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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 514-516

Bilateral hemorrhagic grade 5 disc edema in small vestibular schwannoma with communicating hydrocephalus: An unusual presentation


1 Vision Care, Centre for Retina, Bhubaneswar, Odisha, India
2 Utkal Hospital, Bhubaneswar, Odisha, India

Date of Submission28-May-2021
Date of Acceptance07-Oct-2021
Date of Web Publication13-Apr-2022

Correspondence Address:
Nidhi Sinha
M-1, Samanta Vihar, Near NALCO Chowk, Bhubaneswar, Odisha - 751 017
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_1453_21

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  Abstract 


Bilateral optic disc edema is usually a late manifestation of vestibular schwannoma. Raised intracranial pressure due to obstructive hydrocephalus or a large mass lesion are the known causes. We report a case of a 55-year-old female with decreased vision and bilateral hemorrhagic grade 5 papilledema. MRI revealed a small vestibular schwannoma (<2.5 cm) with mild communicating hydrocephalus. Intraoperative CSF pressure was high most likely due to raised protein level in CSF (1 g/L). Bilateral hemorrhagic grade 5 papilledema causing diminution of vision in a case of small vestibular schwannoma with communicating hydrocephalus prompted us to report this case.

Keywords: Communicating hydrocephalus, papilledema, vestibular schwannoma


How to cite this article:
Penumetcha M, Sinha N, Sahoo SS, Rao GN. Bilateral hemorrhagic grade 5 disc edema in small vestibular schwannoma with communicating hydrocephalus: An unusual presentation. Indian J Ophthalmol Case Rep 2022;2:514-6

How to cite this URL:
Penumetcha M, Sinha N, Sahoo SS, Rao GN. Bilateral hemorrhagic grade 5 disc edema in small vestibular schwannoma with communicating hydrocephalus: An unusual presentation. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 18];2:514-6. Available from: https://www.ijoreports.in/text.asp?2022/2/2/514/342885



Optic disc edema in raised intracranial pressure was first described in 1853, and its pathogenesis is established as a mechanical phenomenon.[1]

It is primarily due to a rise of cerebrospinal fluid pressure (CSFP) in the optic nerve sheath, which produces axoplasmic flow stasis in the optic nerve fibers in the surface nerve fiber layer and prelaminar region of the optic nerve head. Axoplasmic flow stasis then results in swelling of the nerve fibers and consequently of the optic disc.[1]

Papilledema in vestibular schwannoma (VS) is a rare occurrence (8%).[2] The most common cause is obstructive hydrocephalus causing raised intracranial pressure. A large tumor (40 mm) causing bilateral papilledema without hydrocephalus has also been reported.[2]

To the best of our knowledge, hemorrhagic grade 5 papilledema causing diminution of vision in small vestibular schwannoma with communicating hydrocephalus has not been reported.


  Case Report Top


A 52-year-old female presented with gradual, painless, progressive diminution of vision in both eyes (LE > RE) for 5 months. It was associated with headache but no nausea and vomiting. Snellen's visual acuity was 6/18 and 3/60 in the right and left eye, respectively. Ocular evaluation revealed a normal anterior segment. On fundoscopy, bilateral markedly elevated discs with multiple superficial hemorrhages lying within a 3-disc diameter from the disc margin were seen. The artery to vein ratio was 2:3 with no signs of arteriosclerosis [Figure 1]a and [Figure 1]b. Optical coherence tomography (OCT) of both optic nerve heads showed marked elevation suggestive of papilledema [Figure 2].
Figure 1: (a and b) Bilateral hemorrhagic grade 5 papilledema with superficial retinal hemorrhages in first visit. (c and d) Bilateral resolving disc edema 2 months post-neurosurgery. (e and f) Bilateral resolved disc edema at 1 year follow-up

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Figure 2: Bilateral OCT of the disc in the first visit showing disc edema (a) right eye, (b)left eye

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There was a history of diminution of hearing associated with tinnitus and vertigo in left ear 6 months back.

She was nondiabetic, nonhypertensive with normal coagulation and lipid profile on investigations.

MRI Brain reported a well-defined 1.5 × 2.2 × 1.7 cm3 lesion in the left cerebellopontine angle extending into the left internal auditory canal, consistent with a vestibular schwannoma [Figure 3]. It was causing mild mass effect on the middle cerebral peduncle. There was mild hydrocephalus with periventricular hyperintensity. There was no evidence of obstructive pathology suggestive of a communicating hydrocephalus.
Figure 3: MRI report showing a well-defined 1.5 x 2.2 x 1.7 cm3 lesion in the left cerebellopontine angle extending into the left internal auditory canal consistent with a vestibular schwannoma (marked with arrows) (a) Axial view (b) Coronal view.

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A month later, neurosurgical excision of the tumor was done through a left retromastoid craniotomy. A right ventriculoperitoneal shunt (VP shunt) was done prior to the excision. Intraoperative CSF opening pressure was high, and evaluation for protein was 1 gm/L (normal: 0.05–0.15 g/L).[3] Intraoperatively the tumor was firm, densely adherent to the VII/VIII complex with intrameatal extension. The cisternal segment was excised completely, leaving behind a small intrameatal segment.

Postoperatively, the patient had facial nerve palsy (grade II according to House–Brackmann classification).

Follow-up at 2 months post-surgery revealed resolving optic disc edema with visual acuity 6/9, N8 in the right eye and 6/18, N8 in the left eye [Figure 1]c and [Figure 1]d. An OCT showed evidence of macular edema left more than the right eye.

At 1-year, optic disc edema had resolved with visual acuity of 6/9, N6 in the right eye and 6/12, N8 in the left eye [Figure 1]e and [Figure 1]f.


  Discussion Top


Chronic papilledema has been described as a cause of severe visual loss in neurofibromatosis Type 2, often leading to irreversible nerve damage.[4] After evaluation of 100 acoustic neuroma cases, van Meter et al.[5] found papilledema only in tumors that were 4.5 cm or greater in diameter. Mass effect and obstructive hydrocephalus cause raised intracranial pressure and hence papilledema.[6] Our patient presented with bilateral hemorrhagic disc edema (grade 5) with a tumor <2.5 cm in greatest diameter.

Communicating hydrocephalus in vestibular schwannomas (VS) is poorly described. Qasim Al Hinai et al.[3] described four cases of communicating hydrocephalus in small VS similar to the one we found in this case. They concluded elevated CSF protein levels as the cause. However; they did not mention anything about papilledema or visual loss in their patients.

In 1954, Gardner and associates were the first to report the association between small VS, high CSF protein level, and communicating hydrocephalus. They proposed partial obstruction of the absorptive areas of CSF by protein molecules secreted by the tumor as the cause.[7]

In 2003, Bloch et al.[8] stated that elevated ventricular CSF protein concentration impeded the absorption of CSF at the arachnoids. A 2.5- to 3-fold increase in the ventricular CSF protein concentration was necessary to produce communicating hydrocephalus in small VS cases. Our patient's CSF protein level was 1 g/L (a 7-fold increase).

Qasim Al Hinai et al.[3] added that high protein levels may also increase the CSF concentration and cause sludging of flow.

Interestingly, a high CSF protein concentration is a hallmark of VS but not all cases of VS have communicating hydrocephalus. This entreats further studies to solve the enigma.

Bloch et al.[8] hypothesized that older patients (mean: 76 years) have less adaptive capacity and are more prone to hydrocephalus with change in CSF hydrodynamics. However, Qasim Al Hinai et al.[3] had patients between 45 and 66 years. Our patient is 52 years old.

Gender, size, and side of the tumor are not found relevant in the development of communicating hydrocephalus.

Our patient had hydrocephalus management (right VP shunt) followed by the removal of the primary tumor resulting in resolved disc edema and improvement in vision.

We concur with the hypothesis that small VS with raised CSF protein cause communicating hydrocephalus and add that it can present first to an ophthalmologist as disc edema. Prompt neurosurgical management can help restore the vision.


  Conclusion Top


Hemorrhagic papilledema can be a manifestation in small vestibular schwannoma with communicating hydrocephalus. Early neurosurgical intervention may be considered.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hayreh SS. Pathogenesis of optic disc edema in raised intracranial pressure. Prog Retin Eye Res 2016;50:108-44.  Back to cited text no. 1
    
2.
Candanedo C, Moscovici S, Kruger JM, Mizrachi CJ, Eliahou R, Spektor S. Vestibular schwannoma presenting with bilateral papilledema without hydrocephalus: Case study. Cureus 2017;9:e1862.  Back to cited text no. 2
    
3.
Al Hinai Q, Zeitouni A, Sirhan D, Sinclair D, Melancon D, Richardson J, et al. Communicating hydrocephalus and vestibular schwannomas: Etiology, treatment, and long-term follow-up. J Neurol Surg B: Skull Base 2013;74:68-74.  Back to cited text no. 3
    
4.
Thomas DA, Trobe JD, Cornblath WT. Visual loss secondary to increased intracranial pressure in neurofibromatosis type 2. Arch Ophthalmol 1999;117:1650-3.  Back to cited text no. 4
    
5.
van Meter WS, Younge BR, Harner SG. Ophthalmic manifestations of acoustic neurinoma. Ophthalmology 1983;90:917-22.  Back to cited text no. 5
    
6.
Bouzas EA, Parry DM, Elridge R, Kaiser-Kupfer MI. Visual impairment in patients with neurofibomatosis 2. Neurology 1993;43:622-3.  Back to cited text no. 6
    
7.
Gardner WJ, Spitler DK, Whitten C. Increased intracranial pressure caused by increased protein content in the cerebrospinal fluid; an explanation of papilledema in certain cases of small intracranial and intraspinal tumors, and in the Guillain-Barre syndrome. N Engl J Med 1954;250:932-6.  Back to cited text no. 7
    
8.
Bloch J, Vernet O, Aubé M, Villemure J-G. Non-obstructive hydrocephalus associated with intracranial schwannomas: Hyperproteinorrhachia as an etiopathological factor? Acta Neurochir (Wien) 2003;145:73-8.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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