The glossopharyngeal nerve yields a tympanic branch jacobson nerve. 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, or vernets syndrome, is characterized by paresis of the glossopharyngeal, vagal, and accessory with or without the hypoglossal nerves symptoms. Pdf metastatic breast carcinoma of the jugular foramen. Lemierre syndrome is a dangerous potential sequela of pharyngitis that results in. 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. Aan members must change their passwords on the aan site. We report an elderly man who presents with dysphagia, dysarthria, hoarseness, and a 12pound weight loss. Jugular foramen syndrome systemic disease doctor steve. The jugular foramen syndrome represents a minority of skull base syndromes 3. 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. The muscles of the left side of his neck were atrophied, and his left shoulder sagged. Clinicopathologic features of jugular foramen tumors. In a more general meaning, however, any combination of palsies affecting the last four cranial.
Jugular foramen tumors are rare skull base lesions that present diagnostic and complex management problems. The authors experience in managing these rare tumors is presented. Guidelines exist to help physicians decide whether to treat with an antibiotic. Jugular foramen syndrome jfs is an uncommon clinical entity that often presents a diagnostic and management challenge to the otolaryngologist. The jugular foramen is a large foramen opening in the base of the skull, located behind the carotid canal. Vernets syndrome jugular foramen syndrome laryngeal paralysis. Metastatic involvement of the cranial base and jugular foramen generally presents with headache and lower cranial neuropathy but may escape early diagnosis. Accessory nerve neurinoma manifesting with typical jugular. The jugular foramen is the aperture through which the internal jugular vein, and cranial nerves ix, x, and xi exit the skull. 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. On physical examination,nhe had leftsided sensorineural hearing loss with left njugular foramen syndrome.
Jugular foramen syndrome information including symptoms, causes, diseases, symptoms, treatments, and other medical and health issues. 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. Surgical strategy for jugular foramen tumors, in sekhar ln, janecka ip eds. Most cases are nonbacterial in origin, but those that are bacterial are usually the result of group a. Breast cancer is the most common cause of skull base metastases. 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. Aan members 800 8791960 or 612 9286000 international nonaan member subscribers 800 6383030 or 301 2232300 option 3, select 1. The jugular foramen syndrome jfs specifically refers to paralysis to the ixxith cranial nerves. 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. Accessory nerve neurinoma manifesting with typical jugular foramen syndrome. Identification of the normal jugular foramen and lower.
What is jugular foramen syndrome meaning and definition. Jugular foramen syndrome as initial presentation of. 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. We observed a further variant characterized by a styloid elongation coursing adjacent to the transverse process of c1. Examination demonstrated paralysis of the right vagus and hypoglossal nerve. 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. Giant jugular foramen tumor nonpseudomonal malignant otitis externa and jugular foramen syndrome secondary to cyclosporininduced hypertrichosis in a diabetic renal transplant patient. A digital intravenous angiogram showed an occlusion of the left transverse sinus at the level of the jugular foramen. Jugular foramen syndrome has several etiologies, including trauma, tumor growth, and infection. It is formed in front by the petrous portion of the temporal bone, and behind by the occipital bone. Part ii, presented here, focuses on the foramina of the posterior cranial fossa and discusses each foramen. The foramen is divided into two parts by a fibroosseous bridge connecting the jugular. Papilledema in the metastatic jugular foramen syndrome. Differential diagnosis of jugular foramen lesions article pdf available in skull base surgery 191.
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. 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. Our case is the first report of a jugular foramen pecoma mimicking a schwannoma and resulting in the colletsicard syndrome. 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.
Jugular foramen syndrome and allied syndromes neurology. Paragangliomas in the skull base are ubiquitous in their distribution and arise from paraganglia or glomus cells situated at the following sites. Vernets syndrome definition of vernets syndrome by. The eagle jugular syndrome bmc neurology full text. Jugular foramen schwannomas are rare skull base tumours which typically have a variable clinical presentation. The jugular foramen courses anteriorly, laterally, and inferiorly as it insinuates itself between the petrous temporal bone and the occipital bone gross anatomy. Radiation oncologyanatomyclinical syndromes with cn. Internal jugular vein septic thrombophlebitis lemierre. The lesion was visualized only on magnetic resonance imaging, and surgery confirmed that it originated from a cranial root of the accessory nerve. Symptoms of this syndrome are consequences of this paresis. 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.
It is a clinical subtype of jugular foramen syndromes. Avellis syndrome neurological disorder characterized by a peculiar form of alternating paralysis. 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. Aneurysmal bone cyst of the skull base in a 17yearold boy.
Malignant perivascular epithelioid cell tumor mimicking. Plain tomograms demonstrated a metastasis at the base of the skull. Jugular foramen syndrome definition of jugular foramen. Pdf jugular foramen syndrome caused by choleastatoma. Papilledema in the metastatic jugular foramen syndrome francesc graus, md, neal e. Pdf differential diagnosis of jugular foramen lesions. This article presents the microsurgical anatomy of jugular foramen through the retroauricular, transmastoid infralabyrinthine approach. Jugular foramen syndrome as a complication of metastatic cancer of the prostate. A case of neurinoma arising from the accessory nerve is reported. Aan members 800 8791960 or 612 9286000 international nonaan member subscribers 800 6383030 or 301 2232300 option 3, select 1 international. 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. A case of vernet syndrome associated with internal jugular.
Glossopharyngeal syncope syndrome is an unusual clinical presentation. The two jugular foramina are openings in the skull base located on either side, anterolateral to the foramen magnum. 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. 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. The elongation of the styloid process is historically associated with two variants of the eagle syndrome.
This is due to glossopharyngeal, vagal, and accessory nerve palsies, which may occur in. Presents a new contemporary internal design that helps you. Metastatic masses of the skull base are a rare manifestation of systemic malignancies. Colletsicard syndrome is a very rare neurological condition manifesting as unilateral palsies of the 9th12th cranial nerves. 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. A bony growth in relation to jugular foramen has not been reported yet. 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. The tumor was small and located entirely within the jugular foramen. However it is known that many iih sufferers have smaller jugular foramen, and as far as i understand it is made of bone. Metastatic breast carcinoma of the jugular foramen. Paragangliomas glomus jugular tumors the most common tumor to develop in the jf is a paraganglioma. Colletsicard syndrome attributable to extramedullary. Colliver, do a 36yearold woman presented with right shoulder weakness after a left parotid tumor resection.
Vernets syndrome jugular foramen syndrome laryngeal. Colletsicard syndrome css is defined as unilateral palsy of lower cranial nerves ix, x, xi, and xii. Jugular foramen syndrome, or vernets syndrome, is characterized by dysfunction of the ix, x, and xi cranial nerves. 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. Cranial nerve foramina are integral exits from the confines of the skull. There was marked emphysema with cyanosis of lips and nail beds. Sore throat is a common presenting complaint in the outpatient setting. This method is easier than the classical infratemporal approach and provides better exposure for the cranial nerves reaching the neck.
The classic one, mainly characterized by pain and dysphagia, and the carotid variant characterized by pain and sometimes by cerebral ischemia. The bony growth presented here markedly reduced the size of jugular foramen and jugular fossa. 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. Slatkin, md \sb\in a patientwith ewings tumor, bilat eral papilledema developed along with a left jugular foramen syndrome. 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. 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. Background colletsicard syndrome is a rare manifestation of skull base disease involving the jugular and hypoglossal foramina. 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.