Abstract
Introduction
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.
Case presentation
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.
Discussion
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.
Conclusion
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.
1
Introduction
Jugular foramen lesions have various etiologies often associated with pathology of nearby structures due to either compression or direct invasion. Specifically, paragangliomas are the most common jugular foramen tumor . Other common masses include schwannomas and meningiomas. Clinical symptoms, onset and progression of disease, and radiographic findings can help differentiate among possible etiologies. 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 . Indolent symptoms and slow-growing masses are more often associated with benign lesions, whereas rapid progression and constitutional findings are associated with a more ominous sign of malignancy. We describe a patient presenting with headache and visual changes who was found to have papilledema and a jugular foramen mass; treatment and follow-up are further discussed.
2
Case presentation
A 36-year-old obese woman presented to the otolaryngology clinic with new-onset severe, intermittent, and occipital headaches for 1 month. She also reported pulsatile tinnitus, which was worse at night. She has a medical history of hypertension and tobacco abuse. Results of the otologic and head and neck examinations were normal.
The patient was evaluated by her ophthalmologist with concern for a visual contribution to her headache and was noted to have moderate-to-severe papilledema but intact visual fields. Examination revealed acuities of 20/20 in each eye with full motility, and slit lamp examination was unremarkable. Intraocular pressures were 11 in the right eye and 13 in the left eye. The fundus examination revealed bilateral hyperemic optic disk edema, flame-shaped hemorrhages, and mild venous engorgement consistent with papilledema resulting from elevated intracranial pressure. After neuroimaging ( Figs. 1 and 2 ), a lumbar puncture was performed in the lateral decubitus position; the opening pressure was 38 cm of water.
A magnetic resonance imaging (MRI) revealed a small transverse sinus on the left and an enhancing mass filling the jugular foramen on the right. Contrast computed tomographic (CT) scan revealed almost complete compression of the jugular bulb by the mass, without evidence of bone destruction. Magnetic resonance venogram further confirmed left transverse sinus narrowing and a dominant right transverse sinus. Cerebral angiogram was performed, confirming elevation of dural venous sinus pressure. The left transverse sinus exhibited a 17-mm Hg gradient at the left transverse-sigmoid sinus juncture, confirming stenosis ( Figs. 3–9 ).
The patient subsequently underwent endovascular angioplasty of the left distal transverse sinus and left proximal sigmoid sinus, with a decrease in the pressure gradient noted after the procedure. She reported improvement in her headaches at follow-up visit but continued to experience transient visual obscurations. Follow-up ophthalmologic examination revealed continued bilateral optic disk edema. With signs and symptoms consistent with pseudotumor cerebri, she was started on diuretic therapy (acetazolamide 1000 mg/d). After starting medical therapy, her visual symptoms improved; optic disk edema improved, and the visual fields remained stable.
On one occasion, the patient presented with recurrent headaches, and lumbar puncture was performed with an opening pressure of 33 cm of water. Her symptoms subsequently improved without further intervention. The tumor has remained stable on follow-up MRI, with no immediate need for surgical resection, stereotactic radiation, or consideration of an intraluminal transverse sinus stent placement or shunting.
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