Jugular foramen schwannomas are rare skull base tumours which typically have a variable clinical presentation. The elongation of the styloid process is historically associated with two variants of the eagle syndrome. The purpose of this section is to define this syndrome and elucidate the epidemiology, pathogenesis, clinical manifestations, diagnostic workup, and general treatment options for the syndrome. The jugular foramen syndrome represents a minority of skull base syndromes 3. Metastatic masses of the skull base are a rare manifestation of systemic malignancies. The jugular foramen courses anteriorly, laterally, and inferiorly as it insinuates itself between the petrous temporal bone and the occipital bone gross anatomy. Part ii, presented here, focuses on the foramina of the posterior cranial fossa and discusses each foramen. Jugular foramen syndrome definition of jugular foramen. Presents a new contemporary internal design that helps you. The jugular foramen syndrome jfs specifically refers to paralysis to the ixxith cranial nerves. Jugular foramen syndrome and allied syndromes neurology. Colletsicard syndrome css is defined as unilateral palsy of lower cranial nerves ix, x, xi, and xii.
A case of vernet syndrome associated with internal jugular. This article presents the microsurgical anatomy of jugular foramen through the retroauricular, transmastoid infralabyrinthine approach. Pdf jugular foramen syndrome caused by choleastatoma. We report an elderly man who presents with dysphagia, dysarthria, hoarseness, and a 12pound weight loss. Vernet syndrome, also known as jugular foramen syndrome, is a constellation of cranial nerve palsies due to compression from a jugular foramen lesion, such as a glomus jugulare tumor, schwannoma, or metastasis 2 clinical presentation. Surgical strategy for jugular foramen tumors, in sekhar ln, janecka ip eds. In a more general meaning, however, any combination of palsies affecting the last four cranial. Aan members 800 8791960 or 612 9286000 international nonaan member subscribers 800 6383030 or 301 2232300 option 3, select 1. A case of vernet syndrome associated with internal jugular phlebectasia nicholas c. The inferior petrosal sinus drains the cavernous sinus and courses in the petrooccipital fissure adjacent to the clivus prior to its exit through the pars nervosa and subsequent drainage into the internal jugular vein beneath the foramen. What is jugular foramen syndrome meaning and definition. The bony growth presented here markedly reduced the size of jugular foramen and jugular fossa. Aan members must change their passwords on the aan site. Differential diagnosis of jugular foramen lesions article pdf available in skull base surgery 191.
Jugular foramen syndrome 349 introduction 349 definition 349 etiology and epidemiology 350 pathogenesis 350 anatomy of the jugular foramen 350 clinical manifestations 351 diagnosis 351 treatment 353 complications and prognosis 355 references 356. The main structures which pass through this foramen are the glossopharyngeal ix, vagus x, and spinal accessory xi nerves and the internal jugular vein ijv. Jugular foramen syndrome jfs is an uncommon clinical entity that often presents a diagnostic and management challenge to the otolaryngologist. The unique constellation of clinical findings localises the lesion to the region around the jugular foramen, which itself lies between the lower border of the petrous part of the temporal bone and the condylar part of the. The jugular foramen, the most complex of the foramina through which the cranial nerves pass, although the subject of numerous studies, remains poorly understood and difficult to access surgically 5, 15, 19, 23, 29, 33. Breast cancer is the most common cause of skull base metastases. Identification of the normal jugular foramen and lower. Metastatic breast carcinoma of the jugular foramen.
Papilledema in the metastatic jugular foramen syndrome francesc graus, md, neal e. Jugular foramen syndrome systemic disease doctor steve. Pdf metastatic breast carcinoma of the jugular foramen. Accessory nerve neurinoma manifesting with typical jugular. There was marked emphysema with cyanosis of lips and nail beds. The most inclusive syndrome of the jugular foramen is the villaret syndrome, also called the retropharyngeal syndrome, which includes deficits that are indicative of damage to cranial nerves ix, x, and xi. The jugular foramen is usually described as being divided into two parts by a fibrous or bony septum, called the jugular spine, into the pars nervosa. This method is easier than the classical infratemporal approach and provides better exposure for the cranial nerves reaching the neck. The anteromedial compartment pars nervosa contains the cranial nerve ix, the tympanic branch of ix jacobsons nerve, and the inferior petrosal sinus.
Giant jugular foramen tumor nonpseudomonal malignant otitis externa and jugular foramen syndrome secondary to cyclosporininduced hypertrichosis in a diabetic renal transplant patient. The jugular foramen is the aperture through which the internal jugular vein, and cranial nerves ix, x, and xi exit the skull. Most cases are nonbacterial in origin, but those that are bacterial are usually the result of group a. The muscles of the left side of his neck were atrophied, and his left shoulder sagged. The authors experience in managing these rare tumors is presented. The jugular foramen is a large foramen opening in the base of the skull, located behind the carotid canal. Aan members 800 8791960 or 612 9286000 international nonaan member subscribers 800 6383030 or 301 2232300 option 3, select 1 international. Paragangliomas glomus jugular tumors the most common tumor to develop in the jf is a paraganglioma. Jugular foramen tumors are rare skull base lesions that present diagnostic and complex management problems. Vernets syndrome jugular foramen syndrome laryngeal paralysis. Glossopharyngeal syncope syndrome is an unusual clinical presentation. Jugular foramen syndrome information including symptoms, causes, diseases, symptoms, treatments, and other medical and health issues.
Metastatic involvement of the cranial base and jugular foramen generally presents with headache and lower cranial neuropathy but may escape early diagnosis. Jugular foramen syndrome as initial presentation of. The difficulty in comprehension is created by its varying in size and shape in different crania, from side to side in the same cranium, and from its. Accessory nerve neurinoma manifesting with typical jugular foramen syndrome. Pdf jugular foramen syndrome as initial presentation of. The detection of individual cranial nerves in the intraforaminal portion of the jugular foramen and hypoglossal canal is useful in diagnostic imaging 1. Vernets syndrome is characterized by a constellation of unilateral cranial nerve palsies due the compression or narrowing of the jugular foramen involving the 9th, 10th, and 11th cranial nerves nerves that travel within the jugular foramen. The differential diagnosis of skull bases masses is diverse and includes benign and malignant neoplasms, vascular anomalies, congenital lesions as well as infectious and inflammatory processes. Jugular foramen syndrome vernet syndrome glomus jugular tumours. Our case presented with anhistory of hoarseness for 2 years and facial asymmetrynand left sided hearing loss from 5 months ago with nonhistory of trauma or surgery.
Jugular foramen syndrome, or vernets syndrome, is characterized by dysfunction of the ix, x, and xi cranial nerves. Paragangliomas in the skull base are ubiquitous in their distribution and arise from paraganglia or glomus cells situated at the following sites. Clinicopathologic features of jugular foramen tumors. A digital intravenous angiogram showed an occlusion of the left transverse sinus at the level of the jugular foramen. The jugular foramen jf is a bony channel that transmits vessels and cranial nerves ix, x, and xi cnix, cnx, and cnxi through the skull base into the carotid space.
Radiation oncologyanatomyclinical syndromes with cn. Jugular foramen syndrome has several etiologies, including trauma, tumor growth, and infection. We observed a further variant characterized by a styloid elongation coursing adjacent to the transverse process of c1. It is formed in front by the petrous portion of the temporal bone, and behind by the occipital bone. Three major growth patterns of jugular foramen schwannoma were seen, and it is postulated that the position of the tumor depends on its point of origin from the nerves as they pass through the pars nervosa of the jugular foramen.
Our case is the first report of a jugular foramen pecoma mimicking a schwannoma and resulting in the colletsicard syndrome. Avellis syndrome neurological disorder characterized by a peculiar form of alternating paralysis. Aneurysmal bone cyst of the skull base in a 17yearold boy. In 1915, frederic collet initially described this syndrome in a soldier who had been shot during world war i, 1 and some time later, jean a. There is paralysis of the soft palate and vocal cords on one side and loss of pain sensation and temperature sense on the other side, including the extremities, trunk, and neck. Jugular foramen syndrome as a complication of metastatic cancer of the prostate. However it is known that many iih sufferers have smaller jugular foramen, and as far as i understand it is made of bone. Cranial nerve foramina are integral exits from the confines of the skull. It is a clinical subtype of jugular foramen syndromes. Internal jugular vein septic thrombophlebitis lemierre.
The foramen is divided into two parts by a fibroosseous bridge connecting the jugular spine of the temporal bone and jugular process of the occipital bone. Sore throat is a common presenting complaint in the outpatient setting. Papilledema in the metastatic jugular foramen syndrome. Jugular foramen syndrome, or vernets syndrome, is characterized by paresis of the glossopharyngeal, vagal, and accessory with or without the hypoglossal nerves symptoms. We report the first case of colletsicard attributable to extramedullary plasmacytomamultiple myeloma epmm and the second case of epmm precipitating a jugular foramen syndrome jfsspectrum disorder. Examination demonstrated paralysis of the right vagus and hypoglossal nerve. Colliver, do a 36yearold woman presented with right shoulder weakness after a left parotid tumor resection. The lesion was visualized only on magnetic resonance imaging, and surgery confirmed that it originated from a cranial root of the accessory nerve.
Plain tomograms demonstrated a metastasis at the base of the skull. The tumor was small and located entirely within the jugular foramen. Tumors arising from the jugular foramen vary from common tumors, including schwannomas, paragangliomas, meningiomas and metastatic tumors, to rare tumors such as chondrosarcoma and chordoma. Symptoms of this syndrome are consequences of this paresis. The two jugular foramina are openings in the skull base located on either side, anterolateral to the foramen magnum. Pdf differential diagnosis of jugular foramen lesions. On physical examination,nhe had leftsided sensorineural hearing loss with left njugular foramen syndrome. Background colletsicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. The classic one, mainly characterized by pain and dysphagia, and the carotid variant characterized by pain and sometimes by cerebral ischemia. Compression of the cranial nerves results in dysphagia, hoarseness, change in voice, weakness in the sternocleidomastoid and trapezius muscles leading to the jugular foramen syndrome 2 5.
The glossopharyngeal nerve yields a tympanic branch jacobson nerve. Malignant perivascular epithelioid cell tumor mimicking. Colletsicard syndrome is a very rare neurological condition manifesting as unilateral palsies of the 9th12th cranial nerves. Lemierre syndrome is a dangerous potential sequela of pharyngitis that results in. In this report, a patient developed a jugular foramen syndrome as the initial presentation of metastatic lung cancer soon after being diagnosed and treated surgically for extracranial atherosclerotic internal carotid artery disease. A bony growth in relation to jugular foramen has not been reported yet. The purpose of this study was to evaluate a series of patients with jugular foramen tumors who were surgically treated in the past 16 years, and to analyze the surgical technique, complications, and outcomes. This is due to glossopharyngeal, vagal, and accessory nerve palsies, which may occur in. Jugular foramen syndrome article about jugular foramen. The foramen is divided into two parts by a fibroosseous bridge connecting the jugular.
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