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.

Jugular foramen stenosis in external hydrocephalus in infants.

OBJECTIVE: To measure the size of jugular foramina in infants affected by external hydrocephalus (EH) and in a control group, to support the hypothesis that a jugular foramen (JF) stenosis may determine dural venous sinus alterations and increased venous outflow resistance as main pathophysiological factor. METHODS: Minimum, maximum, and mean values of JF areas were measured in a series of phase-contrast magnetic resonance venous angiography (angio MRV PCA3D) performed on 81 infants affected by EH. Results were compared with a group of 54 controls. RESULTS: Smaller JF area was significantly smaller in patients versus controls (43.1 ± 14.6 vs. 52.7 ± 17.8; p < 0.001) resulting in a significantly smaller mean JF areas in patients vs. controls (51.6 ± 15.8 vs. 57.0 ± 18.3; p = 0.043). In patients, smaller JF areas were significantly associated with higher venous obstruction grading score (VOGS) both on the right (p = 0.018) and on the left side (p = 0.005). Positional plagiocephaly (cranial vault asymmetry index > 3.5%) was more frequent among EH patients than controls (38/17) but the difference was not significant (p = 0.07). In the 38 plagiocephalic patients, JF area was smaller on the flattened side than the contralateral in a significant number of cases both in right (21/7) and left (9/1) plagiocephaly (p < 0.0005) as well as the mean area (48.2 + 16.4 mm2 vs. 57.5 + 20.7 mm2, p = 0.002) and VOGS was significantly higher on the plagiocephalic side than on the contralateral side (1.6 ± 1.1 vs. 1.1 ± 0.9, p = 0.019). CONCLUSION: In this series of infants affected by EH, the mean size of the ostium of both JF resulted significantly smaller than controls. JF stenosis was significantly associated with higher degrees of venous obstruction on both sides, suggesting a direct extrinsic effect of JF size on dural sinus lumen and possible consequent effect on venous outflow resistance. Positional plagiocephaly, when present, was associated with a decreased JF area and increased VOGS on the flattened side.

Microsurgical resection of a samii type D jugular foramen schwannoma via the carotid triangle without removal of bony structure: how I do it.

BACKGROUND: Samii Type-D jugular foramen schwannomas (JFSs) are the most challenging for neurosurgeons because of anatomical complexity. Various neurosurgical approaches have been described to gain access to JF. METHODS: We present a female with incidental diagnosis of the Type-D JFS. Complete radical resection was achieved via the carotid triangle approach without any bony structure removal. And the patient was discharged asymptomatic and without new-developed neurological deficits. CONCLUSIONS: The carotid triangle is a secure and appropriate approach for some cases of selected Type-D JFSs. However, the specific indications of this approach should be further explored and investigated.

The intrabulbar or extrabulbar growth pattern and its surgical outcomes of jugular foramen paragangliomas.

OBJECTIVE: This study is to define a subclassification system of jugular foramen paragangliomas (JFPs) and to demonstrate corresponding microsurgical outcomes of JFPs. STUDY DESIGN: Retrospective study. SETTING: A single-center study. METHODS: We conducted a retrospective review of the clinical data of 44 patients with JFPs who underwent surgical management. Extrabulbar(Be) tumor and intrabulbar(Bi) tumor are defined based on the growth patterns, receiver operating characteristic (ROC) curves of the imaging profile were generated and was confirmed based on intraoperative findings. Area Under Curve (AUC), accuracy, sensitivity, and specificity for diagnostic imaging were revealed. We also compared the correlation between the two growth patterns with Fisch's classification, blood loss, lower cranial nerves (LCNs) deficit. RESULTS: There are 27 (69%) cases of Bi tumor and 17 (39%) cases of Be tumor. Significant radiomics features between the two growth patterns were demonstrated, ROC curves achieved excellent AUCs for MRI sequences (T1W1 MRI, MR contrast-enhanced sequence, MR complex sequences and MR complex + DSA by 0.833, 0.833, 0.875, 0.944) and had statistically significant in diagnosis of two growth patterns (P<0.05). There was no statistical correlation between growth patterns of JFPs and intra-operative blood loss. Preoperative LCNs deficits and Fisch's classification of tumors were correlated with the growth patterns of JFPs (P < 0.05). CONCLUSION: We proposetd two growth patterns of JFPs in term of the inferior petrous sinus involvement. Identification of Bi or Be growth patterns preoperatively is helpful to design optimal surgical strategies and minimize postoperative complications.

Microsurgical anatomy of the glossopharyngeal nerve.

The glossopharyngeal nerve is a complicated and mixed nerve including sensory, motor, parasympathetic, and visceral fibers. It mediates taste, salivation, and swallowing. The low cranial nerves, including IXth, Xth, and XIth, are closely related, sharing some nuclei in the brainstem. The glossopharyngeal nerve arises from the spinal trigeminal nucleus and tract, solitary tract and nucleus, nucleus ambiguous, and inferior salivatory nucleus in the brainstem. There are communicating branches forming a neural anastomotic network between low cranial nerves. Comprehensive knowledge of the anatomy of the glossopharyngeal nerve is crucial for performing surgical procedures without significant complications. This review describes the microsurgical anatomy of the glossopharyngeal nerve and illustrates some pictures involving the glossopharyngeal nerve and its connective and neurovascular structures.

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.

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.

Microsurgical resection of a right dumbbell-shaped jugular foramen schwannoma through a modified retrosigmoid infra-jugular approach: how I do it.

BACKGROUND: Surgical treatment of dumbbell jugular foramen schwannomas can be challenging. The main goals of surgery are maximal resection with preservation of function and overall patient quality of life. METHODS: In this paper, we present a step-by-step technical description of a microsurgical resection of dumbbell-shaped JF schwannoma using a modified retrosigmoid infra-jugular approach. CONCLUSION: The modified retrosigmoid infra-jugular is a safe and suitable approach in selected cases. This technique, however, must be limited only to those tumors with minimal extension into the jugular foramen.

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.

Intravenous sinus meningioma with intraluminal extension to the internal jugular vein: case report and review of the literature.

BACKGROUND: Meningiomas often invade venous sinuses, but intravenous sinus meningiomas remain within the intracranial cavity. This case report describes an extremely rare case of tentorial meningioma with venous sinus invasion, extending intraluminally into the lower part of the internal jugular vein in a 59-year-old man. CASE PRESENTATION: The patient's initial surgery involved the supratentorial component of a right tentorial meningioma, which invaded the right transverse and sigmoid sinuses. The supratentorial component of the tumour did not enlarge during the 2-month waiting period for the first surgery. The patient received postoperative radiation therapy for the residual tumour in the intravenous sinus. Despite radiation, the residual tumour developed caudally and ultimately extended into the right internal jugular vein. The average regrowth speed of the extracranial mass was 3.6 mm/month. The patient underwent surgery for the recurrent tumour located in the transverse sinus, sigmoid sinus, jugular bulb, and internal jugular vein, 46 months after the initial surgery. The pathological features of both surgeries were the same; WHO grade I meningothelial meningioma. CONCLUSIONS: To the best of our knowledge, there have been few case reports of benign meningioma with intraluminal extension into the internal jugular vein, and there have been no reports of long-term observation of such cases. Detailed observation of the present case suggests that the difference in growth speed between the intracranial and venous cavity depends on the surrounding environment.

Three-dimensional structure of the facial canal and related blood vessels and nerves in the temporal bone.

PURPOSE: There are only limited anatomical data on nerves, veins, and arteries in the temporal bone. More detailed anatomical data are required to improve planning of treatments targeting the temporal bone region. Herein, we performed a detailed analysis of the facial canal (FC) and the related carotid artery and vein. METHODS: We examined the bony structure of the middle ear and FC, jugular foramen, and carotid canal in 30 Japanese elderly donor cadavers. Three-dimensional reconstruction of the canal structure was achieved using cone beam computed tomography, while macroscopic and histological analyses were also performed. RESULTS: The FC form was classified as either straight (28%) or bent (72%). There were significant differences in the diameter of the FC and the distance between the internal jugular vein, other FC branches, and the FC. Principal component analysis (PCA) was performed for the FC using 29 factors. Two principal components significantly explained 30.9% (component 1, 18.6%; component 2, 12.3%) of the FC. Histological observation showed numerous ganglion cells and shrunken neurons in the geniculate ganglion of the facial nerve of elderly samples. CONCLUSION: FC diameter is an important contributor to the relationship between the FC and the jugular foramen. The FC and the internal jugular vein are located close to each other, which is useful information for the trans-canal surgery of the otology. Furthermore, the geniculate ganglion contains numerous ganglion cells and shrunken neurons, which may affect the FC structure during bone matrix remodeling with aging.

[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.

Surgical treatment of selected tumors via the navigated minimally invasive presigmoidal suprabulbar infralabyrinthine approach without rerouting of the facial nerve.

The feasibility of a novel skull base approach - the navigated minimally invasive presigmoidal suprabulbar infralabyrinthine approach (NaMIPSI-A) without rerouting of the fallopian canal for selected jugular foramen tumors (JFTs) - has been demonstrated in a neuroanatomical laboratory study. Here, we present our clinical experience with the NaMIPSI-A for selected JFTs, with a particular focus on its efficacy and safety. All patients with JFTs who were treated via the NaMIPSI-A were included in this study. The JFTs were classified according to a modified Fisch classification. The neurological and neuroradiological outcome, the extent of tumor resection, and the approach-related morbidity were examined. Five patients (two women, three men; mean age 57 years, range 48-65) were available. According to the modified Fisch classification, two JFTs were graded as C1, one as De1, and two as De2. Gross total resection (GTR) was achieved in three patients and near-total resection (NTR) in two. Postsurgically, no new neurological deficits and no approach-related morbidity and mortality occurred. One case with a postoperative cerebrospinal fluid leak was managed successfully with lumbar drainage. During the follow-up period (mean 67.6 months, range 12-119 months), tumor recurrence was noted in the NTR group but not in the GTR group. The NaMIPSI-A to the jugular foramen without rerouting of the fallopian canal is highly valuable for selected tumors of the jugular foramen. It is less invasive than other skull base approaches, and it allows safe and complete tumor removal in appropriate patients.

Management of non-vestibular schwannomas in adult patients: a systematic review and consensus statement on behalf of the EANS skull base section Part III: Lower cranial nerve schwannomas, jugular foramen (CN IX, X, XI) and hypoglossal schwannoma (XII).

BACKGROUND: Non-vestibular schwannomas are relatively rare, with trigeminal and jugular foramen schwannomas being the most common. This is a heterogenous group which requires detailed investigation and careful consideration to management strategy. The optimal management for these tumours remains unclear, and there are several controversies. The aim of this paper is to provide insight into the main principles defining management and surgical strategy, in order to formulate a series of recommendations. METHODS: A task force was created by the EANS skull base section committee along with its members and other renowned experts in the field to generate recommendations for the surgical management of these tumours on a European perspective. To achieve this, the task force performed an extensive systematic review in this field and had discussions within the group. This article is the third of a three-part series describing non-vestibular schwannomas (IX, X, XI, XII). RESULTS: A summary of literature evidence was proposed after discussion within the EANS skull base section. The constituted task force dealt with the practice patterns that exist with respect to preoperative radiological investigations, ophthalmological assessments, optimal surgical and radiotherapy strategies and follow-up management. CONCLUSION: This article represents the consensually derived opinion of the task force with respect to the treatment of non-vestibular schwannomas. For each of these tumours, the management paradigm is shifting towards the compromise between function preservation and progression free survival.

Jugular foramen capillary hemangioma masquerading as a paraganglioma.

Capillary hemangiomas are benign vascular lesions that are common in head and neck, but hemangiomas of jugular foramen and temporal bone are rare with only a few cases reported in the literature. We present a case report of this rare disease entity highlighting the subtle radiographic nuances that can benefit clinicians when encountered with similar unusual clinical scenario. Although radiographic features of capillary hemangioma can be distinctive, they are not specific. In this case, the lack of significant involvement of the jugular bulb and the absence of the typical pattern of osseous erosion of the jugular carotid spine led to the alternative diagnosis of a rare capillary hemangioma of the jugular foramen.

Three-Corridors Procedure to Operate in the Infralabyrinthine Cervico-Jugulo-Carotico-Tympanic Area.

INTRODUCTION: The aim of the study was to present the results of our experience in three-corridors procedures applied for the tumors and inflammatory lesions of the infralabyrinthine cervico-jugulo-carotico-tympanic area. METHODS: The lesions located in the infralabyrinthine cervico-jugulo-carotico-tympanic area were operated in 13 patients using the 3-corridors technique. The anatomical and functional integrity of the external and middle ears and the facial nerve (FN) could be preserved. RESULTS: The diagnoses were glomus jugulare, infralabyrinthine petrous bone cholesteatoma, jugular foramen schwannoma, and giant-cell tumor. The follow-up duration ranged from 2 to 24 months. No tumor recurrence or growth was encountered in the follow-up. The operations were uneventful. Total surgical excision could be achieved in 10 patients. A second-stage retrosigmoid approach was performed for the total removal of the intracranial tumor remnant in two patients. A wait-and-scan policy has been considered in one patient who had partial resection for a glomus jugulare tumor. CONCLUSION: Three-corridors procedure seems to be a useful technique to operate in the infralabyrinthine, cervico-jugulo-carotico-tympanic area as it takes the advantage of hearing preservation, preservation of the anatomic and functional integrity of the external and middle ear structures as well as the FN.

High bilateral fenestration of the internal jugular vein.

Duplications and fenestrations of the internal jugular vein (IJV) are rare anatomic variations. There were previously found mostly unilateral fenestrated internal jugular veins, either empty, or traversed by the spinal accessory nerves. It was documented the computed tomography angiogram of a 65 year-old female patient and bilateral high fenestrations of the IJVs were found. On both sides, the superior ends of the fenestrations were in the jugular foramina. On the right side the fenestration had 3.8 cm length and was located in front of the transverse processes of the first two cervical vertebrae. On the opposite side the fenestration had 1.5 cm length and was antero-lateral to the transverse process of the atlas vertebra. On both sides the antero-medial arms of the fenestrations received the inferior petrosal sinuses and anterior condylar veins. Such highly placed fenestrations of the IJVs should be carefully documented either preoperatively, or prior to central venous access.

Duplicated posterior inferior cerebellar arteries one of which was supplied by the jugular branch of the ascending pharyngeal artery.

PURPOSE: To describe a case of duplicated posterior inferior cerebellar arteries (PICAs), one of which was supplied by the jugular branch of the ascending pharyngeal artery (APA). CASE REPORT: A 62-year-old man with cerebral infarction underwent cranial magnetic resonance (MR) imaging and MR angiography. MR angiography showed a hyperplastic left APA, that was found to enter the posterior cranial fossa and continue to the PICA. Another left PICA arising from the V4 segment of the left vertebral artery (VA) and a tiny left anterior inferior cerebellar artery (AICA) were also identified. The source images of MR angiography revealed that the anomalous artery was passing through the medial side of the jugular foramen pars vascularis. The two left PICAs did not fuse to each other. DISCUSSION: There are four types of the PICA arising from the carotid system: (1) the PICA arises from the cavernous segment of the internal carotid artery (ICA) (persistent trigeminal artery variant); (2) the PICA arises from the cervical segment of the ICA (persistent hypoglossal artery variant); (3) the PICA arises from the APA via the hypoglossal canal; and (4) the PICA arises from the APA via the jugular foramen. Two PICAs sometimes arise from the V4 segment of the VA. In this common variation, the AICA is usually absent. This is the first reported case involving the association of (4) and a duplicated PICA with a tiny AICA. CONCLUSION: To identify this variation, careful observation of source images and creation of partial maximum-intensity-projection images of MR angiography are important.