Jugular Paraganglioma and Bilateral Vestibular Schwannoma Presenting to an Acute Ophthalmology Service

A19-year-old woman presented with a 6-month history of right-sided parietal headache associated with nausea,vomiting, photophobia, and blurred vision. The headache was worse at night, improved after vomiting but gained no relief with oral analgesia. She did not complain of symptoms of oscillopsia and had no difficulty with reading. On examination, her Snellen visual acuity was 6/6 bilaterally unaided.There was no relative afferent papillary defect, and pupils were equal and reactive. She had full range of eye movements. Using Ishihara color vision testing, her right eye scored normal at 17/17 but her left eye was slightly reduced with a score of 13/17.

Clinical Correspondence Jugular Paraganglioma and Bilateral Vestibular Schwannoma Presenting to an Acute Ophthalmology Service Clare McCloskey, MB BCh, NUI, Edel Cosgrave, MB BCh, NUI A 19-year-old woman presented with a 6-month history of right-sided parietal headache associated with nausea, vomiting, photophobia, and blurred vision. The headache was worse at night, improved after vomiting but gained no relief with oral analgesia. She did not complain of symptoms of oscillopsia and had no difficulty with reading. On examination, her Snellen visual acuity was 6/6 bilaterally unaided. There was no relative afferent papillary defect, and pupils were equal and reactive. She had full range of eye movements. Using Ishihara color vision testing, her right eye scored normal at 17/17 but her left eye was slightly reduced with a score of 13/17. Anterior segment examination was unremarkable and intraocular pressure measured 10 mm Hg bilaterally. On fundus examination, there was evidence of mild indistinct blurred optic disc margins bilaterally (Fig. 1). As per clinical grading of papilledema using the Frisen Scale, the right disc was Grade 2 (early papilledema), and the left disc was Grade 1 (very early papilledema). Dynamic visual acuity and the vestibular-ocular reflex were tested with the rapid head impulse test which were both normal. Right optical coherence tomography (OCT) (Deep Range Imaging OCT Triton; Topcon, The Netherlands) of optic discs was performed, and the peripapillary retinal nerve fiber layer (RNFL) assessment was normal averaging 112 mm in the right eye and 120 mm on the left (Topcon [mean/SD] among normal subjects: 105.171 mm/10.641). Macular RNFL was normal in both eyes. Macular ganglion cell layer averaged normal in the right eye although marginally thinner inferior to the macula. Ganglion cell layer was slightly reduced to 58 mm in the left eye, more so inferiorly (Topcon [mean/SD] among normal subjects: 71.400 mm/5.756). She underwent a full-field 120-point suprathreshold testing that was normal in the right eye, and the left eye revealed a few nonspecific misses in the superotemporal and inferonasal quadrants. FIG. 1. Right and left color disc photographs. As per clinical grading of papilledema using the Frisen Scale, the right disc is Grade 2 (early papilledema) due to blurring of nasal and temporal disc margins but with no major vessel obscuration. The left disc is Grade 1 (very early papilledema) due to blurring of nasal margin but normal temporal margin. There are pigmentary changes noted at left temporal margin. Waterford Regional Hospital, Waterford, Ireland. The authors report no conflicts of interest. Address correspondence to Clare McCloskey, MB BCh, NUI, Waterford Regional Hospital, Dunmore Road Co, Waterford, Ireland, X91 ER8E; E-mail: [e-mail] 252 Motor and sensory examination of the upper and lower extremities, co-ordination, and balance were normal. With further questioning, she did reveal a deepening of her voice over recent weeks, which was also noticed by family members. Radiological investigations included a computed tomography scan of her brain, which showed no acute intracranial McCloskey and Cosgrave: J Neuro-Ophthalmol 2020; 40: 252-254 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence hemorrhage, mass effect, or focal edema, but did reveal a significant rounded expansion of the right-sided jugular foramen without bone destruction. She had an MRI scan of her head and skull base to further evaluate this which demonstrated multiple pathologies. There was bilateral vestibular schwannomas, larger on the left side than the right and a large 3.0 · 2.5 · 2.1 cm lobular lesion expanding the right jugular foramen and extending through the foramen medially to the right cerebellar pontine angle with a "salt and pepper" appearance consistent with a jugular paraganglioma (Fig. 2). Following this, she had multidisciplinary team input from neurology, endocrinology, and neurosurgery. Further investigations included a catecholamine urine test and whole-body positron emission tomography imaging which were normal. Otolaryngological examination with otoscopy revealed no pathology, and audiology testing was normal with no evidence of sensorineural or conductive hearing loss. Informed written consent was obtained from the patient to present this case. Jugular paragangliomas are from a family of tumors known as chromaffin cell tumors derived from the sympathetic and parasympathetic ganglia (1). They arise from the jugular bulb along the tympanic branch of the glossopharyngeal nerve (2). Paragangliomas can develop at other locations within the head and neck, namely along the vagus nerve (vagal paraganglioma), from the auricular branch of the vagus nerve (tympanic paraganglioma), and most commonly at the carotid body (carotid paraganglioma) (1). FIG. 2. Brain MRI. A. T1-weighted axial section showing a right- and left-sided soft tissue mass extending into and expanding the internal auditory meatus (arrows). This is of low signal on T1- and T2-weighted images but demonstrates intense enhancement after contrast without flow voids. These lesions are consistent with vestibular schwannomas. B. T1-weighted axial section showing a separate large lobular lesion measuring 3.0 · 2.5 · 2.1 cm expanding the right jugular foramen and extending through the foramen medially to the right cerebellar pontine angle with a "salt and pepper" appearance (arrowhead). This lesion is consistent with a right jugular paraganglioma. C. T2-weighted coronal image showing the jugular paraganglioma together with the bilateral vestibular schwannomas. D. T2-weighted image after contrast demonstrating the jugular paraganglioma. McCloskey and Cosgrave: J Neuro-Ophthalmol 2020; 40: 252-254 253 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence Head and neck paragangliomas are rare with the incidence ranging between 1 in 30,000-100,000 (3). Jugular paragangliomas are most commonly benign and slow growing but can be malignant in approximately 3% of cases with metastases in 1%-4% (2). Patients may present with dizziness, hearing impairment, disabling tinnitus, or lower cranial nerve palsies (3). Management options include observation, surgical resection with preoperative embolization, radiotherapy, and gamma knife radiosurgery (3). Rarely, these tumors are believed to cause raised intracranial pressure by way of obstructing venous outflow ultimately resulting in papilledema. Lertakyamanee et al in 2015 summarized 7 cases within the literature and reported an additional 3 cases. Of the 10 cases, age ranged between 2 and 56 years and 70% were female. Forty percent had bilateral tumors, 40% right-sided location, and 20% were left-sided. Two patients presented in a similar manner to our case with blurred vision and headache leading to the diagnosis of the paraganglioma. Five patients presented with blurred vision resulting in a diagnosis of papilledema only after they received intervention for the already diagnosed paraganglioma. One patient was misdiagnosed and treated for idiopathic intracranial hypertension before a later revelation of the presence of the paraganglioma. In cases where visual outcome data were well documented, 3 patients had very poor outcomes. One patient remained "blind" in both eyes, and 2 patients had one eye with light perception (1). The mechanism of action resulting in increased intracranial pressure is secondary to the impaired venous drainage, which subsequently increases the dural venous sinus pressure, and this pressure effect is carried across the arachnoid granulations. The pressure gradient between the subarachnoid and dural venous sinus spaces governs the cerebral sinus fluid outflow. In cases where symptoms are acute in nature, especially postsurgical intervention, venous thrombosis is the cause. Gradual symptoms are believed to be associated with venous compression. Interestingly, rightsided paragangliomas are more common, which could be alluded to the fact that the right transverse-sigmoid-jugular drainage system is usually dominant (1). Our patient also had coexistent bilateral vestibular schwannomas. These are benign neuroectodermal tumors arising from the Schwann cells of the vestibular root of the vestibulocochlear nerve (4). Bilateral vestibular schwannomas are characterized as neurofibromatosis Type 2 (NF-2) (5). The incidence is approximately 1 in 100,000. Patients can present with hearing impairment, tinnitus, facial nerve 254 palsy, and vertigo (4). Observation is a suitable option for small, asymptomatic, nongrowing tumors. Interventional options include microsurgical resection or stereotactic radiotherapy. A cochrane review in 2014 concluded that there were insufficient randomized control trials to determine superiority of one treatment option over another. It suggested that management should be determined by patient symptoms and preference, clinician experience, and availability of radiotherapeutic equipment (4). This case is unusual for several reasons. In comparison with cases discussed above, it is surprising that the grade of papilledema was very subtle despite the sizeable tumors. Similar patients have presented with advanced papilledema resulting in visual field defects as a consequence and in severe cases developed optic atrophy. It is more common for jugular paragangliomas to present with hearing problems, tinnitus, and lower cranial nerve palsies, all of which were absent in our case. We found no association between jugular paraganglioma and neurofibromatosis Type 2 within the literature. This case to the best of our knowledge is also the first report of bilateral vestibular schwannomas and simultaneous unilateral jugular paraganglioma. STATEMENT OF AUTHORSHIP Category 1: a. Conception and design: C. McCloskey and E. Cosgrave; b. Acquisition of data: C. McCloskey and E. Cosgrave; c. Analysis and interpretation of data: C. McCloskey and E. Cosgrave. Category 2: a. Drafting the manuscript: C. McCloskey and E. Cosgrave; b. Revising it for intellectual content: C. McCloskey and E. Cosgrave. Category 3: a. Final approval of the completed manuscript: C. McCloskey and E. Cosgrave. REFERENCES 1. Lertakyamanee P, Srinivasan A, De Lott LB, Trobe JD. Papilledema and vision loss caused by jugular paragangliomas. J Neuroophthalmol. 2015;35:364-370. 2. Gandía-González M, Kusak M, Moreno N, Sárraga JG, Rey G, Álvarez RM. Jugulotympanic paragangliomas treated with gamma knife radiosurgery: a single-center review of 58 cases. J Neurosurg. 2014;121:1158-1165. 3. Taïeb D, Kaliski A, Boedeker CC, Martucci V, Fojo T, Adler JR Jr, Pacak K. Current approaches and recent developments in the management of head and neck paragangliomas. Endocr Rev. 2014;35:795-819. 4. Muzevic D, Legcevic J, Splavski B, Cayé-Thomasen P. Stereotactic radiotherapy for vestibular schwannoma. Cochrane Database Syst Rev. 2014;12:CD009897. 5. Gutmann DH, Aylsworth A, Carey JC, Korf B, Marks J, Pyeritz RE, Rubenstein A, Viskochil D. The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA. 1997;278:51-57. McCloskey and Cosgrave: J Neuro-Ophthalmol 2020; 40: 252-254 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.