Jugular Foramen Paragangliomas Research Articles

The surgical outcomes in management of jugular foramen paragangliomas with tension free anterior rerouting of the facial never and tunnel packing of the inferior petrous sinus

Objective:The aim of this study is to evaluate the safety and efficacy of surgical interventions ofjugular foramen paragangliomas（JFP） utilizing modified surgical techniques, tensionfree anterior rerouting of the facial nerve and tunnel-packing or push-packing of the inferior petrous sinus. Methods:A retrospective analysis was conducted on a cohort of 88 patients diagnosed with JFP and treated at the Eye Ear Nose and Throat Hospital of Fudan University（in Shanghai, China） from October 2010 to June 2021. The surgical outcomes were analyzed for tumor classification, intraoperative conditions, and function of the postoperative facial nerve（FN） and lower cranial nerve（LCN）. Results:The study included a total of 88 patients, gross total resection was achieved in 70 patients（79.5%）, near total resection was obtained in 17 patients（19.3%）, and one patient undergoing subtotal resection. The average of intraoperative blood loss was 448.3 mL. Additionally, 24 patients underwent surgical total anterior rerouting（TAR）, 18 patients underwent surgical total FN tension free anterior rerouting（TF-TAR）, and 18 patients underwent surgical FN partial FN tension free anterior rerouting（TF-PAR）. Good postoperative FN function（House-Brackmann Ⅰ-Ⅱ） was achieved in 62.5% of TAR group. In the TF-TAR and PF_TAR groups, good postoperative FN function was demonstrated in 88.9% patients. It showed a significantly improvement of the FN function following application of tension-free FN anterior rerouting technique（P=0.007）. Twenty patients（22.7%） suffered from at least one LCN deficit in the preoperative evaluation. The postoperative LCN deficits was correlated with the Fisch classification of tumors, which showed a lower incidence of LCN dysfunction in classes C1-C2（4.9%, 2/41cases） and poorer outcomes of LCN dysfunction in classes C3-D（8.5%,4/47cases ）, it was likely less impacted the LCN function in the early stage tumor. Conclusion:The application of modified surgical techniques of FN tension-free anterior rerouting and tunnel-packing of the inferior petrous sinus has been shown to effectively preserve the function of the FN and LCN, decrease intraoperative blood loss, and ultimately improve patients' postoperative quality of life.

Exploration of technique for preservation of external-middle ear structure in surgery of jugular foramen paraganglioma （appended 2 case reports）

Objective:To investigate the feasibility and effect of the modified surgery of the classic infratemporal fossa type A approach for the surgical treatment of jugular foramen paraganglioma with preservation of the external and middle ear structures. Methods:The medical data of 2 patients with jugular foraminal paraganglioma treated by sublabyrinthic-transmastoid approach were retrospectively analyzed. The clinical feature, degree of tumor resection, postoperative facial nerve function and hearing retention, and the incidence of postoperative complications were evaluated. Results:Two patients were both female, and were pathologically confirmed as paraganglioma. The tumor of case 1 was staged as C2De1, and case 2 as C1De1. Tumors were completely resected in both patients. Case 1 suffered infection after surgery, with residual tympanic membrane perforation and mixed deafness. Case 2 developed mild facial paralysis（grade Ⅱ） after surgery, and recovered after symptomatic treatment. There was no tumor residue or recurrence during half a year of follow-up. Conclusion:Surgical treatment of certain paragangliomas in the jugular foramen with a combined sublabyrinthic-transmastoid and upper neck approach might achieve both complete resection of the tumor and preserving the structure and function of the outer-middle ear. This procedure is suitable for paragangliomas restricted in the jugular foramen area, with no or limited involvement of the internal carotid artery（C1 or C2）, and with no or mild hearing loss.

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

This study is to define a subclassification system of jugular foramen paragangliomas (JFPs) and to demonstrate corresponding microsurgical outcomes of JFPs. Retrospective study. A single-center study. 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. 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). 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.

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

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. 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. 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. 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.

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. 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. 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. 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. 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.

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

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. 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. Thirty-five paragangliomas (5 male; median 49years) and 14 metastases (9 male; median 61years) 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). Adding DCE-MRI to the head and neck protocol can aid in the precise differentiation between jugular foramen paragangliomas and metastases.

Staged Surgery for Intra-Extracranial Communicating Jugular Foramen Paraganglioma: A Case Report and Systematic Review

Staged surgery strategy was preferred for patients with intra-extracranial communicating jugular foramen paraganglioma (IECJFP). A female patient who presented mild tinnitus, headache, and dizziness, together with preoperative related imaging, was diagnosed with a left intra-extracranial communicating jugular foramen lesion in November 2015 and accepted an initial operation for the intracranial tumor by retrosigmoid approach. The pathologic report was paraganglioma. In November 2021, a subtotal resection of the extracranial tumor was conducted for prominent lower cranial nerves (LCNs) deficit and middle ear involvement by infratemporal approach. In patients with IECJFP accompanied by LCNs deficit and middle ear involvement, an initial surgery for extracranial lesion and a second procedure for intracranial tumor were appropriate. However, the first operation for the intracranial lesion was preferred in IECJFP cases without LCNs deficit and middle ear involvement, as it could remove compression to the neurovascular structure and brain stem, clarify a pathological diagnosis, avoid a CSF leak, and prevent a severe neurological disorder from extracranial lesion excision. Subtotal resection of the extracranial tumor would be performed when lesion became larger combined with obvious LCNs disorder and tympanic cavity involvement. Consideration of specific staged surgical strategy for IECJFP in accordance with preoperative LCNs deficit and tympanic cavity involvement could prevent critical postoperative neurological deficit and improve quality of life in the long term.

Surgical management of embolized jugular foramen paragangliomas without facial nerve transposition: Experience of a public tertiary hospital in Brazil.

Background: Jugular foramen paragangliomas (JFP) treatment represents a challenge for surgeons due to its close relationship with facial nerve (FN), lower cranial nerves (LCN), and internal carotid artery. Due to its hypervascularization, preoperative tumor embolization has been indicated.Methods: Retrospective analysis of the clinical evolution of 26 patients with JFP class C/D previously embolized treated through infratemporal/cervical access without FN transposition.Results: Total and subtotal resections were 50% each, regrowth/recurrence were 25%, and 23%, respectively, and mortality was 3.9%. Postoperatively, 68.4% of patients had FN House and Brackmann (HB) Grades I/II. New FN deficits were 15.4% post embolization and 30.7% postoperatively. Previous FN deficits worsened in 46.1%. Tumor involved the FN in 30.8% and in 62.5% of them these nerves were resected and grafted (60% of them had HB III). Lateral fall, ear murmur, and vertigo improved in all patients. Tinnitus improved in 77.8% and one patient developed tinnitus after surgery. Hearing loss did not improve, eight partial hearing loss remained unchanged and four worsened. New postoperative LCN deficits were 64.3%. Postoperative KPS between 80 and 100 dropped 8.3%. Two patients with secretory paragangliomas with arterial hypertension difficult to control had better postoperative blood pressure control.Conclusion: Although still with significant morbidity due to FN and LCN injuries, the treatment of patients with JFP Fisch C/D has good long-term results. Surgical techniques without FN transposition have less intraoperative nerve damage, lower rates of total resection, and higher recurrence. Preoperative embolization of JFP reduces the intraoperative blood loss but can cause FN deficit.

Tension-Free Anterior Rerouting of the Facial Nerve in Management of Jugular Foramen Paragangliomas.

Tension-Free Anterior Rerouting of the Facial Nerve in Management of Jugular Foramen Paragangliomas.

Jugular foramen paragangliomas: preoperative transcatheter particle embolization.

Jugular foramen paragangliomas (JFP) are benign tumors of neural crest origin that are located along the temporal bone in the region of the jugular bulb and middle ear. The optimal management of these lesions includes surgical excision with or without preoperative embolization as well as stereotactic radiotherapy. The use of preoperative embolization in the treatment of JFP has shown great promise to bridge patients to surgery by diminishing complication rates and decreasing intraoperative bleeding. We present three successful polyvinyl alcohol (PVA) particle embolizations of patients presenting with symptomatic JFPs. All patients recovered completely in the short term with no bleeding during or after resection of paragangliomas and they were discharged free of their presenting symptoms. Early clinical and imaging diagnosis followed by adequate treatment including preoperative transcatheter particle embolization and surgical or radiosurgical interventions can lead to excellent outcomes.

Surgical Candidacy in Skull Base Paragangliomas: An Institutional Experience.

Objective The aim of the study is to determine the surgical candidacy and nuances of skull base paraganglioma surgery in the era of radiotherapy. Materials and Methods This was a retrospective observational study conducted in patients who presented with skull base paragangliomas between January 2017 and December 2019. Primary data, including indication for surgery, the approach used, the extent of resection, complications, and postoperative lower cranial nerve status were studied. Results A total of 21 cases of skull base paragangliomas were analyzed, including seven cases of tympanic paraganglioma, 10 cases of jugular foramen paraganglioma, three cases of multiple paragangliomas, and one case of vagal paraganglioma. Indications for surgery were young age, bleeding from ear, neck mass with upper aerodigestive pressure symptoms, lower cranial nerve paralysis, and patients with intracranial pressure symptoms. Total excision was done in 11 patients, near-total excision in five patients, subtotal in three patients, and surgery was not done in two patients. Facial nerve paralysis was the most common complication observed, followed by bleeding and flap necrosis. Radiotherapy was considered as adjuvant treatment wherever indicated. Conclusion A thorough knowledge and understanding of the pathophysiology of the skull base paragangliomas and its management strategies can help to achieve excellent results in terms of tumor clearance and reduction in complications. A multidisciplinary team approach and meticulous skull base surgical techniques have a significant role to play in the management of paragangliomas, especially in developing countries where availability of radiosurgery is still a challenge.

Combined Transarterial and Transvenous Onyx Embolization of Jugular Foramen Paragangliomas

Jugular foramen paragangliomas are highly vascular tumors known to have significant venous hemorrhage during resection even after conventional transarterial embolization. The authors report a novel technique to the endovascular embolization of jugular foramen paragangliomas using a combined transarterial and transvenous access for better intraoperative control of blood loss and visualization. This is a retrospective data collection of 2 patients diagnosed with jugular foramen paragangliomas with novel embolization technique and surgical resection. Two patients underwent embolization of jugular foramen paragangliomas through combined transarterial and transvenous routes using 2 double-lumen balloon microcatheters. In both cases, single arterial vessel embolization was performed through the occipital artery in Case 1 and the tympanic branch of the ascending pharyngeal artery in Case 2. Simultaneously, balloon microcatheter occlusion in the sigmoid sinus and single venous outflow vessel embolization was performed. Near-complete occlusion was established, with angiographic disappearance of tumor blush. Surgical resection was performed in both cases. Estimated blood loss BL was 600 mL in Case 1 and 200 mL in Case 2. No blood transfusions were required, intraoperatively or postoperatively. There were no cranial nerve deficits post embolization. One patient had a persistent House Brackman 2 facial nerve palsy after resection. The initial experience with simultaneous transvenous and transarterial paraganglioma embolization demonstrated the safety of the technique and superior embolic agent penetration. This was supported by our observations during embolization and intraoperatively during tumor resection. Additional patients need to be treated with this technique for better assessment of long-term efficacy and incidence of embolization-related cranial neuropathies.

Pheochromocytoma, paragangliomas, and pituitary adenoma

Rationale:Pituitary adenomas and paragangliomas are both rare endocrine diseases. Paragangliomas (PGL)/pheochromocytomas (PHEO) are part of an inherited syndrome in about 30% to 40% of cases. Among familial cases, mutations of the succinate dehydrogenase (SDH) subunit genes (succinate dehydrogenase subunit [SDH]B, SDHC, SDHD, succinate dehydrogenase subunit AF2 [SDHAF2] , and SDHA) are the most common cause.[1]Patient concerns:We here report a 31-year-old patient with a known SDHD mutation whose disease has been revealed by a left PHEO during childhood and who presented at age 29 years a large paraganglioma of the right jugular foramen, a concomitant PHEO of the left adrenal and 2 retroperitoneal paragangliomas. A pituitary incidentaloma was found during investigations on a fluorodeoxyglucose (FDG)-positron emission tomography (PET) (FDG-PET).Diagnosis:A pituitary magnetic resonance imaging (MRI) confirmed the presence of a 14 mm pituitary macroadenoma. The pituitary function was normal except for hypogonadotropic hypogonadism. On examination of the fundus, a diagnosis of Pseudo Foster-Kennedy syndrome was made due to a venous compression of the right jugular vein caused by the paraganglioma (PGL). The pituitary adenoma was not compressive to the optic chiasm.Interventions:A treatment with acetazolamide was started in order to improve intracranial hypertension. The patient couldn’t benefit of a surgical approach for the paraganglioma of the right jugular foramen; the patient has been treated with stereotactic radiosurgery (Gamma Knife).Outcomes:The most recent MRI revealed that the right jugular foramen PGL is stable and the latest visual assessment demonstrated stability despite a recent reduction in acetazolamide dosage. A surveillance by MRI of the pituitary adenoma has been planned.Lessons:The association of a pituitary adenoma to paragangliomas within a same patient is very uncommon and raises the question of related physiopathological mechanisms.

Pathophysiology of sensorineural hearing loss in jugular foramen paraganglioma.

Pathologic involvement of the inferior cochlear vein is a mechanism of sensorineural hearing loss in patients with jugular foramen paraganglioma. Retrospective case-control study. The presenting audiograms, magnetic resonance imaging, and computed tomography were reviewed in 46 subjects with jugular foramen paragangliomas. Four-frequency bone conduction average was compared between the tumor and nontumor ears in each subject to establish the presence of sensorineural hearing loss. Imaging findings for each subject were recorded. Univariate and multivariate statistical analyses were performed to determine which radiographic features were associated with sensorineural hearing loss. Hearing data were analyzed as a continuous variable and as a categorical variable. Twenty subjects (43.4%) had a bone-conduction pure-tone asymmetry of greater than 15 dB. Inferior cochlear vein involvement was identified in 19 of the 20 (95%) subjects with sensorineural hearing loss. Inferior cochlear vein involvement was found to be a statistically significant predictor of sensorineural hearing loss using univariate and multivariate analyses. Other imaging findings that were statistically significant predictors of sensorineural hearing loss include Glasscock-Jackson stage, Fisch-Mattox stage, hypoglossal canal involvement, jugulo-carotid spin erosion, and petrous carotid canal erosion. Involvement of the inferior cochlear vein appears to be a plausible mechanism for sensorineural hearing loss in patients with jugular foramen paraganglioma. 4 Laryngoscope, 129:67-75, 2019.

Less-aggressive surgical management and long-term outcomes of jugular foramen paragangliomas: a neurosurgical perspective.

OBJECTIVE The aim of this study was to analyze the neurological functional outcome and recurrent risks in surgically treated jugular foramen paragangliomas (JFPs) and to propose an individualized therapeutic strategy. METHODS Clinical charts and radiological information were reviewed retrospectively in 51 consecutive cases of JFPs. Less-aggressive surgical interventions were adopted with the goal of preserving neurovascular structures. Scheduled follow-up was performed. RESULTS The mean age of the patients in the cases reviewed was 41.6 years, and the group included 27 females (52.9%). The mean preoperative Karnofsky Performance Scale (KPS) score was 78.4. The mean lesion size was 3.8 cm. Forty-three cases (84.3%) were Fisch Type D, including 37 cases (72.5%) of Type Di1 and Di2. Thirty-seven cases (72.5%) were Glasscock-Jackson Type III-IV. Gross-total resection and subtotal resection were achieved in 26 (51.0%) and 22 (43.1%) cases, respectively. Surgical morbidities occurred in 23 patients (45.1%), without surgery-related mortality after the first operation. The mean postoperative KPS scores at discharge, 3 months, 1 year, and most recent evaluation were 71.8, 77.2, 83.2, and 79.6, respectively. The mean follow-up duration was 85.7 months. The tumor recurrence/regrowth (R/R) rate was 11.8%. Compared with preoperative status, swallowing function improved or stabilized in 96.1% and facial function improved or stabilized in 94.1% of patients. A House-Brackmann scale Grade I/II was achieved in 43 patients (84.3%). Overall neurological status improved or stabilized in 90.0% of patients. Pathological mitosis (HR 10.640, p = 0.009) was the most significant risk for tumor R/R. A 1-year increase in age (OR 1.115, p = 0.037) and preoperative KPS score < 80 (OR 11.071, p = 0.018) indicated a risk for recent poor neurological function (KPS < 80). Overall R/R-free survival, symptom progression-free survival, and overall survival at 15 years were 78.9%, 86.8%, and 80.6%, respectively. CONCLUSIONS Surgical outcomes for JFPs were acceptable using a less-aggressive surgical strategy. Most patients could adapt to surgical morbidities and carry out normal life activities. Preserving neurological function was a priority, and maximal decompression with or without radiotherapy was desirable to preserve a patient's quality of life when radical resection was not warranted. Early surgery plus preoperative devascularization was proposed, and radiotherapy was mandatory for lesions with pathological mitosis.

Jugular foramen paragangliomas: management, outcome and avoidance of complications in a series of 75 cases

Jugular foramen paragangliomas are rare skull base tumours posing multiple complex diagnostic and management problems. We did a study to evaluate surgical technique, outcome and complications in 75 cases of tumours treated by multidisciplinary approach (i.e. combined neurosurgery, neuroradiology, ear, nose and throat surgery and intensive care unit team). Retrospective study on 75 consecutive patients with jugular foramen paragangliomas treated surgically from 1989 to 2005. Preoperative balloon occlusion test was performed in all patients as well as embolization (100%). A combined limited infratemporal and juxtacondylar approach was used in all patients. Gross total resection was achieved in 59 patients (78.7%). The most common complication was represented by lower cranial nerve deficits in five patients (6.6%), which was only temporary in three. Postoperative facial nerve weakness occurred in five cases (6.6%) and resolved in three of them. The remaining two patients underwent facial nerve reconstruction by hypoglossal/facial nerve anastomosis. Four patients (5.3%) had a postoperative cerebrospinal fluid leak, which was successfully treated by lumbar drainage. Two patients (2.7%) died because of complications related to surgical injury of lower cranial nerves: one patient developed aspiration pneumonia and septicemia and the second one developed a large cervico-bulbar hematoma that led to severe respiratory distress and ultimately global cerebral hypoxia. Paragangliomas are rare and complex skull base lesions that may be managed with low morbidity and mortality if a multidisciplinary approach is considered. Facial and lower cranial nerve postoperative deficits can be limited.

Early and long-term results of the treatment of jugular paragangliomas using different ranges of surgical approach

Background and purposeThe applied approach to the jugular foramen is a combination of the juxtacondylar approach with the subtemporal fossa approach type A. The purpose of this study is to present our results of treatment of jugular paragangliomas using the aforementioned approach. Material and methodsTwenty-one patients (15 women, 6 men) with jugular paragangliomas were included in the study. The neurological status of the patients was assessed before and after surgery as well as at the conclusion of treatment. The approximate volume of the tumour, its relation to large blood vessels, cranial nerves and brainstem, as well as consistency and vascularity were also assessed. ResultsThe duration of symptoms ranged from 3 to 74 months. In 86% of patients hearing loss was the predominant symptom. The less frequent symptoms included pulsatile tinnitus in the head, dysphagia and dizziness. Approximate volume of the tumours ranged from 2 to 109 cm3. A gross total resection was achieved in 71.5% of patients. The postoperative performance status improved in 38% of patients, did not change in 38% and deteriorated in 24% of patients. ConclusionsA proper selection of the range of the approach to jugular foramen paragangliomas based on their topography and volume reduces perioperative injury without negative consequences for the radicality of the resection.

Skull Base Paraganglioma and Intracranial Hypertension

Paralysis of the seventh or lower cranial nerves is the most common neurologic complication of jugular foramen paragangliomas. It results from the local aggressiveness of these highly vascular tumors, which are located in the vicinity of important neurovascular structures. In contrast, the literature has only rarely reported intracranial hypertension (ICH), 1 usually in association with massive posterior cranial fossa invasion.

Clinical Presentation and Management of Jugular Foramen Paraganglioma

ObjectivesJugular foramen paraganglioma is a locally invasive, benign tumor, which grow slowly and causes various symptoms such as pulsatile tinnitus and low cranial nerve palsy. Complete surgical resection is regarded as the ideal management of these tumors. The goal of this study is to identify the clinical characteristics and most effective surgical approach for jugular foramen paraganglioma.MethodsRetrospective analysis of 9 jugular foramen paraganglioma patients who underwent surgical resection between 1986 and 2005 was performed. Clinical records were reviewed for analysis of initial clinical symptoms and signs, audiological examinations, neurological deficits, radiological features, surgical approaches, extent of resection, treatment outcomes and complications.ResultsMost common initial symptom was hoarseness, followed by pulsatile tinnitus. Seven out of 9 patients had at least one low cranial nerve palsy. Seven patients were classified as Fisch Type C tumor and remaining 2 as Fisch Type D tumor on radiologic examination. Total of 11 operations took place in 9 patients. Total resection was achieved in 6 cases, when partial resection was done in 3 cases. Two patients with partial resection received gamma knife radiosurgery (GKS), when remaining 1 case received both GKS and two times of revision operation. No mortality was encountered and there were few postoperative complications.ConclusionNeurologic examination of low cranial nerve palsy is crucial since most patients had at least one low cranial nerve palsy. All tumors were detected in advanced stage due to slow growing nature and lack of symptom. Angiography with embolization is crucial for successful tumor removal without massive bleeding. Infratemporal fossa approach can be considered as a safe, satisfactory approach for removal of jugular foramen paragangliomas. In tumors with intracranial extension, combined approach is recommended in that it provides better surgical view and can maintain the compliance of the patients.

Cochlear implantation after surgery for bilateral jugular foramen paragangliomas: a case report

This paper describes a case of cochlear implantation for deafness due to bilateral glomus jugulare tumors (paragangliomas) as well as an unusual complication after cochlear implantation in a 31-year-old male. A 31-year-old male with profound sensorineural hearing loss on the right side and deafness on the left side, caused by bilateral jugular foramen paragangliomas, was implanted with a multi-channel cochlear implant (Combi 40+, Med-El) on the right side during subtotal petrosectomy for removal of the right-sided glomus jugulare tumor. No postoperative medical complications were observed. The patient responded to acoustic stimuli. Postoperative computed tomography (CT) did not show any misplacement of the electrode. Three years after implantation, an acute, rapidly progressive hearing impairment with pain to acoustic stimuli was observed. A CT scan at that time showed cochlear bone resorption. No radiologic evidence of paraganglioma recurrence was observed. The implant was removed and a biopsy of the cochlea was performed which on histologic examination showed fibrosis without any sign of any tumor recurrence. Cochlear implantation can restore hearing in deafness due to bilateral glomus jugulare tumors. Cochlear resorption may occur as a late complication in the implanted side in the patients.