Jugular Foramen Paragangliomas.

Paragangliomas are the most common tumors at jugular foramen and pose a great surgical challenge. Careful clinical history and physical examination must be performed to adequately evaluate neurological deficits and its chronologic evolution, also to delineate an overview of the patient performance status. Complete imaging evaluation including MRI and CT scans should be performed, and angiography is a must to depict tumor blood supply and sigmoid sinus/internal jugular vein patency. Screening for multifocal paragangliomas is advisable, with a whole-body imaging. Laboratory investigation of endocrine function of the tumor is necessary, and adrenergic tumors may be associated with synchronous lesions. Preoperative prepare with alpha-blockage is advisable in norepinephrine/epinephrine-secreting tumors; however, it is not advisable in exclusively dopamine-secreting neoplasms. Best surgical candidates are young otherwise healthy patients with smaller lesions; however, treatment should be individualized each case. Variations of infratemporal fossa approach are employed depending on extensions of the mass. Regarding facial nerve management, we avoid to expose or reroute it if there is preoperative function preservation and prefer to work around facial canal in way of a fallopian bridge technique. If there is preoperative facial nerve compromise, the mastoid segment of the nerve is exposed, and it may be grafted if invaded or just decompressed. A key point is to preserve the anteromedial wall of internal jugular vein if there is preoperative preservation of lower cranial nerves. Careful multilayer closure is essential to avoid at most cerebrospinal fluid leakage. Residual tumors may be reoperated if growing and presenting mass effect or be candidate for adjuvant stereotactic radiosurgery.

[Analysis of the effect of different facial nerve managements applied to tumor resection in the jugular foramen region].

Objective:To summarize the results of different facial nerve management modalities applied to tumor resection in the jugular foramen region. Methods:The clinical data of 54 patients with tumors in the jugular foramen region who underwent surgery from January 2015 to March 2023 were retrospectively analyzed: 18 males and 36 females; Age ranges from 21 to 67 years, with an average age of 44.4 years; and median follow-up time: 12 months. The House-Brackmann（HB） grading system was applied to assess the patients' facial nerve function before surgery, 1-2 weeks after surgery and at the final follow-up （HBⅠ-Ⅱ grade for good function）: 42 cases with preoperative HB grades Ⅰ-Ⅱ; partial facial nerve transposition（9 cases）, complete facial nerve transposition（28 cases）, and facial nerve excision and re-construction（17 cases） were used, respectively（stage Ⅰor Ⅱ）. Relevant factors affecting postoperative facial nerve function were analyzed. Results:Postoperative pathology confirmed 39 cases of paraganglioma, 9 cases of nerve sheath tumor, 3 cases of meningioma, and 1 case each of fibromucinous sarcoma, chondrosarcoma, and intravascular myofibroma. Facial nerve function after partial facial nerve transposition was HB grade Ⅰ-Ⅱ in 89%（8/9）; after complete facial nerve transposition was HB grade Ⅰ-Ⅱ in 86%（24/28） in 28 cases; after facial nerve severance and reconstruction was HB grade Ⅰ-Ⅱ in 2/7（Stage Ⅰ） and 0/3（Stage Ⅱ）, respectively. Tumor size and surgical approach were correlated with postoperative facial nerve function in patients with facial nerve transposition（P<0.05）. There was no statistically significant difference in facial nerve function after complete and partial facial nerve transposition（P>0.05）. Conclusion:Intraoperative stretching of the facial nerve may be an important factor affecting facial nerve function during surgical treatment of tumors in the jugular venous foramen region; for patients with facial nerve dissection, facial nerve reconstruction should be adopted according to the situation, aiming at the recovery of facial nerve function.

Computed tomographic features of canine intracranial and jugular foraminal masses involving the combined glossopharyngeal, vagus, and accessory nerve roots.

A chronic cough, gag, or retch is a common presenting clinical complaint in dogs. Those refractory to conservative management frequently undergo further diagnostic tests to investigate the cause, including CT examination of their head, neck, and thorax for detailed morphological assessment of their respiratory and upper gastrointestinal tract. This case series describes five patients with CT characteristics consistent with an intracranial and jugular foraminal mass of the combined glossopharyngeal (IX), vagus (X), and accessory (XI) cranial nerves and secondary features consistent with their paresis. The consistent primary CT characteristics included an intracranial, extra-axial, cerebellomedullary angle, and jugular foraminal soft tissue attenuating, strongly enhancing mass (5/5). Secondary characteristics included smooth widening of the bony jugular foramen (5/5), mild hyperostosis of the petrous temporal bone (3/5), isolated severe atrophy of the ipsilateral sternocephalic, cleidocephalic, and trapezius muscles (5/5), atrophy of the ipsilateral thyroarytenoideus and cricoarytenoideus muscles of the vocal fold (5/5), and an ipsilateral "dropped" shoulder (4/5). Positional variation of the patient in CT under general anesthesia made the "dropped" shoulder of equivocal significance. The reported clinical signs and secondary CT features reflect a unilateral paresis of the combined cranial nerves (IX, X, and XI) and are consistent with jugular foramen syndrome/Vernet's syndrome reported in humans. The authors believe this condition is likely chronically underdiagnosed without CT examination, and this case series should enable earlier CT diagnosis in future cases.

Endoscopic endonasal combined transoral medial approach to the nasopharynx, parapharyngeal space, and jugular foramen.

OBJECTIVE: This study aimed to validate the feasibility of an endoscopic endonasal combined transoral medial approach for treating lesions in the nasopharynx, parapharyngeal space (PPS), and jugular foramen. METHODS: Anatomical and imaging information of six patients who underwent surgery via this approach were reviewed and analyzed. RESULTS: The feasibility and advantages of the endoscopic endonasal combined transoral medial approach, which uses an inside-to-outside medial surgical corridor, were identified. Total resection was achieved in 3 cases with benign tumors. Safe resection margins were obtained in 2 cases with recurrent nasopharyngeal carcinoma (NPC). Pathological biopsy of NPC lesion between the Eustachian tube and arterial sheath was achieved. The internal carotid artery (ICA) was accurately located and protected in all cases and no complications occurred. CONCLUSION: Lesions in the nasopharynx, PPS, and jugular foramen can be directly assessed via this approach. The ICA can be well identified during the surgery.

Anatomic Step-by-Step Dissection of Complex Skull Base Approaches for Trainees: Surgical Anatomy of the Infratemporal Fossa Approach to the Jugular Foramen.

BACKGROUND AND OBJECTIVES: The infratemporal fossa (ITF) is a complex region bounded by the temporal bone, maxilla, sphenoid, pterygoid plates, and mandibular ramus. Containing a high density of neurovascular and musculoskeletal structures, the ITF can house a number of pathologies, and access is challenging. The ITF approach and its variations can be challenging due to complex anatomy and unfamiliarity by many surgeons. The objective of this study was to present a step-by-step 3-dimensional anatomic dissection for the classic Fisch Type A and modified ITF approach from the surgeon's perspective. METHODS: Six sides of 3 formalin-fixed latex-injected specimens were dissected under microscopic magnification (JRD and AMN). Standard Fisch Type A and modified ITF approaches were performed on contralateral sides of each specimen. Representative high-quality 3-dimensional photography was performed for each key step. RESULTS: The ITF approach affords excellent access to the posterior ITF and jugular foramen. Modifications to this approach include preservation of the ear canal and limiting facial nerve transposition, thus limiting morbidity while generally still providing sufficient access to key anatomic structures. CONCLUSION: The ITF approach provides access to the lateral skull base for jugular foramen paraganglioma and other lesions. Modifications of the classic Fisch Type A technique can be used to access pathologies in this region without sacrificing conductive hearing or facial nerve function. Three dimensional operatively oriented neuroanatomy dissections provide surgeons with a valuable resource for learning this complex surgical approach.

Transmastoid/Infralabyrinthine Approach to the Jugular Foramen: 2-Dimensional Operative Video.

INDICATIONS CORRIDOR AND LIMITS OF EXPOSURE: This approach is intended for tumors centered in the jugular foramen with extensions between intracranial and extracranial spaces, possible spread to the middle ear, and variable bony destruction. 1,2. ANATOMIC ESSENTIALS NEED FOR PREOPERATIVE PLANNING AND ASSESSMENT: Jugular foramen paragangliomas are complex lesions that usually invade and fill related venous structures. They present complex relationships with skull base neurovascular structures as internal carotid artery, lower cranial nerves (CNs), middle ear, and mastoid segment of facial nerve. In this way, it is essential to perform an adequate preoperative vascular study to evaluate sinus patency and the tumor blood supply, besides a computed tomography scan to depict bone erosion. ESSENTIAL STEPS OF THE PROCEDURE: Mastoidectomy through an infralabyrinthine route up to open the lateral border of jugular foramen, allowing exposure from the sigmoid sinus to internal jugular vein. Skeletonization of facial canal without exposure of facial nerve is performed and opening of facial recess to give access to the middle ear in way of a fallopian bridge technique. 2-10. PITFALLS/AVOIDANCE OF COMPLICATIONS: If there is preoperative preservation of lower CN function, it is important to not remove the anteromedial wall of the internal jugular vein and jugular bulb. In addition, facial nerve should be exposed just in case of preoperative facial palsy to decompress or reconstruct the nerve. VARIANTS AND INDICATIONS FOR THEIR USE: Variations are related mainly with temporal bone drilling depending on the extensions of the lesion, its source of blood supply, and preoperative preservation of CN function.Informed consent was obtained from the patient for the procedure and publication of his image.Anatomy images were used with permission from:• Ceccato GHW, Candido DNC, and Borba LAB. Infratemporal fossa approach to the jugular foramen. In: Borba LAB and de Oliveira JG. Microsurgical and Endoscopic Approaches to the Skull Base. Thieme Medical Publishers. 2021.• Ceccato GHW, Candido DNC, de Oliveira JG, and Borba LAB. Microsurgical Anatomy of the Jugular Foramen. In: Borba LAB and de Oliveira JG. Microsurgical and Endoscopic Approaches to the Skull Base. Thieme Medical Publishers. 2021.

[Differential diagnosis and surgical management in chondrosarcoma of the jugular foramen].

Objective: To explore the diagnosis, surgical management and outcome of jugular foramen chondrosarcoma (CSA). Methods: Fifteen patients with jugular foramen CSA hospitalized in the Department of Otorhinolaryngology Head and Neck Surgery of Chinese PLA General Hospital from December 2002 to February 2020 were retrospectively collected,of whom 2 were male and 13 were female, aging from 22 to 61 years old. The clinical symptoms and signs, imaging features, differential diagnosis, surgical approaches, function of facial nerve and cranial nerves IX to XII, and surgical outcomes were analyzed. Results: Patients with jugular foramen CSA mainly presented with facial paralysis, hearing loss, hoarseness, cough, tinnitus and local mass. Computed tomography (CT) and magnetic resonance (MR) could provide important information for diagnosis. CT showed irregular destruction on bone margin of the jugular foramen. MR demonstrated iso or hypointense on T1WI, hyperintense on T2WI and heterogeneous contrast-enhancement. Surgical approaches were chosen upon the sizes and scopes of the tumors. Inferior temporal fossa A approach was adopted in 12 cases, inferior temporal fossa B approach in 2 cases and mastoid combined parotid approach in 1 case. Five patients with facial nerve involved received great auricular nerve graft. The House Brackmann (H-B) grading scale was used to evaluate the facial nerve function. Preoperative facial nerve function ranked grade Ⅴ in 4 cases and grade Ⅵ in 1 case. Postoperative facial nerve function improved to grade Ⅲ in 2 cases and grade Ⅵ in 3 cases. Five patients presented with cranial nerves Ⅸ and Ⅹ palsies. Hoarseness and cough of 2 cases improved after operation, while the other 3 cases did not. All the patients were diagnosed CSA by histopathology and immunohistochemistry, with immunohistochemical staining showing vimentin and S-100 positive, but cytokeratin negative in tumor cells. All patients survived during 28 to 234 months' follow-up. Two patients suffered from tumor recurrence 7 years after surgery and received revision surgery. No complications such as cerebrospinal fluid leakage and intracranial infection occurred after operation. Conclusions: Jugular foramen CSA lacks characteristic symptoms or signs. Imaging is helpful to differential diagnosis. Surgery is the primary treatment of jugular foramen CSA. Patients with facial paralysis should receive surgery in time as to restore the facial nerve. Long-term follow-up is necessary after surgery in case of recurrence.

Extraskeletal Myxoid Chondrosarcoma of the Jugular Foramen in a Pediatric Patient: A Case Report and Comprehensive Review of the Literature.

INTRODUCTION: Extraskeletal myxoid chondrosarcoma of the jugular foramen is a rare clinical entity, especially in the pediatric population. Thus, it can be confused with other pathologies. CASE PRESENTATION: We report an extremely rare case of a 14-year-old female patient with jugular foramen myxoid chondrosarcoma that was completely removed through microsurgical resection. CONCLUSION: The primary purpose of the treatment is gross total resection of the chondrosarcomas. However, adjuvant methods such as radiotherapy should additionally be applied in patients who have high-grade diseases or cannot undergo gross total resection because of anatomic localization.

The Anterolateral Approach, Revisited for Benign Jugular Foramen Tumors With Predominant Extracranial Extension: Microsurgical Anatomy and Case Series (SevEN-012).

BACKGROUND AND OBJECTIVES: The anterolateral approach (ALA) enables access to the mid and lower clivus, jugular foramen (JF), craniocervical junction, and cervical spine with added anterior and lateral exposure than the extreme lateral and endoscopic endonasal approach, respectively. We describe the microsurgical anatomy of ALA with cadaveric specimens and report our clinical experience for benign JF tumors with predominant extracranial extension. METHODS: A stepwise and detailed microsurgical neurovascular anatomy of ALA was explored with cadaveric specimens. Then, the clinical results of 7 consecutive patients who underwent ALA for benign JF tumors with predominant extracranial extension were analyzed. RESULTS: A hockey stick skin incision is made along the superior nuchal line to the anterior edge of the sternocleidomastoid muscle (SCM). ALA involves layer-by-layer muscle dissection of SCM, splenius capitis, digastric, longissimus capitis, and superior oblique muscles. The accessory nerve runs beneath SCM and is found at the posterior edge of the digastric muscle. The internal jugular vein (IJV) is lateral to and at the level of the accessory nerve. The occipital artery passes over the longissimus capitis muscle and IJV and into the external carotid artery, which is lateral and superficial to IJV. The internal carotid artery (ICA) is more medial and deeper than external carotid artery and is in the carotid sheath with the vagus nerve and IJV. The hypoglossal and vagus nerves run along the lateral and medial side of ICA, respectively. Prehigh cervical carotid, prejugular, and retrojugular surgical corridors allow deep and extracranial access around JF. In the case series, gross and near-total resections were achieved in 6 (85.7%) patients without newly developed cranial nerve deficits. CONCLUSION: ALA is a traditional and invaluable neurosurgical approach for benign JF tumors with predominant extracranial extension. The anatomic knowledge of ALA increases competency in adding anterior and lateral exposure of extracranial JF.

Posterolateral Routes to the Skull Base, Craniocervical Junction, and Jugular Foramen-The Far Lateral Transcondylar Approach and Combined Transpetrosal Transcervical Approaches.

The far lateral approach provides wide surgical access to the lower third of the clivus, pontomedullary junction, and anterolateral foramen magnum and rarely requires craniovertebral fusion. The most common indications for this approach are posterior inferior cerebellar artery and vertebral arteryaneurysms, brainstem cavernous malformations, and tumors anterior to the lower pons and medulla, including meningiomas of the anterior foramen magnum, schwannomas of the lower cranial nerves, and intramedullary tumors at the craniocervical junction. We provide a stepwise description of how we perform the far lateral approach, as well as how to combine the far lateral approach with other skull base approaches, including the subtemporal transtentorial approach, for lesions involving the upper clivus; the posterior transpetrosal approach, for lesions involving the cerebellopontine angle and/or petroclival region; and/or lateral cervical approaches, for lesions involving the jugular foramen or carotid sheath regions.

From a High School Student to an Otolaryngologist-19 Years With a Jugular Foramen Meningioma.

In this essay, an otolaryngologist reflects on the circular journey from patient to physician to patient again over 19 years living with a left jugular foramen meningioma.

[Jugular foramen anatomy in lateral skull base paraganglioma surgery]. / Anatomiya yaremnogo otverstiya v khirurgii paragangliom lateral'nogo osnovaniya cherepa.

In this article we present the surgical approaches to the temporal bone paraganglioma based on the anatomical studies. OBJECTIVE: To detalize the anatomy of the jugular foramen according to the comparison of cadaver dissections findings and the CT scans data that were performed before the dissections, for improvement of quality of treatment of patients with temporal bone paraganglioma (Fisch type C). MATERIAL AND METHODS: The data of CT scans and the steps of the approaches to the jugular foramen (retrofacial and infratemporal approaches with opening of jugular bulb and identification of the anatomical structures of jugular foramen) were analyzed on 10 cadaver heads, 20 sides. Clinical implementation was demonstrated in case of temporal bone paraganglioma type C. RESULTS: Based on the detail study of the CTs data we revealed the individual features of the temporal bone structures. Due to the results of 3D rendering the average length of the jugular foramen in anterior-posterior direction was 10.1 mm. The length of vascular part was larger than the nervous part. The posterior part had the bigger height wherein the shortest part we detected between jugular ridges, which in some cases caused the dumbbell shape of jugular foramen. According to 3D multiplanar reconstruction the distances between jugular crests (3.0 mm) had the lowest measures and the largest was between internal auditory canal (IAC) and jugular bulb (JB) (8.01mm). At the same time, one of the largest variations of values was also identified between IAC and JB (from 4.39 to 9.84 mm). The distance between the facial nerve in the mastoid segment and JB was variable (from 3.4 to 10.2 mm) and determined by the volume and position of the JB. The results of the dissection corresponded to the measurements according to the CT scans, taking into account the 2-3 mm error due to the massive removal of temporal bone during performing of surgical approaches. CONCLUSION: The detailed knowledge of the surgical anatomy of the jugular foramen based on a thorough analysis of preoperative CT data is the key to an adequate surgical tactic for the removal of different types of temporal bone paraganglioma while preserving the function of vital structures and the quality of life. A larger study on the big data is needed to determine the statistical relationship between the volume of JB and the size of the jugular crest; the correlation between the dimensions of jugular crests and the tumor invasion in the anterior part of the jugular foramen.

Utility of dynamic susceptibility contrast MRI for differentiation between paragangliomas and meningiomas in the cerebellopontine angle and jugular foramen region.

PURPOSE: Differentiation of paragangliomas and meningiomas can be a challenge. This study aimed to assess the utility of dynamic susceptibility contrast perfusion MRI (DSC-MRI) to distinguish paragangliomas from meningiomas. METHODS: This retrospective study included 40 patients with paragangliomas and meningiomas in the cerebellopontine angle and jugular foramen region between March 2015 and February 2022 in a single institution. Pretreatment DSC-MRI and conventional MRI were performed in all cases. Normalized relative cerebral blood volume (nrCBV), relative cerebral blood flow (nrCBF), relative mean transit time (nrMTT), and time to peak (nTTP) as well as conventional MRI features were compared between the 2 tumor types and between meningioma subtypes as appropriate. Receiver operating characteristic curve and multivariate logistic regression analysis were performed. RESULTS: Twenty-eight meningiomas including 8 WHO grade II meningiomas (12 males, 16 females; median age 55 years) and 12 paragangliomas (5 males, 7 females; median age 35 years) were included in this study. Paragangliomas had a higher rate of cystic/necrotic changes (10/12 vs 10/28; P = 0.014), a higher rate of internal flow voids (9/12 vs 8/28; P = 0.013), higher nrCBV (median 9.78 vs 6.64; P = 0.04), and shorter nTTP (median 0.78 vs 1.06; P < 0.001) than meningiomas. There was no difference in conventional imaging features and DSC-MRI parameters between meningioma subtypes. nTTP was identified as the most significant parameter for the 2 tumor types in the multivariate logistic regression analysis (P = 0.009). CONCLUSIONS: In this small retrospective study, DSC-MRI perfusion differences were observed between paragangliomas and meningiomas, but not between grade I and II meningiomas.

Extracranial Jugular Foramen Schwannomas Treated with the Extreme Lateral Juxtacondylar Approach: Surgical Technique and Our Experience.

BACKGROUND: Schwannoma that arises in the jugular foramen (JF) represents an important challenge for neurosurgeons for its precise location, extension, and neurovascular relationship. Nowadays, different managements are proposed. In this study, we present our experience in the treatment of extracranial JF schwannomas (JFss) with the extreme lateral juxtacondylar approach (ELJA). OBJECTIVE: To present our experience in the treatment of extracranial JF schwannomas (JFss) with the ELJA. METHODS: Between January 2013 and January 2017, 12 patients with extracranial JFs underwent surgery by ELJA. All lesions were type C of the Samii classification. Indocyanine green videoangiography was used to evaluate the relationship between the internal jugular vein and the tumor and to control the presence of spasm in the vertebral artery. RESULTS: A complete exeresis was achieved in 9 patients while in 3 patients, it was subtotal. The complete regression of symptoms was obtained in 7 patients with a total resection. The remaining cases experienced a persistence of symptoms. CONCLUSION: The success of this surgery is achieved through a management that starts from the patient's position. We promote an accurate evaluation of JFs through the Samii classification: Type C tumors allow the use of ELJA that reduces surgical complications. Furthermore, we recommend the use of indocyanine green videoangiography to preserve the vessels and prevent vasospasm.

Precise differentiation between jugular foramen paragangliomas and metastases: utility of diffusion-weighted and dynamic contrast-enhanced magnetic resonance imaging.

PURPOSE: This study tested the utility of diffusion-weighted imaging (DWI) and dynamic contrast-enhanced imaging (DCE-MRI) in differentiating paragangliomas and metastases in the jugular foramen in combination with conventional imaging. METHODS: Forty-nine consecutive patients with paragangliomas or metastases between January 2015 and April 2022 were included in this retrospective study. All patients had pretreatment DWI and DCE-MRI. Between paragangliomas and metastases, normalized apparent diffusion coefficient (nADCmean) and DCE-MRI parameters were compared along with conventional imaging features (enhancement pattern, presence of flow voids, cystic/necrotic change, and bone erosion). The diagnostic performance was tested using receiver operating characteristic (ROC) analysis. RESULTS: Thirty-five paragangliomas (5 male; median 49 years) and 14 metastases (9 male; median 61 years) were analyzed. The most common 3 primary cancers included 4 lung cancers, 3 breast cancers, and 3 melanomas. The presence of flow void was significantly different between paragangliomas and metastases (21/35 vs 2/14; P = 0.0047) in conventional imaging features, while fractional plasma volume (Vp) was significantly different between the two tumor types (median 0.46 vs 0.19; P < 0.001) in DWI and DCE-MRI parameters. The areas under the ROC curves (AUCs) of the presence of flow void and Vp were 0.72 and 0.93, respectively. The AUC of the combination of the presence of flow void and Vp was 0.95 and significantly improved compared to that of the presence of flow void (P < 0.001). CONCLUSION: Adding DCE-MRI to the head and neck protocol can aid in the precise differentiation between jugular foramen paragangliomas and metastases.

Endoscope-assisted resection of a Samii type B jugular foramen schwannoma.

BACKGROUND: Tumors involving the jugular foramen region are challenging for surgical resection. With the development of endoscope in the past decade, surgical approaches assisted by endoscope have been widely emerged in the treatment of skull base tumors. METHODS: Herein, we report a case of jugular foramen schwannoma (Samii type B). Surgical resection was applied via a suboccipital retrosigmoidal craniotomy using surgical microscope assisted by endoscope. Gross total resection was achieved. And the patient recovered without obvious neurological deficits. CONCLUSIONS: Samii type B schwannomas involving the jugular foramen is approachable by endoscope-assisted surgery.

Anterolateral (juxtacondylar) approach with limited mastoidectomy to resect a jugular foramen meningioma.

BACKGROUND: The anterolateral (juxtacondylar) approach with limited mastoidectomy is a suitable option to expose the postero-inferior part of the jugular foramen (JF). It is particularly indicated for tumors extending in the neck beyond the jugular foramen, especially in those cases necessitating both neck control as well as control of the mastoid segment of facial nerve. METHOD: We describe here the steps to safely perform an anterolateral approach with mastoidectomy along with a brief description of its indications and limits. CONCLUSION: This approach represents a valid option to reach the JF. Its knowledge can improve the process of optimal approach selection when dealing with complex pathology involving the JF.