Dural sinus malformation presenting with proptosis: report and review of a rare entity.

Dural sinus malformation (DSM) is a rare vascular malformation characterized by the dilatation of a dural venous sinus with or without an anomalous jugular bulb. Its presentation with venous-reflux-related secondary proptosis is anecdotal, with only six such cases reported so far. We report a 17-month-old boy who presented with a progressive proptosis of the right eye secondary to a DSM of the transverse sinus and torcula. Following endovascular embolization of the arterio-venous fistula, complete thrombosis of the venous lake and improvement in proptosis was noted at 6-month follow-up. Prognosis of this rare malformation is variable and dependent on specific angio-architectural features.

Primary intracranial sarcoma masquerading as a chronic subdural haematoma: illustrative case and review of an unusual phenomenon.

It is very unusual for a primary intracranial malignancy to present as a chronic subdural hematoma. This case report describes one such case in a 3-year-old girl who presented with raised intracranial pressure following a mild head injury. Imaging of her brain revealed bilateral chronic subdural hematomas with an enhancing subdural mass and multiple nodular lesions infiltrating the brain parenchyma. She underwent a craniotomy, drainage of the subdural collections and resection of the subdural mass. Histopathology revealed an undifferentiated sarcoma, and she was referred for adjuvant therapy. This case underlines the importance of evaluating paediatric subdural collections for a possible underlying malignancy, even in the background of a preceding head injury. The cause-effect relationship of sarcomas with subdural collections remains unclear because of the scarcity of available literature on the subject.

CNS paraganglioma: 15-year experience in a tertiary care hospital with literature review.

Paraganglioma that involves the CNS may mimic clinico-radiologically many other commoner entities. The current study presents a wide view of the clinical, radiological, and histomorphological spectrum along with rare associations that can occur concurrently with this lesion. The most common site of infliction in CNS is the spine and, in the current series, involvement of the lumbar spine was most frequent. Both clinical and radiological features point towards other more common differentials, including neurofibroma/schwannoma and ependymoma. Some studies suggest rich vascularity (cap sign) and salt pepper appearance in T2-weighted images to serve as soft pointers towards diagnosing it on magnetic resonance imaging, however, in our series we did not encounter the same.

Intradural "limited drill" technique of anterior clinoidectomy and optic canal unroofing for microneurosurgical management of ophthalmic segment and PCOM aneurysms-review of surgical results.

To evaluate the safety and efficacy of intradural "limited drill" technique (ILDT) of anterior clinoidectomy (AC) and optic canal unroofing (OCU) for microneurosurgical management of ophthalmic segment and posterior communicating artery (PCOM) aneurysms. All the patients with ophthalmic segment and PCOM aneurysms who underwent AC and OCU by ILDT for microneurosurgical management of ophthalmic segment and PCOM aneurysms during 4-year period (2013-2016) at our Institute were included in this study. In ILDT of AC and OCU, the use of power drill is restricted to AC only and OCU is done exclusively with 1-mm Kerrison punch. AC and OCU by ILDT were done in 24 patients with 29 ophthalmic segment and 7 PCOM aneurysms. AC and OCU by ILDT helped in mobilization of optic nerve/internal carotid artery (ICA) and provided excellent exposure for all these aneurysms. There was no injury to ICA or optic nerve during drilling. AC and OCU facilitated clip ligation of 34 of these aneurysms. Remaining 2 aneurysms were considered not suitable for clipping. Check angiogram done for 33 aneurysms revealed complete obliteration of 26 aneurysms, very small residual neck in 5 aneurysms, and small residual aneurysm in 2 aneurysms. Deterioration in vision was noted in 1 patient (4.1%). In 6 patients with preoperative visual deficits, significant improvement in vision was noted in 4 patients (4/6-66.6%) after surgery. Good outcome (MRS < 2) was noted in 91.6% (22/24) of these patients. ILDT is a safe and effective technique of AC and OCU which provide good exposure for ophthalmic segment and PCOM aneurysms.

Deep flexor sarcopenia as a predictor of poor functional outcome after anterior cervical discectomy in patients with myelopathy.

BACKGROUND: Paraspinal muscle morphometry has been recognized to be a prognostic factor across various surgical conditions, but its utility in predicting disease-specific outcomes in spine surgery remains under-explored. METHODS: A prospective cohort study was performed on 45 consecutive patients undergoing anterior cervical discectomy (ACD) for single-level, symptomatic cervical degenerative disc disease causing radiculomyelopathy or myelopathy. Previously described predictors of outcome such as age, gender, smoking, comorbidities, duration of symptoms, preoperative Nurick grade, extent of cord compression, and signal intensity change in the cord were recorded. Additionally, MRI-based morphometrics of the superficial and deep paraspinal muscles were recorded. Logistic regression (LR) analysis was performed using a purposeful variable selection process to identify variables that independently predicted Nurick grade improvement (NGI). RESULTS: At a mean follow-up of 20.02 ± 8.63 months after ACD, 37 (82.22%) patients demonstrated NGI. LR analysis yielded three predictors of NGI of which two were related to the deep flexor muscles. While a worse preoperative Nurick grade negatively predicted NGI, a deep flexor area and deep flexor/deep extensor area ratio positively predicted NGI. The regression model demonstrated a good fit and was statistically significant (χ2(3) = 22.18, p < 0.0001). The model explained 64% of the variance in NGI and correctly classified 89% of cases. CONCLUSIONS: This study has for the first time identified the utility of paraspinal morphometrics in predicting disease-specific functional outcome after cervical spine surgery. Our results indicate that in addition to preoperative Nurick grade, an already accepted outcome predictor, the deep flexor cross-sectional area, and the deep flexor/deep extensor ratio are strong predictors of NGI following ACD for single-level, symptomatic cervical degenerative disc disease with myelopathy. Deep muscle morphometrics could be included in future risk stratification algorithms for patients with cervical disc disease.

Comparison of telemedicine with in-person care for follow-up after elective neurosurgery: results of a cost-effectiveness analysis of 1200 patients using patient-perceived utility scores.

OBJECTIVE The utility of telemedicine (TM) in neurosurgery is underexplored, with most of the studies relating to teletrauma or telestroke programs. In this study, the authors evaluate the cost-effectiveness of TM consultations for follow-up care of a large population of patients who underwent neurosurgical procedures. METHODS A decision-analytical model was used to assess the cost-effectiveness of TM for elective post-neurosurgical care patients from a predominantly nonurban cohort in West Bengal, India. The model compared TM care via a nodal center in West Bengal to routine, in-person, per-episode care at the provider site in Bangalore, India. Cost and effectiveness data relating to 1200 patients were collected for a 52-month period. The effectiveness of TM care was calculated using efficiency in terms of the percentage of successful TM consultations, as well as patient-perceived utility values for overall experience of the type of health care access that they received. Incremental cost-effectiveness ratio (ICER) analysis was done using the 4-quadrant charting of the cost-effectiveness plane. One-way sensitivity and tornado analyses were performed to identify thresholds where the care strategy would change. RESULTS The overall utility for the 3 TM scenarios was found to be higher (89%) than for the utility of routine care (80%). TM was found to be more cost-effective (Indian rupee [INR] 2630 per patient) compared to routine care (INR 6848 per patient). The TM strategy "dominates" that of routine care by being more effective and less expensive (ICER value of -39,400 INR/unit of effectiveness). Sensitivity analysis revealed that cost-effectiveness of TM was most sensitive to changes in the number of TM patients, utility and success rate of TM, and travel distance to the TM center. CONCLUSIONS TM care dominates the in-person care strategy by providing more effective and less expensive follow-up care for a remote post-neurosurgical care population in India. In the authors' setting, this benefit of TM is sustainable even if half the TM consultations turn out to be unsuccessful. The viability of TM as a cost-effective care protocol is attributed to a combination of factors, like an adequate patient volume utilizing TM, patient utility, success rate of TM, and the patient travel distance.

Pneumosinus dilatans of the spheno-ethmoidal complex associated with hypovitaminosis D causing bilateral optic canal stenosis.

INTRODUCTION: Pneumosinus dilatans (PSD) is a rare disorder of undetermined etiology characterized by expansion of the paranasal sinuses without bony erosion. Of the few cases of PSD described in indexed pediatric literature, there has been no reported case of this disorder presenting with optic canal stenosis in the setting of a vitamin deficiency. CASE MATERIAL: A 12-year-old girl presented with a 3-month history of progressive, painless, and asymmetric visual deterioration in her eyes. MRI showed prominent perioptic CSF spaces bilaterally and mild atrophy of both the optic nerves. CT head showed hyperpneumatization of the sphenoethmoidal air cells and both anterior clinoid processes with the optic nerves contained within narrowed intact bony canals. Blood investigations showed reduced vitamin D levels, and a subsequent skeletal survey showed diffuse osteopenia. She underwent endoscopic sphenoidotomy and bilateral decompression of the optic nerves. Following surgery, she reported improvement of vision in her left eye. She was started on vitamin D supplements for the endocrine abnormality. At a follow-up visit 6 months later, her visual acuity in both her eyes had improved. CONCLUSION: Pneumosinus dilatans is an unusual cause of progressive optic nerve dysfunction in the pediatric population. In the absence of any associated intracranial pathologies, conditions like hypovitamosis D should be ruled out.

Visual Deterioration and Herniation of the Anterior Cerebral Artery: Unusual Presentation of an Empty Sella Syndrome Complicating Decompression of a Rathke Cleft Cyst.

Clinical manifestations of empty sella syndrome include hypopituitarism cerebrospinal fluid rhinorrhea, headache, and visual abnormalities. A 21-year-old woman reported a 6-month history of worsening vision 3 years after decompression of a sellar-suprasellar Rathke cleft cyst. Her magnetic resonance imaging (MRI) showed a well-defined recurrent cyst in the sellar-suprasellar region causing chiasmatic compression. She underwent an endonasal, endoscopic decompression of the cyst, with subsequent improvement in her vision. A postoperative computed tomography confirmed good decompression of the cyst. Ten days after surgery, she reported sudden loss of vision in both eyes. MRI revealed an empty sella with herniation of both anterior cerebral arteries and optic chiasm into the sella. She underwent transnasal packing of the sellar floor with fat graft and bone plaques, and experienced gradual improvement in vision in her right eye.

Artery of Percheron infarction after endoscopic pituitary surgery.

Infarction in the artery of Percheron territory is a rare phenomenon in which occlusion of an unpaired perforating artery arising from the P1 segment on one side results in infarcts in the bilateral paramedian thalami with or without midbrain infarcts. We describe the case of a 40-year-old male who developed this complication following re-exploratory trans-sphenoidal surgery for a pituitary adenoma. In this first report of its kind in endoscopic pituitary surgery, the pathogenesis and clinico-radiological features of this rare vascular event are discussed.

Moving Beyond Morphometrics and Alignment: Prospective Longitudinal Study on Cognition, Quality of Life, and Diffusion Metrics in Congenital Craniovertebral Junction Anomalies.

BACKGROUND AND OBJECTIVES: Chiari type I malformation (CMI) has been implicated to cause cognitive impairment. Unusual for a craniovertebral junction (CVJ) anomaly, this phenomenon is postulated to be related to microstructural changes across the brain. However, this has not been confirmed in a longitudinal study, in bony CVJ anomalies, or in the context of quality of life (QOL). This study aimed to analyze cognition, QOL, and diffusion tensor imaging (DTI) metrics before and after surgery for congenital CVJ anomalies and evaluate their correlations with conventional clinico-radiological factors. METHODS: Fifty-seven patients with congenital CVJ anomalies-CMI, atlantoaxial dislocation (AAD), and basilar invagination (BI)-were evaluated in this prospective longitudinal study over 3 years with a neuropsychological battery, QOL and functional scores, and DTI metrics. A total of 43 patients (23 with CMI; 20 with AAD or BI) with 1-year postsurgical data were included in the final analysis. RESULTS: Cognitive dysfunction in one or more domains was noted in 15 (65%) of the patients with CMI and 16 (80%) of the patients with AAD/BI. Memory and visual motor perception were the most-affected domains in both the cohorts. At the 1-year follow-up, the proportion of patients demonstrating an overall cognitive dysfunction did not change significantly (P > .05). All patients demonstrated significant postoperative improvement in functional and QOL scores at follow-up. DTI metrics improved in many regions postoperatively, but did not correlate with functional or cognitive changes (P > .05). CONCLUSION: Cognition is affected in a majority of patients with CMI, AAD, or BI. These patients experience good functional and QOL outcomes after surgery but most of them continue to exhibit cognitive impairment. DTI metrics improve after surgery, but do not correlate with cognitive changes. These findings underline the multidimensional nature of the pathology of these anomalies that complicate achieving the concept of a "successful" surgery.

Extensive anterior skull-base mesenchymal chondrosarcoma: unusual cause of multiple indolent masses on the forehead.

Primary intracranial occurrence of an extraskeletal mesenchymal chondrosarcoma (MC) is unusual. The commonly involved sites are the orbit, clivus and temporo-occipital junction. Occurrence of the lesion in the anterior skull-base (ASB) in an infiltrative manner and with extra-calvarial involvement, is anecdotal. We report the case of a 35-year-old woman who presented with two indolent swellings on the forehead for a duration of 1 year. Examination revealed impaired visual acuity and complete external ophthalmoplegia in the right eye and 5 × 6 cm and 2 × 3 cm sized hard masses on the forehead. CT and MRI revealed a large, intensely enhancing ASB mass with extensions into the right orbit, ethmoid sinus, nasal cavity, and anteriorly, into the subcutaneous tissue of the frontal scalp through erosions in the bone. At surgery via a right frontal recraniotomy, the lesion was found to be firm and very vascular. Owing to its extreme vascularity, decompression was limited to excision of the intracranial and extra-calvarial components of the lesion. Histopathology was consistent with the diagnosis of MC. The patient was advised a second stage surgery for excision of the residual lesion. She, however, opted for radiation therapy and was lost to follow-up. This report, with one of the most extensive ASB MCs described to date, adds to the list of rare differentials of indolent forehead masses in the diagnostic armamentarium of the neuropathologist.

Surgical management of thalamic gliomas: case selection, technical considerations, and review of literature.

This study aimed to identify (1) the thalamic gliomas suitable for surgical resection and (2) the appropriate surgical approach based on their location and the displacement of the posterior limb of the internal capsule (PLIC). A retrospective study over a 5-year period (from 2006 to 2010) was performed in 41 patients with thalamic gliomas. The mean age of these patients was 20.4 years (range, 2-65 years). Twenty (49 %) tumors were thalamic, 19 (46 %) were thalamopeduncular, and 2 (5 %) were bilateral. The PLIC, based on T2-weighted magnetic resonance axial sections, was displaced anterolaterally in 23 (56 %) cases and laterally in 6 (14 %) cases. It was involved by lesion in eight (20 %) cases and could not be identified in four (10 %) cases. Resection, favored in patients with well-defined, contrast-enhancing lesions, was performed in 34 (83 %) cases, while a biopsy was resorted to in 7 (17 %) cases. A gross total resection or near total resection (>90 %) could be achieved in 26 (63 %) cases. The middle temporal gyrus approach, used when the PLIC was displaced anterolaterally, was the commonly used approach (63.5 %). Common pathologies were pilocytic astrocytoma (58 %) in children and grade III/IV astrocytomas (86 %) in adults. Preoperative motor deficits improved in 64 % of the patients with pilocytic lesions as compared to 0 % in patients with grade III/IV lesions (P value, 0.001). Postoperatively, two patients (5 %) had marginal worsening of motor power, two patients developed visual field defects, and one patient developed a third nerve paresis. Radical resection of thalamic gliomas is a useful treatment modality in a select subset of patients and is the treatment of choice for pilocytic astrocytomas. Tailoring the surgical approach, depending on the relative position of the PLIC, has an important bearing on outcome.

Subgaleo-peritoneal shunt: An effective and safer alternative to lumboperitoneal shunt in the management of persistent or recurrent iatrogenic cranial pseudomeningoceles.

Subgaleo-peritoneal (SP) shunting for pseudomeningoceles (PMCs) is an effective and safer alternative as compared to the lumboperitoneal (LP) shunt. SP shunting was done in six patients (14-60 years) with persistent or recurrent PMCs using the cranial (ventricular part) and the distal parts of a Chhabra shunt connected by a rigid connector without any intervening chamber or valve. Two patients had undergone a prior LP shunt that had failed. One patient was unsuitable for a LP shunt placement. The PMC subsided completely in all the patients following the SP shunt. In one patient, the shunt got displaced and required repositioning. None of the patients developed symptoms of over-drainage or any other complication. All patients were asymptomatic at a mean follow-up of 15 months. These results suggest that SP shunting is a safe, simple, and effective alternative to the traditional LP shunt in the management of persistent or recurrent cranial PMCs.

A nondysraphic paraspinal mass and a ventricular septal defect: unusual components of diabetes-induced mesodermal derangement.

Solitary paraspinal masses in the pediatric age group commonly occur secondary to spinal dysraphism, chronic inflammatory conditions, and tumors. We describe the unusual case of a 10-year-old boy with a nondysraphic, paraspinal mass that had occurred secondary to congenital spinal epidural lipomatosis (SEL) in the setting of poorly controlled maternal type-I diabetes mellitus. The mass was picked up along with a ventricular septal defect (VSD) on an antenatal ultrasonogram. This is the first report in literature of SEL presenting as a solitary paraspinal mass at birth, and in the unusual setting of an antenatal mesodermal derangement that simultaneously engendered a VSD.

Giant Tumefactive Perivascular Spaces: An Unusual Cause of Holmes Tremor.

A 34-year-old man presented with a 2-year history of medically refractory Holmes tremor in the right upper limb. Magnetic resonance imaging revealed a large, nonenhancing, multiseptate cystic lesion of cerebrospinal fluid intensity in the left thalamopeduncular region causing brainstem compression and hydrocephalus. A diagnosis of giant tumefactive perivascular spaces was made after a biopsy ruled out an infectious or neoplastic etiology. Significant clinicoradiologic improvement was noted following a ventriculoperitoneal shunt. Giant tumefactive perivascular spaces should be included as one of the rare differentials of a large, nonenhancing cystic lesion situated along the course of perforator vessels. Treatment options include cerebrospinal fluid diversion with or without cyst fenestration.

Multiple intracranial de-novo chloromas presenting with Garcin's syndrome.

Intracranial occurrence of a chloroma (myeloid sarcoma, MS) in the absence of a preceding hematological malignancy is unusual. We report the case of a 20-year-old man who presented with Garcin's syndrome of short duration. MRI revealed multiple extra-axial contrast enhancing lesions: two mirror lesions on the skull base, and one in the right parietal convexity. The parietal lesion was excised and histologically and immunohistochemically proved to be a differentiated variant of MS. Peripheral blood smear and bone marrow biopsy ruled out an underlying leukemia or myeloproliferative disorder. With a diagnosis of intracranial de-novo MS, he was referred for chemotherapy and radiation therapy. 15 months later, his clinical status remained the same while his imaging showed marginal decrease in size of the lesions. A repeat bone marrow biopsy remained normal. This is a first-of-its- kind report of multiple intracranial lesions of a de-novo MS presenting as Garcin's syndrome. Radiological differentials, immunohistochemical variants and management options related to MS are discussed in the light of the reported case.

Evaluation of pain as a preference-based health status measure in patients with cervical spondylotic myelopathy undergoing central corpectomy.

BACKGROUND: Assessment of pain in patients with cervical spondylotic myelopathy (CSM) before and after decompressive surgery has not been adequately addressed in the literature. The purpose of this study was to ascertain the intensity of various pain scores in patients with cervical spondylotic myelopathy (CSM) before and after surgery, and to assess their correlation with other outcome measures. METHODS: In this prospective study, 51 patients with CSM were assessed preoperatively and 1 year or more after uninstrumented central corpectomy (CC) using the Visual Analogue Scale (VAS), Nurick grade, patient perceived outcome score (PPOS) and SF-36. RESULTS: At presentation, there was a higher incidence of neck pain (43.1%) and arm pain (51%) than low axial pain (23.5%), with the mean VAS scores being 53.6 ± 27.4, 55.5 ± 27.4 and 34.0 ± 20.3, respectively. Following surgery, the mean neck, arm and low axial pain scores decreased significantly (p < 0.05) to 14.4 ± 22.6, 5.2 ± 11.8 and 16.0 ± 26.1, respectively. Improvement in pain scores demonstrated poor agreement (κ <0.2) with PPOS, Nurick grade recovery rate (NGRR), and the physical component summary (PCS) and mental component summary (MCS) of the SF-36. Pain scores did not influence quality of life as assessed by SF-36. CONCLUSIONS: Pain was reported by about half the patients with CSM, but was not severe in any of them. Following decompressive surgery, the intensity of all these pain components decreased significantly. Low axial pain, a reflection of CSM-related spasticity perceived in the lumbosacral region, became prominent in many patients after surgery.