Supratentorial extra-ventricular ependymoma as a mass lesion in a child: report and literature review.

Supratentorial extra-ventricular ependymoma (SEE) are extremely rare in pediatric population and have varied presentation based on size, location, epicentre and compression on neurovascular structure. The authors report a 7-year-old girl presenting with seizure, who had a lobar SEE on MRI scan, successfully treated by microsurgical resection and adjuvant therapy.

Dorsal bony spur in pediatric split cord malformations: eight-year experience from a tertiary care hospital.

INTRODUCTION: Dorsal spurs in Type I split cord malformations (SCM-I) are infrequent findings. The pathogenesis of the same is debatable. The objective of this study is to analyze our experience with SCM-I patients having dorsal bony spurs. METHODOLOGY: Retrospective analysis of SCM patients operated from 2010 to 2017 was performed. Their demographic profile, clinic-radiological features, operative findings, and outcome following surgery were recorded. RESULTS: Twenty-four cases of Type I SCM harboring dorsal bony spurs were identified with mean age of 4.96 years. The commonest split site was lumbar, documented in 62.5%. Scoliosis was observed in 58.3%. Pre-operative neurological deficits were seen in 66.6% cases with asymmetric weakness of limbs seen in 16.6%. There was no new neurological deficit observed post-operatively. CONCLUSIONS: This is the largest series of dorsal spurs occurring in SCM, reported in literature so far. Meticulous pre-operative evaluation and imaging are important to identify dorsal spurs for appropriate management and good clinical outcome. Differentiating dorsal spur from ventral spur is important as it has a bearing on surgical approach.

WNT-activated medulloblastoma with melanotic and myogenic differentiation: Report of a rare case.

Medulloblastoma (MB) with melanotic and myogenic differentiation, previously known as melanotic medullomyoblastoma, is an extremely rare histological variant of MB showing melanocytic as well as skeletal muscle differentiation. Only 10 cases of this rare tumor have been reported in the literature to date. We report this case of a 2-year-old male child who presented with a midline cerebellar mass, which on histopathological examination showed classic MB intermixed with cells containing melanin pigment, along with rhabdomyoblasts, spindle cells and occasional strap cells, which corresponded to WNT subgroup on molecular classification. The cell of origin of this MB variant is likely to be neural crest-derived stem cells which are capable of multilineage differentiation. Significant findings from previous reports and important differential diagnoses are discussed. Documentation of these tumors is important to characterize the clinical behaviour and to identify distinct genetic features, if any.

C11orf95-RELA fusion present in a primary intracranial extra-axial ependymoma: Report of a case with literature review.

Ependymomas are gliomas that recapitulate the ependymal cells microscopically and ultrastructurally. They commonly occur along the ventricular surfaces and central canal of the brain and spinal cord. Intracranial extra-axial ependymoma (IEAE) is a rare entity and is commonly misdiagnosed clinically and radiologically as a meningioma. The histogenesis of such IEAEs is obscure. A novel recurrent oncogenic fusion involving the C11orf95 and RELA genes was recently described in supratentorial ependymomas. A 9-year-old girl presented with a dural based parafalcine mass that, in addition to exhibiting classical immunohistochemical features of an ependymoma, also demonstrated C11orf95-RELA fusion, characteristic of supratentorial ependymomas. We suggest that IEAEs share their histogenesis with their intra-axial counterparts, arising either from dural extension of subcortical, subependymal rests or directly from ectopic dural rests.

Bedside computed tomography in traumatic brain injury: Experience of 10,000 consecutive cases in neurosurgery at a level 1 trauma center in India.

INTRODUCTION: Patients with traumatic brain injury (TBI) need frequent computed tomography (CT) of the head for assessment and management. In view of the associated polytrauma, hemodynamic instability, and various in-dwelling catheters and tubes, shifting of patients for CT scans may be difficult. AIMS AND OBJECTIVES: To assess the role of mobile CT (Ceretom®; NeuroLogica Corporation, Boston, MA, USA) in a trauma center with respect to patient management. MATERIALS AND METHODS: In this retrospective study over 67 months (June 2009 to January 2015), the number of CT scans done, the time taken for CT and downtime were evaluated. Also, for the first 1000 mobile CT scans, the clinical and radiological records of all patients with TBI who underwent imaging using the mobile CT scanner in the intensive care units (ICUs) were analyzed. OBSERVATIONS AND RESULTS: A total of 10,000 mobile CT scans were done on the mobile CT scanner till January 5, 2015. Of the first 1000 patients evaluated, 75.3% had severe TBI, 15.1% had moderate TBI, and 9.6% had mild TBI. 78.1% patients were on ventilator, with 80.2% requiring sedation and 8.4%, an inotropic support. An in situ intracranial pressure monitoring was present in 21.1% of patients. In all, 12.4% of patients had long-bone fractures requiring skeletal traction; and, the tube thoracostomy was in-situ in 7.4%. No adverse events related to line malfunction/pullout occurred. The mean time for the performance of imaging using the mobile CT scan was 11.6 minutes compared with 47.8 minutes when patients were shifted to a conventional CT scan suite. The machine was nonfunctional 94 times, with an average downtime of 4.2 hours (range 2-72 hours). The life-cycle cost per mobile CT scan was Rs. 1340. CONCLUSIONS: A mobile CT has considerably changed the management response time in the neurosurgical intensive care unit (ICU) setup and decreased patient transfer times and the associated complications. Inclusion of a mobile CT scanner in the armamentarium of a neurosurgeon as a "bedside tool" can dramatically change decision making and the response time. It should be considered as the standard of care in any large-volume emergency department or neurosurgical facility.

Cerebellar Liponeurocytoma, an Unusual Tumor of the Central Nervous System--Ultrastructural Examination.

Cerebellar liponeurocytoma is a rare tumor of the central nervous system which shows neuronal and variable astrocytic differentiation, along with foci of lipomatous differentiation. It is usually located in the cerebellum, and may be mistaken for medulloblastoma with lipidized cells or lipomatous ependymoma. Histopathological examination, supplemented by immunohistochemistry and electron microscopy, is required to distinguish between these entities. This 35-year-old male presented with vomiting and headache for three months, followed by gait imbalance. Neurological examination showed positive cerebellar signs with ataxic gait. Magnetic resonance imaging showed a lesion measuring 4.4 cm× 4.3 cm× 3.9 cm involving the cerebellum. The patient underwent midline suboccipital craniotomy to excise the tumor. Histopathological examination showed a circumscribed, cellular tumor composed of round to polygonal cells with moderate cytoplasm and minimal pleomorphism. Clear intracytoplasmic vacuoles were seen within the tumor cells. These tumor cells were immunopositive for synaptophysin, NSE, and MAP-2, confirming their neurocytic origin. On ultrastructural examination, lipid vacuoles as well as dense-core neurosecretory granules were identified within these neurocytic cells, confirming the diagnosis of liponeurocytoma. No cilia, microvilli, or gap junctions were identified in the tumor cells, ruling out the possibility of lipomatous ependymoma. The differentiation of liponeurocytoma from its morphological mimics is imperative, as their treatment differs drastically. The role of electron microscopy is extremely important in this differential diagnosis.

Surgical management of odontoid fractures at level one trauma center: a single-center series of 142 cases.

INTRODUCTION: Odontoid fractures constitute 9-20% of all adult cervical spine fractures. The present study was carried out to focus on the nuances involved in the surgical management of odontoid fractures. MATERIALS AND METHODS: Patients with an odontoid fracture, admitted for surgical stabilization, between January 2008 and March 2014, were included in the study. RESULTS: Among 142 patients [127 male and 15 female patients; median age: 28 years range 4-75 years], type II odontoid fractures were present in 111, type IIA fractures in 8, and type III fractures in 23 patients. 58.5% patients had been involved in a motor vehicular accident while 38.7% had sustained a fall. Eighty-five patients (59.9%) with a well-reduced fracture and an intact transverse ligament underwent anterior odontoid screw (OS) placement; the other 57 patients (40.1%) underwent posterior fixation (PF). The mean follow-up duration was 22 months (range: 6 months-5.4 years). OS placement was successful in 82 patients (96.5%) with a fusion rate of 95% (95.8% in type II, 100% in type III, and 75% in type IIA odontoid fractures). The procedure-related morbidity was 11.7%. One patient died of sub-arachnoid hemorrhage (SAH) that occurred during OS placement. The PF procedures had a better fusion rate (96.5%). The latter patients, however, had significant restriction of their neck movements and an overall morbidity of 8.7%. The revision surgery rates after OS placement and PF fixation were 7% and 3.5%, respectively. CONCLUSIONS: Anterior OS fixation shows excellent fusion rates and should be the first-line management in reduced/non-displaced acute type II (including type IIA) and high type III odontoid fractures as it preserves cervical motion. PF, that has also been associated with an excellent fusion rate, should be reserved for patients where OS fixation has either failed or has not been feasible.

Cisternostomy as an Adjuvant or Standalone Approach for Management of Traumatic Brain Injury: A Systematic Review and Network Meta-Analysis.

BACKGROUND: Traumatic brain injury (TBI) is a leading cause of morbidity in all age groups worldwide. Decompressive craniectomy (DC) is a salvage procedure in patients with TBI. The outcome and quality of life following DC is questionable. Basal cisternostomy (BC) has been proposed to reduce edema and lead to brain relaxation. It was initially used as an adjunct in TBI patients, thereby improving outcomes. With gaining popularity among neurosurgeons, BC was used as a standalone approach in TBI patients. This network meta-analysis aims to analyze the role of BC either as an adjunct or as a standalone approach in managing TBI patients. METHODS: A comprehensive search of electronic databases (PubMed and SCOPUS) was performed using the search strategy using the field terms and medical subheading terms (MeSH Terms) to retrieve studies describing the role of BC in patients with TBI either as an adjunct with DC or standalone treatment and their outcome. RESULTS: Thirty-one articles were selected for full-text review and 18 articles were selected for the final analysis. BC alone group was found to have minimum in-hospital mortality (odds ratio [OR], 0.348; 95% credible interval [CrI], 0.254 to 0.477) followed by DC combined with BC group (OR, 0.645; 95% CrI, 0.476 to 0.875). DC combined with the BC group was found to have a minimum duration of mechanical ventilation (OR, 0.114; 95% CrI, 0.005 to 2.451) followed by the BC alone group (OR, 0.604; 95% CrI, 0.024 to 15.346). DC combined with the BC group were found to have the maximum Glasgow Outcome Scale score (OR, 1.661; 95% CrI, 0.907 to 3.041) followed by the BC alone group (OR, 1.553; 95% CrI, 0.907 to 3.041). CONCLUSIONS: Our analysis showed that BC alone was associated with lower in-hospital mortality rates in TBI patients. DC with BC had decreased the requirement of mechanical ventilation. However, larger multi-centric studies from other parts of the world are required to confirm these findings.

"Rabbit ear" scalp deformity caused by massive subdural effusion in infant following bilateral bur-hole drainage failure: successfully managed with subduro-peritoneal shunt.

INTRODUCTION: Subdural effusion in an infant is a rare clinical scenario which may be secondary to a variety of etiologies. Massive subdural effusion is an extremely rare complication of head injury. CASE REPORT: Authors report a rare case of progressive massive subdural effusion which, despite bilateral bur-hole placement and drainage, failed and presented with visual deterioration and massive bulge of the scalp at bur-hole sites, producing a rabbit ear appearance in a 10-month-old infant. Ultimately, cystoperitoneal shunt was carried out in a desperate attempt to prevent impending rupture of scalp sutures at sites of previous bur-hole placement. This was followed by not only complete resolution of hygroma but also visual recovery. The patient is doing well at 6 months following shunt, regaining normal vision and appropriate developmental milestones. A MRI scan of the brain was carried out at last follow-up, which revealed mild ventriculomegaly with the rest of the brain being unremarkable, and subduro-peritoneal shunt in situ. Such a case has not been reported in the literature till date. DISCUSSION: Subdural effusion usually runs a self-limiting course. Though neurosurgical intervention is occasionally needed, different methods of surgical procedure for management include bur hole alone, bur holes with subdural drain placement, twist drill craniotomy with drain, and even craniotomy. Various methods of management are discussed along with a review of pertinent literature.

O-Arm Assisted Anterior Odontoid Screw Fixation in Type II and Rostral Type III Odontoid Fractures: Single Center Surgical Series of 50 Patients.

Background: Accuracy of screw placement is one of the important factors necessary for adequate union in odontoid fractures with malposition rates as high as 27.2% with standard techniques. Objective: To evaluate efficacy of intraoperative O-arm assistance in improving accuracy of anterior odontoid screw placement and clinco-radiological outcome in type II and III odontoid fractures. Material and Methods: In this retrospective study, surgery consisted of anterior odontoid screw fixation under intraoperative O-arm assistance over 5 years. Demographical, clinical, radiological, operative details and postoperative events were retrieved from hospital database and evaluated for fusion and surgical outcome. Results: 50 patients (Mean age 34.6 years, SD 14.10, range: 7-70 years; 44 males and 6 females) with Type II and Type III odontoid fracture underwent O-arm assisted anterior screw placement. The mean interval between injury and surgery was 12 days (range 1-65 days). Mean operating time was 132.2 min ± SD 33.56 with average blood loss of 93 ml. ±SD 61.46. With our technique, accurate screw placement was achieved in 100% patients. At the mean follow-up of 26.4 month (SD13.75), overall acceptable fusion rate was 97.8% with non-union in 2.2% patients. Morbidity occurred in two patients; one patient developed fixation failure while other patient had nonunion which was managed with posterior C1-C2 arthrodesis. We had surgical mortality in one patient due to SAH. So overall our procedure was successful in 94% patients and among patients whose follow-up was available, acceptable fusion rates of 97.8% were achieved. Conclusion: We conclude that use of intraoperative three-dimensional imaging using O-arm for anterior odontoid screw fixation improves accuracy and leads to improved radiological and clinical outcomes. It further enables us to extend the indications of odontoid screw fixation to selected complex Type II and rostral Type III odontoid fractures.

Anterior Odontoid Screw Fixation for Pediatric and Adolescent Odontoid Fractures: Single-Center Experience Over a Decade.

OBJECTIVE: Management of pediatric odontoid fractures is tricky and controversial. This study will enrich world literature with intricacies of anterior odontoid screw (OS) fixation in the pediatric population learned over the last decade. METHODS: In this retrospective study, all patients with pediatric odontoid fracture who underwent anterior odontoid screw fixation from January 2010 to December 2019 were included and evaluated for surgical outcome. RESULTS: Thirteen patients were included in this study (mean age, 15 years; range, 6-18 years; male/female, 11:2; type II, 10; type IIA, 1; type III, 2). Common causes of injury were motor vehicle accidents (61.5%) followed by fall from height (38.5%) and all were acute fractures (2-30 days). Five patients had neurologic deficits. Accurate placement of screw was achieved in 92.3% of patients, including all 9 patients who used intraoperative O-arm. K wire migration during bicortical drilling resulted in neurovascular injury, with 1 mortality (7.7%). The remaining 12 patients were available for follow-up (mean, 36 months; range, 20-72 months) and all had preservation of neck movements. Successful OS fixation was achieved in 84.6% of patients, including 1 patient (7.7%) who had a fibrous union. One patient (8.3%) had nonunion because of migration of the screw head in the C2 body. CONCLUSIONS: Anterior odontoid screw fixation in the pediatric population provides good functional outcomes with instant fixation by direct osteosynthesis. However, the surgeon should be meticulous in the surgical approach and should achieve a lag effect. The surgeon should stop after engaging the outer cortex of the odontoid peg with K wire to avoid cranial migration. Intraoperative O-arm guidance is useful.

External Occipital Protuberance Projecting as Downward Curved Horn Presenting with Intractable Occipital Pain: Report of a First Case.

External occipital protuberance is normal anatomical entity, rarely it may show hyperostosis and may get prominent and causing pain and examination reveals presence of tender bony swelling. However, such occurrence is extremely uncommon.

Adult Achondroplasia Patient with Extensive Long Segment Severe C1 to C6 Segment Spinal Canal Stenosis: Unique Presentation.

Achondroplasia represents the common variety of skeletal dysplasia causing short-limb dwarfism. It may be associated with congenial craniovertebral junction anomaly, including atlantoaxial dislocation, Os odointoideum or basilar invagination or acquired as a result of the previous foramen magnum region surgery. However, long segment cervical compression from the first cervical vertebra region to sixth cervical region, such extensive compression producing myelopathy is not reported, and current case represents first case in the western literature. He was managed surgically with a good outcome. Perioperative management along with brief review of pertinent literature along with the management of such case is discussed in brief. Anesthetic management of such patient with coexisting extensive cervical compression presents unique challenges in view of the multisystem involvement along with preexisting anatomical and physiological constraints.

Persistent Enlarged Occipital Sinus with Absent Unilateral Transverse Sinus.

The occipital sinus may occasionally remain patent, but the incidence is extremely low and observed in less than 10% of cases. A persistent patent occipital sinus (POS) may be associated with other venous sinus abnormality. The absence of transverse sinus in association with POS is an extremely rare condition and not reported yet. The neuroradiologist, neurosurgeons, otolaryngologist, and neurologist must be aware of the possible existence of POS and other associated venous sinus anomaly, as its warrants very crucial modification of surgical planning, selection of appropriate approaches, and, additionally, may also critically limit the extent of surgical exposure of target, and may hinder intended extent of surgical excision of tumor and associated possibility of injury to POS, which may produce catastrophic hemorrhage, brain swelling, and neurosurgical morbidity. The authors report a 35-year-old male who underwent suboccipital craniotomy for right-side giant acoustic schwannoma. Following the raising bone flap, a markedly prominent, turgid, occipital sinus was observed, not placed exactly in the midline but deviated to the right side, causing further restraining of dural opening. Surgical nuances and intraoperative difficulty encountered along with pertinent literature is reviewed briefly.

Management and Outcome Analysis of Conus and Filum ependymoma: A Tertiary Center Study.

BACKGROUND: Spinal ependymomas constitute approximately 2%-8% of primary adult central nervous system tumors. Authors analyzed demographic, clinical, radiological, surgical, and histopathological factors which correlated with the postoperative neurological outcome of patients who underwent surgery for conus and filum ependymoma (CFE). MATERIALS AND METHODS: A retrospective analysis of 31 patients regarding clinical feature, imaging study, surgical management, and McCormick grading system for assessing functional neurological status was carried out, who underwent surgical management for CFE between January 2009 and April 2014. Final neurological outcome at follow-up period was correlated with various factors in search to find out probable prognostic factors affecting final neurological outcome following surgical management. RESULTS: The myxopapillary ependymoma was observed in 55% of cases (n = 17), while 39% cases (n = 12) had Grade II ependymoma and rest 6% (n = 2) cases had anaplastic ependymomas. The mean age was 30 years (range 7-60 years) with male to female ratio of 1:0.82. Patients predominantly presented with pain (80.65%); mean duration of symptoms was 28.61 months. Only, the preoperative McCormick grade was found to be the statistically significant prognostic factor (P = 0.045), affecting neurological outcome however, the age, sex, duration of symptoms, location of the tumor, extent of the tumor, extradural spread, degree of surgical excision, vascularity of tumor, and histopathological World Health Organization grades were not found to be significant prognostic factors in the current study. CONCLUSION: The preoperative McCormick score was found to be the only statistically significant factor predicting the functional and neurological outcome after surgery, so surgical treatment should be offered early in the course of the disease to provide chance of preservation and good neurological recovery.

Neurogenic Fever in Severe Traumatic Brain Injury Treated with Propranolol: A Case Report.

The causes of intractable fever in severe traumatic brain injury (TBI) patients can be diverse. Neurogenic fever (NF) which is a rare entity can develop due to autonomic dysregulation in the absence of infection or any other cause of fever. It manifests as fever, tachycardia, paroxysmal hypertension, dilated pupils, tachypnea, and extensor posturing in cases of severe TBI, brain neoplasms or brain haemorrhage. We found propranolol to be effective in controlling many of the manifestations of neurogenic fever in our patients with severe TBI. Fever in severe TBI patients is not an uncommon phenomenon, but when intractable with negative fever workup, a central cause should be considered. Propranolol is deemed as one of the most efficacious drugs for managing NF due to dysautonomia. We want to apprise the readers about this entity and its treatment with beta-blockers.