: A Single Institution Experience.

BACKGROUND: Cerebral bypass is a valuable surgical technique in well-selected patient populations. Updated clinical guidelines and improved surgical techniques warrant a contemporary reevaluation of the complications and patency to inform clinical practice and enhance postoperative patient care. OBJECTIVE: To assess the complication rates and postoperative graft patency for the 3 most common indications for bypass surgery: moyamoya disease, intracranial atherosclerosis, and intracranial aneurysms. METHODS: Perioperative notes of 175 consecutive bypass patients at a single institution were retrospectively identified to evaluate the clinical course and complications of surgery. RESULTS: The rate of total postoperative complications between moyamoya disease (9 of 98, 9.2%), intracranial atherosclerotic disease (7 of 57, 12.3%), and intracranial aneurysm (4 of 20, 20%) was not statistically different (P = .33). Immediate postoperative bypass patency was significantly higher in moyamoya disease (90 of 96, 93.8%) and intracranial atherosclerotic disease (48 of 51, 94.1%) than in intracranial aneurysm (13 of 18, 72.2%; P = .02). Intravenous heparin administration during bypass suturing was negatively associated with immediate postoperative patency (87% heparin patency vs 99% no heparin patency; P = .02). Double-barrel bypass trended toward an increased risk of wound healing complications (2 of 13, 15.4%) compared with the single-barrel bypass technique (4 of 156, 2.6%; P = .07). CONCLUSION: Cerebral bypass surgery remains an excellent surgical treatment for moyamoya disease, intracranial atherosclerosis, and intracranial aneurysms. This study suggests bypass is safer in moyamoya disease and intracranial atherosclerosis. Additional studies to clarify the risk of single-barrel vs double-barrel bypass and intraoperative heparin-stratified complications may be beneficial.

The Final Cut: Evolution of Cavernous Sinus Decompression Beyond the Dolenc Cut.

BACKGROUND: Meningiomas involving the cavernous sinus (CS) represent a formidable challenge to neurosurgeons. Because of high morbidity, aggressive tumor resection within the CS has been largely replaced by extracavernous tumor resection and decompression of the CS. The widely used Dolenc method involves blind dural transection over the oculomotor nerve, potentially placing the crossing trochlear nerve at risk. OBJECTIVE: To provide a safer way for the decompression of the CS and at the same time, circumferential resection of the temporal lobe dura. METHODS: Cadaveric dissection of 8 cadaveric heads (16 sides) was performed. Frontotemporal craniotomy with zygomatic osteotomy was performed. Extradural and intradural dissection of the lateral wall of the CS and free edge of tentorium was performed. Photographic documentation, artistic illustrations, and illustrative video cases are provided. RESULTS: Three tether points were released: anterior-the meningo-orbital band, lateral-dura over the V2 and V3, and medial-superficial layer of the free edge of tentorium. Transection of the superficial layer of the free edge of the tentorium along its edge, in conjunction with a lateral cut over the temporal convexity, constitutes the final cut, allowing a resection of the lateral CS wall and the circumferential resection of temporal lobe dura. CONCLUSION: Using the "final cut" technique allows for a circumferential resection of the temporal lobe dura and lateral CS wall while avoiding blind cuts that could put cranial nerves at risk. All dural transections are performed in anatomically separated layers under constant visualization.

Spontaneous resolution of cerebral arteriovenous malformation after liver transplant: illustrative case.

BACKGROUND: Cerebral arteriovenous malformations (AVMs) have historically been considered congenital lesions with treatment options including surgery, radiation therapy, and observation. Spontaneous resolution of cerebral AVMs remains an exceedingly rare event with poorly understood pathophysiology. MATERIALS AND METHODS: Herein we report a retrospective case review of a 28-year-old man with alcoholic cirrhosis who presented with a seizure 3 weeks after liver transplantation. Neuroimaging confirmed the presence of a Spetzler-Martin grade 2 AVM in the right frontal lobe. Due to the recent liver transplantation, treatment was deferred at the time of initial diagnosis and the patient was observed for a course of 1 year. Follow-up imaging 1 year later showed resolution of the AVM, confirmed by a catheter angiogram. CONCLUSION: Spontaneous resolution of cerebral AVMs is a rare event. Treatment of chronic liver disease resulted in the normalization of angiogenic factors that likely led to AVM resolution. This case provides valuable insight into the vital role of angiogenesis in the natural history of AVMs.

Bilateral Nervus Intermedius Sectioning for Geniculate Neuralgia: Case Report and Operative Video.

BACKGROUND AND IMPORTANCE: Geniculate neuralgia is a rare condition characterized by excruciating ear pain. Surgical options for geniculate neuralgia include microvascular decompression and sectioning of the nervus intermedius. We report herein a case of bilateral geniculate neuralgia treated by nervus intermedius sectioning without prior microvascular decompression. To our knowledge, this is the first report of this treatment strategy with a subsequent description of the side effects of bilateral nervus intermedius disruption. CLINICAL PRESENTATION: A 54-yr-old woman presented with bilateral geniculate neuralgia, worse on the left, refractory to medical therapy. Surgical treatment options were reviewed, including microvascular decompression and sectioning of the nervus intermedius. She opted for left nervus intermedius sectioning. The procedure was uncomplicated and no compressive vascular loop was identified during surgery. Postoperatively, she had complete symptom resolution with no discernable side effects. Three years later, the patient developed worsening geniculate neuralgia on the contralateral side. After the discussion of treatment options, she opted again for sectioning of the contralateral nervus intermedius with successful resolution of all symptoms after surgery. Following surgery, the patient identified partial impairment of lacrimation and gustation. She continued to have functional taste of the anterior two-thirds of the tongue, lacrimation, and hearing bilaterally. CONCLUSION: Bilateral sectioning of nervus intermedius may provide benefit in patients with bilateral geniculate neuralgia without egregious side effects. However, lacrimatory and gustatory alterations are a potentially significant side effect with a wide range of symptomatology.

Endoscopic Endonasal Versus Transoral Odontoidectomy for Non-Neoplastic Craniovertebral Junction Disease: A Case Series.

BACKGROUND: Odontoidectomy is a challenging yet effective operation for decompression of non-neoplastic craniovertebral junction disease. Though both the endoscopic endonasal approach (EEA) and the transoral approach (TOA) have been discussed in the literature, there remain few direct comparisons between the techniques. OBJECTIVE: To evaluate the perioperative outcomes of EEA vs TOA odontoidectomy. METHODS: A retrospective review of all cases undergoing odontoidectomy by either the EEA or TOA was performed. Attention was paid to the need for prolonged nutritional support, prolonged respiratory support, and hospitalization times. RESULTS: During the study period between 2000 and 2018, 25 patients underwent odontoid process resection (18 TOA and 7 EEA). The most common indication for surgery was basilar invagination. Hospital length of stay, intensive care unit length of stay, and intubation days were all significantly shorter in the EEA group compared to the TOA group (P < .01, P = .01, P < .01, respectively). Prolonged nutritional support in the form of a gastrostomy tube was required in 5 patients and tracheostomy was required in 4 patients; all of these underwent odontoidectomy by the TOA. There was no statistical difference in neurological outcomes between the EEA and TOA groups (P = .17). CONCLUSION: Odontoidectomy can be performed safely through both the EEA and TOA. The results of this study suggest the EEA has shorter hospitalizations and a lower probability of requiring prolonged nutritional support. These advantages are likely the results of decreased oropharyngeal mucosa disruption as compared to the TOA.

Changing Enhancement Pattern and Tumor Volume of Vestibular Schwannomas After Subtotal Resection.

BACKGROUND: Surgical strategy in vestibular schwannomas may require subtotal resection to preserve neurologic function. Residual tumor growth pattern and contrast enhancement in the immediate postresection period remain uncertain. We sought to evaluate changes in the enhancement pattern and volume of vestibular schwannomas after subtotal resection in the immediate postoperative period. METHODS: Volumetric analysis of tumor size and enhancement patterns of vestibular schwannomas were measured on magnetic resonance imaging (MRI) scans obtained within 3 days of surgery, 3 months after surgery, and 1 year after surgery. RESULTS: Nineteen patients were eligible for inclusion in the study (9 male and 10 female) with an average age of 47 years. Contrast enhancement was absent in 6 of 19 (32%) of cases on the immediate postresection MRI with return of expected enhancement on subsequent studies. Volumetric analysis identified that tumors decreased in size by an average of 35% in the first 3 months (P = 0.025) after resection and 46% in the first year after resection (P < 0.01). CONCLUSIONS: Vestibular schwannomas that undergo subtotal resection tend to decrease in size over the first 3 months after resection. Residual tumor volume may fail to enhance on the immediate postresection MRI. Both of these findings could lead surgeons to misinterpret degree of resection after surgery and have implications for clinical decision making and research reporting in the scientific literature for vestibular schwannomas after subtotal resection.

Facial nerve outcomes following gamma knife radiosurgery for subtotally resected vestibular schwannomas: Early versus delayed timing of therapy.

OBJECTIVES: Initially treating vestibular schwannomas (VSs) with subtotal resection (STR) followed by Gamma Knife radiosurgery (GKRS) for progression of tumor residual is a strategy that balances maximal tumor resection with preservation of neurological function. The effect of timing of GKRS for residual and recurrent VSs remains poorly defined. We developed a simple and practical treatment algorithm for the timing of GKRS after STR of VSs and reviewed our follow-up results to determine outcomes between patients treated with early vs. late GKRS. PATIENTS AND METHODS: Patients that underwent STR between 1999 and 2017 for a VS at Tufts Medical Center were identified and included in the study cohort. Patients who received GKRS ≤ 12 months after STR were included in the early intervention group. Patients who received GKRS > 12 months after STR or did not have tumor progression on follow-up thus not requiring GKRS were included in the observation/delayed intervention group. RESULTS: STR of VSs was performed on 23 patients. Mean patient age at the time of STR was 53.0 years (range: 20-86.2). The mean follow-up was 4.2 years (range: 1 month-15.5 years). Patients most frequently presented with hearing loss. There were 5 patients (21.7 %) in the early intervention group and 18 (78.3 %) patients in the observation/delayed intervention group. Ten of 23 patients (43.5 %) required GKRS. Thirteen (56.5 %) did not receive GKRS. None of the patients in the early intervention group or the observation/delayed intervention group had changes in House-Brackmann (HB) Grade either after GKRS or at the end of the study period. CONCLUSIONS: GKRS of residual or recurrent tumor is safe following STR of VS and appears to carry a low risk of worsening facial nerve function when performed for progressive tumor growth.

Colloid Cysts: Evolution of Surgical Approach Preference and Management of Recurrent Cysts.

BACKGROUND: Optimal management of third ventricular colloid cysts remains debated. While microsurgery offers greater resection rates and lower recurrences, endoscopy offers a perceived less invasive option. OBJECTIVE: To describe the evolution of our practice to favor microsurgery and determine the optimal management of recurrent colloid cysts. METHODS: Any patient having undergone surgery for a colloid cyst by the senior author was identified and included in the study cohort. Clinical, radiographic, and operative records were reviewed, with attention paid to those patients requiring recurrent surgery. RESULTS: Thirty-three patients were treated for intracranial colloid cysts between 1995 and 2017. Two patients had initial surgical treatment at an outside institution prior to presentation at our institution. Microsurgery was used in 15/31 initial cases, endoscopy in 13 cases, and ventriculoperitoneal shunting in 3 cases. Between 1995 and 2005, 89% of colloid cysts (8/9 cases) were resected endoscopically, whereas 74% of colloid cysts (14/19 cases) were resected by microsurgery after 2005. Of the 13 patients treated endoscopically, 6 (46%) required surgery for recurrent cysts. Incomplete cyst wall resection at the initial operation increased the recurrence rate to 55%. There were no recurrences in the microsurgery cohort. CONCLUSION: Surgical resection of recurrent colloid cysts should focus on complete removal of the cyst wall to minimize the chance of recurrence. Microsurgery has been shown to provide the highest success rates for cyst wall resection and lowest rates of recurrence and is therefore recommended for patients undergoing surgery for primary and recurrent colloid cysts.

Occipital teratoma in a neonate with CHARGE syndrome: a case report.

INTRODUCTION: Teratomas of the head and neck region are rare lesions, representing just 5% of all congenital teratomas. Usually found in the pineal region orneurohypophysis, teratomas are uncommonly located in the posterior occiput. CASE PRESENTATION: Herein, we present a case of a female neonate born at 37-week gestation with hydrocephalus, an occipital scalp lesion, and several craniofacial abnormalities consistent with a diagnosis of coloboma, heart defect, atresia choanae, retarded growth, genital abnormality, and ear abnormality (CHARGE) syndrome. The occipital scalp lesion was initially thought to be an encephalocele. On day of life 3, the neonate was taken to the operating room for placement of a ventriculoperitoneal shunt and repair of the occipital lesion. Intra-operatively, the lesion resembled a dural-based meningocele; however, during histologic evaluation, it was found to contain tissue derived from all three germ layers and thus, it was determined to be more consistent with a teratoma. CONCLUSION: We hypothesize that a germline mutation in CHD7 or other similar regulatory gene causative of CHARGE syndrome and craniofacial developmental abnormalities may have contributed to the unusual location of the teratoma in this case.

High p16 Expression Is Associated with Malignancy and Shorter Disease-Free Survival Time in Solitary Fibrous Tumor/Hemangiopericytoma.

Objective Solitary fibrous tumors (SFT) and hemangiopericytomas (HPC) are now classified along a single spectrum of fibroblastic mesenchymal tumors with NAB2-STAT6 fusion. This fusion acts as a driver mutation that constitutively activates EGR1, which is known to be involved in the p16 pathway. Overexpression of p16 is associated with malignancy and worse prognosis in multiple mesenchymal tumors. The authors sought to investigate p16 immunoexpression in association with malignancy and prognosis of SFT/HPC tumors. Design Twenty-three SFT/HPC tumors (central nervous system [CNS]: 12, non CNS: 11) diagnosed at our institution from 2002 to 2016 were assigned into 3 grades. Data from microarray immunohistochemistry for STAT6, synaptophysin, CD56, chromogranin, SST2A, EGR1, Ki67, and p16, grade and survival were analyzed. Results CNS SFT/HPCs tend to be malignant (grade 3; 67 vs. 18%, p = 0.036) and more likely to express synaptophysin (33 vs. 0%, p = 0.035) than non CNS tumors. Overexpression of p16 (immunopositivity ≥ 50% tumor cells) was associated with malignant (grade 3) tumors, and has a sensitivity of 70% (7/10), and a specificity of 77% (10/13), as a predictive marker for malignancy. SFT/HPC patients with low p16 expression demonstrated significantly longer disease-free survival time (median survival > 113 months) than those with high p16 expression (median survival = 30 months, p = 0.045). Conclusions SFT/HPCs in the CNS are more likely to be malignant than the tumors in other sites. High p16 expression is also associated with malignancy and shorter disease-free survival time in SFT/HPC tumors in our study cohort. Clinically, p16 overexpression can be used as predictive marker for malignancy and prognosis and a possible therapeutic target.

Orbital reconstruction for tumor-associated proptosis: quantitative analysis of postoperative orbital volume and final eye position.

OBJECTIVE: Surgical resection of sphenoid wing tumors and intraorbital pathology carries the dual goal of appropriately treating the target pathology as well as correcting proptosis. Residual proptosis following surgery can lead to cosmetic and functional disability. The authors sought to quantitatively assess the effect of orbital volume before and after reconstruction to determine the optimal strategy to achieve proptosis correction. METHODS: All surgeries involving orbital wall reconstruction for orbital or intracranial pathology that preoperatively resulted in proptosis between 2007 and 2017 were reviewed. Proptosis was measured by the exophthalmos index (EI): the ratio of the distance of the anterior limit of each globe to a line drawn between the anterior limit of the frontal processes of the zygomas, comparing the pathological eye to the normal eye. Postoperative radiographic measurements were taken at least 60 days after surgery to allow surgical swelling to abate. The orbit contralateral to the pathology was used as an internal control for normal anatomical orbital volume. Cases with preoperative EI < 1.10, orbital exenteration, or enucleation were excluded. RESULTS: Twenty-three patients (16 females and 7 males, with a mean age of 43.6 ± 22.8 years) were treated surgically for tumor-associated proptosis. Nineteen patients harbored meningiomas (11 en-plaque; 8 sphenoid wing), and one patient each harbored an orbital schwannoma, glomangioma, arteriovenous malformation, or cavernous hemangioma. Preoperative EI averaged 1.28 ± 0.10 (range 1.12-1.53). Median time to postoperative imaging was 19 months. Postoperatively, the EI decreased to a mean of 1.07 ± 0.09. Greater increases in size of the reconstructed orbit were positively correlated with greater quantitative reductions in proptosis (p < 0.01). Larger volume of soft tissue pathology was also associated with achieving greater proptosis correction (p < 0.01). Residual exophthalmos (defined as EI > 1.10) was present in 8 patients, while reconstruction in 2 patients resulted in clinically asymptomatic enophthalmos (defined as EI < 0.95). Tumor invasion into the superior orbital fissure sinus was associated with residual proptosis (p = 0.04). CONCLUSIONS: Proptosis associated with intracranial and orbital pathology represents a surgical challenge. The EI is a reliable and quantitative assessment of proptosis. For orbital reconstruction in cases of superior orbital fissure involvement, surgeons should consider rebuilding the orbit at slightly larger than anatomical volume.

Regression of a nonfunctioning pituitary macroadenoma on the CDK4/6 inhibitor palbociclib: case report.

A 71-year-old female patient was referred in 2013 for evaluation of an asymptomatic nonsecreting pituitary adenoma. The adenoma, measuring 13 mm in height by 10 mm in width, was discovered incidentally on imaging in 2012. Biochemical testing demonstrated a nonfunctioning adenoma. Given the relatively small lesion size and the lack of symptoms, observation was preferred over surgical intervention. The patient was monitored with routine MRI, which until 2016 demonstrated minimal growth. In early 2016, the patient developed recurrence of metastatic breast cancer and was treated with palbociclib, a cyclin-dependent kinase (CDK) 4/6 inhibitor. This inhibitor acts on a pathway believed to be involved in pituitary adenoma tumorigenesis. One year after starting palbociclib, routine imaging demonstrated significant regression of her pituitary adenoma. The authors hypothesize that inhibition of the CDK4/6 pathway by palbociclib contributed to adenoma regression in this patient, and that palbociclib may represent a possible adjuvant therapy for the treatment of nonfunctioning pituitary adenomas.

Secondary overtriage of pediatric neurosurgical trauma at a Level I pediatric trauma center.

The authors looked at all of the pediatric patients with a head injury who were transferred from other hospitals to their own over 12 years and tried to identify factors that would allow patients to stay closer to home at their local hospitals and not be transferred. Many patients with isolated, nondisplaced skull fractures or negative CT imaging likely could have avoided transfer. While hospitals should be cautious, this may help families stay closer to home.

Chiari-related scoliosis: a single-center experience with long-term radiographic follow-up and relationship to deformity correction.

OBJECTIVE Previous reports have addressed the short-term response of patients with Chiari-related scoliosis (CRS) to suboccipital decompression and duraplasty (SODD); however, the long-term behavior of the curve has not been well defined. The authors undertook a longitudinal study of a cohort of patients who underwent SODD for CRS to determine whether there are factors related to Chiari malformation (CM) that predict long-term scoliotic curve behavior and need for deformity correction. METHODS The authors retrospectively reviewed cases in which patients underwent SODD for CRS during a 14-year period at a single center. Clinical (age, sex, and associated disorders/syndromes) and radiographic (CM type, tonsillar descent, pBC2 line, clival-axial angle [CXA], syrinx length and level, and initial Cobb angle) information was evaluated to identify associations with the primary outcome: delayed thoracolumbar fusion for progressive scoliosis. RESULTS Twenty-eight patients were identified, but 4 were lost to follow-up and 1 underwent fusion within a year. Among the remaining 23 patients, 11 required fusion surgery at an average of 88.3 ± 15.4 months after SODD, including 7 (30%) who needed fusion more than 5 years after SODD. On univariate analysis, a lower CXA (131.5° ± 4.8° vs 146.5° ± 4.6°, p = 0.034), pBC2 > 9 mm (64% vs 25%, p = 0.06), and higher initial Cobb angle (35.1° ± 3.6° vs 22.8° ± 4.0°, p = 0.035) were associated with the need for thoracolumbar fusion. Multivariable modeling revealed that lower CXA was independently associated with a need for delayed thoracolumbar fusion (OR 1.12, p = 0.0128). CONCLUSIONS This investigation demonstrates the long-term outcome and natural history of CRS after SODD. The durability of the effect of SODD on CRS and curve behavior is poor, with late curve progression occurring in 30% of patients. Factors associated with CRS progression include an initial pBC2 > 9 mm, lower CXA, and higher Cobb angle. Lower CXA was an independent predictor of delayed thoracolumbar fusion. Further study is necessary on a larger cohort of patients to fully elucidate this relationship.

Dorsal Cervical Spinal Cord Herniation Precipitated by Kyphosis Deformity Correction for Spinal Cord Tethering.

INTRODUCTION: Cervical spinal cord herniation is a rare clinical entity. Reported after previous intradural surgery or surgery complicated by durotomy, patients return several months to years later with symptoms of worsening myelopathy. Herein is presented a case of a 51-year-old female patient with spinal cord herniation in the cervical spine after kyphosis deformity correction. CASE DESCRIPTION: A 51-year old female patient presented to the neurosurgery clinic with worsening cervical myelopathy due to cervical spinal cord tethering and adhesions from previous intradural surgery for Chiari malformation. Conservative treatment initially was recommended but ultimately unsuccessful, as her neurologic function continued to deteriorate. Follow-up imaging demonstrated progressive cervical kyphotic deformity with stability of the tethered spinal cord. The patient underwent cervical deformity correction to reduce tension on the spinal cord, after which her neurologic symptoms stabilized and began to improve with physical therapy. Four months after surgery, she returned to clinic with recurrence of cervical myelopathy. Repeat imaging demonstrated herniation of the cervical spinal cord through a dorsal defect, and the patient was treated successfully with a wide cervical duraplasty to recreate an intact thecal sac. CONCLUSIONS: The inability of the spinal cord to compensate for changes in spinal alignment in cases of tethering makes it susceptible to increased pressure and tension at the point of tethering. Caution is urged when attempting deformity correction in the presence of spinal cord tethering, which may limit the capacity of the spinal cord and surrounding tissue to compensate for alterations in spinal alignment.

Flow Diverter Therapy With the Pipeline Embolization Device Is Associated With an Elevated Rate of Delayed Fluid-Attenuated Inversion Recovery Lesions.

BACKGROUND AND PURPOSE: Flow diversion using the Pipeline Embolization Device is reported as a safe treatment of aneurysms. Complete aneurysm occlusion, however, occurs in a delayed fashion with initial persistent filling of the aneurysm dome. We hypothesized that this transflow across metallic struts may be associated with thromboembolic events. METHODS: Forty-one consecutive patients undergoing aneurysm treatment with the Pipeline Embolization Device and a comparison group of 78 Neuroform stent-mediated embolizations were studied. Patients' charts, procedure notes, platelet function, and anticoagulation state were analyzed. Serial magnetic resonance images were assessed for the presence of newly occurring diffusion-weighted imaging and fluid-attenuated inversion recovery (FLAIR) lesions at multiple postprocedure time ranges (average days post procedure [Pipeline Embolization Device/Neuroform]: T1=1, T2=73/107, T3=174, T4=277/335, and T5=409). In addition, diffusion-weighted imaging or FLAIR burden was estimated by lesional diameter summation. RESULTS: Pipeline patients were more likely to have new ipsilateral FLAIR lesions at all time points studied (30.6% versus 7.2% of patients at T=2 and 34.5% versus 6.2% at T=4). The mean FLAIR burden was significantly increased for Pipeline patients (10.1 versus 0.7 mm at T=2 and 8.8 versus 1.9 mm at T=4). Overall 34% (14/41) of Pipeline patients experienced a new FLAIR lesion at anytime when compared with 10% (8/78) of Neuroform stent-coil patients. Postprocedural diffusion-weighted imaging did not predict future FLAIR lesions suggesting a nonprocedural cause. CONCLUSIONS: The Pipeline Embolization Device is associated with increased rate of de novo FLAIR lesions occurring in a delayed fashion and distinct from perioperative diffusion-weighted imaging lesions. The cause and clinical effect of these lesions are unknown and suggest the need for prudent follow-up and evaluation.

Benefit of cone-beam CT angiography in visualizing aneurysm shape and identification of exact rupture site.

While high-resolution cone-beam computational tomographic (CBCT) angiography has gained use in intracranial vascular imaging, digital subtraction angiography (DSA) and 3-dimensional-rotational angiography (3D-RA) remain the preferred acquisition modalities for intracranial aneurysm imaging. This case report highlights the utility of the greater spatial resolution afforded by CBCT for cerebral aneurysm imaging. A 54-year-old man presenting with subarachnoid hemorrhage was confirmed to harbor a ruptured anterior communicating artery aneurysm by conventional angiography. Due to varying contrast opacification captured by different acquisition methods, dramatic aneurysm shape difference was observed between 2- and 3-dimensional-angiographic and CBCT models. The greater resolution of CBCT revealed in an unequivocal fashion the exact site of rupture on the aneurysm dome, visualized as a discrete irregular and elongated bleb that was not seen on either 3D-RA or DSA. High-resolution CBCT visualized the shape of the target aneurysm in greater detail than the more conventional 2D-DSA and 3D-RA, enabling more precise computational fluid dynamics (CFD) simulations. Given that aneurysms most likely change shape either prior to rupture or upon rupture, future studies evaluating fluid dynamics using computer reconstructions should be cognizant of the differences in resolution provided by various imaging modalities.