Incidence of cranial nerve palsy after preoperative embolization of glomus jugulare tumors using Onyx.

OBJECT: The resection of glomus jugulare tumors can be challenging because of their inherent vascularity. Preoperative embolization has been advocated as a means of reducing operative times, blood loss, and surgical complications. However, the incidence of cranial neuropathy associated with the embolization of these tumors has not been established. The authors of this study describe their experience with cranial neuropathy following transarterial embolization of glomus jugulare tumors using ethylene vinyl alcohol (Onyx, eV3 Inc.). METHODS: The authors retrospectively reviewed all cases of glomus jugulare tumors that had been treated with preoperative embolization using Onyx at their institution in the period from 2006 to 2012. Patient demographics, clinical presentation, grade and amount of Onyx used, degree of angiographic devascularization, and procedural complications were recorded. RESULTS: Over a 6-year period, 11 patients with glomus jugulare tumors underwent preoperative embolization with Onyx. All embolization procedures were completed in one session. The overall mean percent of tumor devascularization was 90.7%. No evidence of nontarget embolization was seen on postembolization angiograms. There were 2 cases (18%) of permanent cranial neuropathy attributed to the embolization procedures (facial nerve paralysis and lower cranial nerve dysfunction). CONCLUSION: Embolizing glomus jugulare tumors with Onyx can produce a dramatic reduction in tumor vascularity. However, the intimate anatomical relationship and overlapping blood supply between these tumors and cranial nerves may contribute to a high incidence of cranial neuropathy following Onyx embolization.

A comparative review of multidetector CT angiography and MRI in the diagnosis of jugular foramen lesions.

AIM: To compare the efficiency of multidetector computed tomography (MDCT) and magnetic resonance imaging (MRI) in the diagnosis of jugular foramen lesions. MATERIALS AND METHODS: The imaging of 15 patients with tumours predominantly occurring at the jugular foramen was retrospectively reviewed, with postoperative pathology data available for 11 patients. MDCT was performed at arterial phase and MRI with standard sequences and contrast enhancement. All imaging was blindly re-reported by an experienced neuroradiologist. RESULTS: Pathology reported six glomus jugulare tumours and five neuromas, which were all correctly diagnosed using MDCT. A confident diagnosis was also made in the remaining four cases based on the pattern of enhancement. Only glomus tumours enhanced in the arterial phase. Overall, MRI was used to make a confident diagnosis in eight patients. One showed no enhancement and was correctly diagnosed as a neuroma, and seven demonstrated the tumour flow voids characteristic of a glomus tumour. The remaining seven cases all showed a similar enhancement pattern and could not be confidently differentiated between a neuroma or a glomus tumour. MDCT angiography enabled a confident assessment of the jugular vein in all cases, but MRI was inconclusive in a third of cases. Also, in the nine cases of glomus tumour diagnosed using MDCT, an enlarged feeding artery was identified in eight patients. CONCLUSION: MDCT is more accurate than MRI in diagnosing glomus tumours, and in particular, neuromas. It also offers valuable preoperative vascular information to the surgeon.

Surgical management of glomus jugulare tumors: a proposal for approach selection based on tumor relationships with the facial nerve.

OBJECT: The goal of this paper is to analyze the extension and relationships of glomus jugulare tumor with the temporal bone and the results of its surgical treatment aiming at preservation of the facial nerve. Based on the tumor extension and its relationships with the facial nerve, new criteria to be used in the selection of different surgical approaches are proposed. METHODS: Between December 1997 and December 2007, 34 patients (22 female and 12 male) with glomus jugulare tumors were treated. Their mean age was 48 years. The mean follow-up was 52.5 months. Clinical findings included hearing loss in 88%, swallowing disturbance in 50%, and facial nerve palsy in 41%. Magnetic resonance imaging demonstrated a mass in the jugular foramen in all cases, a mass in the middle ear in 97%, a cervical mass in 85%, and an intradural mass in 41%. The tumor was supplied by the external carotid artery in all cases, the internal carotid artery in 44%, and the vertebral artery in 32%. Preoperative embolization was performed in 15 cases. The approach was tailored to each patient, and 4 types of approaches were designed. The infralabyrinthine retrofacial approach (Type A) was used in 32.5%; infralabyrinthine pre- and retrofacial approach without occlusion of the external acoustic meatus (Type B) in 20.5%; infralabyrinthine pre- and retrofacial approach with occlusion of the external acoustic meatus (Type C) in 41%; and the infralabyrinthine approach with transposition of the facial nerve and removal of the middle ear structures (Type D) in 6% of the patients. RESULTS: Radical removal was achieved in 91% of the cases and partial removal in 9%. Among 20 patients without preoperative facial nerve dysfunction, the nerve was kept in anatomical position in 19 (95%), and facial nerve function was normal during the immediate postoperative period in 17 (85%). Six patients (17.6%) had a new lower cranial nerve deficit, but recovery of swallowing function was adequate in all cases. Voice disturbance remained in all 6 cases. Cerebrospinal fluid leakage occurred in 6 patients (17.6%), with no need for reoperation in any of them. One patient died in the postoperative period due to pulmonary complications. The global recovery, based on the Karnofsky Performance Scale (KPS), was 100% in 15% of the patients, 90% in 45%, 80% in 33%, and 70% in 6%. CONCLUSIONS: Radical removal of glomus jugulare tumor can be achieved without anterior transposition of the facial nerve. The extension of dissection, however, should be tailored to each case based on tumor blood supply, preoperative symptoms, and tumor extension. The operative field provided by the retrofacial infralabyrinthine approach, or the pre- and retrofacial approaches, with or without closure of the external acoustic meatus, allows a wide exposure of the jugular foramen area. Global functional recovery based on the KPS is acceptable in 94% of the patients.

Intraoperative monitoring of intraarterial paraganglioma embolization by indocyaningreen fluorescence angiography.

Preoperative arterial embolization (AE) of paraganglioma (PG) is widely used to diminish intraoperative blood loss. Thereby conditions for a resection of the tumor shall be improved and risks for facial, vagal or hypoglossus nerve injuries are reduced. The vascularization of jugular and tympanic PGs is particularly complex due to collaterals with the vertebral and internal carotid arteries. Thus AE is often not complete and intraoperative blood loss may still be considerable. The postinterventional perfusion is of interest for the surgeon. We evaluated the arterial perfusion after AE using indocyaningreen (ICG) angiography. Six patients with PG, two carotid PGs, two jugular PGs, one vagal PG and one tympanic PG underwent surgery 1 day after AE. After tumor was exposed, ICG was intravenously applied followed by fluorescence angiography. Residual perfusion was assessed on the video clip and the perfusion index was automatically calculated by the IC-CALC software. This index was compared with the radiologist's assessment of arteriographic control after AE. Two of the six patients showed only marginal residual perfusion. These were patients with carotid PGs. The patient with the vagal PG showed 20%, the patients with jugular PGs 80 and 60% and the patient with the tympanic PG had 70% residual blood flow. The preoperative AE is rarely complete in PGs of the petrous bone. Intraoperative fluorescence angiography is a reliable procedure to evaluate the efficiency of preoperative embolization and can help the surgeon to estimate intraoperative bleeding favouring risks.

[Selective embolization and surgical resection for head and neck glomus tumours--clinical outcome analysis]. / Präoperative Embolisation mit chirurgischer Resektion von Glomustumoren im Kopf-Hals-Bereich: Analyse der klinischen Ergebnisse.

The effectiveness and safety of angiographic embolization was investigated as a preliminary step prior to surgical excision of glomus tumours in the head and neck region. Embolization was performed in 54 patients presenting with a total of 58 chemodectomas, jugular (n=30), tympanicum (n=24) and caroticum (n=4) between the years 1988 and 2006. Embolization was considered successful if complete occlusion of all tumor-feeding vessels was achieved. The procedure was performed using polyvinylalcohol particles and microcoils and lasted for a median duration of 159 minutes. Complete tumor embolization was achieved in 72 % of patients. In 23%, it was partly successful and in 4% it was unsuccessful. 16% of patients experienced minor events during the procedure including hypotension, bradycardia, and vertigo. Following embolization, almost all patients (98%) had their tumour completely excised. Although the majority experienced minor postoperative complications (69%), one patient developed meningitis. There were no reported deaths. Angiographic embolization of glomus tumours in the head and neck before definitive excision can be safe and effective, resulting in an improved surgical outcome and tumour resectability.

Management of cervical paragangliomas: review of a 15-year experience.

BACKGROUND AND AIMS: Cervical paragangliomas are highly vascular neoplasms and should be considered in the evaluation of all lateral neck masses. The aim of this study is to review an institutional experience in the management of these tumors. MATERIALS AND METHODS: Thirteen patients with 14 paragangliomas were treated in our institution during a period of 15 years. There were eight women (61.5%) and five men (38.5%) with a mean age of 41.3+/-15 years. A painless lateral neck mass was the main finding in 69.2% of patients. There was no evidence of a functional tumor. Carotid angiography was performed in all patients to define the vascular anatomy of the lesion. The 78.6% of paragangliomas underwent selective embolization of the major feeding arteries. Surgical resection followed within the next 48 h. RESULTS: The majority of the lesions were paragangliomas of the carotid bifurcation (85.7%), while one patient was diagnosed with a jugular and one with a vagal paraganglioma. In one patient, bilateral paragangliomas in the carotid bifurcation were detected. There was no evidence of malignancy in any case. Preoperative embolization has proven successful in reducing tumor vascularity. Vascular reconstruction was necessary in one patient. The main postoperative complication was transient cranial nerve deficit in seven (53.8%) patients, and a permanent Horner's syndrome was documented in one patient. No stroke occurred. The jugular paraganglioma was treated with irradiation due to skull base extension with significant symptomatic relief. CONCLUSION: Combined therapeutic approach with preoperative selective embolization followed by surgical resection by an experienced team offers a safe and effective method for complete excision of the tumors with a reduced morbidity rate.

Intratumoral injection of cyanoacrylate glue in head and neck paragangliomas.

BACKGROUND AND PURPOSE: Substantial intraoperative bleeding during surgical removal of head and neck paragangliomas may be a major problem in the management of these highly vascularized tumors. Traditional preoperative embolization via a transarterial approach has proved beneficial but is often limited by complex vascular anatomy and unfavorable locations. We report our experience with the preoperative devascularization of head and neck paragangliomas by using direct puncture and an intralesional injection of cyanoacrylate. METHODS: We retrospectively analyzed nine consecutive patients with head and neck paragangliomas who were referred for preoperative devascularization. Three patients were treated for carotid-body tumors; two for vagal lesions; and four, for jugular paragangliomas. Direct puncture of the lesion was performed by using roadmap fluoroscopic guidance. Acrylic glue was injected by using continuous biplane fluoroscopy. All patients underwent postembolization control angiography and immediate postoperative CT scanning. RESULTS: Angiograms showed that complete devascularization was achieved in all cervical glomus tumors, whereas subtotal devascularization was achieved in jugular paragangliomas. In this latter location, the injection of acrylic glue was limited by the potential risk of reflux into normal brain territory via feeders from the internal carotid or vertebral artery. The tumors were surgically removed and histologically examined. No technical or clinical complications related to the embolization procedure occurred. CONCLUSION: Percutaneous puncture of paragangliomas in the head and neck region and their preoperative devascularization by intralesional injection of acrylic glue is a feasible, safe, and effective technique.

The basic science of glomus jugulare tumors.

Glomus tumors are a fascinating group of lesions. It is a challenge for neurosurgeons and otolaryngologists to resect them completely with minimal morbidity. Laboratory researchers have discovered extremely interesting genetic and molecular biology factors involved in the development and growth of glomus tumors. In this article the author reviews the genetics, protein mutations, angiogenesis and apoptosis associated with tumor formation, and the secretion of vasoactive substances is discussed as well. It is hoped that with further research less invasive measures may be developed to treat these tumors.

Ossified glomus jugulare tumour: case report.

A huge, ossified and highly vascular glomus jugulare tumour in a 19-year-old boy was radically and successfully resected. External carotid artery embolization and intermittent internal carotid artery trapping during surgery were the principle methods employed to control the operative blood loss. Extensive petrous bone resection, and adequate and wide exposure were necessary. The case and the operative steps in this unusual and difficult surgical problem are discussed.

Avascular tympanojugular paraganglioma.

The avascular paraganglioma described in this article appears to be the second such tumor reported in the international literature and the first to be reported in the tympanojugular region. Despite a highly suggestive history and clinical appearance, the tumor showed no signs of vascularization on radiologic studies. The pathologic postoperative study confirmed the diagnosis of paraganglioma with extensive stromal fibrosclerosis and without the typical well-vascularized thin fibrous septa. In the authors' opinion, this observation is notable because of the difficulties encountered in the correct diagnostic interpretation of an avascular mass in the tympanojugular region. In such cases, the possibility of a paraganglioma should always be considered.

[Pulsatile tinnitus as a key symptom of glomus tumor: diagnostic value of magnetic resonance tomography]. / Pulsatiler Tinnitus als Leitsymptom des Glomustumors: Diagnostische Wertigkeit der Magnetresonanztomographie.

The aim of this study was to evaluate the diagnostic value of modern imaging tools in pulsatile tinnitus, which might be apparent in a variety of different diseases. While computed tomography (CT) and digital subtraction angiography (DSA) have been the methods of choice in the diagnostic management of this symptom and its related diseases, the technical progress in the development of MRI and MRA highly recommends these techniques as additional yet sufficient imaging tools. The authors demonstrate 2 representative cases of pulsatile tinnitus (one patient with glomus tumour, one patient with jugular bulb deformity) and the adequate diagnostic procedure in terms of imaging tools. It is concluded that a routine MRI-examination might allow a diagnosis with high sensitivity and specificity. Still, digital subtraction angiography and computed tomography remain indispensable in the diagnostic management of pulsatile tinnitus.

Intratumoral embolization of intracranial and extracranial tumors: technical note.

A new technique of intratumoral embolization is described. An intratumoral injection of N-butylcyanoacrylate is performed either perioperatively by direct puncture of the tumor or preoperatively through the nose or through the skin. The indications for this technique are hypervascularized tumors, such as juvenile angiofibroma, hemangiopericytoma, or paraganglioma. This technique has been applied in 21 patients with excellent results in terms of devascularization.

Surgical management of previously untreated glomus jugulare tumors.

The treatment of glomus jugulare tumors is controversial. Changes in the surgical treatment of glomus jugulare tumors at The House Ear Clinic have allowed complete resection in 85% of patients with minimal morbidity and no surgical mortalities. Our experience with 52 previously untreated patients with glomus jugulare tumors is reviewed. Two primary surgical techniques were used. The mastoid/neck approach was used in 9 patients with small tumors limited to the jugular bulb. The infratemporal fossa approach was used in the remaining 43 tumors. Lower cranial nerve preservation was possible in the majority of patients with normal preoperative function. Modifications in the management of the facial nerve during the infratemporal fossa approach have resulted in good recovery of facial function (House grade I/VI or II/VI) in 95% of patients. Most patients (85%) were able to fully resume all preoperative activities. Our results suggest that surgical management is the treatment of choice in younger patients with glomus jugulare tumors.

Facial palsy after glomus tumour embolization.

A case is presented of a patient undergoing pre-operative embolization of a glomus tumour who developed a facial palsy one hour after embolization. At the time of surgery it was found to be due to the embolization material (polyvinyl alcohol foam) blocking the stylomastoid artery. The blood supply of glomus tumours and the variations in the blood supply of the facial nerve are discussed.

Recent advances in surgery of the temporal bone and skull base.

Remarkable advances have been made in the field of neuro-otologic and skull base surgery within the past decade. Each component of the "disease model"--prevention, diagnosis, therapy, and rehabilitation--is undergoing rapid progress. The purpose of this paper is to highlight only a few of these achievements. Each topic chosen has witnessed recent advancement in one aspect of the disease model. Neurofibromatosis (prevention) has been subjected to chromosomal mapping, allowing for genetic counseling. Intraoperative facial nerve monitoring (diagnosis) has allowed improved anatomic and functional preservation of this nerve during surgery. Embolization of glomus tumors (therapy) has rendered these difficult lesions more surgically manageable, and cochlear implantation (rehabilitation) has allowed the profoundly deaf to play a more active role in society. Each of these topics is briefly discussed as it relates to the temporal bone surgeon.

[Contribution of angiography in the pre-operative evaluation of 24 jugulo-tympanic paragangliomas with otoneurologic manifestations]. / Contribuição da angiografia na avaliação pré-cirúrgica de 24 paragangliomas jugulotimpânicos com manifestações otoneurológicas.

The contribution of carotid and vertebral angiography for surgical planning, particularly for the infratemporal approach, in 24 jugulotympanic paragangliomas with otoneurological symptoms is discussed. These symptoms included peripheral facial palsy, sensorineural hypoacusis, labyrinthine and nervous syndrome of the jugular foramen. Angiography was essential for the diagnosis of these tumors and, above all, for the rigorous evaluation of intrapetrous and apical invasion of the carotid artery, and also of extra and intradural posterior fossa extensions. Presurgical embolization, a relevant step in treatment, was based on the criterious evaluation of the angiotopographic pattern of the tumoral hypervascularization.