Huge hydrocephalus: a rare case series with insights into management and outcomes of this neglected cohort.

PURPOSE: This study aims to discuss the management challenges of huge hydrocephalus (HH), a severe subset of hydrocephalus, presenting predominantly in underprivileged backgrounds. Insights into the condition's characteristics, factors affecting outcomes, and associated morbidity are discussed. METHODS: A retrospective review of all operated cases of hydrocephalus with head circumference greater than body length (HC>L) was conducted (January 2019-January 2023). Data on epidemiological parameters, associated cranial abnormalities, cortical mantle thickness, peri-conceptional folic acid intake, surgical interventions, age-appropriate milestones, and complications were collected. Follow-up was conducted for at least 12 months or until expiration. RESULTS: The cohort consisted of 7 males and 5 females with age ranging from 3 to 48 months. 33% of them had associated neurological abnormalities, and 18 surgical interventions were needed for these 12 cases, including ventriculoperitoneal shunt or endoscopic diversion. A 17% wound breakdown rate requiring re-suturing was present, and 33% of cases had postoperative CSF infection, with 33% mortality, with only one case having age-appropriate development seen. The average hospital stay was 11.9 days, six times our center's average. All cases with an Evans index with an average of 0.7 expired within 12 months. None of the 12 mothers took peri-conceptional folic acid, and no case agreed to reduction cranioplasty. CONCLUSION: Huge hydrocephalus is a rare cohort with significant management challenges and poor prognosis even after treatment. Factors such as delayed intervention and low socioeconomic status contribute to adverse outcomes. Prevention through peri-conceptual folic acid intake and addressing socioeconomic disparities is crucial in reducing disease burden and improving prognosis.

Prevalence and predictors of preoperative cardiac autonomic dysfunction among elective neurosurgical patients: A prospective observational study.

Background and Aims: The autonomic nervous system (ANS) is cardinal for systemic homeostasis. Autonomic dysfunction is prevalent in as high as 65% of patients presenting for cardiac surgery in the Indian scenario. Pre-existing cardiac autonomic dysfunction (CAD) in surgical patients can accentuate perioperative haemodynamic fluctuations during stressful intraoperative events, predispose to adverse cardiac events, and contribute to morbidity and mortality. The prevalence and predictors of CAD in the elective neurosurgical population are unknown in the Indian scenario. The current study was conducted to bridge this knowledge gap. Methods: In this single-centre prospective observational study conducted at a tertiary care neurosciences centre, among 400 consenting adult patients of either gender, between 18 and 80 years of age, undergoing elective neurosurgery, the preoperative ANS function at the bedside was assessed as the primary outcome measure. The ANS status was evaluated using ANSiscope™-derived indices of heart rate variability. The diagnosis of CAD was made when the ANS index exceeded a threshold of 13.5. Data regarding predictors of CAD were collected from patient records as the secondary outcome measure. Statistical analysis was done using the R software. A P-value of <0.05 was considered statistically significant. Results: The prevalence of preoperative CAD in our study population was 79.7% (319/400 patients). None of the demographic and baseline clinical characteristics we studied predicted CAD in our study. Conclusion: We observed a significant prevalence of preoperative CAD among elective neurosurgical patients. None of the parameters we evaluated predicted CAD in our study.

Role of Optical Coherence Tomography in Predicting Visual Outcome after Surgery for Sellar and Supra-Sellar Tumors.

BACKGROUND: Almost one-fifth of patients undergoing surgery for sellar/supra-sellar tumors do not gain a significant improvement in their vision. Various methods have been described to predict prospective visual outcomes in them, although they lack uniformity. OBJECTIVE: The study was conducted to predict visual outcomes following surgery for sellar and supra-sellar tumors compressing the anterior optic pathway based on pre-operative optical coherence tomography (OCT) parameters. METHODS AND MATERIALS: This was a record-based observational descriptive longitudinal study done in a tertiary care center in India. Thirty-seven patients (74 eyes) diagnosed with sellar supra-sellar lesions were included in the study. Patients' ophthalmic evaluations, done pre-operatively and 3 months post-operatively, were reviewed. Spectral-domain OCT and segmentation were done using the automated segmentation technology of Spectralis software. The thickness of the respective layers was measured. RESULTS AND CONCLUSIONS: The mean age of the study population was 42.68 years. Eyes with a pre-operative visual acuity component of VIS (visual impairment score) ≤61, pre-operative ganglion cell layer thickness ≥26.31 um, a pre-operative inner plexiform layer thickness of ≥25.69 um, a pre-operative ganglion cell inner plexiform layer thickness of 52.00 um, pre-operative ganglion cell complex thickness ≥84.47 µm, and a pre-operative inner retinal layer thickness of ≥205.25 µm were more likely to have an improved visual outcome. Eyes with a pre-operative duration of visual symptoms of ≥15 months, VIS ≥126.50, a pre-operative decimal visual acuity of <0.035, a pre-operative visual field index of ≤8%, a pre-operative macular thickness of ≤287.06 um, a pre-operative macular RNFL (retinal nerve fiber layer) thickness ≤66.00 µm, and a pre-operative peri-papillary RNFL thickness ≤64.62 µm were unlikely to have visual improvement.

Bell's mania as a clinical presentation in a neurosurgical setting from a tertiary care neuropsychiatric hospital in India.

Bell's mania is the co-occurrence of delirium and mania. We present two cases of Bell's mania in a neurosurgical setting. The first case is of a 52-year-old male who presented with holocranial headache, disorientation, and manic symptoms for five months. He was found to have suprasellar craniopharyngioma. He significantly improved with olanzapine, but re-emergence of mood symptoms was noted after surgery. The second case is of a 42-year-old male who presented with a 15-day history of seizures and disorientation. He was found to have a dural arteriovenous fistula. He developed Bell's mania in the post-procedural period, which improved with olanzapine. Compression of the hypothalamo-pituitary stalk in the first case and vascular and inflammatory changes in the second case could have led to Bell's mania. Atypical age of onset and presence of neurological symptoms in patients presenting with psychiatric symptoms should raise the suspicion of an underlying organicity. Atypical anti-psychotics can be a useful management strategy for Bell's mania.

Factors That Predict Recurrence Following Endoscopic Repair of Cerebrospinal Fluid Rhinorrhea.

BACKGROUND: The endoscopic approach has gained popularity in cerebrospinal fluid (CSF) rhinorrhea repair with high success rates, yet recurrence is frequent. We analyzed our cases to determine the outcomes of endoscopic repair of CSF rhinorrhea and the effect of several perioperative factors on the success of repair. METHODS: A retrospective review of 50 patients who underwent endoscopic repair of CSF rhinorrhea between January 2013 and July 2023 was performed, collecting details of presentation, surgery, and postoperative period. RESULTS: The most frequent etiology was nontraumatic CSF rhinorrhea (76%), in which the defect was most commonly located at the left cribriform plate, followed by traumatic CSF rhinorrhea (24%), in which sphenoid defects were most frequent. Traumatic CSF rhinorrhea was more common among male patients and was significantly associated with anosmia. Success rate at first repair attempt was 84%. Persistent CSF rhinorrhea was present in 3 patients (6%), and 5 patients (10%) developed recurrence of CSF rhinorrhea. Overall, 7 patients required reoperation, with 100% success rate after the second surgery. The use of 3-layered repair with fat, fascia lata, and mucosal flap was protective against repair failure, whereas bilateral defects and duration of symptoms >1 year were significantly associated with repair failure. The use of lumbar drain did not demonstrate a difference in repair success rate. CONCLUSIONS: Endoscopic repair of CSF rhinorrhea appears to be safe and effective when performed with accurate localization of the site of the lesion and multilayered repair. Potential predictors of recurrence include bilateral and long-standing defects.

Pediatric shunt failure: finding predictability in the sea of uncertainty.

OBJECTIVE: Shunt malfunction is a complication that can have devastating implications. In this study, the authors aimed to evaluate the rate of shunt revision in a single institution over 5 years and to determine the factors associated with shunt revision in the pediatric population. METHODS: This retrospective report assimilated data from all patients ≤ 18 years old who underwent shunt surgery between January 2015 and April 2021 at the authors' institute with a minimum of 3 months of follow-up. Patient data regarding demographic characteristics, indications, clinical status, point of entry, operative and CSF findings, revision interval, and cause of failure were collected. RESULTS: Between January 2015 and April 2021, 1112 pediatric patients underwent initial shunt surgery at the authors' institute, among whom 934 patients met the inclusion criteria. Ninety-five patients underwent revision (shunt revision rate 10.2%). The cohort comprised 562 male and 368 female patients (no sex was recorded in 4 cases), with infratentorial tumors (37.8%) being the most common indication for the shunt. Multivariate analyses revealed that younger patient age, right-sided shunt, single surgeon, and shunt placement done in the evening and night were significantly associated with shunt failure. Among all the factors analyzed, female sex had the greatest risk of early shunt failure (OR 2.90 [95% CI 1.09-8.16], p = 0.037). The presence of prior external ventricular drainage was associated with an increased risk of multiple revisions (OR 6.67 [95% CI 1.60-32.52], p = 0.012). The most common cause of failure was obstruction, usually at the cranial end. The most common cause of distal failure was malposition of the abdominal end. CONCLUSIONS: This study identifies various factors associated with shunt failure. Various goal-directed strategies toward modifiable risk factors can significantly improve shunt survival.

Fitness to re-join job: Neuropsychiatric perspective.

Objectives: Evaluating "Fitness to Re-join Job" and certification in persons with Neuro-Psychiatric Disorders is an indispensable professional responsibility. However, there is little documented guidance on clinically approaching this particular issue. This study aimed to study the sociodemographic, clinical, and employment profile of patients who sought fitness to re-join their job from the tertiary neuropsychiatric center. Materials and Methods: This study was carried out at the National Institute of Mental Health and Neurosciences in Bengaluru, India. A retrospective chart review was adapted for the purpose. One hundred and two case files referred to medical board for fitness to rejoin the duty were reviewed from January 2013 to December 2015. Apart from the descriptive statistics, the Chi-square test or Fisher exact test was used to test the association between categorical variables. Results: Patients' mean (standard deviation) age was 40.1 (10.1) years; 85.3% were married, and 91.2% were male. Common reasons for seeking "fitness certification" were work absenteeism (46.1%), illness affecting the work (27.4%), and varied reasons (28.4%). The presence of neurological disorders, sensory-motor deficits, cognitive decline, brain damage/insult, poor drug compliance, irregular follow-ups, and poor or partial treatment response were associated with an unfitness to rejoin the job. Conclusion: This study shows that work absenteeism and the impact of illness on work are common reasons for referral. Irreversible neurobehavioural problems and deficits impacting work are common reasons for unfitness to rejoin the job. There is a need for a systematic schedule to assess the fitness for the job in patients with neuropsychiatric disorders.

Gamma Knife Radiosurgery in Partially Embolised Arteriovenous Malformations: Management Dilemmas and Outcomes.

Introduction: The role of Gamma Knife radiosurgery (GKRS) in partially embolized arteriovenous malformations (AVMs) has always remained a subject of debate. The aim of this study was to evaluate the efficacy of GKRS in partially embolized AVMs and to analyze factor that influence its obliteration. Methods: This was a retrospective study from a single institute performed over a period of 12 years (2005-2017). It included all patients who underwent GKRS for partially embolized AVMs. Demographic characteristics, treatment profiles, and clinical and radiological data were obtained during treatment and follow-up. Obliteration rates and factors affecting the same were sought and analyzed. Results: A total of 46 patients with a mean age of 30 years (range: 9-60 years) were included in the study. Follow-up imaging was available for 35 patients either by digital subtraction angiography (DSA) or magnetic resonance imaging (MRI). We found complete AVM obliteration in 21 patients (60%): one had near total obliteration (>90% obliteration), 12 had subtotal obliteration (<90%), and one had no change in the volume following GKRS. Following embolization alone, an average of 67% of the AVM volume was obliterated which resulted in an average 79% final obliteration rate after Gamma Knife radiosurgery. Mean duration to complete obliteration was found to be 3.45 years (range: 1-10 years). There was a significant difference (P = 0.04) in the mean interval between embolization and GKRS among cases with complete obliteration (12 months) and those with incomplete obliteration (36 months). There was no significant difference (P = 0.49) in the average obliteration rate between the ARUBA-eligible unruptured AVMs (79.22%) and ruptured AVMs (79.04%). Bleeding after GKRS during the latency period had a negative impact on obliteration (P = 0.05). Other factors like age, sex, Spetzler-Martin (SM)-grade, Pollock Flickinger score (PF-score), nidus volume, radiation dose, or presentation before embolization had no significant influence on obliteration. Three patients had permanent neurological deficits after embolization and none after radiosurgery. Six out of nine patients (66%) presenting with seizures were seizure-free after the treatment. Hemorrhage was noted in three patients following combined treatment and were managed non-surgically. Conclusion: Obliteration rates in partially embolized AVM after Gamma Knife are inferior when compared to Gamma Knife alone; moreover with volume staging and/or dose staging being increasingly plausible due to the new ICON machine, embolization may be completely replaced. However we have shown that in complicated and carefully chosen AVMs, embolization followed by GKRS is a valid modality of management. This study represents a real-world picture of individualized AVM treatment depending on patient choices and resources available.

Spectrum of Surgically Resected Lesions of the Cavernous Sinus: A Neuropathologic Audit.

Background The cavernous sinus is a complex space composed of extradural venous plexus within dural folds. Several important structures like the carotid artery, cranial nerves, and sympathetic nerve fibers traverse through this space. Radiological diagnosis may not be definitive and in the context of discordance between clinical and neuroimaging diagnosis, histopathological evaluation becomes essential for diagnosis and management. Literature on the pathological spectrum of lesions is scarce as, with a shift in the treatment paradigm, most small lesions of cavernous sinus are treated with radiosurgery. However, surgical management still plays a role for larger lesions and in radiologically ambiguous cases for planning the definitive management. Materials and Methods We retrospectively reviewed all surgically resected lesions of the cavernous sinus over the last two decades (1998-2019). The clinical presentation, neuroimaging features, and histopathological findings were reviewed. Lesions extending from sella and other adjacent areas were excluded. Results Thirty-eight cases of isolated cavernous sinus mass lesions were diagnosed over the last two decades (1998-2019). Cavernous hemangiomas (19 cases, 50%) constituted the most frequent pathology, followed by aspergilloma, meningioma, schwannoma, metastatic adenocarcinoma, chondrosarcoma, and chordoma. Overall, 29.4% (10/34) could not be accurately diagnosed on neuroimaging. Of these, four cases of cavernous hemangiomas were mistaken for either meningioma (three cases) or schwannoma (one case). Neither chordoma nor chondrosarcoma was suspected. Conclusion This is the first study in literature, enumerating the pathological and imaging spectrum of surgically resected cavernous sinus lesions. Cavernous hemangiomas, metastases and chordomas, and chondrosarcoma posed the greatest difficulty in diagnosis on neuroimaging and the reasons for the same are analyzed. In the context of clinical and neuroimaging discordance in diagnosis, pathological characterization becomes essential for appropriate and timely management.

Surgical Outcomes in Rare Movement Disorders: A Report of Seventeen Patients from India and Review of Literature.

Background: Rare movement disorders (RMDs) throw remarkable challenges to their appropriate management particularly when they are medically refractory. We studied the outcome of functional neurosurgery among patients with RMDs. Methods: Retrospective chart-review from 2006 to 2021 of patients with RMDs who underwent either Deep brain Stimulation (DBS) or lesional surgeries in the department of Neurology and Neurosurgery at a tertiary care centre. Results: Seventeen patients were included. Generalized dystonia (11 patients, 64.7%) and tremor (5 patients, 29.4%) were the most common indication for surgery whereas, Wilson's disease (8 patients, 47.1%) and Neurodegeneration with brain iron accumulation (5 patients, 29.4%) were the most common aetiology. Sixteen patients (94.1%) had objective clinical improvement. Significant improvement was noted in the dystonia motor scores both at 6-months and 12-months follow-up (n = 11, p-value of <0.01 and 0.01 respectively). Comparison between DBS and lesional surgery showed no significant difference in the outcomes (p = 0.95 at 6-months and p = 0.53 at 12-months), with slight worsening of scores in the DBS arm at 12-months. Among five patients of refractory tremor with Wilson's disease, there was remarkable improvement in the tremor scores by 85.0 ± 7.8% at the last follow-up. Speech impairment was the main complication observed with most of the other adverse events either transient or reversible. Discussion: Surgical options should be contemplated among patients with disabling medically refractory RMDs irrespective of the aetiology. Key to success lies in appropriate patient selection. In situations when DBS is not feasible, lesional surgeries can offer an excellent alternative with comparable efficacy and safety.

Gamma Knife Radiosurgery for Large Vestibular Schwannoma More Than 10 cm 3 : A Single-Center Indian Study.

Introduction Gamma Knife radiosurgery (GKRS) is an effective treatment for benign vestibular schwannomas (VSs). The established cutoffs have recently been challenged, as recent literature expanded the horizon of GKRS to larger tumors. Even though microsurgery remains the primary option for large VS, GKRS can provide reasonable tumor control and is more likely to avoid cranial neuropathies associated with open surgery. Methods We analyzed patients with VS with volume exceeding 10 cm 3 who underwent GKRS at our center from January 2006 to December 2016. Clinicoradiological and radiosurgical data were collected from medical records for statistical analysis. Follow-up was performed every 6 months with a clinical assessment along with magnetic resonance imaging (MRI) of the brain and audiometric evaluation in patients with serviceable hearing. Results The study included 34 patients (18 males and 16 females) with an average age of 45.5 years. The mean tumor volume was 10.9 cm 3 (standard deviation [SD], ± 0.83), with a median tumor dose of 12 Gy (interquartile range, 11.5-12) and a mean follow-up of 34.7 months (SD, ± 23.8). Tumor response was graded as regression in 50%, stable in 44.1%, and increase or GKRS failure in 2 cases (5.8%). Treatment failure was noted in five cases (14.7%), requiring microsurgical excision and a ventriculoperitoneal shunt post-GKRS. The tumor control rate for the cohort is 85.3%, with a facial preservation rate of 96% (24/25) and hearing loss in all (5/5), while three patients developed new-onset hypoesthesia. We noted that gait ataxia and involvement of cranial nerve V or VII at initial presentation were associated with GKRS failure in univariate analysis. Conclusion Microsurgery should remain the first-choice treatment option for large VSs. GKRS is a viable alternative with good tumor control and improved or stabilized cranial neuropathies with a low complication rate.