Lumbar Epidural Lipomatosis is Increased in Patients With Morbid Obesity and Subsequently Decreases After Bariatric Surgery.

BACKGROUND: Spinal epidural lipomatosis (SEL) is characterized by symptomatic neurogenic compression from adipose tissue in the spinal canal. The question arises whether patients with morbid obesity have higher volumes of epidural adipose tissue (EAT) in their lumbar spinal canal compared with patients with a normal weight, and to what extent this decreases after bariatric surgery. METHODS: In this explorative study the lumbar EAT volume was assessed in 25 patients with morbid obesity (body mass index [BMI] >40) using available lumbar magnetic resonance imaging (MRI) prior to their bariatric surgery. An age- and sex-matched control group (n = 25) of patients with a normal weight (BMI 20-25) was used for comparison. Participants from the obesity group underwent a postoperative control MRI. RESULTS: The mean volume of EAT per MRI slice of the group of patients with obesity was significantly higher than for the group of patients with normal weight (mean: 83.3 ± 30.7 mm3 vs. mean 56.5 ± 25 mm3; P < 0.001). Fifteen participants with obesity (15 of 25) agreed to undergo a follow-up MRI. There was a significant decrease in EAT volume per MRI slice for these 15 participants (mean 82 ± 25.5 mm3 vs. 46 ± 20.0 mm3; P < 0.001) over time up to 3.6 (range: 1.2-6.0) years after bariatric surgery. CONCLUSIONS: Patients with obesity have significantly larger volumes of EAT in comparison with patients with normal weight. After bariatric surgery, a significant weight loss coincided with a significant volumetric reduction of this adipose tissue in the spinal canal. Future prospective studies in patients with symptomatic SEL may elucidate whether decreases in EAT volume influence concurrent neurogenic claudication symptoms.

Cerebral large artery occlusion in chronic graft-versus-host disease: A case report.

RATIONALE: Cerebral large artery occlusion in chronic central nervous system graft-versus-host disease after allogeneic hematopoietic stem cell transplantation (allo-HSCT) was very scarce. We described a young patient with bilateral white matter lesions and symptomatic internal carotid artery occlusion after allo-HSCT with the history of aplastic anemia. PATIENT CONCERNS: A 17-year-old girl with the history of aplastic anemia developed recurrent headache and sudden hemiplegia of right limbs 2 years after allo-HSCT. DIAGNOSES: She was diagnosed with skin chronic graft-versus-host disease 19 months after allo-HSCT. Brain magnetic resonance imaging showed bilateral subcortical white matter abnormal signals and hyperintensity of left fronto-parietal lobe on diffusion weighted imaging and corresponding hypointense apparent diffusion coefficients indicating acute infarction. CT angiography revealed thrombosis in left internal carotid artery. Carotid plaque high-resolution magnetic resonance imaging showed annular enhancement of vascular wall revealing signs of vasculitis. INTERVENTIONS: Intravenous immunoglobulin, methylprednisolone, and anticoagulant therapy were used to treat the patient. OUTCOMES: The patient's symptoms gradually resolved and she could walk with assistance after 3 weeks before returned home. LESSONS: Chronic graft-versus-host disease-associated vasculitis could involve cerebral large vessels which warrants further study.

The Evolving Role of Induced Pluripotent Stem Cells and Cerebral Organoids in Treating and Modeling Neurosurgical Diseases.

Over the past decade, the use of induced pluripotent stem cells (IPSCs), as both direct therapeutics and building blocks for 3D in vitro models, has exhibited exciting potential in both helping to elucidate pathogenic mechanisms and treating diseases relevant to neurosurgery. Transplantation of IPSCs is being studied in neurological injuries and diseases, such as spinal cord injury and Parkinson's disease, whose clinical manifestations stem from underlying neuronal and/or axonal degeneration. Both animal models and clinical trials have shown that IPSCs have the ability to regenerate damaged neural tissue. Such evidence makes IPSCs a potentially promising therapeutic modality for patients who suffer from these neurological injuries/diseases. In addition, the cerebral organoid, a 3D assembly of IPSC aggregates that develops heterogeneous brain regions, has become the first in vitro model to closely recapitulate the complexity of the brain extracellular matrix, a 3-dimensional network of molecules that structurally and biochemically support neighboring cells. Cerebral organoids have become an exciting prospect for modeling and testing drug susceptibility of brain tumors, such as glioblastoma and metastatic brain cancer. As patient-derived organoid models are becoming more faithful to the brain, they are becoming an increasingly accurate substitute for patient clinical trials; such patient-less trials would protect the patient from potentially ineffective drugs, and speed up trial results and optimize cost. In this review, we aim to describe the role of IPSCs and cerebral organoids in treating and modeling diseases that are relevant to neurosurgery.

Molecular Association of Medulloblastoma and Sarcoidosis: Case Report and Review of the Literature.

BACKGROUND: Medulloblastoma (MB) is a malignant tumor of the central nervous system (CNS), and sarcoidosis is a chronic inflammatory disease of many organ systems, commonly affecting the lungs. No association between MB and sarcoidosis was described in the literature. MB and sarcoidosis have mutual molecular and signaling pathways that may predispose patients with sarcoidosis to develop MB. We describe a patient with sarcoidosis who developed MB. CASE DESCRIPTION: The patient is a 36-year-old diagnosed with pulmonary sarcoidosis presented with ataxia, bilateral horizontal nystagmus, diplopia, and bilateral upper limb dysmetria was found to have a cerebellar mass on magnetic resonance imaging (MRI). He was initially treated with corticosteroids as a case of neurosarcoidosis. The patient's symptoms worsened, and repeat MRI showed an increase in the tumor size with hydrocephalus. External ventricular drain insertion plus midline suboccipital craniotomy and resection of the tumor was performed. Pathology revealed MB classic type, sonic hedgehog-activated. There was no cerebrospinal fluid dissemination. He received craniospinal radiation and chemotherapy. Follow-up 20 months after radiation revealed residual neurologic symptoms and no recurrence on MRI brain. CONCLUSIONS: The exceedingly rare coexistence of adult MB and sarcoidosis may have a causal relationship based on specific common molecules. Leukotrienes, stimulation of astrocytes and Purkinje neurons, and the sonic hedgehog signaling pathway can be considered. Further genetic and molecular studies are merited.

A rare case of spinal epidural sarcoidosis: case report and review of the literature.

Sarcoidosis is a rare systemic disease characterized by growth and organization of inflammatory cells in a granuloma. Granulomas can localize in any parts of the human body. The main localization is represented by lungs, lymph nodes, eyes and skin. Any organ, however, can be affected. Central nervous system (CNS) represents a rare localization of sarcoidosis, in fact, only 1% of patient with sarcoidosis present brain and/or spinal cord localization of the granulomas associated with this disease. This condition takes the name of Neurosarcoidosis. Its diagnosis and management pose a significant challenge, as its clinical manifestation and appearance in imaging studies are difficult to distinguish from more common lesion of the spinal cord. In this paper, we present the case of a 45-year-old woman who presented back pain associated with neurologic signs of myelopathy. Spinal cord MRI documented a T2 hyperintense signal around medullary conus. She underwent a gross total removal of the lesion. Histopathological examination revealed spinal sarcoidosis. Next total body CT scan did not show other localization of the disease. The aim of the present paper is to report a very rare case of spinal epidural sarcoidosis, actually only five cases have been described, without other localization of the disease. The present article underlines the difference between this forms of spinal sarcoidosis compared to intradural extramedullary and intramedullary spinal sarcoidosis.

Differences in Electrophysiologic Values between Preoperative and Intraoperative Neurophysiologic Monitoring.

AIM: To compare preoperative and intraoperative electrophysiologic values in patients who underwent brain and spine surgeries. MATERIAL AND METHODS: A retrospective medical record review was conducted on patients who underwent brain and spine surgery between January 2014 and July 2018. Patients underwent preoperative electrophysiologic monitoring within a week before the operation and intraoperative monitoring during the surgery. Monitoring parameters included the onset latency (msec) and amplitude (mV) of motor evoked potential (MEP), somatosensory evoked potential (SEP), electroneuronography, and brainstem auditory evoked potential (BAEP). RESULTS: The latency of the MEP and SEP were significantly prolonged while the amplitude was decreased during operation. Specifically, patients with abnormal findings in their preoperative assessments showed more prominent differences compared to those with normal findings. However, there was no significant difference between preoperative and intraoperative results based on which side of the hemisphere was affected or unaffected. Unlike the MEP and SEP parameters, there were no significant changes in the facial electroneuronography and BAEP parameters. CONCLUSION: There were differences in the preoperative and intraoperative monitoring parameters. Further studies are necessary to understand the underlying mechanisms behind these changes during surgery.

Endoscopic Management for Recurrent Hydrocephalus Associated with Neurosarcoidosis.

BACKGROUND: Recurrent hydrocephalus may occur as a complication of neurosarcoidosis with chronic inflammation. We present a case that required a combination of multistage endoscopic diversion of the cerebrospinal fluid pathway and shunt surgery. CASE DESCRIPTION: A 34-year-old man presented with progressive nausea and vomiting. Magnetic resonance imaging revealed hydrocephalus with leptomeningeal enhancement along the base of the fourth ventricle and the bilateral foramina of Luschka. Concurrent endoscopic third ventriculostomy and biopsy were performed. The diagnosis was neurosarcoidosis. Immediately after the procedure, the endoscopic third ventriculostomy stoma was occluded, and a right ventriculoperitoneal shunt was urgently performed. However, left unilateral hydrocephalus developed during the late phase of immunosuppressive therapy for neurosarcoidosis. Endoscopic septostomy with repositioning of the ventricular catheter was indicated. Intraoperative findings included a white pasty tissue with nodules that covered the ventricular wall close to the foramen of Monro and sealed the side holes of the catheter. Chemotherapy with a tumor necrosis factor-α inhibitor was initiated after the surgical procedure. The patient had an uneventful course without recurrence of hydrocephalus for >6 months. CONCLUSIONS: Endoscopic diversion of the cerebrospinal fluid pathway should be actively considered for treating hydrocephalus without a shunt and performing biopsy simultaneously. Even if a subsequent shunt is needed, complex hydrocephalus can be avoided with a combination of endoscopic techniques.

COVID-19 Impact on Neurosurgical Practice: Lockdown Attitude and Experience of a European Academic Center.

BACKGROUND: The coronavirus disease 2019 (COVID-19) pandemic is an unprecedented challenge. Different models of reorganization have been described aiming to preserve resources and ensure optimal medical care. Limited clinical neurosurgical experience with patients with COVID-19 has been reported. We share organizational experience, attitudes, and preliminary data of patients treated at our institution. METHODS: Institutional guidelines and patient workflow are described and visualized. A cohort of all neurosurgical patients managed during the lockdown period is presented and analyzed, assessing suspected nosocomial infection risk factors. A comparative surgical subcohort from the previous year was used to investigate the impact on surgical activity. RESULTS: A total of 176 patients were admitted in 66 days, 20 of whom tested positive for COVID-19. Patients initially admitted to the neurosurgical ward were less likely to be suspected for a COVID-19 infection compared with patients admitted for critical emergencies, particularly with neurovascular and stroke-related diseases. The mortality of patients with COVID-19 was remarkably high (45%), and even higher in patients who underwent surgical intervention (77%). In addition to the expected decrease in surgical activity (-53%), a decrease in traumatic emergencies was noted. CONCLUSIONS: By applying infection prevention and resource-sparing logistics measures shared by the international medical community, we were able to maintain essential neurosurgical care in a pandemic with controlled nosocomial infection risk. Special consideration should be given to medical management and surgical indications in patients infected with severe acute respiratory syndrome coronavirus 2, because they seem to show a problematic hemostatic profile that might result in an unfavorable clinical and surgical outcome.

Risk Factors for Patient-Reported Olfactory Dysfunction After Endoscopic Transsphenoidal Hypophysectomy.

Importance: Iatrogenic olfactory dysfunction after endoscopic transsphenoidal hypophysectomy (ETSH) is an overlooked complication without elucidated risk factors. Objective: To assess the independent prognostic role of demographic, comorbidity, cephalometric, intraoperative, histological, and postoperative parameters in patient-reported postoperative olfactory dysfunction, and to explore the association between anatomical measurements of the skull base and sinonasal cavity and postoperative olfactory dysfunction. Design, Setting, and Participants: This retrospective cohort study in a tertiary care medical center enrolled consecutive patients with primary sellar lesions who underwent ETSH between January 1, 2015, and January 31, 2019. Patients were excluded if they underwent multiple sinonasal surgical procedures, presented with a sellar malignant neoplasm, required an expanded transsphenoidal approach, had nasal polyposis or a neurodegenerative disease, or sustained traumatic brain injury. After undergoing medical record review and telephone screening, patients were asked to participate in a 3-item telephone survey. Main Outcomes and Measures: The primary outcome was the Clinical Global Impressions change in smell rating, a validated transitional patient-reported outcome measure. Patients rated their change in smell before and after ETSH on a 7-point Likert scale, with the following response options: (1) much better, (2) somewhat better, (3) slightly better, (4) neither better nor worse, (5) slightly worse, (6) somewhat worse, or (7) much worse. Responses of slightly worse, somewhat worse, and much worse were surrogates for postoperative olfactory dysfunction status. Patient medical records, preoperative imaging scans, operative notes, and pathology reports were reviewed. Results: Of the 147 patients (mean [SD] age, 54 [15] years; 79 women [54%]) who responded to the telephone survey, 42 (29%) reported olfactory dysfunction after ETSH. Median (interquartile range [IQR]) time between the ETSH completion and survey response was 31.1 (21-43) months. On multivariable analysis, abdominal fat grafting (adjusted relative risk [aRR], 2.95; 95% CI, 1.89-4.60) was associated with postoperative olfactory dysfunction, whereas smoking history (aRR, 1.54; 95% CI, 0.95-2.51) demonstrated a clinically meaningful but imprecise effect size. A more obtuse angle between the planum sphenoidale and face of the sella turcica on sagittal imaging was protective (aRR, 0.98; 95% CI, 0.96-0.99). Increased number of months after the ETSH was associated with patient-reported normosmia (aRR, 0.93; 95% CI, 0.91-0.95). In contrast, other comorbidities; intraoperative variables such as turbinate resection, nasoseptal flap, and mucosal or bone grafting; histological variables such as pathology and proliferative index; and postoperative variables such as adjuvant radiotherapy were not associated with postoperative olfactory dysfunction. Conclusions and Relevance: This study found that abdominal fat grafting, acute skull base angle, and smoking history appeared to be clinically significant risk factors for patient-reported postoperative olfactory dysfunction. Increased time after ETSH may be associated with better olfactory outcomes.

Epidermoid Cyst of the Lumbar Spine After Lumbar Puncture: A Clinical, Radiographic, and Pathologic Correlation.

BACKGROUND: Epidermoid cysts can rarely arise as a late complication of lumbar puncture. We describe a young man who had a remote history of a lumbar puncture and who was subsequently found to have a lumbar spinal epidermoid cyst on imaging, after presenting with lower extremity radicular pain. CASE DESCRIPTION: A 24-year-old man with a remote history of lumbar puncture presented with lower back pain and radicular leg pain which had been ongoing for over a year. Despite conservative management, the patient's symptoms progressed to worsening back pain and left L4 radiculopathy. Magnetic resonance imaging of the lumbar spine demonstrated a peripherally enhancing, intradural, extramedullary lesion at L4-5. Diffusion-weighted imaging revealed diffusion restriction within the lesion, characteristic of an epidermoid cyst. The patient underwent an L4-5 laminectomy for resection of the intradural tumor. The lesion was noted to contain pearly white granules consistent with the appearance of an epidermoid cyst. Histopathology confirmed the diagnosis. On follow-up examination, the patient demonstrated improvement of his back pain and resolution of radicular symptoms. CONCLUSIONS: Lumbar spinal epidermoid cysts may be either congenital or secondary to an iatrogenic cause. This patient had a remote history of lumbar puncture during workup for meningitis as a child. As a complication of a lumbar puncture, the formation of an epidermoid cyst can occur and is thought to be the result of implanted cutaneous tissue. This case provides a comprehensive illustration of the clinical, radiographic, intraoperative, and pathologic findings consistent with an iatrogenic epidermoid cyst.

Hospital utilization for orbital and intracranial complications of pediatric acute rhinosinusitis.

BACKGROUND: Orbital and intracranial complications of pediatric acute rhinosinusitis (ARS) are uncommon. With a risk of significant morbidity, hospital utilization and the financial burden of these entities are often high. We sought to assess utilization trends for complicated ARS and elucidate which factors influence cost. METHODOLOGY: Analysis of Kids' Inpatient Database (2006, 2009 and 2012). Children were selected based on diagnosis codes for ARS and grouped as: uncomplicated ARS, orbital complications (OC), or intracranial complications (IC). Patients with IC were subdivided into abscess (ICa), meningitis, or sinus thrombosis. Length of stay (LOS), cost and management information were analysed. Data presented as median [IQR]. RESULTS: A weighted total of 20,775 children were included. OC and IC were observed in 10.9% and 2.7% of these patients. LOS was longer for IC compared to OC (9 [8] v 4 [3]days, p < 0.001). Daily cost for IC was greater than OC ($2861 [4044] v $1683 [1187], p < 0.001), likely due to differences in need for surgery (IC 66.3% v OC 37.1%, p < 0.001). Within the ICa group, patients who received both otolaryngologic (ENT) and neurosurgery, compared to neurosurgery alone, had higher total cost ($41,474 [41,976] v $32,299 [18,235], p < 0.001) but similar LOS (12 [10] v 11 [9] days, p = 0.783). CONCLUSIONS: Children with IC required more surgery than their OC counterparts, resulting in a longer LOS and increased cost. Within the ICa group, the addition of ENT surgery to neurosurgery resulted in higher costs, but with a similar LOS. Considering the increased costs, the additional benefit of ENT surgery to those with children with IC should be investigated further.

Neurosarcoidosis Presenting as Isolated Bilateral Cerebellopontine Angle Tumors: Case Report and Review of the Literature.

OBJECTIVES: To describe a unique case of isolated bilateral sarcoidosis of the cerebellopontine angle as well as the related imaging in the case. To conduct a literature review of the published articles regarding sarcoidosis of the cerebellopontine angle. DATA SOURCES: Representative case report from a single institution as well as PubMed and Scopus database searches. METHODS: In addition to a retrospective review, all published case reports and case series of sarcoidosis involving the cerebellopontine angle from 1960 to July 2018 in the English language were reviewed. Demographic data, presenting symptoms, and outcomes were collected. RESULTS: We identified 8 total cases with pertinent clinical information that were included. CONCLUSIONS: Isolated neurosarcoidosis of the cerebellopontine angle is an exceptionally rare phenomenon that, on history and imaging, presents similar to more common retrocochlear pathologies. Surgery may be required in large lesions unresponsive to traditional medical therapy with immunosuppression.

An [18F]-Positron Emitting Fluorophore Allows Safe Evaluation of Small Molecule Distribution in the CSF, CSF Fistulas, and CNS Device Placement.

The small molecule fluorescein is commonly used to guide the repair of cerebral spinal fluid leaks (CSFLs) in the clinic. We modified fluorescein so that it is also visible by positron emission tomography (PET). This probe was used to quantitatively track the fast distribution of small molecules in the CSF of rats. We tested this probe in models relevant to the clinical diagnosis and treatment of central nervous system (CNS) diseases that affect CSF flow. In this study, fluorescein was radiolabeled with fluorine-18 to produce Fc-AMBF3. [18/19F]-Fc-AMBF3 was introduced at trace quantities (13.2 nmols, 100 µCi) intrathecally (between L5 and L6) in rats to observe the dynamic distribution and clearance of small molecules in the CSF by both [18F]-PET and fluorescence (FL) imaging. Murine models were used to demonstrate the following utilities of Fc-AMBF3: (1) utility in monitoring the spontaneous CSFL repair of a compression fracture of the cribriform plate and (2) utility in quantifying CSF flow velocity during neurosurgical lumboperitoneal shunt placement. Fc-AMBF3 clearly delineated CSF-containing volumes based on noninvasive PET imaging and in ex vivo FL histology. In vivo morbidity (n = 16 rats, <2.7 mg/kg, 77 times the PET dose) was not observed. The clearance of the contrast agent from the CNS was rapid and quantitative (t1/2 = 33.8 ± 0.6 min by FL and t1/2 = 26.0 ± 0.5 min by PET). Fc-AMBF3 was cleared from the CSF through the vasculature and/or lymphatic system that supplies the cribriform plate and the temporal bone. Fc-AMBF3 can be used to diagnose CSFLs, image CSFL repair, and determine the CSF flow velocity in the CNS or through lumboperitoneal shunts by PET/FL imaging. In conclusion, Fc-AMBF3 PET imaging has been demonstrated to safely and dynamically quantitate CSF flow, diagnose fistulas associated with the CSF space, and approximate the clearance of small molecules in the CSF.

Radiological and clinical findings of isolated meningeal Rosai-Dorfman disease of the central nervous system.

Rosai-Dorfman disease (RDD) with isolated central nervous system (CNS) involvement is an extremely rare disease. Most RDD of the CNS present as dural-based mass mimicking meningioma and other common lesions, which makes preoperative accurate diagnosis of great difficulty. We searched the pathology database in our hospital and 3 cases of RDD with isolated CNS involvement were finally included in our study. Radiological and clinical findings of these three cases were retrospectively analyzed. The lesions of 2 cases were dura-based against the cerebral convexity, presenting as a sheet-shaped thickened dura mater, another case was located just across the cerebral falx, the dural display in the center was intact. The 3 cases showed low signal intensity on T2-weighted image, obviously enhanced, significantly surrounding edema and finger-like protuberance but no invasion of the brain parenchyma or no sign of hyperplasia or sclerosis of the surrounding cranial bones. In conclusion, when we come across a disease that mimicking meningioma, especially when it manifests as the above radiological features, we should considered it might be a kind of proliferative disease of the meninges, such as RDD.

Accuracy of Intraoperative Examination in Central Nervous System Lesions: A Study of 133 Cases.

OBJECTIVE: Intraoperative examination is a highly valuable tool for the evaluation of central nervous system (CNS) lesions, helping the neurosurgeon to determine the best surgical management. This study aimed to evaluate the accuracy and to analyze the diagnostic disagreements and pitfalls of the intraoperative examinations through correlation with the final histopathological diagnosis in CNS lesions. STUDY DESIGN: Retrospective analysis of intraoperative examination of CNS lesions and their final diagnosis obtained during 16 consecutive years. All diagnoses were reviewed and classified according to World Health Organization (WHO) grading for CNS tumors. Squash was performed in 119 cases, while frozen section and both methods were done in 7 cases each. RESULTS: Among the 133 intraoperative examinations considered, 114 (85.7%) presented concordance and 19 (14.3%) diagnostic disagreement when compared with subsequent histopathological examinations. The sensitivity and specificity for the detection of neoplasia in intraoperative examination was 98 and 94%, respectively. The positive and negative predictive values were 99 and 88%, respectively. The accuracy for neoplastic and nonneoplastic disease was 85.7%. Disagreements were more frequent among low-grade (WHO grades I and II) neoplasms and nonmalignant cases. CONCLUSIONS: Our results showed good accuracy of the intraoperative assessments for diagnosis of CNS lesions, particularly in high-grade (grades III and IV) lesions and metastatic neoplasms.

Surgical management of sacral meningeal cysts by obstructing the communicating holes with muscle graft.

BACKGROUND: The surgical indication and treatment of sacral meningeal cyst have not been well established and current methods are usually accompanied by complications and recurrence. The aim of this study is to discuss the treatment of symptomatic sacral meningeal cyst, by investigating the surgical results of our surgically treated patients, and minimize the complications and recurrence. METHODS: We retrospectively reviewed all patients with symptomatic sacral meningeal cysts who were surgically treated by a single surgeon in the same institution from 2002 to 2017. All patients underwent the same operation by incising the cyst wall and obstructing the communicating hole with muscle graft, while the cyst wall was left untreated instead of resected or imbricated. The obstruction was verified by doing a Valsalva-like maneuver. The preoperative symptoms and signs, and the outcomes at most recent follow-up were rated and compared by Neurological Scoring System. RESULTS: A total of 18 patients (7 male patients and 11 female patients, average age 42.3 years) were followed up for an average of 51.7 months. All patients had communicating holes linking the cysts and the dural sacs. The average preoperative neurological score was 19.7 ± 2.2, and it was improved to 23.2 ± 2.8 at the most recent follow-up (p < 0.01). CONCLUSIONS: The sacral meningeal cyst originated from the communication with the dural sac. Surgical treatment of symptomatic sacral meningeal cysts can yield a long-term resolution of the appropriately selected patient's symptoms. Obstructing the communicating hole with muscle graft is an effective and simple method to obliterate the cyst. The incised cyst wall can be left untreated instead of resected or imbricated.

Neurosarcoidosis Presenting with Obstructive Hydrocephalus Successfully Treated with Endoscopic Third Ventriculostomy.

A 58-year-old Japanese woman complained of unstable gait and dizziness lasting for a month. She had been diagnosed histologically with pulmonary and cutaneous sarcoidosis and attended outpatient clinics for routine checkups. Head computed tomography and magnetic resonance imaging (MRI) indicated obstructive hydrocephalus caused by a contrast-enhanced lesion in the cerebral aqueduct. The patient underwent endoscopic third ventriculostomy and a biopsy of the lesion, leading to the diagnosis of neurosarcoidosis. This was a rare case of neurosarcoidosis presenting with obstructive hydrocephalus that was treated with endoscopic third ventriculostomy and diagnosed histologically via an intraoperative biopsy.

Neurosarcoidosis Presenting Initially as Idiopathic Vocal Cord Paralysis.

INTRODUCTION:: The presentation, course, and management of a rare laryngeal manifestation of neurosarcoidosis due to central nervous system (as opposed to peripheral nervous system) injury are described. METHODS:: The authors present 3 cases of vocal cord paralysis as the initial symptom of isolated neurosarcoidosis at a tertiary care laryngology clinic. RESULTS:: Laryngoscopy diagnosed unilateral vocal cord paralysis. Laryngeal electromyography revealed high vagal injury, prompting workup on brain magnetic resonance imaging. On magnetic resonance imaging, 2 cases showed basilar leptomeningeal inflammation and 1 case showed a brainstem mass. Patients were found at follow-up to have severe, progressive vagal injury, with patients developing severe quality of life impairments and medical complications. CONCLUSIONS:: Neurosarcoidosis is not usually considered in the differential diagnosis of vocal cord paralysis. At initial presentation, all patients lacked other cranial neuropathies and systemic sarcoidosis manifestations, making diagnosis difficult. Otolaryngologists should be aware of this rare presentation, as prompt diagnosis by brain magnetic resonance imaging with or without central nervous system biopsy, as opposed to traditional chest radiography or computed tomography for the workup of peripheral nerve injury, is necessary. Serial laryngeal examinations are recommended for close monitoring of progressive disease and recommending treatment. Injection or medialization laryngoplasty can provide improvements in voicing but not swallow.

Laryngotracheal separation in pediatric patients: 13-year experience in a reference service / Separação laringotraqueal em pacientes pediátricos: 13 anos de experiência em um serviço de referência

ABSTRACT Objective: To evaluate clinical stability of neurologically impaired children and adolescents with recurrent pneumonia submitted to laryngotracheal separation. Methods: Between October 2002 and June 2015, 92 neurologically impaired children from a reference service, with median age of 68.5 months were submitted to laryngotracheal separation. Data were evaluated and statistical analysis was made by Student's t test and Pearson's χ2 test (significance level adopted of 95%). Results: Fifty-three children were male (57.6%). Forty-six children required admission to intensive care, and 42.4% needed mechanical ventilation. We observed that 90.2% of patients were exclusively fed by gastrostomy and 72.4% of the gastrostomies were performed before the tracheal surgery. Thirteen (14.1%) children had postoperative complications as follows: fistulae (5.4%), bleeding (4.3%), granuloma (2.2%) and stenosis (3.2%). A total of 24 patients had pneumonia in the postoperative period (26.1%), but there was a significant drop in occurrence of this condition after surgery (100% versus 26.1%; p<0.001). Twenty-three patients (25%) died. Postoperative complications were similar when comparing patients who died and those that presented good outcome (16.7% versus 13.2%; p=0.73). Conclusion: When well-indicated, the laryngotracheal separation reduces the incidence of postoperative pulmonary infections, thus improving quality of life and reducing admissions to hospital. Laryngotracheal separation should be indicated as a primary procedure in patients with cerebral palsy and recurrent aspiration pneumonia.

RESUMO Objetivo: Avaliar a estabilidade clínica da criança e do adolescente neuropata com episódios de pneumonia de repetição submetidos a procedimento cirúrgico de separação laringotraqueal. Métodos: Entre outubro 2002 a junho 2015, 92 crianças neuropatas de um único serviço com idade mediana de 68,5 meses foram submetidas à separação laringotraqueal. Os dados foram avaliados e foi realizada análise estatística pelo teste t de Student e pelo teste do χ2 de Pearson, com nível de significância adotado de 95%. Resultados: Dentre as 92 crianças, 53 eram do sexo masculino (57,6%). Quarenta e seis crianças necessitaram de internação em unidade de terapia intensiva, e 42,4% fizeram uso de ventilação mecânica. Dessas crianças, 90,2% alimentavam-se exclusivamente via gastrostomia, e 72,4% foram realizadas antes da separação laringotraqueal. As complicações pós-operatórias ocorreram em 13 crianças (14,1%), na seguinte ordem: fístula (5,4%), sangramento (4,3%), granuloma (2,2%) e estenose (3,2%). Observaram-se 24 episódios de pneumonia no período pós-operatório (26,1%). Houve diminuição significativa de ocorrência de pneumonias após a cirurgia (100% versus 26,1%, p<0,001). Óbito foi registrado em 23 pacientes (25%). A frequência de complicações pós-operatórias foi semelhante entre os pacientes que evoluíram ou não para óbito (16,7% versus 13,2%; p=0,73). Conclusão: A cirurgia bem indicada reduz o número de infecção pulmonar após o procedimento, melhorando a qualidade de vida desses pacientes e, consequentemente, reduzindo o número de internações. A separação laringotraqueal deve ser indicada como procedimento primário nos pacientes com paralisia cerebral e episódios repetidos de pneumonia aspirativa.

Imaging-Ambiguous Lesions of Meckel's Cave-Utility of Endoscopic Endonasal Transpterygoid Biopsy.

INTRODUCTION: Meckel's cave is a dural-lined cavity in the middle fossa skull base in which lies the Gasserian ganglion, a potential site for tumors and inflammatory lesions. A variety of lesions can be predominantly isolated to Meckel's cave, including extension from head and neck cancers, other malignant tumors, as well as benign lesions. Clinical presentation and imaging findings are often insufficient to establish a diagnosis. Hence, histologic confirmation is required to determine the appropriate treatment strategy. Several surgical approaches have been used to reach this deep-seated area, often with significant morbidity and prolonged recovery. Given advancements in endoscopy and greater facility with the technique, the endoscopic endonasal approach has been used increasingly to reach lesions in the region. METHODS: A single-institution, retrospective chart review over a 10-year period was performed to identify and describe patients with pathologically differing but imaging-similar lesions with their epicenter in Meckel's cave. RESULTS: Of a total of 21 cases of lesions in Meckel's cave approached by an endoscopic endonasal transpterygoid approach, we present 6 patients with imaging-ambiguous lesions involving Meckel's cave that were biopsied via the extended endoscopic endonasal approach. Among this diverse group, pathology included B-cell lymphoma, squamous cell carcinoma, adenocarcinoma, malignant schwannoma, benign schwannoma, and neurosarcoidosis. CONCLUSIONS: We explore not only the relevance of this approach in the armamentarium of the modern skull-base surgeon but also its limitations and conclude that the endoscopic endonasal approach provides a safe and relatively direct, minimally invasive corridor to many lesions of Meckel's cave.