Multifocal spinal malignant peripheral nerve sheath tumor in an immunocompromised individual: case report and review of literature.

PURPOSE: Primary intraosseous spinal malignant peripheral nerve sheath tumor (MPNST) is exceedingly rare. MPNST with multifocal origin has been described to occur in the extremities. Such a lesion has not been described to occur in the spine. We describe a case of multifocal spinal MPNST and to review the literature relevant to this rare entity and its management. METHODS: A 40-year-old immunodeficient patient presented with rapidly progressive paraparesis and mid back ache. RESULTS: Despite aggressive surgical decompression, he developed multiple metastases 3 months after surgery. However, he remained stable for 1 year without any adjuvant therapy. Presently, he has received palliative radiotherapy for spinal recurrence and cerebral metastasis. CONCLUSION: Multifocal spinal MPNST is a rare lesion. In this instance, the multifocality of the disease and its odd location could be attributed to the immunodeficiency state. The prolonged survival could be due to an improvement in his immune status due to HAART.

Challenges in management of Candida auris meningitis secondary to infected ventriculoperitoneal shunt - A case report.

Meningitis in patients with ventriculo-peritoneal shunt (VP shunt) caused by various species of Candida have been widely described in literature. However, reports describing Candida auris as a cause of meningitis is limited. In this case report we describe a case of multidrug resistant Candida auris meningitis secondary to VP shunt infection successfully treated with intrathecal amphotericin B deoxycholate and intravenous liposomal amphotericin B. This is the second case report of successful treatment of Candida auris meningitis from India. More literature regarding the use of intrathecal/intraventricular echinocandins including optimal dosing and duration of therapy is needed.

Idiopathic intraventricular aneurysm presenting with intraventricular hemorrhage: case report and review of the literature.

Intraventricular hemorrhage (IVH) is a relatively commonly encountered problem in neurosurgical practice. The underlying causes could include hypertension, arteriovenous malformations (AVM), angiomas, trauma, tumors, aneurysms and moyamoya disease. Truly idiopathic intraventricular aneurysms (IVA) are rare. A high index of suspicion needs to be maintained since, with the appropriate treatment, the outcome is generally good. We report the case of a 14-year-old boy who presented with sudden onset headache and vomiting. CT angiogram pointed to the possibility of an IVA. This was confirmed by MRI with three-dimensional constructive interference in steady state (CISS-3D) sequences and digital subtraction angiography. The patient underwent microsurgical excision of the aneurysm and is doing well on follow-up. Idiopathic IVA can present with IVH at any age. MRI with CISS-3D and MR angiography would be the imaging modality of choice since it can also rule out other causes of intraventricular bleeding such as tumors and AVM. These lesions could be managed effectively by microsurgical excision/clipping. The necessity of investigating every patient who presents with IVH is debatable.

Microsurgical Excision of Vestibular Schwannoma with Facial Nerve Preservation: A Retrosigmoid Approach.

Background: This video highlights the standard surgical steps of retrosigmoid craniotomy and excision of a moderately sized vestibular schwannoma with facial nerve preservation, with basic technical nuances to guide young neurosurgeons. Objective: A systematic step-by-step approach to microsurgical dissection and removal of vestibular schwannoma that achieves gross total excision with good facial nerve preservation. Methods: The video starts with a note on clinical presentation of a 45-year-old male with unilateral sensorineural hearing loss and headache with MRI brain showing a 3.8 × 3.0 cm schwannoma arising from the left vestibular nerve. The surgery was performed in lateral position with neuromonitoring, using a conventional retrosigmoid craniotomy. The essential surgical steps of arachnoid opening and extra capsular dissection, identification and preservation of facial nerve, and drilling of the internal acoustic meatus (IAM) are demonstrated step by step achieving a gross total excision. Results: A systematic and meticulous approach to microsurgical excision of vestibular schwannoma is pivotal in the preservation of facial nerve and in preventing damage to surrounding neurovascular structures. Conclusion: Adopting microsurgical techniques in vestibular schwannoma with intraoperative facial nerve monitoring aids in achieving good functional outcome and tumor control.

Simplifying the Surgical Strategy for Excising Medial Sphenoid Wing Meningiomas: A Stepwise Approach.

Background: Medial sphenoid wing meningiomas constitute 15%-20% of all intracranial meningiomas. These lesions have a propensity to encase the vessels of the circle of Willis and the surrounding cranial nerves. Thus, radical excision is a difficult proposition. Objectives: In this paper, we analyzed our series of sphenoid wing meningiomas. We describe our surgical strategy, which was based on zone-wise dissection of the tumor. We describe the complications and outcomes of surgery. Materials and Methods: This case series is a retrospective analysis of a single surgeon series of medial sphenoid wing meningiomas operated over a 13-year period. Clinical, radiographic, and outcome variables were studied. The surgical videos were analyzed in detail. The meningioma and its extensions were divided into several zones and a zone-wise strategy for tumor excision was evolved. Results: Twenty-four patients with medial sphenoid wing meningiomas were operated. In 14 patients, Simpson grade 3 excision could be achieved; 5 patients had Simpson grade 4 and 1 patient, grade 5 excision. Four (of 24 patients, 16.7%) had vessel injuries. Conclusions: Medial sphenoid wing meningiomas are difficult lesions to excise radically. Close follow-up of residual lesions (especially if attached to the basal dura) is warranted. Additional modalities of treatment like radiosurgery may be required in case of any progression and for higher-grade lesions.

Analysis of Factors Associated with Long-Term Survival in Patients with Glioblastoma.

BACKGROUND: Some patients with glioblastoma multiforme (GBM) survive 3-5 years (or longer) after diagnosis. The goal of this study was to identify differences between the long-term survivors (LTS) and those who had a shorter overall survival (non-LTS groups). METHODS: This study was a retrospective analysis of prospectively maintained surgical databases. All patients who underwent safe maximal resection for GBM were included. Demographic, clinical, radiologic, and pathologic data were obtained from electronic medical records. Values of the biomarkers of systemic inflammation were computed from the preoperative hemogram reports. Patients with an overall survival (OS) ≥36 months were defined as the LTS group and were compared with the non-LTS groups (OS<36 months). RESULTS: Patients in the LTS group were younger, had a better baseline performance status, and were more likely to have undergone near- or gross-total resection. LTS was associated with lower Ki67 labeling, MGMT methylation, IDH mutation, and lack of p53 overexpression. Several novel findings were generated by this study. A longer pretreatment duration of symptoms was associated with a longer OS. Higher pretreatment levels of the absolute neutrophil count, neutrophil-lymphocyte ratio, monocyte-lymphocyte ratio, derived neutrophil-lymphocyte ratio and systemic index of inflammation, and lower levels of the absolute eosinophil count and eosinophil-lymphocyte ratio all correlated with a shorter OS. CONCLUSIONS: Several differences were identified between the LTS and non-LTS groups. These differences will likely be incorporated into future prognostic models. They may also aid in differentiation between recurrent disease and treatment-related changes.

Supratentorial enterogenous cyst: a report of two cases and review of literature.

Enterogenous cyst is extremely rare in the supratentorial compartment. Two adult patients with histologically variefied supratentorial entrogenous cyst are described. Light microscopy and immunohistochemistry examination revealed the endodermal origin of the cyst. Enterogenous cyst should be considered in the differential diagnosis of non-enhancing cyst in the supratentorial compartment. Total excision of the cyst wall should be done wherever feasible. Preventing spillage of the cyst contents during surgery is mandatory to avoid postoperative intractable seizures and craniospinal dissemination.

Awake microvascular decompression with fat-teflon sandwich technique: Clinical implications of a novel approach for cranial nerve neuralgias.

Re-appearance of trigeminal neuralgia (TN) pain following microvascular decompression (MVD) is a challenging issue. A selective ablation with MVD provides the best response in such recurrences. The absence of intra-operative indicator for immediate correction of sub-optimal decompression is the primary factor for failure. We analysed the effectiveness and safety of awake MVD in minimizing failure, by tailoring the procedure according to intra-operative response with re-exploration or additional procedure like internal neurolysis in the same setting, especially in patients without vascular compression and those unfit for General Anesthesia (GA). The prospective study from June 2016 to June 2017 includes one glossopharyngeal neuralgia (GPN) and 6 trigeminal neuralgia (TN). Five cases responded with immediate complete pain relief but in 2 cases, incomplete pain relief resulted in alteration of intraoperative decision. In one case, a partial pain relief, mandated an additional internal neurolysis in the same setting, resulting in complete pain relief while in the other, re-exploration revealed a hidden venous conflict, not identified on MRI following which an additional IN was performed. All cases were followed up with BNI PIS for a minimum of one year without recurrence. Awake MVD is safe and reliable intraoperative neurophysiological prognostic marker of immediate pain relief and provides a window for an immediate correction of sub-optimal decompression with Internal Neurolysis when needed, in the same setting, especially in neuroimaging negative and elderly cases unfit for GA. It has the potential to reduce the rate of re-intervention and increase the overall effectiveness of MVD by specifically ameliorating the pain burden and quality of life.

Retinal nerve fiber layer thickness analysis in cases of papilledema using optical coherence tomography ­ A case control study.

BACKGROUND: Papilledema is defined as an optic disk swelling that is secondary to elevated intracranial pressure. Early diagnosis of papilledema can help in early intervention thus preventing visual loss and even death. Optical coherence tomography (OCT) is a non-invasive imaging technique which can detect and quantify diffuse thickening of the retinal nerve fiber layer (RNFL) in eyes with optic disk edema. AIMS: To assess the difference in the RNFL thickness in patients with papilledema from controls using OCT and to correlate the RNFL thickness with the degree of papilledema. SETTING AND DESIGN: A case control study was conducted from August 2011 to July 2013 in a tertiary care medical college of south India. METHODS AND MATERIALS: All adult patients diagnosed or suspected to have papilledema were included in the study. Disk photographs were graded according to modified Frisen criteria. Fast RNFL protocol on time-domain OCT was used. Cases and controls were compared. STATISTICAL ANALYSIS: RNFL thickness was compared using an independent samples t-test. Correlation between RNFL thickness and modified Frisen scale of papilledema was done using Spearman correlation. GraphPad InStat 3 version was used. RESULTS: A total of 100 cases and 126 controls were studied. Statistically significant thickening of retinal nerve fiber layer (RNFL) was seen in all quadrants in patients with papilledema as compared to controls. A positive correlation was found between Frisen grading of papilledema RNFL thickness measurements. CONCLUSIONS: RNFL thickening was mainly in the inferior and superior peripapillary region and was greater in higher grades of papilledema. A strong positive correlation was found between RNFL thickness and the Frisen scale for grading of papilledema. OCT can be included as a routine non-invasive quantitative tool for detection of early papilledema.

Atlantoaxial dislocation in a patient with nonsyndromic symmetrical dwarfism: Report of a rare case.

Congenital anomalies of the craniovertebral junction (CVJ) are complex developmental defects. We describe a patient with atlantoaxial dislocation (AAD) and short stature whose morphopathologydid not fit into any of the previously described syndromic constellations. The patient underwent a reduction of the AAD followed by fixation with C1-C2 transarticular screws. Although numerous syndromes have been linked to both dwarfism and craniovertebral junction anomalies, this patient did not fit into any of these patterns. It is possible that this may be one of the many as yet unrecognized patterns of congenital anomalies.

Actinomycotic osteomyelitis of the cranial vault presenting with headache: an unusual presentation.

A case of left parietal calvarial actinomycotic osteomyelitis in a young woman is described. She had no predisposing illnesses. She had delivered a live child at term and presented in the puerperal period. No extracranial focus of infection was identified. She responded well to a combination of surgery and medical therapy and had an excellent outcome. The authors emphasise the importance of establishing a histopathological diagnosis since radiological signs are non-specific and unreliable.

Vascular endothelial growth factor expression and angiogenesis in various grades and subtypes of meningioma.

BACKGROUND: Vascular endothelial growth factor (VEGF) expression has been extensively studied in astrocytoma, whereas relatively less literature exists on VEGF expression in meningioma. MATERIALS AND METHODS: Patients operated for meningioma from 2006 to 2011 (n = 46) were included. Tumor was subtyped and graded as per WHO grading. Immunohistochemistry was performed for MIB labeling index, VEGF, and CD 34 staining. The patterns of VEGF expression in various histological subtypes and grades and its correlation with microvascular density were analyzed. RESULTS: This series consisted of 40 Grade I meningioma, 4 Grade II tumors, and 2 Grade III tumors. While 14 (30.4%) tumors showed no staining with VEGF antibody, 32 (69.6%) were positive for VEGF. Sixty five percent of Grade I tumors showed VEGF positivity, while 100% of Grade II and Grade III tumors were VEGF positive (P = 0.157). The mean microvascular density in VEGF-negative tumors was 9.00, while that of VEGF-positive tumors was 17.81(P = 0.013). There was a gradual increase in microvascular density from tumors which are negative for VEGF to tumors which expressed moderate to strong VEGF, the difference being statistically significant (P = 0.009). CONCLUSIONS: VEGF expression correlated with the microvascular density in meningioma irrespective of tumor grade, with a gradual increase in microvascular density in relation to the VEGF score.

Spinal aneurysms: clinicoradiological features and management paradigms.

OBJECT: Spinal aneurysms (SAs) are rare lesions. The clinicoradiological features and the exact degree of their association with comorbid conditions such as arteriovenous malformations (AVMs) and coarctation of the aorta have not been definitively described. The ideal management paradigm has not been established. The authors reviewed literature to determine the clinical patterns of presentation, management, and outcome of spinal aneurysms. METHODS: A systematic review of literature was performed using 23 separate strings. A total of 10,190 papers were screened to identify 87 papers that met the inclusion criteria. A total of 123 SAs could be included for analysis. RESULTS: The mean age of patients at presentation was 38 years; 10% of patients were aged less than 10 years and nearly 50% were greater than 38 years. Spinal aneurysms can be divided into 2 groups: those associated with AVMs (SA-AVMs, or Type 1 SAs) and those with isolated aneurysms (iSAs, or Type 2 SAs). Patients with Type 2 SAs were older and more likely to present with bleeding than those with Type 1 SAs. The acute syndromes can be divided into 3 groups of patients: those with spinal syndrome, those with cranial/craniospinal syndrome, and those with nonspecific presentation. Overall, 32.6% presented with angiography-negative cranial subarachnoid hemorrhage (SAH). Presentation with evidence of cord dysfunction (myelopathy/weakness/sensory loss/bladder involvement) correlated with poor outcome, as did presentation with hemorrhage and association with other comorbid conditions. Surgery and endovascular therapy both led to comparable rates of complete aneurysm obliteration for Type 2 SAs, whereas for the AVM-associated Type 1 SAs, surgery led to better rates of lesion obliteration. The authors propose a classification scheme for spinal aneurysms based on whether the lesion is solitary or is associated with a coexistent spinal AVM; this would also imply that the ideal therapy for the aneurysm would differ based on this association. CONCLUSIONS: The clinical and radiological patterns that influence outcome are distinct for Type 1 and Type 2 SAs. The ideal treatment for Type 1 SAs appears to be excision, whereas surgery and endovascular therapy were equally effective for Type 2 SAs.

Cerebral revascularization with superficial temporal - middle cerebral artery anastomosis for complete carotid occlusion: An emerging modality for preventing recurrent stroke.

Complete long segment carotid occlusion presents a treatment challenge. These patients cannot be managed adequately by endarterectomy or stenting. Despite best medical management, many continue to develop recurrent strokes. In this select group of patients, there may be role for flow augmentation techniques like superficial temporal-middle cerebral artery bypass. We report a patient who was thus successfully treated and remains asymptomatic. The relevant literature is reviewed.