Case report: atypical presentation of jugular foramen mass

Jugular foramen lesions are often associated with pathology of adjacent structures due to either compression or direct invasion. Common presenting symptoms include pulsatile tinnitus, a neck mass, hearing loss, and cranial nerve palsies, leading to changes in taste, vocal cord paralysis, dysphagia, and sternocleidomastoid/trapezius weakness (A. Hakuba, K. Hashi, K. Fujitani, et al., Jugular foramen neurinomas. Surg Neurol 1979; 11:83-94). This patient was found to have a jugular foramen mass after presenting with the unusual constellation of visual changes and headache.

A jugular foramen mass in a young woman was discovered after presenting with visual changes and headache; the patient was found to have papilledema on initial examination. Otologic and head and neck examination were normal. Subsequent imaging demonstrated a mass at the right jugular foramen with compression of this structure; a contralateral transverse sinus stenosis was also seen. This latter abnormality (along with obstruction of the jugular foramen) impeded venous drainage leading to papilledema and visual changes.

In a patient presenting with papilledema and severe headache with an associated jugular foramen mass, a multidisciplinary approach benefits the patient with input from interventional neuroradiology, neurosurgery, and neuro-ophthalmology. Venous outflow was compromised through the left stenotic transverse sinus, and the normal outflow on the right side through the jugular bulb was impeded by the tumor; obstructions of both led to symptomatic impeded venous outflow. This compromise in venous outflow led to an increase in superior sagittal sinus pressure, with subsequent increase in intracranial pressure and resultant papilledema. In an attempt to increase blood flow, an angioplasty was performed on the patient's affected transverse sinus. In addition, symptomatology consistent with pseudotumor cerebri prompted the use of acetazolamide for medical management. After both therapies, the patient's symptoms dramatically improved and were stable. The tumor has also remained stable, with no immediate need for surgical resection, stereotactic radiation, or consideration of an intraluminal transverse sinus stent placement or shunting.

The unique presentation of a jugular foramen mass in a young woman leading to papilledema highlights the need for high clinical suspicion of potential etiologies necessary for diagnosis. Despite the benign nature of her disease process, an unusual constellation of anatomical factors lead to the need for acute intervention.