Granulomatosis With Polyangiitis of Spinal Dura Presenting With "Bottle Brush Sign" on 18 F-FDG PET/CT.

ABSTRACT: A 72-year-old man with fever and weakness in both lower limbs underwent thoracolumbar MRI and 18 F-FDG PET/CT. The PET/CT scan revealed diffused FDG uptake along the spinal dura mater from T7 to S2 level like a "bottle brush." Pathologic examination after biopsy of spinal canal lesions manifested granulomatous inflammation. The blood test showed cytoplasmic antineutrophil cytoplasmic antibody (ANCA) and myeloperoxidase-ANCAs were positive, whereas the perinuclear ANCA was negative. Eventually, he was diagnosed with granulomatosis with polyangiitis.

Duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression for pediatric Chiari malformation type 1 with syrinx.

PURPOSE: In posterior fossa decompression for pediatric Chiari malformation type 1 (CM-1), duraplasty methods using various dural substitutes have been reported to improve surgical outcomes and minimize postoperative complications. To obtain sufficient posterior fossa decompression without cerebrospinal fluid-related complications, we developed a novel duraplasty technique using a combination of a pedicled dural flap and collagen matrix. The objective of this study was to describe the operative nuances of duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression for pediatric CM-1. METHODS: We reviewed the clinical and radiographic records of 11 consecutive pediatric patients who underwent posterior fossa decompression with duraplasty using a combination of a pedicled dural flap and collagen matrix followed by expansile cranioplasty for CM-1. The largest area of the syrinx and the size of the posterior fossa were calculated. RESULTS: The maximum syrinx area was reduced by a mean of 68.5% ± 27.3% from preoperatively to postoperatively. Four patients (36.4%) had near-complete syrinx resolution (> 90%, grade III reduction), five (45.5%) had 50% to 90% reduction (grade II), and two (18.2%) had < 50% reduction (grade I). The posterior fossa area in the midsagittal section increased by 8.9% from preoperatively to postoperatively. There were no postoperative complications, including cerebrospinal fluid leakage, pseudomeningocele formation, or infection. CONCLUSION: Duraplasty using a combination of a pedicled dural flap and collagen matrix in posterior fossa decompression is a promising safe and effective surgical technique for pediatric CM-1 with syrinx.

An Intraosseous Pterygopalatine Fossa Dural Arteriovenous Fistula.

Intraosseous dural arteriovenous fistulas (DAVFs) are distinct in that the fistula is located within the bone rather than the dura through which the dural vessels pass. It has been stated that only fistulas within marrow should be considered as intraosseous DAVFs rather than DAVFs with traditional angioarchitecture that erode into bone or are located within a bony foramen. The ambiguity in the definition may have contributed to the oversight and scarcity of relevant cases reported in the literature. Three- or four-dimensional digital subtraction angiography is useful for determining the location of the fistula and developing treatment plans. We present an intraosseous DAVF around the pterygopalatine fossa using a transvenous approach.

A selected case series of idiopathic hypertrophic pachymeningitis in a single center: Pathological characteristics and case-oriented review.

Hypertrophic pachymeningitis (HP) is a relatively rare disease of the central nervous system characterized by local or diffuse fibrous thickening of the dura mater. At present, there is still insufficient research on the pathogenesis and treatment strategies of this disease. We reported a continuous case series of seven patients with idiopathic HP (IHP), and also details one case of immunoglobulin G4-related HP requiring surgical intervention. Early diagnosis and appropriate surgical intervention for IHP could prevent the progression of permanent neurological damage and spinal cord paraplegia.

IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma.

Hypertrophic pachymeningitis is a rare disorder of the dura mater of the spine or brain. It can be caused by inflammatory, infective or neoplastic conditions or can be idiopathic. We report a man with hypertrophic pachymeningitis and bilateral chronic subdural haematoma caused by IgG4-related disease. We highlight the diagnostic challenges and discuss possible underlying mechanisms of subdural haematoma formation in inflammatory conditions. Isolated IgG4-related hypertrophic pachymeningitis with chronic subdural haematoma is very rare; previously reported cases have suggested a possible predilection for men in their sixth decade, presenting with headache as the dominant symptom. Given the rarity and complexity of the condition, it should be managed in a multidisciplinary team setting.

A Three-Dimensional Anterior and Middle Cranial Fossa Model for Skull Base Surgical Training with Two Layers of the Colored Dura Mater.

BACKGROUND: Adequate epidural procedures and anatomical knowledge are essential for the technical success of skull base surgery. We evaluated the usefulness of our three-dimensional (3D) model of the anterior and middle cranial fossa as a learning tool in improving knowledge of anatomy and surgical approaches, including skull base drilling and dura matter peeling techniques. METHODS: Using a 3D printer, a bone model of the anterior and middle cranial fossa was created based on multi-detector row computed tomography data, incorporating artificial cranial nerves, blood vessels, and dura mater. The artificial dura mater was painted using different colors, with 2 pieces glued together to allow for the simulation of peeling the temporal dura propria from the lateral wall of the cavernous sinus. Two experts in skull base surgery and 1 trainee surgeon operated on this model and 12 expert skull base surgeons watched the operation video to evaluate this model subtlety on a scale of 1 to 5. RESULTS: A total of 15 neurosurgeons, 14 of whom were skull base surgery expert, evaluated, scoring 4 or higher on most of the items. The experience of dural dissection and 3D positioning of important structures, including cranial nerves and blood vessels, was similar to that in actual surgery. CONCLUSIONS: This model was designed to facilitate teaching anatomical knowledge and essential epidural procedure-related skills. It was shown to be useful for teaching essential elements of skull-base surgery.

Assessment of Meningeal Lymphatics in the Parasagittal Dural Space: A Prospective Feasibility Study Using Dynamic Contrast-Enhanced Magnetic Resonance Imaging.

OBJECTIVE: Meningeal lymphatic vessels are predominantly located in the parasagittal dural space (PSD); these vessels drain interstitial fluids out of the brain and contribute to the glymphatic system. We aimed to investigate the ability of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in assessing the dynamic changes in the meningeal lymphatic vessels in PSD. MATERIALS AND METHODS: Eighteen participants (26-71 years; male:female, 10:8), without neurological or psychiatric diseases, were prospectively enrolled and underwent DCE-MRI. Three regions of interests (ROIs) were placed on the PSD, superior sagittal sinus (SSS), and cortical vein. Early and delayed enhancement patterns and six kinetic curve-derived parameters were obtained and compared between the three ROIs. Moreover, the participants were grouped into the young (< 65 years; n = 9) or older (≥ 65 years; n = 9) groups. Enhancement patterns and kinetic curve-derived parameters in the PSD were compared between the two groups. RESULTS: The PSD showed different enhancement patterns than the SSS and cortical veins (P < 0.001 and P < 0.001, respectively) in the early and delayed phases. The PSD showed slow early enhancement and a delayed wash-out pattern. The six kinetic curve-derived parameters of PSD was significantly different than that of the SSS and cortical vein. The PSD wash-out rate of older participants was significantly lower (median, 0.09; interquartile range [IQR], 0.01-0.15) than that of younger participants (median, 0.32; IQR, 0.07-0.45) (P = 0.040). CONCLUSION: This study shows that the dynamic changes of meningeal lymphatic vessels in PSD can be assessed with DCE-MRI, and the results are different from those of the venous structures. Our finding that delayed wash-out was more pronounced in the PSD of older participants suggests that aging may disturb the meningeal lymphatic drainage.

An unusual finding of the Porus trigeminus: Case report with histological findings.

Many anatomical variants can potentially contribute to trigeminal neuralgia. These include compression by the superior cerebellar artery and less commonly, bony compression near the trigeminal cave. Herein, we report the gross and histological findings of a cadaveric specimen found to have a bony roof of the trigeminal cave. During the routine dissection of a male cadaver, an unusual finding of the skull base was observed. Palpation over the porus trigeminus identified a completely ossified roof. The bony spicule was 1.22 cm long and 0.76 mm wide. The trigeminal nerve was noted to have an indented region just below its contact with the ossified roof of the porus trigeminus. No frank nerve degeneration was noted with histological analysis. Normal mature bone tissue was noted surrounded by a sheath of dura mater. Future radiographic research is needed to better elucidate if ossification of the roof of the trigeminal cave is related to clinical symptoms of trigeminal neuralgia (TN). However, physicians should be cognizant of radiographic ossification of the trigeminal cave as a potential cause of TN.

Immune-Mediated Hypertrophic Pachymeningitis and its Mimickers: Magnetic Resonance Imaging Findings.

Hypertrophic pachymeningitis (HP) is a rare and chronic inflammatory disorder presenting as localized or diffuse thickening of the dura mater. It can be idiopathic or an unusual manifestation of immune-mediated, infectious, and neoplastic conditions. Although some cases may remain asymptomatic, HP can lead to progressive headaches, cranial nerve palsies, hydrocephalus, and other neurological complications, which makes its recognition a fundamental step for prompt treatment. Regarding the diagnosis workup, enhanced MRI is the most useful imaging method to evaluate dural thickening. This article addresses the MR imaging patterns of immune-mediated HP, including immunoglobulin G4-related disease, neurosarcoidosis, granulomatosis with polyangiitis, rheumatoid pachymeningitis, and idiopathic HP. The main infectious and neoplastic mimicking entities are also discussed with reference to conventional and advanced MR sequences.

Extensive Foreign Body Reaction to Synthetic Dural Replacement After Decompressive Craniectomy with Radiological and Histopathology Evidence: Observational Case Series.

BACKGROUND: Though the indications are quite varied, decompressive craniectomy is considered a life-saving procedure. Maximal effectiveness of craniectomy is achieved when, in addition to bone removal, the dura mater is opened properly and is augmented with duraplasty. Different synthetic materials have been used over the decades to replace the dura during decompressive craniectomy. We have used different synthetic dural replacements at our institution, including Neuro-Patch, DuraGen, and Lyoplant. In this case series, we described 4 cases that had excessive granulation tissue formation in response to a newly used synthetic dural substitute (ReDura) after emergent decompressive craniectomy. During follow-up brain imaging at different intervals, these cases were found to have foreign body reaction in the form of excessive granulation tissue formation; additionally, 1 case had a sterile pus-like collection. The granulation tissue diagnosis was affirmed by histopathology in all 4 cases. METHODS: This study was an observational retrograde case series, with data obtained from electronic medical records. RESULTS: The study showed extensive foreign body giant cell reactions on preoperative computed tomography scans, indicating a very high occurrence rate of 72.4%, when ReDura was used as dural replacement. CONCLUSIONS: Our experience showed that patients are prone to develop severe foreign body giant cell reactions with ReDura. Neurosurgical centers using this material should monitor patients for possible abnormal foreign body reaction and report it to establish the safety and efficacy profile of this material.

Parasagittal Cystic Lesions May Arise from the Pial Sheath around the Cortical Venous Wall.

It has been reported that perivenous cystic structures near the parasagittal dura are associated with the leakage of gadolinium-based contrast agents at 4 hours after intravenous administration. The origin of such cystic structures remains unknown. While reading many cases of MR cisternography, we noticed that some of the cystic structures appeared to connect to the perivenous subpial space. This new imaging finding might facilitate future research of the waste clearance system for the central nervous system.

Traumatic acute convexity interdural hematoma: a case report and literature review.

INTRODUCTION: Interdural hematoma (IDH) is an extremely rare hemorrhage between the outer periosteal dura mater and the inner meningeal dura mater. There are 8 cases of convexity IDH reported previously but none of them were acute post traumatic one. We report the case of a patient with an initial diagnosis of acute epidural hematoma (EDH) that was eventually revealed to be an acute convexity IDH. CASE REPORT: A 57-year-old man presented to the emergency department with a complaint of falling from 2 meters. Imaging findings revealed an expanding intracranial hematoma with a linear skull fracture extending to sagittal suture, which was mistaken as an EDH. Emergent surgical evacuation was performed; the hematoma was lodged between two dural layers. DISCUSSION: This is the first case of acute convexity IDH following trauma that has been reported. In chronic and sub-acute hematomas MRI scans could be very useful, but in acute cases under emergent circumstances, CT scan cannot make a proper differentiation between an EDH and IDH. IDH should be considered during craniotomy when extradural findings do not explain the CT scan findings. This case showed a linear skull fracture expanding to the sagittal suture, and this can be a possible explanation for IDH formation. Surgical evacuation of the hematoma without inner nor outer layer resection and leaving the outer layer open, to connect the interdural space to epidural space was first described in this report.

Comparison of Clinical and Radiologic Outcomes between Dural Splitting and Duraplasty for Adult Patients with Chiari Type I Malformation.

BACKGROUND: The most used surgical procedure in the treatment of patients with Chiari type I malformation (CIM) is posterior fossa decompression. However, no consensus has been reached regarding the superiority of either dural splitting or duraplasty. Thus, the aim of this study was to compare clinical and radiologic outcomes between the two techniques used in consecutive patients. METHODS: We retrospectively reviewed 74 adult patients with CIM who were diagnosed and treated surgically between 2015 and 2020 at our neurosurgery department. The patients were divided into two groups: dural splitting in group 1 and duraplasty in group 2. Clinical outcomes based on Chicago Chiari Outcome Scale (CCOS) scores at the last control visits were compared between the groups. Radiologic outcomes were compared in terms of tonsillar regression rate based on 12-postoperative-month magnetic resonance images. RESULTS: Overall improved, unchanged, and worsened neurologic statuses were observed in 75.6% (n = 56), 17.5% (n = 13), and 6.7% (n = 5) of our patients, respectively. The mean last visit CCOS scores in groups 1 and 2 were 12.3 ± 2.1 and 13.5 ± 1.7, respectively. The difference between the groups was statistically significant (p < 0.01). The mean tonsillar regression rates were 34.7 ± 17.0% and 52.1 ± 15.3% in groups 1 and 2, respectively, with a statistically significant difference (p < 0.001). CONCLUSION: Adult patients undergoing duraplasty had better clinical and radiologic outcomes than those treated with dural splitting. Therefore, we recommend decompression with duraplasty for adult CIM patients.

Optic nerve sheath diameter in intracranial hypertension: Measurement external or internal of the dura mater?

BACKGROUND AND PURPOSE: Optic nerve sheath diameter (ONSD) is a promising metric to estimate intracranial pressure (ICP). There is no consensus whether ONSD should be measured external (ONSDext) or internal (ONSDint) of the dura mater. Expert opinion favors ONSDint, though without clear evidence to support this. Adjustments of ONSD for eye diameter (ED) and optic nerve diameter (OND) have been suggested to improve precision. We examined the diagnostic accuracy of ONSDext and ONSDint for estimating ICP, unadjusted as well as adjusted for ED and OND. METHODS: We performed an observational cohort study, measuring ONSDext and ONSDint in patients with invasive ICP monitoring at Karolinska University Hospital in Stockholm, Sweden. We used ONSDext and ONSDint unadjusted as well as adjusted for ED and for OND. We compared the area under the receiver operator characteristics curve (AUROC) for these methods. Thresholds for elevated ICP were set at ≥20 and ≥22 mmHg, respectively. RESULTS: We included 220 measurements from 100 patients. Median ONSDext and ONSDint were significantly different at 6.7 and 5.2 mm (p = .00). There was no significant difference in AUROC for predicting elevated ICP between ONSDext and ONSDint (.67 vs. .64, p = .31). Adjustment for ED yielded better diagnostic accuracy (AUROC, cutoff, sensitivity, specificity) for ONSDext/ED (.76, .29, .81, .62) and ONSDint/ED (.71, .24, .5, .89). CONCLUSIONS: ONSDext and ONSDint differ significantly and are not interchangeable. However, there were no significant differences in diagnostic accuracy between ONSDext and ONSDint. Adjustment for ED may improve diagnostic accuracy of ONSD.

Intracranial dural arteriovenous fistula: a comprehensive review of the history, management, and future prospective.

Dural arteriovenous fistulas (DAVF) are abnormal acquired intracranial vascular malformations consisting of pathological connections located within the dura between the pial arteries and the veno vasora, comprising the walls of the dural sinuses, bridging veins, or transosseous emissary veins. Dural arteriovenous fistulas are distinguished from arteriovenous malformations by their arterial supply from the vessels that perfuse the dura mater and lack of a parenchymal nidus. They are most commonly situated at the transverse and cavernous sinuses. The mechanism of development behind dural arteriovenous fistula can be explained by the molecular and anatomical factors. Multiple classification systems have been proposed throughout history including; Djindjian and Merland, Cognard, and Borden classification systems. The aggressiveness of the clinical course in intracranial dural arteriovenous fistula can be predicted through the angiographic patterns of venous drainage, more specifically, the presence of cortical venous drainage, the presence of venous ectasia, and the aggressiveness of clinical presentation. Intracranial dural arteriovenous fistulas might be discovered incidentally. However, if symptomatic, the clinical presentation ranges from mild neurological deficits to severe, lethal intracranial hemorrhage. Angiography is the imaging of choice to investigate, diagnose, and plan treatment for intracranial dural arteriovenous fistula. The management algorithm of intracranial dural arteriovenous fistula can be broadly divided into conservative, surgical, endovascular, and/or radiosurgical options. With the advent of endovascular therapies, surgery has fallen out of favor for managing intracranial dural arteriovenous fistulas. In the present article, the pathophysiology, classifications, natural history, clinical manifestations, radiological features, management, and complications are comprehensively reviewed.