Left Jugular Foramen Research Articles

Primary jugular foramen fibrous dysplasia: surgically nuanced video of extradural infratemporal transjugular approach. Illustrative case.

Fibrous dysplasia of the bone is a disease caused by a somatic GNAS mutation that affects craniofacial bones and can have a mass effect on different neurovascular structures. The authors present the first case of primary jugular foramen fibrous dysplasia with occlusion of the transverse and sigmoid sinuses. A 33-year-old man presented with a history of dizziness and occasional dysphagia over the past year. Magnetic resonance imaging showed a uniform enhanced mass in the left jugular foramen, with complete blockage of the adjacent transverse and sigmoid sinuses. The computed tomography scan revealed a cystic bone lesion of the jugular foramen. The patient underwent a gross-total removal of the tumor through an infratemporal transjugular approach with complete preservation of the lower cranial nerves. This first reported case of primary jugular foramen fibrous dysplasia highlights the importance of considering this diagnosis when evaluating jugular fossa lesions. Understanding the anatomy of the infratemporal and jugular fossae, along with proficiency in microsurgical techniques, is essential for removing such tumors while preserving cranial nerve functions and the patient's quality of life. https://thejns.org/doi/10.3171/CASE24396.

Are Chiari Malformation and Basilar Invagination Associated with Jugular Foramen Stenosis?

ObjectiveTo compare the diameter of the jugular foramen (JF) between normal individuals and those with the diagnosis of craniovertebral junction abnormalities, such as Chiari malformation type I (CMI) and/or basilar invagination (BI). MethodsThis cross-sectional analytical study measured the diameter of right and left JF in magnetic resonance imaging of patients that were split into four groups: 68 with combined CMI and BI (CMI+BI), 42 with isolated BI, 45 with isolated CMI, and 102 control cases. The t test determined sex differences, while univariate ANOVA with Tukey's post-hoc test assessed JF differences between groups. ResultsConsidering all combinations of laterality and sex, CMI+BI had JF narrower than their respective controls. The JF was bilaterally narrower in BI than the control, only in females. Regardless of laterality, CMI had wider JF than the CMI+BI in males, and it did not differ from the control. In the females of CMI, the right JF did not differ from the control, and it was wider than the CMI+BI; while the left JF was narrower than the control, and it did not differ from the CMI+BI. ConclusionJF narrowing is associated with CMI and BI when they are found combined in the same patient. When CMI or BI are detected separately, they do not imply evident changes in JF morphometry. It is expected that these findings could bring empiric substrate to support theories that claims a possible role of intracranial pressure disturbances as a triggering factor for CMI.

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.

Left ‐ Right Asymmetry of the Jugular Foramen in Multisuture Syndromic Craniosynostosis

IntroductionApert and Crouzon syndromes are among the most common craniosynostosis syndromes found in children. Mutations have been found in the fibroblast growth factor receptor 2 (FGFR2) gene in both syndromes. Although different mutations, the common mutated receptor raises suspicion for its involvement in the cranial morphogenesis found in these syndromic craniosynostosis patients.The shapes of these children’s skulls have long been thought to be the result of early fusion of the synchondroses. The syndromic morphology of the skulls in these patients, in particular Apert, results from a combination of abnormal development of the skull base, neural tissue, and calvaria. Investigations of these effects has led multiple studies to find decreased volumes of the posterior fossa in craniosynostosis patients and effects on the basicranial foramina of the skull, in particular the jugular foramen.Craniosynostotic children develop elevated intracranial pressure (ICP), as well as, other complications such as hydrocephalus and tonsillar herniation related to this increased pressure and skull dysmorphology. Narrowing of the jugular foramen is a potential cause of venous hypertension and may contribute to the ICP.This study analyzes the size of the jugular foramina in an effort to relate the severity of the foraminal stenosis to timing of synchondroses fusion, seen earlier in Crouzon patients. Additionally, as right jugular foramina have been found to be larger than left in the general population, our study analyzes the right and left jugular foramen sizes in relationship to each other in each Apert and Crouzon patient.MethodsTwelve preoperative CT scans of individuals diagnosed with either Apert or Crouzon syndrome were individually segmented and volume of the right and left jugular foramina estimated in Avizo. Volumes were analyzed using Wilcoxon Rank Sum and Kruskal‐Wallis one‐way ANOVA tests in SPSS. Scatter plots of the ratio of right to left foraminal volumes were created using Excel. This study was conducted under LSUHSC IRB #920.Results and DiscussionInitial results found no significant differences between right and left jugular foramina, however, absolute measurement of volumes most often recorded larger volumes on the left, contrary to that seen in the normal population. Scatter plots revealed two distinct clusters based on ratio of left‐right asymmetry which may reflect a difference in diagnosis.

Symptoms: Middle Ear Mass and Hearing Loss

Symptoms: Middle Ear Mass and Hearing Loss

Fatal Case of Dysphagia Induced by Neurofibromatosis Type II

A 17-year-old boy was found in an unconscious state. He had had neurofibromatosis type 2 (NF2) since 9 years old. His history included multiple operations for NF2 at the left jugular vein foramen, thoracic spinal cord, and cervical spinal cord at 9 and 10 years old. However, remaining bilateral acoustic tumors resulted in the onset of aspirated pneumonia within a few months. He was found unconscious in his dormitory. When emergency medical technicians checked him, he was in cardiac arrest. Saburra was identified in his mouth. He did not achieve return of spontaneous circulation with advanced cardiac life support. Autopsy imaging revealed residual bilateral acoustic tumors compressing the brain stem. NF2 is a serious disease that can rapidly become life-threatening. We hope new treatments that will improve the survival of NF2 patients will be developed soon.

Relationship between jugular foramen dimensions and neurocranium shape

BackgroundSeveral studies reported that the jugular foramen (JF) dimensions are related to the size of the internal jugular vein and morphology of the superior bulb. The present study raises the hypothesis that the JF dimensions may also be influenced by the skull dimensions. This study aims to investigate whether there is a relationship between the JF dimensions and the neurocranium shape. MethodsThe greater depth of the jugular fossa (DJF), the greater diameter of the lateromedial axis (DLM), and the largest diameters of the neural compartment (DNC) and vascular compartment (DVC) were measured in a sample of 97 adult skulls. Using these measures 3 J F indices were proposed: index 1 = 100 * DVC/DLM; index 2 = 100 * DJF/DLM; index 3 = 100 * DVC/DJF. The neurocranium shape was obtained by cranial index (CI) as being 100 * bitemporal width/fronto-occipital length. ResultsThe skulls had the CI with mean values 82.64 ± 3.74 (brachycephalic) and the analyzes showed that the left DJF tended to be shallower in the skulls with high CI values (p = 0.023). A significant correlation was observed for the CI and index 1 at the right side (p = 0.007), and CI and index 3 at right (p = 0.008) and left (p = 0.038) sides. All JF indices showed that right and left JF are proportionally symmetrical between sides (p ≥ 0.05). ConclusionThe JF proportions were weak but significantly related to the neurocranium shape, mainly in the vascular compartment. Indices that assess the JF proportions have been described, and may be useful in future studies.

Observable Differences in Symmetry and Volume of Jugular Foramen in Children with Craniosynostosis

Craniosynostosis is the premature fusion of one or more calvarial sutures. Craniosynostosis can present as part of a genetic syndrome, as in Apert or Crouzon Syndrome, or it may be an isolated finding in non‐syndromic patients. The premature closure of these sutures can lead to morphologic and physiologic anomalies. Synostotic patients present with craniofacial malformations, increased intracranial pressure and intracranial hypertension. These patients often present with anomalous vascular and osseous anatomy, specifically, large emissary veins and stenotic cranial foramina.CT scans were used to generate three‐dimensional models of the patients. The jugular foramen was manually modeled, using the intracranial foramen and the level of the odontoid process as anatomical landmarks. Each measurement was repeated three times to ensure consistency.This case series serves as a basis for continued research into the anomalous anatomy of synostotic patients. When compared to their normal counterparts, synostotic patients present with markedly asymmetric jugular foramina. A high degree of variability was observed between patients, and bilaterally within each patient. Large differences in shape and volume are noted between the right and left foramen. At this point, due to the small size of the study, inferences about the patient population at large cannot be made. However, some general similarities were observed.Our preliminary data show that in all subjects, the right jugular foramen was longer and wider than the left. It was noted that the right foramen has a more curved trajectory, moving in the posterior to anterior direction, while the left is relatively straight. This may be related to the left jugular foramen's apparent propensity to merge with the carotid canal.The observed anomalies likely contribute to the physiologic sequelae of craniosynostosis. We hypothesize that the right jugular foramen is consistently larger due to the backwards pulse pressure coming from the right atrium of the developing fetus. We propose that the timing of the pathophysiologic insult determines the character of the osseous anomaly. Measuring the surface area two‐dimensionally leads to an incomplete and potentially inaccurate representation of the jugular foramen in synostotic patients due to the irregularity of caliber throughout the jugular foramen's course. It is proposed that this osseous irregularity is in part due to diminished post‐natal remodeling. We propose that the most appropriate and rigorous method for examining the jugular foramen in synostotic patients is by obtaining the volume of the jugular foramen by three‐dimensional modeling.This abstract is from the Experimental Biology 2018 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Ewing`s Sarcoma of Temporal Bone : Case Report

Primary Ewing`s Sarcoma (ES) is a small round tumor, more likely to present in long bones and rarely in skull bones. ES commonly present in the second decade of life, and it is usually treated by multi‐modality including surgery, chemotherapy, and radiotherapy. In this case, a 13 years old boy presented with left facial palsy and hearing loss for two weeks, treated with steroid but no improvement. MRI was done and showed left jugular foramen and cerebellopontine mass measuring 4.8*4.4* 3 cm. Debulking surgery of the tumor was done with multiple biopsies reported by histopathology as malignant small round cell tumor consistent with Ewing sarcoma.

Severe skull base osteomyelitis caused by Pseudomonas aeruginosa with successful outcome after prolonged outpatient therapy with continuous infusion of ceftazidime and oral ciprofloxacin: a case report

BackgroundSkull base osteomyelitis is an uncommon disease that usually complicates a malignant external otitis with temporal bone involvement. It affects predominantly diabetic and immunocompromised males and has a high mortality rate. Pseudomonas aeruginosa is the most common causative organism. Currently, there is no consensus about the best therapeutic option. Here we describe a case of severe skull base osteomyelitis caused by Pseudomonas aeruginosa with progressive palsy of cranial nerves that was successfully managed with prolonged outpatient continuous infusion of ceftazidime plus oral ciprofloxacin.Case presentationA 69-year-old Caucasian man presented with dysphagia, headache, and weight loss. He complained of left earache and purulent otorrhea. Over the following weeks he developed progressive palsy of IX, X, VI, and XII cranial nerves and papilledema. A petrous bone computed tomography scan showed a mass in the left jugular foramen with a strong lytic component that expanded to the cavum. A biopsy was then performed and microbiological cultures grew Pseudomonas aeruginosa. After 6 weeks of parenteral antibiotic treatment, our patient was discharged and treatment was continued with a domiciliary continuous infusion of a beta-lactam through a peripherally inserted central catheter, along with an oral fluoroquinolone for 10 months. Both radiological and clinical responses were excellent.ConclusionsSkull base osteomyelitis is a life-threating condition; clinical suspicion and correct microbiological identification are key to achieve an accurate and timely diagnosis. Due to the poor outcome of Pseudomonas aeruginosa skull base osteomyelitis, prolonged outpatient parenteral antibiotic therapy administered by continuous infusion could be a valuable option for these patients.

A Woman With Chronic Postprandial Abdominal Pain and Vomiting

Question: A 35-year-old woman presented with a 3-month history of postprandial abdominal discomfort and vomiting. The patient’s history included left jugular foramen schwannoma with involvement of the vagus and spinal accessory nerves (cranial nerves X and XI) treated with left level II/III neck dissection 4 months before presentation; intraoperatively, electromyography (EMG) monitoring confirmed transection of both nerves in the process of tumor resection. Physical examination yielded a soft, nondistended abdomen with bowel sounds present; no guarding, rebound, or bruits were found. The only laboratory abnormality included a mildly elevated leukocyte count of 11.7 10/L (normal range, 3.5-10.5 10/L). Abdominal computed tomographic imaging demonstrated pronounced gallbladder contraction (Figure A [transverse plane] and Figure B [coronal plane]; arrows) without additional intra-abdominal pathology. Subsequent abdominal ultrasound performed in the fasting state also demonstrated gallbladder contraction (Figure C). The patient proceeded to cholecystectomy for diagnostic and therapeutic intent. Postoperative surgical pathology revealed chronic inflammation and thickened muscularis mucosa of the gallbladder (Figure D; star indicates thickened muscularis mucosa). After cholecystectomy, the patient reported complete symptomatic resolution. What are the diagnosis and the likely etiology? Look on page 47 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.

Role of "major" and "minor" lambdoid arch sutures in posterior cranial fossa changes: mechanism of cerebellar tonsillar herniation in infants with multisutural craniosynostosis.

This study aimed to explain the functional role of lambdoid arch sutures in the development of cerebellar tonsillar herniation. Posterior cranial fossa (PCF) changes were investigated in infants with premature synostosis of the major and minor sutures of the lambdoid arch without premature synostosis of the PCF synchondroses. Morphometric and volumetric PCF measurements were performed on preoperative high-resolution CT studies in 12 infants with multisutural craniosynostosis involving the lambdoid arch and compared with those of 12 age-matched healthy subjects. All 12 patients had hypoplasia of PCF bone structures and normal volumes of the PCF and neural structures. PCF hypoplasia was related to exocciput length in infants with isolated involvement of major sutures, while it was related to posterior skull base hemifossae in infants with isolated involvement of minor lambdoid arch sutures. Foramen magnum AP diameter was reduced in babies with major suture involvement and tonsillar herniation, while foramen magnum AP and LL diameters were reduced in babies with minor suture involvement without tonsillar herniation. Right and left jugular foramen (JF) areas differed in all infants; however, the area of the smaller JF was significantly reduced only in infants with involvement of minor lambdoid arch sutures. Hypoplasia of PCF bone structures due to sutural synostosis of the lambdoid arch is a required predisposing but not sufficient factor for the development of cerebellar tonsillar herniation through the foramen magnum. Normal PCF volume and foramen magnum anatomy may partly explain the development of cerebellar tonsil herniation in infants with lambdoid arch synostosis.

GE-01 * IDH1-MUTATED PITUITARY ADENOMA CHALLENGES TRADITIONAL CHARACTERIZATION OF MAFFUCCI SYNDROME

Maffucci syndrome is a rare, non-inheritable condition of mesodermal dysplasia, presenting in childhood with formation of multiple enchondromas and soft-tissue hemangiomas. Approximately, 40% of enchondromas undergo malignant transformation into chondrosarcomas. The underlying genetic etiology lies in somatic mosaicism of mutations in isocitrate dehydrogenase 1 (IDH1) or IDH2, which have been detected simultaneously in both tumors and blood leukocytes of affected patients. We describe a 7-year old male who presented with extensive enchondromatosis and subcutaneous hemangiomas, clinically diagnosed as Maffucci syndrome. Years later, he developed sub-acute dysphasia, dysarthria, and loss of left visual acuity. A brain MRI demonstrated two heterogeneously enhancing lesions, one centered in the left jugular foramen and the other located in the supra-sellar region. Surgical resections of both intracranial tumors were performed over a period of ten months. Histopathological review revealed diagnoses of a grade II chondrosarcoma as well as a non-functional pituitary adenoma after subsequent neuroendocrine studies were unremarkable. Genotypic studies of the tumors were performed to identify common IDH1/2 mutations. DNA sequencing utilized standard Sanger sequencing in conjunction with a target-specific peptide nucleic acid (PNA) to detect IDH1 mutations in tumor tissues. The results demonstrated identical IDH1 mutations (c.394C > T; R132C) in both tumors. This result represents the first genetic evidence to establish causality between pituitary adenoma formation and tumor predisposition in Maffucci syndrome. As neuro-ectodermal tissue, this IDH1-mutated pituitary adenoma challenges the notion that Maffucci syndrome only involves mesodermal tissues. As such, in pediatric patients newly diagnosed with Maffucci syndrome, it may be appropriate to monitor for development of pituitary pathology and neuroendocrine dysfunction. Conversely, in the rare sub-population of pediatric neuro-oncology patients with concomitant non-nervous system tumors, further investigation is warranted to rule out Maffucci syndrome, as there may be considerable risk for malignant transformation as well as additional tumor formation.

Isolated Hypoglossal Nerve Schwannoma: An Uncommon Presentation of Schwannoma.

Isolated Hypoglossal Nerve Schwannoma: An Uncommon Presentation of Schwannoma.

Variations in jugular foramen of human skull

Background: Jugular foramen is one of the foramen at the base of skull lies between the occipital bone and the petrosal portion of the temporal bone. It allows passage of important nervous and vascular elements, such as the glossopharyngeal, vagus accessory nerves, and the internal jugular vein. Glomic tumors, schwannomas, metastatic lesions and infiltrating inflammatory processes are associated with this foramen, which can account for injuries of related structures. Variability in anatomical aspect of jugular foramen has been studied by many workers in different part of the world. Aims & Objective: To study the variability in shape and size of jugular foramen which has clear cut relationship with size of internal jugular vein and presence or absence of prominent superior bulb. The study is also aimed to confirm the description given in most of the text book of Anatomy that mostly right jugular foramen is larger than left (Figure 1). Materials and Methods: Present study has been designed to study on 68 skulls (68×2=136 foramen). Result: In the study Bilateral presence of dome has been found in 57.35% of cases whereas bilateral absence in 4.41% of cases. In 64.7% of case larger right and in 19.1% of cases larger left foramen have been observed. In remaining 16.1% of cases both left and right jugular foramen are almost equal in size. Both unilateral and bilateral complete septation have been observed. DOI: http://dx.doi.org/10.3126/ajms.v6i2.10940 Asian Journal of Medical Sciences Vol.6(2) 2015 95-98

Variations in the Structure of the Jugular Foramen of Human Skull

Context: The jugular foramen is one of the most fascinating foramen present at the base of the skull attracting the imagination of many Anatomists worldwide as many important structures pass through it, and amongst them the intriguing structure is the internal jugular vein. The shape and size of the jugular foramen is related to the size of the internal jugular vein and the presence or absence of a prominent superior bulb. As most of the textbooks of Anatomy describe that the right jugular foramen is usually larger than the left jugular foramen. Henceforth the present study was undertaken in 55 skulls from the dept. of Anatomy. Measurements were taken with the help of sliding vernier caliper. Study type: Cross-sectional descriptive type. Place and period of study: Department of Anatomy, Rajshahi Medical College, Rajshahi and Pabna Medical College, Pabna from April 2010 to June 2011. Materials and Methods: Total fifty five (55) human adult skulls were collected from the Anatomy department of Rajshahi Medical College, Rajshahi and Pabna Medical College, Pabna at different times of the study period. The study was conducted to observe variations in the structure of the jugular foramen of the human’s skull. Result: Out of 55 skulls (110 foramina) studied, the presence of dome indicating the presence of jugular bulb was found bilaterally in 100% of cases. 58.18% of cases showed that the size of right foramina were larger than the left foramina whereas 20% of cases showed that right foramina were equal to the left and in 21.82% of cases the left foramina were larger than the right side foramina. An important observation in the present study was the presence of either complete or partial septation in the jugular foramen. Conclusion: The findings of the study reveals that there are some differences among some parameters. The variations are might be due to the geographical variations of the skeletons. It needs further study with larger sample size from different geographical areas of Bangladesh. DOI: http://dx.doi.org/10.3329/bja.v10i2.17281 Bangladesh Journal of Anatomy, July 2012, Vol. 10 No. 2 pp 45-49

Dandy-Walker Malformation, Papillary Thyroid Carcinoma, and SDHD-Associated Paraganglioma Syndrome

A 43-year-old female presented to a local hospital with the recent onset of intermittent dizziness suggestive of intermittent rotatory vertigo, but no abnormal findings on examination. Magnetic resonance imaging of the brain demonstrated a large cerebrospinal fluid collection in the posterior fossa, incomplete fusion of the posterior elements of the cerebellum and vermis, and dilatation of the third and lateral ventricles, indicative of a significant Dandy-Walker malformation (Figures 1 and 2). The patient reported a history of papillary thyroid carcinoma diagnosed at 16 years of age and an abdominal paraganglioma diagnosed at the age of 28 in the immediate postoperative period after a cesarean section. Her 2 samples of 24-hour urinary normetanephrine was 10 578 and 19 409 nmol/24 h (normal, 600); the tumor was 4-cm in maximum diameter, I-MIBG positive, and at the level of the lower pole of the right kidney. It was successfully removed. There were no cutaneousfeaturessuggestingahereditarysyndrome.Family history revealed two daughters who had both undergone craniofacial surgery. Genetic analysis demonstrated a pathogenic germline succinate dehydrogenase (SDH) type D mutation (c.60_ 63delGCTT). Further investigation revealed bilateral head and neck paragangliomas at the left jugular foramen and on the right just above the carotid bifurcation; these are currently being considered for therapy. The asymptomatic Dandy-Walker malformation in our patient is an uncommon finding, and few cases have been diagnosed in adults (1). SDHx mutations cause familial paraganglioma syndromes but have also been related to other neoplasias such as renal and pituitary tumors (2) and thyroid cancer (3). It seems probable that SDH may be involved in central nervous system organogenesis as well as acting as a tumor suppressor.

Zespół Verneta w przebiegu wieloogniskowego przyzwojaka szyjnego – opis przypadku

Cervical paragangliomas are rare tumors of the neuroectodermal origin. Extensive paragangliomas press on the regional bone and neurovascular structures. 90-years-old woman was admitted to our department with sinister deafness and smooth painless tumor in the left external auditory meatus. The patient has suffered from sinister tinnitus and progresive sinister hypacusis for 11 years, hoarseness for 9 years, dysphagia for 4 years and the left humeral articulation's mobility dysfunction for 1 year. In diagnostic imaging two good vascularized tumors were shown in the left jugular foramen and in the left carotid bifurcation. We diagnosed the sinister glossopharyngeal, vagus and accessory nerves paralysis in multifocal cervical paraganglioma. Authors concluded that in a case of the one side hypacusis and the ipsilateral lower cranial nerve lesion the temporal bones CT shuold be the first step in the diagnostic process. The characteristic clinical picture of cervical paragangliomas permits diagnose them without histopathology.

Morphometric analysis of the foramen magnum and jugular foramen in adult skulls in southern Nigerian population

A morphometric analysis of the foramen magnum and jugular foramen of adult skulls in southern Nigeria was carried out to demonstrate the anatomical variations in morphology. A total number of 120 dry skulls were used for this study. Measurements were performed by using a digital vernier caliper to span across the lengths and widths of the two foramina. Results revealed that the mean length and width of the foramen magnum was 36.11±0.24mm and 29.65± 0.24mm respectively. The mean length of the right and left jugular foramen was 15.76±0.22mm and 13.39±0.23mm respectively, while the mean width of the right and left jugular foramen was 9.34±0.18mm and 7.54±0.20mm respectively. There was a significant difference between the right and the left jugular foramen. The right jugular foramen was found to be larger than the left in Southern Nigeria.

Skull Base Aneurysmal Bone Cyst Presented with Foramen Jugular Syndrome and Multi-Osseous Involvement

Aneurysmal bone cyst (ABC) is an expansile bone lesion that usually involves the long bones. Skull base involvement is rare. Hereby, we describe a 17-year-old man with hoarseness, facial asymmetry, left sided sensorineural hearing loss and left jugular foramen syndrome. CT scan and MRI showed a skull base mass that was confirmed as ABC in histopathology. The case was unusual and interesting due to the clinical presentation of jugular foramen syndrome and radiological findings such as severe enhancement and multiosseous involvement.