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.

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.

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.

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.

Changes in Sinonasal and Overall Quality of Life Following Endoscopic Endonasal Surgery for Non-functioning Pituitary Adenomas: Results of A Prospective Observational Study.

Introduction: Quality of life (QOL) assessment is increasingly being recognized as an important parameter while evaluating outcomes after endoscopic endonasal transsphenoidal surgery (EETS). There has been no study that has evaluated this in the Indian context. Objectives: We aimed to analyze sinonasal and overall QOL before and after EETS for non-functioning pituitary adenomas (NFPAs), and to evaluate possible correlations between changes in QOL and various clinicoradiological, hormonal, and surgical factors. Methods: This prospective observational study included 62 patients who underwent EETS for NFPAs. The Anterior Skull Base Nasal Inventory-12 (ASK-12) and Short Form-12 (SF-12) were used to assess QOL. Changes in QOL were recorded at 2 weeks, 3 months, and 1-year follow-up periods. Results: While the nasal crusting and nasal discharge components of the ASK-12 worsened 2 weeks after EETS, the headache component demonstrated significant improvement (P < 0.05). The overall ASK-12 score (6.69+/-7.98) did not change significantly at follow-up. The SF-12 physical component summary (PCS) score (42.14+/-8.54) improved progressively after surgery, while the SF-12 mental component summary (MCS) score (42.48+/-7.95) improved 1 year later. The SF-12 PCS correlated with visual field improvement at 3-month follow-up (P = 0.04). Conclusions: EETS affects some components of the ASK-12 without affecting the overall sinonasal QOL in patients with NFPAs. These patients demonstrate progressive postoperative improvement in physical well-being, while their mental functioning improves only a year later. Improvement in physical functioning correlates with visual field improvement at a 3-month follow-up. Hormonal dysfunction and surgical factors do not have any impact on QOL in these patients.

Posterior Reversible Encephalopathy Syndrome Complicating the Postoperative Clinical Course in a Child with a Suprasellar Craniopharyngioma.

Posterior reversible encephalopathy syndrome (PRES) is an unusual neurological condition known to occur in the setting of various risk factors such as acute hypertension, renal failure, sepsis, multiorgan failure, autoimmune disease, and immunosuppression. Twenty cases of PRES have been previously reported in neurosurgical literature, almost all of which were attributed to either hypertension or chemotherapeutic drugs. Herein, we report a case of PRES in a neurosurgical patient in the absence of the commonly described risk factors for the syndrome. The patient was a 9-year-old boy who underwent uneventful transcranial resection of a suprasellar craniopharyngioma. His postoperative clinical course was complicated by hepatic failure and hypothalamic dysfunction, both of which were managed conservatively. Ten days after the onset of these complications, he developed clinical and radiological features suggestive of PRES which eventually resolved at follow-up. We discuss the pathogenesis of this unusual neurological syndrome in our patient in the light of a literature review.

Cervical Flexor-Extensor Muscle Disparity in Monomelic Amyotrophy (Hirayama Disease): Evidence from a Comprehensive Morphometric Evaluation of Subaxial Paraspinal Musculature.

Background Monomelic amyotrophy (Hirayama disease) has been established to have accompanied biomechanical abnormalities such as flexion hypermobility and sagittal imbalance. Paraspinal muscles, the major contributor to cervical biomechanics, have, however, not been comprehensively evaluated in the disease. The objective of this study was to compare the morphology of the subaxial cervical paraspinal musculature in patients with and without Hirayama disease. Materials and Methods A retrospective case-control study of 64 patients with Hirayama disease and 64 age- and sex-matched controls was performed . Cross-sectional areas (CSAs) of the superficial and deep flexors and extensors from C3 to C7 were measured on T2-weighted magnetic resonance imaging sequences. Student's t -test was used to compare differences between the paraspinal muscle CSAs in the study and control groups. Results Compared with controls, patients with Hirayama disease were found to have larger flexors and smaller extensors at all levels. The overall subaxial muscle area values for superficial flexors and deep flexors were significantly larger ( p < 0.0001) in patients, while the corresponding superficial extensor and deep extensor area values were significantly smaller than in controls ( p = 0.01 and < 0.0001, respectively). The patient group demonstrated stronger subaxial deep flexor-deep extensor, superficial flexor-superficial extensor, and total flexor-total extensor ratios ( p < 0.0001). Conclusion Patients with Hirayama disease have morphometric alterations at all levels of their subaxial cervical paraspinal musculature. These patients have abnormally large flexors and small extensors compared with controls. This flexor-extensor muscle disparity could be utilized as a potentially modifiable factor in the management of the disease.

Spinal Morphometry As A Novel Predictor For Recurrent Lumbar Disc Herniation Requiring Revision Surgery: Results of A Case Control Study.

Introduction: There is conflicting data on the risk factors for recurrent lumbar disc herniation (rLDH). Most of the predictors for rLDH identified so far are acquired risk factors or radiological factors at the level of the herniation. Whole lumbar spine (WLS) morphometry has not been evaluated as a possible predictor of rLDH. Objectives: We aimed to evaluate if preoperative spinal morphometry can predict the occurrence of rLDH requiring revision surgery. Methods: This retrospective case-control study on 250 patients included 45 patients operated for rLDH, 180 controls without rLDH who had previously undergone microdiscectomy for a single level lumbar disc prolapse, and a holdout validation set of 25 patients. Morphometric variables related to the WLS were recorded in addition to previously identified predictors of rLDH. Logistic regression (LR) analysis was performed to identify independent predictors of rLDH. Results: LR yielded four predictors of which two were WLS morphometric variables. While increasing age and smoking positively predicted rLDH, increasing WLS interfacet distance and WLS dural-sac circumference negatively predicted rLDH. The LR model was statistically significant, χ2 (4) =15.98, P = 0.003, and correctly classified 80.3% of cases. On validation, the model demonstrated a fair accuracy in predicting rLDH (accuracy: 0.80, AUC: 0.70). Conclusions: Larger mean lumbar bony canals and dural sacs protect from the occurrence of symptomatic rLDH. These WLS morphometric variables should be included in future risk stratification algorithms for lumbar disc disease. In addition to the previously recognized risk factors, our study points to an underlying developmental predisposition for rLDH.

Are Two-Dimensional Morphometric Measures Reflective of Disease Severity in Adult Chiari I Malformation?

BACKGROUND: Two-dimensional (2D) morphometric measures have been previously used to diagnose Chiari I malformation (CMI) and distinguish patients from healthy subjects. There is, however, a paucity of literature regarding whether morphometric differences exist across the range of CMI disease severity. We evaluated whether 2D morphometrics demonstrate significant changes across standardized grades of CMI severity in adults. METHODS: This retrospective observational study comprised 76 patients with symptomatic CMI with or without syringomyelia. Patients matched for age, sex, and body mass index were selected from each of the 3 severity grades of the Chiari Severity Index (CSI). The study used 17 2D craniocervical and posterior fossa morphometric variables that were previously found to differentiate patients with CMI from healthy subjects. The measurements were performed on midsagittal T1-weighted magnetic resonance imaging sequences using 2 custom in-house software tools, MorphPro and CerePro, and compared across CSI grades. RESULTS: Analysis of variance showed that none of the 17 morphometric variables were significantly different across the 3 CSI grades (P > 0.003). Bayes factor 10 scores ranged from 0.11 to 0.82. Of variables, 9 had Bayes factor 10 scores between 0.10 and 0.30, while 8 had scores between 0.30 and 1. None of the Bayes factor 10 scores provided support for the alternative hypothesis that the morphometric measures differed across the CSI grades. CONCLUSIONS: Our study indicated that 2D morphometrics are not reflective of CMI disease severity as indicated by the CSI grading system. The findings of our study support the need for further investigation into whether non-2D morphometric variables can be used as markers of disease severity.

Occipital Interhemispheric Transtentorial Approach for Tumors of Posterior Third Ventricular Region: Review of Surgical Results.

Background: Controversies exist regarding the ideal surgical approach for tumors in posterior third ventricular region (PTV). Objective: To evaluate the results of occipital interhemispheric transtentorial (OITT) approach for tumors in PTV. Materials And Methods: Thirty-three patients underwent surgery via OITT approach for PTV tumors at Sri Sathya Sai Institute of Higher Medical Sciences during the study period of 5 years (June 2011-May 2016). Ideal trajectory for OITT approach was determined by neuronavigation. Endoscope was used for removing any residual lesion at the blind spots. Results: Postoperative magnetic resonance imaging (MRI) performed in all the patients revealed gross total or near-total (>95%) excision of tumor in 31 patients (93.9%). Preoperative neurological deficits improved either completely or significantly following excision of the tumor in 73.3% (11/15) of the patients. Outcome was good (modified Rankin scale ≤2) at discharge in 93.9% (31/33) and at a final follow-up of 3 months or more in 96.8% (30/31) of the patients. None of the patients died during the postoperative period. Complications included upgaze palsy (transient- 6.1% [2/33], persisting- 3% [1/33]), visual field defects (transient- 3% [1/33], persisting- 3% [1/33]), transient third nerve paresis (1/33-3%), transient hemiparesis (1/33-3%), operative site hematoma (1/33-3%), small posterior cerebral artery (PCA) territory infarct (1/33-3%), and small venous infarct (1/33-3%). At least one follow-up MRI could be performed in 23 patients. Final follow-up MRI revealed no recurrence or increase in the size of the residual lesion compared to postoperative images in 20 patients (20/23-87%) and large recurrence in 3 patients (3/23-13%) with high-grade lesions. Conclusion: Gross total/near-total excision can be performed in majority of the PTV tumors through OITT approach with minimal morbidity and mortality.

An Unusual Case of a Conus Hanging by a Thread.

We report a rare cause of paraparesis in a 2-year-old girl. Along with lower limb weakness, she presented with a neurogenic bladder and lower limb deformities. Her magnetic resonance imaging showed multiple dorsolumbar segmentation defects and a dysgenetic spinal cord segment between T10 and S1. A thin septum connected the lower end of the normal cord to a bulky conus at S1-2. These features were suggestive of type 1 segmental spinal dysgenesis. The findings were confirmed at surgery, and the child was referred for supportive care.

Clinical and Laboratory Markers of Brain Abscess in Tetralogy of Fallot ('BA-TOF' Score): Results of a Case-Control Study and Implications for Community Surveillance.

Background Cardiogenic brain abscess (CBA) is the commonest noncardiac cause of morbidity and mortality in cyanotic heart disease (CHD). The clinical diagnosis of a CBA is often delayed due to its nonspecific presentations and the scarce availability of computed tomography (CT) imaging in resource-restricted settings. We attempted to identify parameters that reliably point to the diagnosis of a CBA in patients with Tetralogy of Fallot (TOF). Methods From among 150 children with TOF treated at a tertiary care institute over a 15-year period from 2001 to 2016, 30 consecutive patients with CBAs and 85 age- and sex-matched controls without CBAs were included in this retrospective case-control study. Demographic and clinical features, laboratory investigations, and baseline echocardiographic findings were analyzed for possible correlations with the presence of a CBA. Statistical Analysis Variables demonstrating significant bivariate correlations with the presence of a CBA were further analyzed using multivariate logistic regression (LR) analysis. Various LR models were tested for their predictive value, and the best model was then validated on a hold-out dataset of 25 patients. Results Among the 26 variables tested for bivariate associations with the presence of a CBA, some of the clinical, echocardiographic, and laboratory variables demonstrated significant correlations ( p < 0.05). LR analysis revealed elevated neutrophil-lymphocyte ratio and erythrocyte sedimentation rate values and a lower age-adjusted resting heart rate percentile to be the strongest independent biomarkers of a CBA. The LR model was statistically significant, (χ 2 = 23.72, p = <0.001), and it fitted the data well. It explained 53% (Nagelkerke R 2 ) of the variance in occurrence of a CBA, and correctly classified 83.93% of cases. The model demonstrated a good predictive value (area under the curve: 0.80) on validation analysis. Conclusions This study has identified simple clinical and laboratory parameters that can serve as reliable pointers of a CBA in patients with TOF. A scoring model-the 'BA-TOF' score-that predicts the occurrence of a CBA has been proposed. Patients with higher scores on the proposed model should be referred urgently for a CT confirmation of the diagnosis. Usage of such a diagnostic aid in resource-limited settings can optimize the pickup rates of a CBA and potentially improve outcomes.