Sellar Reconstruction With a Bioabsorbable Plate After Endoscopic Transsphenoidal Pituitary Adenoma Resection: Safe and Efficacious.

OBJECTIVE: To report outcomes of a large cohort of patients who underwent endoscopic endonasal transsphenoidal surgery (EETS) for resection of a pituitary adenoma with subsequent Resorb-X plate (RXP) sellar reconstruction. METHODS: A retrospective review of 620 EETS operations performed at a single academic center between 2005 and 2020 was conducted. RESULTS: A total of 215 EETS operations of 208 patients were identified between 2012 and 2020 who underwent reconstruction with the RXP after EETS for pituitary tumor resection with a final pathologic diagnosis of pituitary adenoma. Analysis of pooled data revealed a mean preoperative tumor volume of 6.8 cm3 (range: 0.038-51.03 cm3). Postoperative cerebrospinal fluid leak occurred in 2 patients (0.93%). Postoperative meningitis occurred in 1 patient (0.47%). There were no cases of RXP extrusion. CONCLUSIONS: The rate of postoperative CSF leak and meningitis after use of the RXP for sellar reconstruction compares favorably to other methods, including use of autologous grafts and flaps. Use of RXP during EETS is a safe and efficacious method of sellar reconstruction and may obviate the need for autologous tissue reconstruction after pituitary adenoma resection.

Granulomatosis with polyangiitis presenting headache: A case report and review of literature.

RATIONALE: Central nervous system involvement is a rare manifestation of active-phaselocalized Granulomatosis with polyangiitis (GPA). In hypertrophic dura meningitis, GPA with headache is typical. In this case, cerebral magnetic resonance (MR) enhancement revealed no meningeal thickening, to our knowledge, this manifestation had not been found previously. PATIENT CONCERNS: The patient presented to the Rheumatology and Immunology Clinic with severe headache and hearing loss, and central nervous system granulomatosis with polyangiitis was confirmed after a series of examinations. The patient had no significant effect after treatment with cyclophosphamide (CTX), but after the use of rituximab, the headache and hearing loss were significantly improved, and laboratory indicators returned to normal levels. DIAGNOSIS: We comprehensively screened for craniocerebral infection and malignant tumors, diagnosed central nervous system granulomatosis with polyangiitis. INTERVENTIONS: We gave sequential treatment of rituximab. OUTCOMES: All indicators are mostly back to normal when the patient was monitored at the outpatient clinic. LESSONS: GPA and severe headache are more prevalent in hypertrophic dura meningitis, but the patient early headache could not be explained by hypertrophic dura meningitis or localized granulomatous lesions that invaded the central nervous system. Patients with severe headaches likely have vascular inflammation and local bone destruction at the base of the skull.

Primary diffuse leptomeningeal primitive neuroectodermal tumor presenting as chronic meningitis.

Primary diffuse leptomeningeal primitive neuroectodermal tumor is a rare meningeal neoplasm which can masquerade as chronic meningitis. While the clinical presentation and radiological features may provide a clue to this condition, meningeal biopsy is essential to clinch the diagnosis. A high index of suspicion and a low threshold for re-evaluating cases of neuroinfection that do not respond to empirical therapy are essential in this scenario. We present the case of a nine year old boy who was initiated on antituberculous treatment for chronic meningitis with hydrocephalus. Meningeal biopsy revealed a primary diffuse leptomeningeal primitive neuroectodermal tumor.

An overview of the therapeutic strategies for neoplastic meningitis due to breast cancer: when and why?

INTRODUCTION: Neoplastic meningitis (NM), also known as leptomeningeal carcinomatosis, is characterized by the infiltration of tumor cells into the meninges, and poses a significant therapeutic challenge owing to its aggressive nature and limited treatment options. Breast cancer is a common cause of NM among solid tumors, further highlighting the urgent need to explore effective therapeutic strategies. This review aims to provide insights into the evolving landscape of NM therapy in breast cancer by collating existing research, evaluating current treatments, and identifying potential emerging therapeutic options. AREAS COVERED: This review explores the clinical features, therapeutic strategies, recent advances, and challenges of managing NM in patients with breast cancer. Its management includes multimodal strategies, including systemic and intrathecal chemotherapy, radiation therapy, and supportive care. This review also emphasizes targeted drug options and optimal drug concentrations, and discusses emerging therapies. Additionally, it highlights the variability in treatment outcomes and the potential of combination regimens to effectively manage NM in breast cancer. EXPERT OPINION: Challenges in treating NM include debates over clinical trial end points and the management of adverse effects. Drug resistance and low response rates are significant hurdles, particularly inHER2-negative breast cancer. The development of more precise and cost-effective medications with improved selectivity is crucial. Additionally, global efforts are needed for infrastructure development and cancer control considering the diverse nature of the disease.

[A Surgical Case of Arachnoid Cyst With Rheumatoid Meningitis].

Rheumatoid meningitis is a rare neurological manifestation that is difficult to diagnose. Here we report a 77-year-old woman who developed a left hemiparesis because of an arachnoid cyst with rheumatoid meningitis. The patient had a 13 year history of rheumatoid arthritis, which had been successfully treated. She developed left hemiparesis and visited our hospital. MRI showed a high signal intensity lesion in the right frontal subarachnoid space and an arachnoid cyst on Fluid attenuated inversion recovery (FLAIR). A gadolinium enhanced T1 weighted image revealed associated abnormal enhancement. We removed the arachnoid cyst and diagnosed the patient as having rheumatoid meningitis. Her symptoms and MRI findings improved rapidly by methylprednisolone pulse therapy. Rheumatoid meningitis is rare, but a case of arachnoid cyst associated with rheumatoid meningitis was extremely rare. This is the first report of such a case.

Dural and Leptomeningeal Diseases: Anatomy, Causes, and Neuroimaging Findings.

Meningeal lesions can be caused by various conditions and pose diagnostic challenges. The authors review the anatomy of the meninges in the brain and spinal cord to provide a better understanding of the localization and extension of these diseases and summarize the clinical and imaging features of various conditions that cause dural and/or leptomeningeal enhancing lesions. These conditions include infectious meningitis (bacterial, tuberculous, viral, and fungal), autoimmune diseases (vasculitis, connective tissue diseases, autoimmune meningoencephalitis, Vogt-Koyanagi-Harada disease, neuro-Behçet syndrome, Susac syndrome, and sarcoidosis), primary and secondary tumors (meningioma, diffuse leptomeningeal glioneuronal tumor, melanocytic tumors, and lymphoma), tumorlike diseases (histiocytosis and immunoglobulin G4-related diseases), medication-induced diseases (immune-related adverse effects and posterior reversible encephalopathy syndrome), and other conditions (spontaneous intracranial hypotension, amyloidosis, and moyamoya disease). Although meningeal lesions may manifest with nonspecific imaging findings, correct diagnosis is important because the treatment strategy varies among these diseases. ©RSNA, 2023 Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article. Quiz questions for this article are available through the Online Learning Center.

Rituximab for treatment of pachymeningitis in granulomatosis with polyangiitis.

Pachymeningitis is a rare manifestation of granulomatosis with polyangiitis (GPA), and there is a paucity of literature regarding the efficiency of rituximab (RTX) in such cases. We present a woman in her early 50s with GPA who initially responded adequately to conventional therapy. However, the treatment course was complicated by pulmonary cryptococcosis. The patient later developed non-infectious pachymeningitis, which failed to respond to steroids and methotrexate. However, using RTX upfront led to good clinical response and radiological resolution.

The Risk of Meningitis After Cochlear Implantation: A Systematic Review and Meta-Analysis.

OBJECTIVE: This study aims to estimate the rate of postoperative meningitis (both immediate and long-term) in patients following cochlear implants (CIs). It aims to do so through a systematic review and meta-analysis of published studies tracking complications after CIs. DATA SOURCES: MEDLINE, Embase, and Cochrane Library. REVIEW METHODS: This review was performed in line with Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Studies tracking complications following CIs in patients were included. Exclusion criteria included non-English language studies and case series reporting <10 patients. Bias risk was evaluated using the Newcastle-Ottawa Scale. Meta-analysis was performed through DerSimonian and Laird random-effects models. RESULTS: A total of 116/1931 studies met the inclusion criteria and were included in the meta-analysis. Overall, there were 112 cases of meningitis in 58,940 patients after CIs. Meta-analysis estimated an overall rate of postoperative meningitis of 0.07% (95% confidence interval [CIs], 0.03%-0.1%; I2 = 55%). Subgroup meta-analysis showed this rate had 95% CIs crossing 0% in implanted patients who received the pneumococcal vaccine, antibiotic prophylaxis, those with postoperative acute otitis media (AOM), and those implanted less than 5 years. CONCLUSION: Meningitis is a rare complication following CIs. Our estimated rates of meningitis after CIs appear lower than prior estimates based on epidemiological studies in the early 2000s. However, the rate still appears higher than the baseline rate in the general population. The risk was very low in implanted patients who received the pneumococcal vaccine, antibiotic prophylaxis, received unilateral or bilateral implantations, developed AOM, those implanted with a round window or cochleostomy techniques, and those under 5 years.

Leukocyte-Rich and Platelet-Rich Fibrin for Skull Base Reconstruction After Endoscopic Endonasal Skull Base Surgery.

BACKGROUND: Postoperative cerebrospinal fluid (CSF) leak is one of the most common complications after endoscopic endonasal approach to the skull base. OBJECTIVE: To analyze the effect of leukocyte-rich and platelet-rich fibrin (L-PRF) as part of a standardized endonasal skull base repair protocol on postoperative infection and CSF leak rate. METHODS: One hundred three patients who underwent an endoscopic endonasal approach and were reconstructed using a standard technique (Group A) were compared with 139 patients using the same protocol with the addition of L-PRF (Group B). Postoperative intracranial infection and CSF leak at 6 months were analyzed. RESULTS: In patients with intraoperative CSF leak, postoperative leak occurred in 10.8% in Group A and 2.6% in Group B ( P .024), and in patients with intraoperative high-flow leaks, the incidences were 11.7% and 3.1%, respectively ( P .048). L-PRF reduced postoperative CSF leak by 76% in cases with intraoperative CSF leak (relative risk 0.24, 95% CI 0.06-0.87) and by 73% of patients with high-flow leak (relative risk 0.27, 95% CI 0.07-0.99). In patients undergoing surgery for diagnoses other than adenomas, there were no cases of postoperative leak in Group B, whereas in Group A occurred in 13.4% of those with intraoperative leaks ( P .047) and 15.8% with high-flow intraoperative leaks ( P .033). No significant differences were found in patients with pituitary adenoma. Meningitis occurred in 0.97% in Group A vs 2.16% in Group B ( P .639), without differences between subgroups. CONCLUSION: L-PRF reduced the rate of postoperative CSF leaks in patients with intraoperative leaks, without differences on postoperative meningitis.

Impact of an external ventricular shunt (EVD) handling protocol on secondary meningitis rates: a historical cohort study with propensity score matching.

BACKGROUND: External ventricular drainage (EVD) is frequently used in neurosurgical procedures for cerebrospinal fluid (CSF) drainage. It is, however, associated with high infection rates, namely secondary meningitis and ventriculitis. Based on a previous high prevalence of these infections among patients with EVDs, we have proposed and implemented a protocol in an effort to decrease the infection rate. The aim of this study was to measure the effect of hospital-wide implementation of the EVD handling protocol on secondary EVD infections. PATIENTS AND METHODS: We included 409 consecutive patients who received a new EVD for other indications than infectious pathologies from January 2000 until June 2012. Patients above 18 years of age were divided into pre- (n = 228) and post-protocol (n = 181) groups. Patient and disease demographics, as well as EVD data together with confounders for secondary meningitis were recorded in a database. Propensity score matching was then performed to create groups matched for sex, age, reason for drainage, type of shunt, time in situ and duration of surgery to place the EVD. Binomial logistic regression for confounder adjustment and regression discontinuity analyses were then performed on the matched cohort. RESULTS: Infections occurred more frequently in the pre-protocol group (23% vs 9%, p <  0.001). The incidence of infection was 33/1000 drain-days pre-protocol and 9/1000 drain-days post-protocol. Regression analysis in a propensity score-matched cohort (n = 103 in the pre- and n = 178 in the post-protocol groups) showed that the pre-protocol period was independently associated with more infections (OR 2.69; 95%-CI 1.22-5.95, p = 0.01). CONCLUSIONS: The incidence of secondary EVD infections can be reduced significantly by the implementation of a strict hospital-wide EVD handling protocol.

A review of rheumatoid meningitis with case studies.

Rheumatoid arthritis (RA) is an autoimmune disease involving synovial joints, and it is known that extra-articular manifestations that may affect the central and peripheral nervous systems may develop during its course. Rheumatoid meningitis is very rare among all neurological involvements. In this study, cases diagnosed as rheumatoid meningitis with clinical, imaging, laboratory, and histopathological features are presented, and the aim of the study is to present current approaches in the diagnosis and treatment of rheumatoid meningitis in the light of case studies and current literature. The data of the patients who were followed up with the diagnosis of rheumatoid meningitis in neurology clinic between 2017 and 2021 were reviewed retrospectively. Three cases diagnosed with rheumatoid meningitis are presented in detail. In the first case, the diagnosis was reached by clinical, imaging, and laboratory findings as well as treatment response, while the diagnosis was made by histopathological verification in the second case. The third case shows that spontaneous remission can be observed in the course of rheumatoid meningitis. Rheumatoid meningitis, which is one of the rarest involvements in the course of RA, may present with headaches, focal neurological deficits, seizures, and altered consciousness. A meningeal biopsy is recommended when the differential diagnosis cannot be ruled out with imaging and laboratory findings. In the differential diagnosis Mucobacterium tuberculosis, syphilis, neuro-sarcoidosis, immunoglobulin G4-related disease, lymphoproliferative diseases, and systemic metastasis should be kept in mind. Aggressive RA management is recommended for treatment.

Difficulties of diagnosing idiopathic hypertrophic pachymeningitis in children: Case report and literature review.

Hypertrophic pachymeningitis (HP) is a rare inflammatory disorder characterised by local or diffuse thickening of the cranial and spinal dura mater. HP occurs owing to idiopathic or secondary causes, including autoimmune disease, infection, and trauma. HP has mainly been reported in adults, with few reported cases in children. We encountered an 11-year-old boy with idiopathic HP who presented with chronic inflammation and daily occipital headache. Gadolinium (Gd)-enhanced magnetic resonance imaging (MRI) helped us to diagnose him with HP. He was successfully treated with corticosteroids and azathioprine with no recurrence. We also conducted a literature review of childhood-onset HP and found only 16 cases, including our patient. Seven patients had idiopathic HP, and the remaining nine had secondary HP, including two with rheumatic disease. The most common clinical symptoms were headache (68.8%) and cranial nerve-related symptoms (68.8%). Inflammatory laboratory markers were elevated in 60% of patients with available data. Fifteen cases were diagnosed using Gd-enhanced MRI. The main initial treatment was steroids and/or immunosuppressants, to which 87.5% of patients responded. However, two patients with HP associated with trauma and neuroblastoma (12.5%) died, and seven patients (43.8%) had left cranial nerve-related sequelae. As the prognosis for childhood HP is poor, early diagnosis and treatment are essential. Children with headache, cranial nerve symptoms, and elevated inflammatory marker levels should be suspected of having HP and Gd-enhanced MRI should be considered.

Is Hypertrophic Pachymeningitis Really Idiopathic?

Background: Hypertrophic pachymeningitis (HPM) is a unique disorder characterized by thickening and fibrosis of the dura mater. Clinically it presents with headache, cranial nerve palsies, and other focal neurological deficits. Two forms exist, one is primary, where all other causes have been excluded and the other is secondary where an identifiable cause exists. It is important to recognize these secondary causes as treatment depends on the etiology. Objective: To elucidate the various characteristics of HPM. To delineate clinical-radiological features that help differentiate secondary from primary causes and to understand treatment response and disease outcomes of HPM. Methods: This retrospective observational study included 33 patients who presented with radiological diagnosis of HPM from January 2014 to July 2019. Spontaneous intracranial hypotension patients were excluded. All patients were extensively evaluated for secondary causes and treatment outcomes were analyzed on follow-up. Results and Conclusions: Secondary causes of HPM were present in 48% cases. The clue for primary causes is an associated Tolosa-Hunt syndrome. Secondary causes in our series are immunological, infection, and malignancy. Clues to differentiate primary from these secondary causes are clinical like myelopathy, seizures, poor response to immunosuppression; radiological like hypertrophic cranial nerves, infarcts, bony erosion, and leptomeningeal involvement. There are case reports in literature but large Indian studies are lacking. This manuscript presents a large cohort of cases with HPM, which helps differentiate primary from secondary causes, as management and prognosis depend on etiology. An algorithm depicting the approach to the management of HPM has been presented.

A simple surgical technique for sellar closure after transsphenoidal resection of pituitary adenomas in the context of risk factors for cerebrospinal fluid leaks and meningitis.

OBJECTIVE: The transsphenoidal approach is the standard for most pituitary tumors. Despite low morbidity, postoperative CSF fistulas and meningitis are specific complications. Various surgical closure techniques for intraoperative CSF (iCSF) leak and sellar reconstruction have been described. For many years the authors have applied synthetic materials for iCSF leak repair and sellar closure in a standardized fashion in their department. Here they analyze the surgical outcome as well as risk factors for iCSF leak and meningitis. METHODS: All patients with transsphenoidal resection of a pituitary adenoma performed by the same surgeon between January 2013 and December 2019 were screened retrospectively. A small amount of iCSF flow without a diaphragmatic defect was classified as a minor leak, and obvious CSF flow with or without a diaphragmatic defect was classified as a major leak. In case of iCSF leak, a fibrin- and thrombin-coated sponge was used to cover the diaphragmatic defect and another one was used for the sellar opening. A gelatin sponge was placed in the sphenoid sinus as an abutment. The primary and secondary outcomes were the number of postoperative CSF (pCSF) leaks and meningitis, respectively. Clinical, histological, and perioperative data from medical records were collected to identify risk factors for CSF leak and meningitis. RESULTS: Of 417 transsphenoidal surgeries, 359 procedures in 348 patients with a median age of 54 years were included. There were 96 iCSF leaks (26.7%; 37.5% major, 62.5% minor). In 3 of 359 cases (0.8%) a pCSF fistula occurred, requiring revision surgery in 2 patients and a lumbar drain in 1 patient. Meningitis occurred in 3 of 359 cases (0.8%). All 3 patients recovered without sequelae after antibiotic therapy. According to univariate analysis, risk factors for iCSF leak were macroadenoma (p = 0.006) and recurrent adenoma (p = 0.032). An iCSF leak was found less often in functioning adenomas (p = 0.025). In multivariate analysis recurrent tumors remained as a risk factor (p = 0.021) for iCSF leak. Patients with iCSF leak were at increased risk for a pCSF leak (p = 0.005). A pCSF leak in turn represented the key risk factor for meningitis (p = 0.033). CONCLUSIONS: Patients with macroadenomas and recurrent adenomas are especially at risk for iCSF leak. An iCSF leak in turn increases the risk for a pCSF leak, which carries the risk for meningitis. The authors' surgical technique leads to a very low rate of pCSF leaks and meningitis without using autologous graft materials. Hence, this technique is safe and improves patient comfort by avoiding the disadvantages of autologous graft harvesting.

Case Report: Rheumatoid meningitis with positive NMDAR antibody: A case treated with intravenous immunoglobulin.

As a rare complication of rheumatoid arthritis (RA) in the central nervous system (CNS), rheumatoid meningitis (RM) mainly affects the meninges and has various clinical symptoms. The diagnostic and treatment approaches currently used are not practical. RM cases with positive NMDAR antibodies (Abs) have never been reported. In the present study, a 66-year-old man with a 1-year history of RA presented recurrent left lower limb weakness during activities for 1 month. The results showed that rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibody (ACPA) were positive in the serum, and NMDAR Abs were present in cerebrospinal fluid (CSF). Hyperintensity was observed in the leptomeninges of the right frontal and parietal lobes, and subtle hyperintensity was observed in the left frontal and parietal lobes, as indicated by brain MRI. A meningeal biopsy revealed non-specific inflammation with the absence of rheumatoid nodules. The patient was given IVIg on day 7 after admission. The clinical symptoms were relieved, the lesions were alleviated, and abnormal biochemical indicators were gradually recovered 1 week after initiation of the treatment, while NMDAR Abs were present in CSF even after treatment. After 5 months of follow-up, the patient's serum and CSF ACPA and IL-6 levels were still high. The findings showed that brain MRI was adequate for the diagnosis of RM. ACPA and IL-6 might be the specific biomarkers for disease activity in RM. IVIg was effective as induction therapy for RM. Further studies should explore whether the presence of NMDAR Abs is associated with RM.

Endoscopic Repair of Round Window Cochleocele.

OBJECTIVE: To describe the novel presentation, implications, and endoscopic management of a congenital round window cochleocele. PATIENT: A 16-month old girl with profound hearing loss from bilateral incomplete partition type 1 (IP1) anomaly of the cochlea plus left-sided cochlear nerve aplasia and cochleocele. INTERVENTION: Anomalies were identified with computed tomographic scanning and magnetic resonance imaging during cochlear implant candidacy assessment. While under general anesthesia for right-sided cochlear implantation, the cochleocele was removed and packed with temporalis fascia using transcanal endoscopic ear surgery. The endoscope was held by an assistant while the surgeon packed the round window using a two-handed technique to counter the gush of cerebrospinal fluid. RESULTS: The child made an uneventful recovery with no cerebrospinal fluid leak or meningitis. CONCLUSIONS: Cochleocele can protrude through the round window of a cochlea with incomplete partition type 1 anomaly. An increased risk of meningitis secondary to acute otitis media is expected given the known risk from cochleocele arising through a stapes footplate fistula. Care should be taken to check for the presence of a cochleocele in hearing loss from congenital malformations involving the basal turn of the cochlea on imaging and also at the time of cochlear implant surgery. Repair should be considered at the first opportunity to prevent meningitis. If early cochlear implant surgery is not feasible or appropriate (as, for example, with cochlear nerve aplasia), transcanal endoscopic ear surgery provides good access for a low-morbidity approach.

[Meningitis, Encephalitis, and Myelitis].

Infections of the central nervous system such as meningitis, encephalitis, and myelitis are a life-threatening neurological emergences. Early recognition, efficient decision-making, and rapid initiation of therapy can be lifesaving. Adequate clinical management of central nervous system infections is important for neurosurgeons during neurological emergences. This article describes recent trends in central nervous system infections and the pitfalls faced by neurosurgeons.

IgG4-related hypertrophic pachymeningitis with tumor-like intracranial and intracerebral lesions.

OBJECTIVE: IgG4-related hypertrophic pachymeningitis is a rare fibroinflammatory disorder that may cause localized or diffused thickening of the dura mater. Misinterpretations of the clinical and imaging findings are common. Clinical manifestations depend on the location of the inflammatory lesion and on compression of neural structures leading to functional deficits. A dural biopsy is commonly needed for a definitive diagnosis. Immunomodulatory therapy is considered the therapy of choice. METHODS: Four patients with IgG4-related hypertrophic pachymeningitis were identified over a 5-year period. Patient-related characteristics including age, preoperative workup, signs and symptoms of patients, and diagnostic procedures were evaluated. Furthermore, the surgical treatment and 5-year follow-up outcomes were analyzed. RESULTS: There were two adults and two adolescents (mean age 32 years; range 15 to 67 years). Two patients were male, and two were female. No history of disease was known in any of the patients. Clinical symptoms were epilepsy (n = 2), ataxia and nausea (n = 1), and facial nerve palsy (n = 1). MR imaging studies showed contrast enhancing lesions in the temporal region in two patients, and in the cerebellar region in the other two patients. Subtotal resection was performed in two instances and a biopsy via a suboccipital retrosigmoid approach was obtained in the other two patients. Histochemical and immunohistochemical investigations revealed an IgG 4 disease in all of these patients. Immunomodulatorry therapy led to clinical stability during follow-up of 5 years in all four cases. CONCLUSION: The diagnosis of IgG4-related hypertrophic pachymeningitis is challenging, but is of great relevance as treatment differs significantly from other forms of pachymeningitis and a specific therapeutic approach may avoid long-term neurological complications. Our series contributes to a better clinical characterization of this rare disease.