Cavernous Research Articles

Clinical Presentation, Investigation Findings, and Outcomes of IgG4-Related Pachymeningitis

ImportanceImmunoglobulin G4 (IgG4)–related disease is an increasingly recognized fibroinflammatory condition that can involve multiple organs, including the pachymeninges. The understanding of IgG4-related pachymeningitis (IgG4-RP) remains limited because of its rarity and the predominance of knowledge derived from case reports and case series.ObjectiveTo systematically review and synthesize the clinical presentation, investigation findings, and prognosis of IgG4-RP to better understand its diagnosis and management.Evidence ReviewA comprehensive systematic review was conducted following guidelines from the Preferred Reporting Items for Systematic Reviews and Meta-analyses. PubMed/MEDLINE, Embase, and Scopus were searched from their inception until May 30, 2023, using terms related to IgG4-related disease and pachymeningitis without language or publication restrictions. Case reports and series that met the 2020 Revised Comprehensive Diagnostic Criteria or the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria were included. Data on clinical presentations, investigation findings, and treatment outcomes were extracted and summarized.FindingsA total of 148 case reports contributed data from 208 patients. Their median (IQR) age was 52 (39-62) years; 132 patients were male (63.5%) and 76 female (36.5%). Headache and cranial nerve dysfunctions were the most common neurological manifestations. Systemic involvement was identified in nearly half of the patients. Diagnostic imaging often showed preferential involvement of cavernous sinus and middle fossa. Laboratory results highlighted elevated serum IgG4 levels in 97 of 147 patients (65%) of patients and cerebrospinal fluid pleocytosis in 43 of 82 patients (52%). Storiform fibrosis or obliterating phlebitis were uncommon pathological findings. Mortality was below 1% (1/134; 0.7%), but only a third of patients presented complete clinical improvement, and the recurrence rate was 60 patients (40%) in a median (IQR) follow-up time of 9 (1-20) months. Glucocorticoids were the most commonly prescribed treatment, in 143 of 169 patients (85%); rituximab was prescribed as maintenance therapy in 53 of 169 patients (31%).Conclusions and RelevanceIgG4-RP commonly presents with headaches and cranial nerve dysfunction, posing diagnostic challenges due to the significant absence of systemic manifestations, low IgG4 serum levels, and atypical pathological findings. Current treatment outcomes are limited by incomplete recovery and frequent relapses underscoring the necessity for new treatment strategies.

Invasive Aspergillosis with Cavernous Sinus Thrombosis Following High-Dose Corticosteroid Therapy: A Challenging Case of Rhino-Orbital-Cerebral Mycosis

Invasive aspergillosis is a rare but severe fungal infection primarily affecting immunocompromised individuals. The Coronavirus Disease-2019 (COVID-19) pandemic has introduced new complexities in managing aspergillosis due to the widespread use of corticosteroids for treating COVID-19-related respiratory distress, which can increase susceptibility to fungal infections. Here, we present a challenging case of progressive cerebral aspergillosis complicated by cavernous sinus thrombosis (CST) in a 67-year-old male with a history of COVID-19. The patient, initially misdiagnosed with temporal arteritis, received pulse corticosteroid therapy twice before presenting with persistent left-sided headaches and vision loss. Cranial imaging revealed findings consistent with fungal sinusitis, Tolosa–Hunt syndrome, and orbital pseudotumor, which progressed despite initial antifungal therapy. Subsequent magnetic resonance imaging indicated an invasive mass extending into the left cavernous sinus and other intracranial structures, raising suspicion of aspergillosis. A transsphenoidal biopsy confirmed Aspergillus infection, leading to voriconazole therapy. Despite aggressive treatment, follow-up imaging revealed significant progression, with extension to the right frontal region and left cavernous sinus. The patient then developed visual impairment in the right eye and was diagnosed with CST secondary to fungal sinusitis. Management included a combination of systemic antifungals and antibiotics; however, the patient declined surgical intervention. This case underscores the diagnostic challenges and rapid progression associated with cerebral aspergillosis in post-COVID-19 patients treated with corticosteroids. This report highlights the need for heightened clinical suspicion and prompt, targeted interventions in similar cases to improve patient outcomes. Further research is required to understand the optimal management of invasive fungal infections.

Intracranial angioleiomyoma mimicking meningioma: an uncommon tumor with favorable outcome and frequent GJA4 mutation.

Intracranial angioleiomyoma (IALM) is a rare neoplasm mimicking meningioma. We aimed to explore the clinical and molecular characteristics of IALMs. We included 40 patients with IALMs who had tumor resection at our center from 2009 to 2022. Clinicopathological and radiological characteristics were extracted and analyzed thoroughly. GJA4 mutation status was detected and correlated with clinical characteristics. IALMs accounted for about 10% of all angioleiomyoma and had a significant male predominance compared to extracranial angioleiomyoma (p=0.04). However, there was no age difference between extracranial angioleiomyomas and IALMs. In our IALM cohort, orbital (35%) were the most common tumor location, followed by tentorium (25.0%), cavernous sinuous (17.5%). Vision decrease, diplopia and exophthalmos (40%) were the most common symptom. The radiological characteristics of ILAMs were similar to meningiomas; 20 patients (57.1%) in our cohort were misdiagnosed as meningioma preoperatively. Gross total resection was achieved in all patients. The postoperative pathology showed median Ki-67 index was 1% (range: 0-10%). Vision improvement or exophthalmos relief was achieved in 12 of 16 patients (75.0%). During the long-term follow-up (mean 70.3 months, range 30 - 128 months), no patient experienced tumor recurrence or died of tumor progression, indicating that IALM was clinically benign. GJA4 mutation (p. Gly41Cys) was detected in 17 (42.5%) patients. Of note, the correlations analysis revealed that orbital and cavernous sinus areas (64.7%) were the leading location that harbor GJA4 mutations. Tumors with mutant GJA4 were associated with positive progesterone receptor (PR) expression (p=0.02).This first large case series demonstrated that IALM predominantly affected males, was located in the cavernous sinus and orbital areas, and often manifested visual impairment and diplopia. IALM had favorable outcome. Notably, IALMs frequently exhibited a GJA4 mutation, which was linked to the cavernous sinus and orbital locations, as well as PR expression.

RADT-42. DISSEMINATED TUMORAL AMYLOIDOSIS OF THE CRANIAL NERVES IN THE ABSENCE OF SYSTEMIC DISEASE: CASE REPORT

Abstract Tumoral amyloidosis, characterized by tumor-like deposits without systemic disease, is rare in the central nervous system. Most often located in the periventricular white matter, tumoral amyloidosis typically consist of immunoglobulin light chains (AL). While prior reports describe cases of tumoral amyloidosis isolated to the trigeminal nerve, we report herein a first known case of diffuse cranial nerve involvement. The patient is a 43-year-old man who presented with left trigeminal dysesthesias, which led to self-excoriation and erosion of the left naris. On exam, he had absent sensation in the V1-V3 distributions, left trochlear nerve palsy, and bilateral abducens nerve palsies. Imaging demonstrated significant thickening of the left trigeminal nerve and diffuse cisternal contrast enhancement of the other cranial nerves, including the oculomotor, abducens, facial, and glossopharyngeal nerves. Work-up began with a lumbar puncture that found elevated protein with otherwise benign results, including flow cytometry. He was therefore recommended to undergo open biopsy of the left trigeminal nerve and cavernous sinus. Pathology from this surgery demonstrated congo red- positive AL amyloid deposits. Following this pathologic diagnosis, he subsequently underwent a full workup for systemic amyloidosis including bone marrow biopsy, abdominal fat pad biopsy, and a PET CT scan. Biopsy of a long nodule was negative for amyloidosis. Systemic amyloid testing did not demonstrate non-CNS sites of amyloid deposition. Medical therapy with daratumumab, cyclophosphamide, bortezomib, and dexamethasone was started but then discontinued due to ineffectiveness. He underwent radiation treatment of 4500 cGy to the left cavernous sinus where the tumoral amyloidosis was most severe. He has had no further progression of his disease. Tumoral amyloidosis of the cranial nerves remains a rare and poorly-understood disease. Prior reports have described the disease isolated to the trigeminal nerve. This report raises attention to this diagnosis in cases of rare multi-nerve involvement and suggests that radiation may be a useful first-line treatment.

Unilateral papilledema: illustrative case.

Idiopathic intracranial hypertension is caused by an elevation of intracranial pressure and can present with slowly progressive visual deterioration from bilateral papilledema. Unilateral papilledema is an exceedingly rare phenomenon, and the mechanisms underlying asymmetric optic nerve involvement remain unknown. A 42-year-old woman presented with acute left-eye blurred vision and extraocular pain. Examination revealed unilateral left optic disc edema with an enlarged blind spot, while the right optic disc appeared flat. Neuroimaging examination was suggestive of intracranial hypertension, characterized by flattening of the posterior globes and narrowing of bilateral distal transverse sinuses. Increased intracranial pressure was confirmed by lumbar puncture. Acetazolamide and weight loss yielded initial improvement, but recurrence prompted cerebral arteriography, revealing a transstenotic gradient in the right distal transverse sinus and hypoplastic left sigmoid and transverse sinuses with extensive collateralization to the left cavernous sinus. Stenting of the right transverse sinus successfully alleviated the optic disc swelling. This case highlights the intricate interplay between venous sinus dynamics and unilateral papilledema, underscoring the importance of tailored interventions. https://thejns.org/doi/10.3171/CASE24385.

SURG-56. TRANSCRIPTION FACTOR CELL LINEAGE DETERMINES AGGRESSIVE BEHAVIOR AMONG NONFUNCTIONAL PITUITARY ADENOMAS

Abstract INTRODUCTION Pituitary neuroendocrine tumors (PitNETs) are the second most common intracranial neoplasm. Silent or non-functional PitNETs represent a common neuro-oncological challenge due to their size and unpredictable invasion of parasellar structures. This makes their complete surgical resection difficult and puts patients at higher risk for recurrence. OBJECTIVE To characterize differences in tumor volume, cavernous sinus invasion, and recurrence in nonfunctional PitNETs. METHODS Out of 615 transsphenoidal surgeries from 2012 to 2024, there were 309 overall PitNETs and 181 nonfunctional PitNETs. Based on new WHO criteria for transcription factor profiling (PIT-1, TPIT, and SF-1), there were 98 silent gonadotroph adenomas (SGA), 31 silent corticotroph adenomas (SCA), 10 silent pleurihormonal tumors (PHT), and 2 null cell adenomas. Tumor volume was calculated using (AxBxC/2). Knosp grade was calculated. Cavernous sinus invasion was defined as Knosp ≥ 3. Residual disease at 3 months postoperative was recorded and surgical intervention for growing residual disease was captured. RESULTS Average tumor volume of PHT (18.08 cm3) was significantly higher than SGA (8.44 cm3, p=0.007) and similar to SCA (10.33 cm3, p=0.11). Percentage of cavernous sinus invasion was significantly different between SCA (31.82%) and SGA (10.20%, p=0.003), but not PHT (33.33%; p>0.05). There was a significantly higher residual rate in SCA compared to SGA (31.71% vs. 11.22%, p=0.006). There were significantly more reoperations in the SCA group compared to SGA and PHT. CONCLUSIONS Despite similar tumor volume between SCA and SGA, there was a significantly higher rate of cavernous sinus invasion and residual disease among SCA. This has important neuro-oncological implications regarding surgical technique, postoperative surveillance, and need for adjuvant therapy to mitigate aggressive recurrent disease.

Recurrent Patterns in Patients With Nasopharyngeal Caricinoma and Risks Leading to Inaccurate Delineation in Marginal Failure in the Era of Intensity-Modulated Radiotherapy.

To investigate the failure patterns of recurrent nasopharyngeal carcinoma (NPC), especially to identify the relationship between the recurrent-prone anatomic structures and the tumor regression sites after the introduction of chemotherapy (IC). A cohort of 1121 non-metastatic patients with NPC was retrospectively enrolled. The pretreatment and recurrent images of each patient were registered to the planning CT. Tumor regression sites after IC (Vicr) overlapped with recurrent tumor (rGTV) were evaluated for the delineation accuracy and dose sufficiency in marginal failure patients. A total of 126 (11.24%) experienced tumor recurrence. Re-evaluation of 12 patients with local marginal recurrence, their rGTV within Vicr predominantly located at choanae, sphenoidal sinus, and cavernous sinus. The regression sites did not receive the full 70 Gy but over half receiving with 60Gy. Analysis from marginal failure that exempts tumor regression sites from GTV or an insufficient prescription dose of less than 70 Gy may contribute to marginal failure.

Surgical anatomy of the carotid sympathetic plexus in endoscopic endonasal approaches: strategies for preventing Horner syndrome.

Endoscopic endonasal approaches (EEAs) specifically for procedures involving manipulation of the internal carotid artery (ICA), such as the transcavernous and translacerum approaches, confer a potential risk of carotid sympathetic plexus injury, potentially leading to postganglionic Horner syndrome. The primary aim of this study was to delineate the surgical anatomy of the carotid sympathetic plexus from an endoscopic endonasal perspective, offering insights to facilitate intraoperative anatomical identification and injury prevention. A comprehensive dissection was conducted on 20 silicone-injected, lightly embalmed postmortem human heads. The segments, patterns, and surgical landmarks of the carotid sympathetic plexus were investigated in a stepwise manner. In addition, 3 illustrative cases highlighting the involvement of the carotid sympathetic plexus in EEAs are included to contextualize the anatomical findings. The carotid sympathetic plexus can be categorized into 3 segments: 1) the cavernous sinus (CS) segment, beginning at the upper surface of the petrolingual ligament and ascending anterosuperiorly along the inferior compartment of the CS (mean ± SD length 5 ± 0.5 mm); 2) the lacerum segment, starting at the exit of the petrous carotid canal and ascending laterally and vertically to the upper surface of the petrolingual ligament (mean ± SD length 10 ± 1 mm); and 3) the petrous segment, originating at the external opening of the carotid canal and terminating at the foramen lacerum (mean ± SD length 18 ± 1 mm). Two primary morphological patterns of the sympathetic plexus at the CS and lacerum segments were identified: a plexus-like pattern in 12 (30%) hemispheres, and a nerve-like pattern that was subcategorized as double (18 [45%] hemispheres) or single (10 [25%] hemispheres) trunks. Surgical strategies emphasize the importance of recognizing key landmarks when approaching the carotid sympathetic plexus, including the pterygosphenoidal triangle, lingual process, petrolingual ligament, lateral parasellar ligament, and abducens nerve. This article provides a comprehensive anatomical description of the carotid sympathetic plexus in EEAs, highlighting key anatomical segments and patterns for intraoperative identification. A better understanding of anatomical landmarks for the carotid sympathetic plexus could help reduce the incidence of postganglionic Horner syndrome, augmenting the safety and efficacy of endoscopic endonasal transcavernous surgery.

A study of the cerebral venous drainage patterns in craniosynostosis: nonsyndromic cases and the induction effect of Virchow's law on venous sinuses.

Surgical intervention is commonly necessary for craniosynostosis. One of the preoperative concerns revolves around the cerebral venous drainage pattern and its potential involvement during surgery. Although there have been reports regarding venous drainage patterns in syndromic craniosynostosis, studies of nonsyndromic cases have been rare. In the present study, the aim was to study venous drainage patterns in nonsyndromic craniosynostosis. Nonsyndromic cases at a single institute were retrospectively reviewed, and cerebral venous drainage in the posterior (transverse sinus [TS]) and anterior (cavernous sinus [CS] and para-CS [ParaCS]) venous routes was systematically investigated. The occipital sinus (OS) and emissary veins were also evaluated. A total of 89 nonsyndromic cases were evaluated, including 12 right coronal synostosis (RCS), 14 left coronal synostosis (LCS), 15 bilateral coronal synostosis (BCS), 36 sagittal synostosis, 6 metopic synostosis, and 6 combined metopic-sagittal synostosis cases. All venous studies were performed using MR venography. There was a significant difference among all six groups in TS dominance (p = 0.0108). In unilateral coronal synostosis (UCS; including RCS and LCS) cases, 76.9% had TS dominance on the opposite side of the synostotic suture (20 of 26 UCS, including 10 of 12 RCS and 10 of 14 LCS). There was a significant difference in the incidence of OS, with the highest incidence observed in the BCS group (33.3%, p = 0.027). CS/ParaCS venous drainage was observed in 94.4% of cases on the right side and 95.5% on the left side, showing no significant difference among the groups on both sides. No visible emissary vein was observed in any of the groups. A significantly higher predominance of left TS was found in RCS cases, in contrast with the typical right-side predominance seen in the normal population. In addition, the majority of UCS cases exhibited TS dominance on the opposite side of the synostotic suture. Furthermore, the present results showed a significant difference in the prevalence of OS, which was predominantly observed in BCS cases. These findings could be explained by the induction effect on venous sinuses by the compensatory growth of the skull according to Virchow's law, suggesting that synostotic sutures induce compensatory skull expansion in regions farthest (diagonally) from the affected sutures, thereby enlarging nearby venous sinuses.

Preoperative predictors of biochemical remission in somatotroph adenoma resections: a single-institution retrospective review.

There is persistent debate in the literature surrounding the true predictors of biochemical remission after resection of somatotroph adenoma. A multimodal analysis of a large number of patients is needed to better understand which patients may be at higher or lower risk for remission failure after surgery. A retrospective review was performed on patients undergoing somatotroph adenoma resection. Biochemical remission was defined as age- and sex-adjusted normalization of serum insulin growth factor-1 (IGF-1) levels at least 6 months after surgery. Patient case characteristics and clinicopathologic variables were tested for statistical associations with remission and were included in a random forest machine learning model to assess for their importance in determining remission status. Preoperative variables found to be significant remission predictors on statistical testing and important in the random forest model were subsequently assessed via receiver operating characteristic (ROC) analysis to determine numeric thresholds that optimally predicted preoperative likelihood of remission success or failure. Eighty patients were identified with somatotroph adenoma who underwent transsphenoidal resection, with 60 patients (75%) achieving biochemical remission. Statistical testing found that patients with failed remission were more likely to have larger tumors (1.9 vs 1.6 cm by the largest axis, p = 0.014; and 3.61 vs 2.66 cm3 by 3D volume, p = 0.013) that invaded the cavernous sinus more frequently (70% vs 22% of patients, p < 0.001) and have higher preoperative IGF-1 level (860 vs 660 ng/ml, p = 0.044). An optimized random forest machine learning model with 10,000 iterations found that tumor size, preoperative growth hormone and IGF-1 levels, and cavernous sinus invasion were important preoperative predictors of remission status. ROC analysis revealed that 96% of patients with preoperative 3D tumor volume less than 1.51 cm3 (area under the curve [AUC] 0.691, p = 0.003) and 100% with nonadjusted preoperative IGF-1 level less than 718.5 ng/ml (AUC 0.736, p = 0.002) achieved remission. Important preoperative predictors of postoperative remission for somatotroph adenoma resection include serum IGF-1 level, cavernous sinus invasion, and tumor size. Ninety-five percent of patients who achieved postoperative remission had preoperative 3D tumor volume less than 1.51 cm3.

Lung transplantation for alpha-1-antitrypsin deficiency and Behçet's disease: A case report

INTRODUCTIONAlpha-1-Antitrypsin deficiency (A1ATD) is a genetically determined anti-proteinase deficiency which predisposes to early onset emphysema and liver disease. Lung transplantation (LTx) is the final therapeutic option. Behçet's disease (BD) is a rare autoimmune disease characterized by oral and genital ulcers, deep venous thrombosis associated with large and small-vessel vasculitis and aneurysms. The association of A1ATD and BD is unknown. We aim to describe a rare case of concomitant presentation of these pathologies in a patient submitted to LTx. CASE REPORTA 31-years-old female presented with oral and genital ulcers associated with cavernous sinus thrombosis in 2012, being diagnosed with BD. In 2018 debuted with progressive respiratory symptoms. CT-scan revealed extensive pulmonary emphysema associated with decreased pulmonary function. A1ATD was identified with a heterozygous “MZ” allelic combination. She was referred to LTx center in 2021. After additional investigation, the patient was considered to LTx. She underwent standard bilateral LTx in July of 2022. During post-operative time, special attention was given to the risk of vascular complications, and pos-operative angio-CT was performed actively searching for this possible outcome. Nevertheless, no major events occurred. After 1 year, she is fully functional with no signs of BD activity. CONCLUSIONTo our knowledge, this is the first description of LTx for A1ATD in a patient with BD, proving its feasibility in a highly selected patient at an experienced and specialized center. Nonetheless, solid organ transplantation in patients with BD remains a high risk procedure and should be indicated with caution.

Evaluation of Clinical and Laboratory Findings of Sinusitis Complications in Children

Abstract Objective This retrospective study evaluates the demographic characteristics, clinical symptoms, laboratory results, treatment approaches, and outcomes of children with complications arising from bacterial sinusitis. Methods Fifty-four pediatric inpatients with sinusitis complications admitted between September 2013 and October 2022 were included in the study. These patients were categorized into two groups: those with preseptal cellulitis (group 1) and those with orbital–intracranial complications (group 2). Results The median age of the patients was 10.8 years, and 61.1% were males. Maxillary sinusitis (n = 40.74%) was the most common type of sinus involvement. Half of the patients exhibited preseptal cellulitis. The median age of our patients with intracranial complications was 13.7 years, while the median age of patients with orbital complications was 8 years. The predominant orbital–intracranial complication was meningitis, which accounted for 41% of the cases. All patients with a subdural abscess (n = 6) presented with frontal sinusitis, while all cases of cavernous sinus thrombosis (n = 3) involved the sphenoidal sinus. Surgical intervention was undertaken in 37% of cases. Streptococcus pneumoniae was the most frequently isolated microorganism. The median duration of hospital stay was 14 days, with a total median treatment duration of 21 days. There were no fatalities, although neurological sequelae were observed in 7.4% of patients (n = 4). Conclusion Preseptal cellulitis and meningitis remain the most common complications of sinusitis in children. Adolescents with sinusitis should be monitored closely for intracranial complications. The prevalence of S. pneumoniae as the most frequently detected pathogen underscores the importance of vaccination.

Thrombophlebitis of the cavernous sinus in children: about a case

We report the case of a girl transferred to our department for treatment of sepsis. On admission, physical examination revealed right eyelid edema and frank meningeal syndrome. The brain CT scan then performed returned to normal. After 10 days of antibiotic therapy, in a persistent infectious context, ptosis, ophthalmoplegia and arereactive mydriasis appeared in the right eye. Brain MRI showed thrombophlebitis of the cavernous sinus. Adapted antibiotic therapy and anticoagulant treatment have been instituted. After four weeks, the outcome was favourable, marked by a complete regression of the signs, particularly ophthalmological, and a disappearance of the radiological signs of cavernous sinus thrombosis. However, sequelae quadriventricular hydrocephalus was noted. Keywords: thrombophlebitis, cavernous sinus, heparinotherapy, Senegal.

Analysis of the impact of a rhinologist-assisted endoscopic approach on a single neurosurgeon’s outcomes in transsphenoidal resection of pituitary adenomas

ObjectivesDetermine the improvements in outcomes of transsphenoidal pituitary adenoma resection following addition of a skull base rhinologist to the neurosurgical team. DesignAll patients that underwent transsphenoidal resection of pituitary adenoma were identified. Demographic data, Otolaryngology (ENT) involvement, initial adenoma size, extent of resection, hormonal secretion status, visual field exam, post-operative DI, CSF leak, recurrence, need for nasoseptal flap (NSF), re-operation rate were all collected. A linear-regression model adjusteds for confounders. SettingA single academic institution Participants271 patients who underwent transsphenoidal surgery between 2015 and 2020.Main Outcome MeasuresThe main outcome measures were Extent of Resection and Endocrinologic remission. ResultsOf the 271 patients identified, 228 (84 %) patients had macroadenomas, one-third of patients presented with bitemporal hemianopsia, and 27 % (72) were hormonally active. For these and demographics there were no statistically significant differences between the microscopic or endoscopic approach (EEA) groups. For patients with cavernous sinus invasion of >50 %, EEA was more likely to achieve gross total resection (GTR) (OR 2.6; p<0.001). For patients who had prior sellar surgery, EEA was 2.5x more likely to get a GTR (p=0.05). The EEA approach resulted in an extra hospital day (1–2 vs 2–3 p<0.05) and surgery lasted an additional hour (1.17 vs 2.5, p<0.05), but did not require fat grafts or lumbar drains on a routine basis. ConclusionsWhile the operating time and LOS were longer, the EEA approach performed by two specialized co-surgeons demonstrates superior value for adenomas that have had a prior surgical attempt or with significant cavernous sinus invasion.

Origin and Course Cavernous Internal Carotid Artery Branches from the Endoscopic Endonasal Perspective: Cadaveric Study and Analysis Clinical Implications.

Endoscopic endonasal approaches to treat cavernous sinus lesions require detailed knowledge of the origin, course, and anatomic variations of the branches of the cavernous internal carotid artery (cICA) because inadvertent avulsion can cause intraoperative ICA injury. We aim to study the origin and course of the branches of the cICA from an endoscopic endonasal perspective and relate these branches to surgically relevant anatomic references. Sixty sides of 30 formalin-fixed specimens were dissected to identify the origin and course of cICA branches, including the inferolateral trunk (ILT), the meningohypophyseal trunk (MHT), anterior and inferior McConnell's capsular arteries (MCAs), and the superolateral trunk (SLT). The ILT and MHT were bilaterally in all specimens, whereas the anterior and inferior MCAs were identified in 28% and 25% of dissected sides, respectively. The SLT was only found in 3% of sides dissected. The MHT was the most proximal branch of the cICA, and its origin was an average of 8.9 mm anterior to the foramen lacerum and 3.8 mm superior to the sellar floor. The ILT was the second most proximal branch of the cICA, which originated 6.4 mm from the MHT on average. The anterior and inferior MCAs were present in 28% and 25% of specimens, respectively. The SLT, when present, was the second most proximal branch of the cICA, which originated at a mean height of 2.7 mm from the sellar floor. Overall, complete ILT and MHT were identified in 68% and 77% of cases, respectively. The MHT and ILT are constant branches of the cICA, with the MHT originating from the medial cICA posterior bend 4 mm superior to the sellar floor and 9 mm anterior to the foramen lacerum, whereas the ILT arises from the lateral horizontal cICA, 2.3 mm superior to the sellar floor and 6 mm anterior to the MHT.

Stand-alone transarterial flow diversion for treatment of carotid cavernous fistulas

ObjectiveCarotid-cavernous fistulas (CCFs) are rare arteriovenous communications allowing blood to flow from the carotid artery into the cavernous sinus. Although currently coil and/or liquid embolization remain the preferred treatment methods for CCFs, flow diverters (FD) stents represent a viable alternative to traditional embolization techniques. This study explores both the technical aspects and rationale behind using FD stents as a stand-alone treatment approach for CCFs. MethodsThe study includes records of 7 patients with CCFs treated at two comprehensive stroke centers from 2019 to 2023. Patients treated with FD stenting in conjunction with coil and/or liquid embolization were excluded from the study. ResultsFive patients were diagnosed with direct CCFs and 2 patients with indirect CCFs. Six patients were treated with the Surpass Evolve FD stent and 1 patient with the Pipeline FD stent. DSA follow-up was performed for an average duration of 14.4 months following FD placement. Complete fistula obliteration with no residual shunting was observed in all patients. Furthermore, all patients experienced a complete resolution of symptoms following treatment. ConclusionsThe authors’ experiences suggest the efficacy and safety of FD stenting as a stand-alone treatment option for CCFs. Compared to embolization, FD stents can better preserve the parent vessel and promote healing with less associated mass effect. Despite being a retrospective self-assessment with a relatively small sample size, to the authors’ knowledge, this study represents the largest individual case series of patients with CCF treated with stand-alone FD stenting.

PATTERNS OF RADIOTHERAPY PRACTICE FOR FUNCTIONING PITUITARY TUMOURS - A SURVEY OF UK CENTRES

Abstract AIMS Pituitary adenomas are benign tumours that are classified as functioning or non-functioning based on the production of excess pituitary hormones. Functioning adenomas (FA) typically present as hypersecretory syndromes including acromegaly and Cushing’s disease. In FA, radiotherapy is used when surgery fails to achieve hormone normalisation and/or when medical treatment does not control hormone excess. In the absence of prospective studies that clearly define the optimal radiotherapy technique in FA, it is expected that there will be variability in practice. A survey of UK neuro-oncology centres was undertaken, aiming to establish the extent of this variability in FA. METHOD A survey was distributed electronically to 20 neuro-oncology departments across the UK. Data were collected on patient numbers, radiotherapy indications, techniques, dosing, and target volume delineation. RESULTS Responses were received from eight centres (40% response rate), treating a total of approximately 32 FA per annum. There was variability in techniques employed to treat FA. While four centres treated most FA with IMRT, two treat most with stereotactic radiosurgery (SRS), and two with fractionated stereotactic radiotherapy (fSRT). The most common dose used for IMRT treatment was 45Gy in 25 fractions (n=7), with 50.4Gy in 28 fractions, 50Gy in 30 fractions and 54Gy in 30 fractions also reported. Use of margins around residual disease (n=5) and inclusion of involved cavernous sinuses and surgical tract (n=3) within clinical target volumes also varied. Most centres (n=7) would agree to a change in target volume delineation practices for the purpose of a clinical trial in FA. CONCLUSION This survey found that there is variability in radiotherapy dosing, technique, and target volume delineation practices for radiotherapy to FA across the UK. All centres but one would accept changes in their practice within the context of a prospective clinical trial of radiotherapy for FA.

Classification, angioarchitecture and treatment outcomes of medullary bridging vein-draining dural arteriovenous fistulas in the foramen magnum region: a multicenter study.

This study aimed to classify medullary bridging vein-draining dural arteriovenous fistulas (MBV-DAVFs) located around the foramen magnum (FM) according to their location and characterize their angioarchitecture and treatment outcomes. Patients with MBV-DAVFs diagnosed between January 2013 and October 2022 were included. MBV-DAVFs were classified into four groups. Jugular vein-bridging vein (JV-BV) DAVF: located in proximity to jugular fossa, Anterior condylar vein (ACV)-BV DAVF: proximity to anterior condylar canal, Marginal sinus (MS)-BV DAVF: lateral surface of FM and Suboccipital cavernous sinus (SCS)-BV DAVF: proximity to dural penetration of vertebral artery. Twenty patients were included, three JV-BV, four ACV-BV, three MS-BV and ten SCS-BV DAVFs, respectively. All groups showed male predominance. There were significant differences in main feeders between JV (jugular branch of ascending pharyngeal artery) and SCS group (C1 dural branch). Pial feeders from anterior spinal artery (ASA) or lateral spinal artery (LSA) were visualized in four SCS and one MS group. Drainage pattern did not differ between groups. Transarterial embolization (TAE) was performed in three, two, one and two cases and complete obliteration was obtained in 100%, 50%, 100% and 0% in JV, ACS, MS and SCS group, respectively. Successful interventions without major complications were finally obtained in 100%, 75%, 100%, and 40% in JV, ACS, MS and SCS group, respectively. JV-BV DAVFs were successfully treated using TAE alone. SCS-BV DAVFs were mainly fed by small C1 dural branches of vertebral artery often with pial feeders from ASA or LSA, and difficultly treated by TAE alone.

Fluorescence detection of pituitary neuroendocrine tumour during endoscopic transsphenoidal surgery using bevacizumab-800CW: a non-randomised, non-blinded, single centre feasibility and dose finding trial [DEPARTURE trial

Achieving endocrine remission by gross total resection is challenging in pituitary neuroendocrine tumours (PitNETs) with cavernous sinus invasion. This study aims to assess the safety, feasibility, and optimal dose for intraoperative fluorescence imaging as an added instrument to discriminate PitNET from surrounding tissue using bevacizumab-800CW, targeting vascular endothelial growth factor A (VEGF-A). In part I, dose-escalation (0-4∙5-10-25mg) was performed in 4 groups of 3 patients with PitNETs Knosp grade 3-4. In part II, after interim analysis, the 10mg and 25mg groups were expanded to a total of 6 patients. Quantitative fluoroscence molecular endoscopy consisted of wide field fluorescence molecular endoscopy and multi-diameter single fiber reflectance / single fiber fluorescence spectroscopy. Mean fluorescence intensity (MFI) of the fresh surgical specimen was calculated and VEGF-staining was performed. Eighteen patients were included. All doses were well tolerated. Three serious adverse events were registered, but none were tracer-related. Part I showed an adequate in-vivo tumour-to-background ratio for both 10mg (TBR 2∙00 [1∙86, 2∙19]) and 25mg (TBR 2∙10, [1∙86, 2∙58]). Part II revealed a substantially higher MFI in the 25mg group. With both 10mg and 25mg a statistically significant difference between tumour and surrounding tissue was detected (p < 0∙0001). All surgical specimens had VEGF-A expression. This study demonstrates the safety and feasibility of quantitative fluorescence molecular endoscopy during PitNET surgery. Both 10mg and 25mg bevacizumab-800CW result in clear differentiation in-vivo, with improved contrast ex-vivo (MFI) in the 25mg group. NCT04212793 / Study Details| Detection of PitNET Tissue During TSS Using Bevacizumab800CW| ClinicalTrials.gov.

Retinal vascular abnormality of non-perfusion and neovascularization in a toddler with neurofibromatosis type 1.

This report describes the case of a 13-month-old boy diagnosed with neurofibromatosis type 1, who presented with retinal vascular abnormalities including extensive non-perfusion and neovascularization. We also discuss the observed changes following photocoagulation treatment. A 13-month-old boy presented to the Department of Ophthalmology at Peking University People's Hospital with a reduction in the width of the left palpebral fissure for the past 6 months. The boy exhibited more than six café-au-lait spots larger than 5 mm in diameter on his trunk and legs. Fundus examination of the left eye revealed significant neovascularization in the temporal periphery of the retina, with late leakage and non-perfusion also noted temporally in fluorescein angiography (FA). Magnetic resonance imaging of the brain and orbits showed an enlarged left sphenoid body, a widened left cavernous sinus, and a large plexiform neurofibroma. Laser treatment was performed on the left eye. Five months later, the neovascularization was controlled. Careful fundus examinations and systemic reviews, especially FA, are essential. Timely laser treatment is crucial for controlling disease progression and preventing retinal detachment.