Cavernous Sinus Lesions Research Articles

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.

Application of Topographical Anatomy of the Trochlear Nerve in Transtentorial Approaches: An Anatomic Study.

Transection of the free edge of tentorium (FET) might be necessary when approaching cavernous sinus lesions, clip placement in certain posterior communicating artery aneurysms, or during transpetrosal and retrosigmoid transtentorial approaches. No anatomic study has investigated the relationship of the trochlear and oculomotor nerve and FET in an attempt to identify a safe zone for such maneuver. Ten embalmed specimens (20 sides) were studied and the following morphometric measurements were taken using digital microcalipers: trochlear (TP) to oculomotor porus (OP), length of the intratentorial segment of cranial nerve (CN) IV, nerve free FET, and distance along the oculomotor (CN III) from its OP to CN III/trochlear (CN IV) crossing and angle between the long-axis petrous ridge and TP (petrotrochlear angle). The CN IV pierced the deep layer of the FET in all cases at a mean distance 8.11 mm (4.43-11.33) posterior to the OP, measured along the FET. CN IV continued within the tentorial edge for a mean of 6.17 mm (3.18-11.33) before entering the cavernous sinus at a mean distance of 1.9 mm (0-5.46 mm) from the posterior-most aspect of the OP. A nerve-free portion of FET was 1.93 mm (mean) (0-5.46). No nerve-free FET segment (<1 mm) was observed in 4 sides (20%), and a nerve-free FET segment <2.00 mm was seen in 55% of cases. The mean distance from OP to the CN III/IV crossing was 10.17 mm (1.00-15.57). The mean petrotrochlear angle was 164.49° (140.01-183.85), and >175° in 4 specimens (20%). Our data confirm presence of the intratentorial segment of CN IV. Transection of the FET carries the lowest risk of CN IV injury if performed < 2 mm or >10 mm posterior to the OP, or posterior to intersection of the FET and a virtual extension of a petrous ridge.

Transorbital approach to the cavernous sinus: an anatomical study of the related cranial nerves.

The cavernous sinus (CS) is a demanding surgical territory, given its deep location and the involvement of multiple neurovascular structures. Subjected to recurrent discussion on the optimal surgical access, the endoscopic transorbital approach has been recently proposed as a feasible route for selected lesions in the lateral CS. Still, for this technique to safely evolve and consolidate, a comprehensive anatomical description of involved cranial nerves, dural ligaments, and arterial relations is needed. Detailed anatomical description of the CS, the course of III, IV, VI, and V cranial nerves, and C3-C7 segments of the carotid artery, all described from the ventrolateral endoscopic transorbital perspective. Five embalmed human cadaveric heads (10 sides) were dissected. An endoscopic transorbital approach with lateral orbital rim removal, anterior clinoidectomy, and petrosectomy was performed. The course of the upper cranial nerves was followed from their apparent origin in the brainstem, through the middle fossa or cavernous sinus, and up to their entrance to the orbit. Neuronavigation was used to follow the course of the nerves and to measure their length of surgical exposure. The transorbital approach allowed us to visualize the lateral wall of the CS, with cranial nerves III, IV, V1-3, and VI. Anterior clinoidectomy and opening of the frontal dura and the oculomotor triangle revealed the complete course of the III nerve, an average of 37 (±2) mm in length. Opening the trigeminal pore and cutting the tentorium permitted to follow the IV nerve from its course around the cerebral peduncle up to the orbit, an average of 54 (±4) mm. Opening the infratrochlear triangle revealed the VI nerve intracavernously and under Gruber's ligament, and the extended petrosectomy allowed us to see its cisternal portion (27 ± 6 mm). The trigeminal root was completely visible and so were its three branches (46 ± 2, 34 ± 3, and 31 ± 1 mm, respectively). Comprehensive anatomic knowledge and extensive surgical expertise are required when addressing the CS. The transorbital corridor exposes most of the cisternal and the complete cavernous course of involved cranial nerves. This anatomical article helps understanding relations of neural, vascular, and dural structures involved in the CS approach, essential to culminating the learning process of transorbital surgery.

The cavernous sinus: An anatomic study with clinical implication.

The management of lesions involving the cavernous sinus remains a formidable challenge. To optimize care for patients with tumors extending into this skull base region a detailed understanding of the surrounding osteology as well as neural and vascular relationships is requisite. This thesis examines the gross anatomy of the region and highlights important surgical implications drawn from these as well as previously published studies. A review of the historical scientific, anatomic, clinical, and surgical literature extending to the present (1992) relating to the cavernous sinus has been performed and discussed. Additionally, the author has performed and described cadaveric dissections revealing novel details about the macroscopic (dural and neurovascular anatomic relationships) and microscopic structure of the cavernous sinus. A series of cases of cavernous sinus pathologies that were addressed in an interdisciplinary surgical approach at the author's institution is also reported. Included in this report is a comprehensive review of the embryology of the cavernous sinus and its associated neurovascular structures. Cadaveric dissections have also revealed novel details about dural/meningeal compartments of the cavernous sinus as well as well as associated arterial, venous, and neural relationships. Microscopic observations also reveal novel fundamental insights into the components and structure of the cavernous sinus. Clinical examples from 20 patients illustrate the critical importance for clinical application of cavernous sinus anatomic knowledge to the surgical treatment of pathologies in this region. The cavernous sinus is a tripartite venous osteomeningeal compartment intimately neighboring vital structures including the optic tracts, pituitary gland, cranial nerves III, IV, V, V, VI, and the internal carotid artery. Surgical management of cavernous sinus lesions has and continues to evolve with increasing anatomic and clinical study as well as advancements in diagnostic and surgical methodologies. NA.

Neuroanesthesia Management in Cavernous Sinus Meningioma Craniotomy Patients

The most prevalent primary cavernous sinus (CS) lesion is cavernous sinus meningioma (CSM). Of all intracranial neoplasms, 1% are tumors in CS, and 41% are CSM. For contemporary neurosurgeons, orbital involvement in cavernous sinus meningiomas (CSMs) poses special difficulties. The condition is known as cavernous sinus meningioma (CSM) gradually impairs vision and may ultimately result in chiastic compression. Since January 2023, a male 55-year-old had been admitted to the hospital with cephalgia and mild diplopia in his right eye. Cavernous meningiomas were discovered using CT scans, and a craniotomy procedure was scheduled to remove the tumor. In order to facilitate intubation, the patient was given a premedication of sufentanyl for analgesia and was then given general anesthesia. Rocuronium was used to relax the muscles. Desflurane is an attractive option available to anesthesiologists to maintain general anaesthesia. This surgical procedure of removing intracranial tumours requires proper induction and monitoring of the patient's condition during surgery to prevent increased intracranial pressure. Intracranial elevation can cause systemic changes such as hypertension and changes in heart rhythm, as well as cerebral artery spasm, and lead to cerebral infarction and cerebral ischemia. An effective neuroanesthesia management program can help preserve hemodynamic stability and improve results during craniotomy surgery for the removal of meningiomas

Abducens Nerve Palsy Caused by Cavernous Sinus Meningioma Mimicking Tolosa-Hunt Syndrome

Purpose: To report a case of abducens nerve palsy caused by cavernous sinus meningioma mimicking Tolosa-Hunt syndrome (THS).Case summary: A 59-year-female visited to the ophthalmic department for sudden onset of horizontal diplopia for 20 days. The extraocular examination showed 10 prism diopters (PD) esotropia at distant fixation and ortho at near fixation without definitive abduction limitation. At 2 months of follow-up, she showed the 25 PD left esotropia with abduction limitation of the left eye. The ocular pain and headache were newly developed. Magnetic resonance imaging (MRI) with contrast was performed and revealed the enlargement and enhancement of left cavernous sinus. THS was suspected and high dose steroid treatment was performed. The ocular findings and headache were improved after treatment. These symptoms showed waxing and waning with steroid tapering during follow-up. The follow-up MRI showed no definitive improvement of cavernous sinus lesion compared with previous study. The cavernous sinus meningioma was considered. There was no improvement of ocular motility findings during follow-up and the strabismus surgery was performed.Conclusions: The abducens nerve palsy with headache can occur caused by cavernous sinus meningioma. It may be difficult to differentiate it from THS in certain cases.

Endoscopic Transorbital Surgery for Trigeminal Schwannoma: Introduction of a Novel Approach: 2-Dimensional Operative Video.

The major indications of endoscopic transorbital approach include spheno-orbital meningiomas, cavernous sinus lesions, and Meckel cave lesion such as trigeminal schwannomas. It can avoid excessive brain retraction and allows for a fast recovery to the normal daily living activity. To access the cavernous sinus, the sagittal crest and meningo-orbital band should be identified and cut. 1. Skin incision along the superior eyelid is performed. 2. Careful dissection of the soft tissue under the orbicularis is required not to injure the orbital septum. 3. After the lateral orbital rim is exposed, the periosteum and periorbita are elevated from the lateral orbital wall. 4. Drilling of the zygomatic bone within the orbit exposes the temporalis muscle first followed by the exposure of the temporal dura. It is essential to obtain adequate working room when the base of the greater sphenoidal wing is drilled. The sagittal crest should be removed, and the meningo-orbital band should be cut to expose the lateral cavernous sinus wall. For successful access through the orbit, endoscopic transorbital approach needs to minimize the retraction of the orbit. To achieve this goal, retraction of the orbit should be limited to a maximum of 10 minutes. This approach can be combined with lateral orbitotomy. It provides a wider working room and makes surgery easier to access the lateral temporal lobe.The patient consented to the procedure, and the participants and any identifiable individuals consented to publication of his/her image. Images at 1:29 reproduced from Corrivetti F, de Notaris M, Di Somma A, et al, "Sagittal crest": definition, stepwise dissection, and clinical implications from a transorbital perspective, Operative Neurosurgery, 22(5), p e206-e212, ©2022, by permission from the Congress of Neurological Surgeons. Video screen capture from The Neurosurgical Atlas at 1:06 used with permission from Aaron Cohen-Gadol; ©The Neurosurgical Atlas, all rights reserved.

SAT589 A Case Of Lymphocytic Hypophysitis In A Young Female With Unilateral Abducens Nerve Palsy

Abstract Disclosure: J.H. Yu: None. M.L. Nguyen: None. M.H. Horani: None. M. Shahlapour: None. Lymphocytic hypophysitis is a neuroendocrine disorder characterized by autoimmune inflammation of the pituitary gland. Lymphocytic hypophysitis is the most common form of hypophysitis with the cause usually unknown. It often occurs in late pregnancy or in the postpartum period with affected individuals presenting with symptoms of headaches of an intensity out of proportion to the size of the lesion and with hypopituitarism. Other manifestations include visual field defects and ocular motility disturbances. We present a case of a 24 year old Female who presents with headache, diplopia, and left-sided CN6 nerve palsy of 4 day duration. She has a known history of amenorrhea for the past year and obesity. She also presents with polydipsia and polyuria. Further workup included MRI of the brain showing a sellar mass with mass effect on optic chiasm and left cavernous sinus lesion. Pituitary hormonal workup showed ACTH 13, LH 0.42, FSH 2.1, TSH 0.442, free T4 1.0, random cortisol 6.3, and prolactin 78. Lumbar puncture with basic CSF studies showed glucose 56, protein 48, nucleated cells 38 (lymph 91%, poly 4%, mono 5%), and RBC 4. ACE, AFP, IgG4, ANA, ANCA, and CEA workup was negative. Biopsy of the mass was performed with results suggesting lymphocytic hypophysitis. Additional immunohistochemistry stains will be performed to evaluate for IgG4 related diseases. The patient would later develop diabetes insipidus and central hypothyroidism weeks later. Discussion: The lymphocytic infiltration causes inflammation, enlargement and fibrosis of the pituitary gland which can produce a mass effect on surrounding tissues. The definitive diagnosis is made via histopathology staining of biopsied sellar or suprasellar tissue. The condition predominantly affects women more than men at a ratio of 8:1.Treatment involves evaluation of pituitary function with management of any hormone deficiencies and alleviating any mass effects due to pituitary inflammation and enlargement. First line treatment usually involves high dose corticosteroids with refractory cases being treated with immunosuppressive medications. Surgery or fractionated radiotherapy can be used if medical therapy fails. In our patient, a biopsy was performed that confirmed lymphocytic hypophysitis with unmasked diabetes insipidus and hypothyroidism presenting weeks after starting on high-dose corticosteroids. Conclusion: This case highlights the unique presentation of lymphocytic hypophysitis including left-sided CN6 nerve palsy, headaches, and hypogonadotropic hypopituitarism. From our knowledge, it is one of the first presentations with unilateral CN6 nerve palsy as a mass effect from lymphocytic hypophysitis. Presentation: Saturday, June 17, 2023

A New Perspective on the Cavernous Sinus as Seen through Multiple Surgical Corridors: Anatomical Study Comparing the Transorbital, Endonasal, and Transcranial Routes and the Relative Coterminous Spatial Regions.

Background: The cavernous sinus (CS) is a highly vulnerable anatomical space, mainly due to the neurovascular structures that it contains; therefore, a detailed knowledge of its anatomy is mandatory for surgical unlocking. In this study, we compared the anatomy of this region from different endoscopic and microsurgical operative corridors, further focusing on the corresponding anatomic landmarks encountered along these routes. Furthermore, we tried to define the safe entry zones to this venous space from these three different operative corridors, and to provide indications regarding the optimal approach according to the lesion's location. Methods: Five embalmed and injected adult cadaveric specimens (10 sides) separately underwent dissection and exposure of the CS via superior eyelid endoscopic transorbital (SETOA), extended endoscopic endonasal transsphenoidal-transethmoidal (EEEA), and microsurgical transcranial fronto-temporo-orbito-zygomatic (FTOZ) approaches. The anatomical landmarks and the content of this venous space were described and compared from these surgical perspectives. Results: The oculomotor triangle can be clearly exposed only by the FTOZ approach. Unlike EEEA, for the exposure of the clinoid triangle content, the anterior clinoid process removal is required for FTOZ and SETOA. The supra- and infratrochlear as well as the anteromedial and anterolateral triangles can be exposed by all three corridors. The most recently introduced SETOA allowed for the exposure of the entire lateral wall of the CS without entering its neurovascular structures and part of the posterior wall; furthermore, thanks to its anteroposterior trajectory, it allowed for the disclosure of the posterior ascending segment of the cavernous ICA with the related sympathetic plexus through the Mullan's triangle, in a minimally invasive fashion. Through the anterolateral triangle, the transorbital corridor allowed us to expose the lateral 180 degrees of the Vidian nerve and artery in the homonymous canal, the anterolateral aspect of the lacerum segment of the ICA at the transition zone from the petrous horizontal to the ascending posterior cavernous segment, surrounded by the carotid sympathetic plexus, and the medial Meckel's cave. Conclusions: Different regions of the cavernous sinus are better exposed by different surgical corridors. The relationship of the tumor with cranial nerves in the lateral wall guides the selection of the approach to cavernous sinus lesions. The transorbital endoscopic approach can be considered to be a safe and minimally invasive complementary surgical corridor to the well-established transcranial and endoscopic endonasal routes for the exposure of selected lesions of the cavernous sinus. Nevertheless, peer knowledge of the anatomy and a surgical learning curve are required.

Complications of the Superior Eyelid Endoscopic Transorbital Approach to the Skull Base: Preliminary Experience With Specific Focus on Orbital Outcome.

The endoscopic superior eyelid approach is a relatively novel mini-invasive technique that is currently investigating for skull base cancers. However, questions remain regarding specific approach-related complications when treating different skull base tumors. This study aims to analyze any surgical complications that occurred in our preliminary consecutive experience, with specific focus on orbital outcome. A retrospective and consecutive cohort of patients treated via a superior eyelid endoscopic transorbital approach at the Division of Neurosurgery of the Hospital Clinic in Barcelona was analyzed. Patients features were described in detail. Complications were divided into 2 groups to analyze separately the approach-related complications, and those resulting from tumor removal. The ocular complications were subdivided into early ocular status (<3 weeks), late ocular status (3-8 weeks), and persistent ocular complications. The "Park questionnaire" was used to determine patient's satisfaction related to the transorbital approach. A total of 20 patients (5 spheno-orbital meningiomas, 1 intradiploic Meningioma, 2 intraconal lesions, 1 temporal pole lesion, 2 trigeminal schwannoma, 3 cavernous sinus lesions, and 6 petroclival lesions) were included in the period 2017-2022. Regarding early ocular status, upper eyelid edema was detected in all cases (100%) associated with diplopia in the lateral gaze in 30% of cases, and periorbital edema in 15% of cases. These aspects tend to resolve at late ocular follow-up (3-8 weeks) in most cases. Regarding persistent ocular complications, in one case of intraconal lesion, a limitation of eye abduction was detected (5%). In another patient with intraconal lesion, an ocular neuropathic pain was reported (5%). In 2 cases of petroclival menigioma, who were also treated with a ventriculo-peritoneal shunt, slight enophthalmus was observed as a persistent complication (10%). According to the Park questionnaire, no cosmetic complaints, no head pain, no palpable cranial irregularities, and no limited mouth opening were reported, and an average of 89% of general satisfaction was encountered. The superior eyelid endoscopic transorbital approach is a safe and satisfactory technique for a diversity of skull base tumors. At late follow-up, upper eyelid edema, diplopia, and periorbital edema tend to resolve. Persistent ocular complications are more frequent after treating intraconal lesions. Enophthalmus may occur in patients with associated ventriculo-peritoneal shunt. According to patient's satisfaction, fairly acceptable results are attained.

Lateral Transorbital Endoscope-Assisted Approach to the Cavernous Sinus.

Surgical access to the cavernous sinus (CS) poses a unique challenge to the neurosurgeon given the concentration of delicate structures in the confines of a very small anatomic space. The lateral transorbital approach (LTOA) is a minimally invasive, keyhole approach that can provide direct access to the lateral CS. A retrospective review of CS lesions treated by a LTOA at a single institution was performed between 2020 and 2023. Patient indications, surgical outcomes, and complications are described. Six patients underwent a LTOA for a variety of pathologies including a dermoid cyst, schwannoma, prolactinoma, craniopharyngioma, and solitary fibrous tumor. The goals of surgery (ie, drainage of cyst, debulking, and pathological diagnosis) were achieved in all cases. The mean extent of resection was 64.6% (±34%). Half of the patients with preoperative cranial neuropathies (n = 4) improved postoperatively. There were no new permanent cranial neuropathies. One patient had a vascular injury repaired endovascularly with no neurological deficits. The LTOA provides a minimal access corridor to the lateral CS. Careful case selection and reasonable goals of surgery are critical to successful outcome.

Neurofibroma of the internal carotid artery cavernous sympathetic plexus: illustrative case.

Intracranial carotid sympathetic plexus (CSP) nerve sheath tumors have rarely been reported in the literature. This study describes the first reported case of a CSP neurofibroma and the first case of a CSP nerve sheath tumor treated via an endoscopic endonasal approach followed by adjuvant radiosurgery. A 53-year-old man presented with 3 days of headaches and diplopia and was found to have a complete left abducens nerve palsy. Computed tomography (CT) revealed a smoothly dilated left carotid canal, CT angiography revealed a superiorly displaced left internal carotid artery (ICA), and magnetic resonance imaging revealed a T2-hyperintense and avidly enhancing lesion in the left cavernous sinus encasing the ICA. The patient underwent subtotal resection via an endoscopic transsphenoidal transcavernous approach followed by Gamma Knife radiosurgery. Nerve sheath tumors arising from the CSP are extremely rare but need to be considered when assessing unusual cavernous sinus lesions. The clinical presentation is dependent on the anatomical location of the tumor and its relationship to the ICA. The optimal treatment paradigm is unknown.

Endoscopic Endonasal Biopsy for Diagnosis of Undifferentiated Lesions of the Cavernous Sinus

Radiologically undifferentiated lesions of the cavernous sinus can pose a diagnostic challenge. Although radiotherapy is the mainstay for treatment of cavernous sinus lesions, histologic diagnosis allows access to a wide variety of alternative treatment modalities. The region is considered a high-risk area for open transcranial surgical access, and the endoscopic endonasal approach presents an alternative technique for biopsy. A retrospective case series was performed of all patients undergoing endoscopic endonasal biopsy of isolated cavernous sinus lesions at 2 tertiary institutions. The primary outcomes were the percentage of patients in whom a histologic diagnosis was achieved and the proportion of patients in whom therapy differed from radiotherapy alone. Secondary outcomes included preoperative and postoperative 22-item Sino-Nasal Outcome Test symptom scores, as well as perioperative adverse outcomes. Eleven patients underwent endoscopic endonasal biopsy, with a diagnosis achieved in 10 patients. The most common diagnosis was perineural spread of squamous cell carcinoma, followed by perineuroma and single cases of metastatic melanoma, metastatic adenoid cystic carcinoma, mycobacterium lepri infection, neurofibroma, and lymphoma. Six patients had treatments other than radiotherapy, including immunotherapy, antibiotics, corticosteroids, chemotherapy, and observation alone. There was no significant difference in prebiopsy and postbiopsy 22-item Sino-Nasal Outcome Test scores. There was 1 case of epistaxis requiring return to theater for cautery of the sphenopalatine artery and there were no mortalities. In a limited case series, endoscopic endonasal biopsy was safe and effective in obtaining diagnosis for cavernous sinus lesions and had a significant impact on therapeutic decision making.

Germline genetic mutations in pediatric cerebrovascular anomalies: a multidisciplinary approach to screening, testing, and management.

Genetic alterations are increasingly recognized as etiologic factors linked to the pathogenesis and development of cerebrovascular anomalies. Their identification allows for advanced screening and targeted therapeutic approaches. The authors aimed to describe the role of a collaborative approach to care and genetic testing in pediatric patients with neurovascular anomalies, with the objectives of identifying what genetic testing recommendations were made, the yield of genetic testing, and the implications for familial screening and management at present and in the future. The authors performed a descriptive retrospective cohort study examining pediatric patients genetically screened through the Pediatric Neurovascular Program of a single treatment center. Patients 18 years of age and younger with neurovascular anomalies, diagnosed radiographically or histopathologically, were evaluated for germline genetic testing. Patient demographic data and germline genetic testing and recommendation, clinical, treatment, and outcome data were collected and analyzed. Sixty patients were included; 29 (47.5%) were female. The mean age at consultation was 11.0 ± 4.9 years. Diagnoses included cerebral arteriovenous malformations (AVMs) (n = 23), cerebral cavernous malformations (n = 19), non-neurofibromatosis/non-sickle cell moyamoya (n = 8), diffuse cerebral proliferative angiopathy, and megalencephaly-capillary malformation. Of the 56 patients recommended to have genetic testing, 40 completed it. Genetic alterations were found in 13 (23%) patients. Four patients with AVMs had RASA1, GDF2, and ACVRL1 mutations. Four patients with cavernous malformations had Krit1 mutations. One with moyamoya disease had an RNF213 mutation. Three patients with megalencephaly-capillary malformation had PIK3CA mutations, and 1 patient with a cavernous sinus lesion had an MED12 mutation. The majority of AVM patients were treated surgically. Patients with diffuse cerebral proliferative angiopathy were treated medically with sirolimus. At-risk relatives of 3 patients positive for genetic anomalies had also been tested. This study demonstrates a role for exploring genetic alterations in the identification and treatment of pediatric neurovascular disease pathogenesis. Germline genetic mutations were found in almost one-quarter of the patients screened in this study, results that helped to identify medically targeted treatment modalities for some pediatric neurovascular patients. Insight into the genetic etiology of vascular anomalies may provide broader clinical implications for risk assessment, family screening, follow-up surveillance, and medical management.

Pictorial Review of Cavernous Sinus Hemangiomas (Venous Malformations)

Cavernous venous malformations of the cavernous sinus are rare, benign tumors that account for 2%‐3% of all cavernous sinus lesions. An accurate pretreatment diagnosis is important due to the highly vascular nature of these lesions and the risk of major intraoperative hemorrhage. A shifting preference toward radiosurgery as a primary treatment, increasingly without a pretreatment biopsy, further underscores the need for an accurate imaging diagnosis. The aim of this pictorial review is to illustrate the imaging findings of cavernous sinus venous malformations on CT and MR imaging, the use of red blood cell scintigraphy as an adjunct imaging technique, relevant differential diagnoses, and management.Learning Objective: To describe the main imaging features of cavernous sinus venous malformations using CT, MR imaging, and red blood cell scintigraphy to differentiate them from other common lesions of the cavernous sinus

Modified lateral orbitotomy approach for resection of anterior temporal cavernous malformation

Background: The lateral orbitotomy approach (LOA) provides a direct and minimally invasive corridor to orbital apex, cavernous sinus, and middle cranial fossa (MCF) lesions. Removal of the lateral orbital wall and retraction of the orbital contents, as performed with a traditional LOA, can cause diplopia and enophthalmos and affect visual acuity. The modified LOA (mLOA) preserves the lateral orbital wall to limit this morbidity.Case Description: A 58-year-old man experienced new-onset headaches and anxiety attacks that improved with anti-seizure medication. He was neurologically intact on examination. Magnetic resonance imaging demonstrated a 2-cm right anterior temporal cavernous malformation with an associated hemosiderin ring. Electroencephalogram revealed right temporal intermittent rhythmic delta activity suspicious for anterior temporal lobe epilepsy. He underwent an endoscopic-assisted keyhole mLOA for resection of the cavernoma and hemosiderin-stained brain. Key steps included a Y-shaped incision in the upper eyelid/lateral canthus, removal of a 1.5-cm segment of the lateral orbital rim, drilling of the lateral orbital wall with preservation of the medial cortex, drilling the lateral sphenoid ridge to access the anterior temporal lobe, resecting the cavernoma with endoscopic assistance for removal of all potentially epileptogenic abnormal brain, and plating the orbital rim as part of a layered closure. Postoperatively, he remained neurologically intact. He was discharged on postoperative day 4 after resolution of a cerebrospinal fluid leak with lumbar drainage. On follow-up, his anxiety attacks had completely resolved, and his incision was well-healed.Conclusion: The mLOA is an ideal keyhole technique for selected lesions of the MCF.

Surgical Planning and Simulation of Endonasal Endoscopic Surgery for Pituitary Adenoma With Cavernous Sinus Invasion.

Transsphenoidal endoscopic endonasal surgery (EES) provides effective treatment for patients with lesions of the sella turcica. The endoscopic technique requires different instrumentation, which depends on the gross anatomy of the nasal cavity. The treatment of lateral lesions is more challenging in EES. To evaluate the effect of preoperative simulation using multiple anatomic landmarks. Pre- and postoperative tumor volumes were analyzed in 33 patients with nonfunctioning pituitary adenomas who underwent EES (Knosp grades 3 and 4). The surgical working angle and space were three-dimensionally simulated at the plane of the anterior/posterior surgical field (tuberculum sellae/posterior clinoid process) using multiple anatomic landmarks of high-resolution computed tomography scans, such as nasal piriform aperture (proximal surgical corridor), and the width of bilateral vidian canals or lamina perpendicularis of palatine bone (distal surgical corridor). Receiver operating characteristic curves for the removed tumor volume were used to determine the cutoff value for the simulated working angle and space. Simulated working space at the plane of tuberculum sellae using piriform aperture and lamina perpendicularis of palatine bone was associated with the removed tumor volume in the cavernous sinus. Patients with a larger working space (≥42.7 mm) significantly showed a higher removed tumor volume ( P = .023). There was no relationship between other parameters and the removed tumor volume. A new method to predict the surgical field for cavernous sinus lesions around sella turcica was successfully established. Further studies are needed to define and expand applications of this simulation method.

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

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

Clinical and imaging features in surgically verified patients over 11 years and literature review

Background: Controversy continues in the treatment decisions despite advanced imaging techniques. Though specific diagnosis by imaging is not precise. Diffusion weighted imaging is useful in a small proportion of patients. We evaluated the features of magnetic resonance imaging (MRI) with histopathological findings in patients with lesions of the cavernous sinus (CS). Materials and methods: Retrospective analysis of clinical, imaging and histopathological findings of lesions involving cavernous sinus (CS) in 27 consecutive patients was done. Results: The average age of the study population was 41.12 ± 14.49 (13-63) years; with 16 (59.2%) males. Visual disturbances were the most common complaints, reported in 62.0% and cranial nerve involvement was observed in 55 % of the patients. Complete excision was done in nine (33.3%) patients. Post-operative histopathology revealed meningiomas and hemangiomas in six (22.2%) patients each. While, five (18.5%) patients had schwannoma; fungal granuloma was observed in three (11.1%). Imaging based diagnosis showed concordance with histopathology in five (85.0%) patients with hemangioma. Among fungal granuloma, schwannoma and meningiomas, the concordance was 66.6%, 40.0% and 33.3% respectively. In the entire study population, concordance was 44.4%. Conclusions: MR signal intensities are similar in neoplasms, infections, vascular lesions and inflammatory lesions. Cavernous hemangiomas are most often mistaken for other lesions but may be characterized by intense contrast enhancement and absence of restriction of DWI and blooming on GRE sequence. In lesions of cavernous sinus, accuracy of diagnosis on MRI is less than 50%. Diagnosis on MRI is more accurate in hemangiomas and fungal granulomas. Non-invasive diagnosis of granulomatous lesions may help plan appropriate management strategy. Keywords: cavernous sinus; magnetic resonance imaging; diffusion weighted imaging

Revisiting Surgery in the Current Era of Gamma Knife for Cavernous Sinus Lesions.

The challenges associated with surgeries of cavernous sinus (CS) lesions have shifted the management trend towards upfront gamma knife radiosurgery (GKRS). Although GKRS is effective in arresting the progression of certain small residual/recurrent lesions, its efficacy in alleviating neurological deficits is less evident. Furthermore, GKRS without establishing the histopathological diagnosis, at times can be detrimental. We present our clinical experience to reemphasize the role of surgery for CS lesions in the current era of upfront GKRS. We reviewed our database of 32 patients with various CS lesions treated by surgery for progressive cranial nerve deficits. The follow-up data were analyzed for the extent of resection, and in particular for improvement in their symptoms. The lesions were confirmed as hemangioma (CSH)-8, meningioma-8, trigeminal schwannoma-6, chordoma-3, residual pituitary with CS extension-3, fungal granuloma-3, and dysgerminoma-1. Symptoms improved in 23 (complete in 13) and remained at least static in six patients. Follow-up ranged from 4-36 months. The nature of pathology should determine the management modality in CS lesions. Excision of CS schwannomas and chordomas yields rapid clinical improvement and good long-term outcomes. Resection is preferred for large CSH and functioning pituitary tumors. Although the clinical improvement may be less dramatic, surgery debulks the meningiomas. Most importantly, surgery also establishes the histopathological diagnosis of CS lesions. Even with an easy alternative of upfront GKRS, resection has a definite role in the primary management of most CS pathologies.