|Year : 2015 | Volume
| Issue : 1 | Page : 41-43
Retrobulbar Neuritis with Headache: A Case of Invasive Fungal Sphenoethmoidal Sinusitis
Sonal Dangda1, Kirti Singh1, Ishwar Singh2, Kshitij Aditya1
1 Guru Nanak Eye Centre, Lok Nayak Hospital, Maulana Azad Medical College, New Delhi, India
2 Department of ENT, Lok Nayak Hospital, Maulana Azad Medical College, New Delhi, India
|Date of Web Publication||27-Jan-2015|
L-57, Sector-25, Noida - 201 301
Source of Support: None, Conflict of Interest: None
Retrobulbar neuritis is classically defined as a disease where "neither the patient nor the doctor sees anything." The present case was diagnosed as retrobulbar neuritis associated with extreme retrobulbar pain, an unusual constellation of features which led us to suspect an unusual etiology. Detailed investigations revealed invasive fungal sinusitis masquerading as retrobulbar neuritis. This case highlights the need to keep a high index of suspicion and caution while labeling a case as pure retrobulbar neuritis without performing neuroimaging to rule out life-threatening conditions.
Keywords: Fungal, headache, sudden vision loss, spheno-ethmoidal sinusitis
|How to cite this article:|
Dangda S, Singh K, Singh I, Aditya K. Retrobulbar Neuritis with Headache: A Case of Invasive Fungal Sphenoethmoidal Sinusitis. MAMC J Med Sci 2015;1:41-3
|How to cite this URL:|
Dangda S, Singh K, Singh I, Aditya K. Retrobulbar Neuritis with Headache: A Case of Invasive Fungal Sphenoethmoidal Sinusitis. MAMC J Med Sci [serial online] 2015 [cited 2020 Jun 3];1:41-3. Available from: http://www.mamcjms.in/text.asp?2015/1/1/41/150063
| Introduction|| |
Optic Neuritis is an acute inflammatory condition of the optic nerve resulting in a sudden loss of vision usually associated with mild pain on eye movement. Although most commonly idiopathic, it can also be associated with a variety of conditions and pathologies of the sphenoid sinus have lately been noted to be an important causative factor.
| Case Report|| |
A 35-year-old female patient presented to the emergency services with complaints of severe headache and nausea of 7-8 days duration along with a diminution of vision over last 2-3 days. Examination showed a visual acuity of light perception with two quadrants inaccurate projection of rays in right eye (R/E) and 6/6 left eye (L/E). Ocular motility was full with no proptosis or adnexal lesion. Anterior segment examination was essentially normal with only relative afferent pupillary defect (RAPD) seen in R/E [[Figure 1]a]. Posterior segment revealed normal fundus with no evidence of optic disc edema or pallor and no signs suggestive of compression like macular/choroidal folds [[Figure 1]b]. Visual acuity was previously documented as 6/24 in R/E with a hypermetropic refractive error of 4.5 dioptre sphere is and 6/6 in emmetropic left eye (L/E).
|Figure 1: (a) Anterior segment photograph showing relative afferent pupillary defect, (b) Fundus photograph showing no evidence of optic disc edema or pallor, (c) Magnetic resonance imaging brain and orbit revealed an ill-defined lesion in the sphenoethmoidal sinus area, (d) Visual evoked potential showed increased P100 latency on the right side, (e) Visual evoked potential showing improvement in P100 latency on the right side, (f) Contrast-enhanced computed tomography of para-nasal sinuses showing invasive fungal sinusitis with intracranial extension into the middle cranial fossa due to bone erosion|
Click here to view
Clinical evaluation did not reveal fever, evidence of upper respiratory tract or paranasal sinus infection. Neurological examination was essentially normal with only right-sided second cranial nerve being involved. Electrophysiological testing documented abnormal visual evoked potential (VEP) of R/E with increased P100 latency [[Figure 1]c]. Magnetic resonance imaging of the brain and orbit revealed an ill-defined lesion in the spheno-ethmoidal sinus area [[Figure 1]d]. A clinical suspicion of spheno-ethmoidal mass of unclear etiology causing optic neuritis was entertained. After ear, nose, and throat consult, intravenous methyl prednisolone under antibiotic and antifungal cover (intravenous monocef 500 mg 12 h and oral fluconazole 150 mg BD) was started. Patient's vision recovered to 5/60 with an accurate projection of rays within 3 days, and VEP of optic nerve conductance elicited a response with improvement of P100 latency from 125.70 to 88.20 [[Figure 1]e]. A nasal endoscopy revealed purulent discharge in the spheno-ethmoidal recess giving an impression of pyocele. Repeat contrast-enhanced computed tomography of paranasal sinuses revealed complete opacification of sphenoid and posterior ethmoidal sinuses with hyperdense content. Mild bulkiness of right cavernous sinus along with intracranial extension into middle cranial fossa with bone erosion was noted. Extension to superior orbital fissure and involvement of right optic nerve was also documented [[Figure 1]f]. Provisional diagnosis of invasive fungal sinusitis was made, and endoscopic removal of the spheno-ethmoidal mass was performed, followed by anti-fungal course of intravenous amphotericin B (1 g/kg/day). The patient recovered best-corrected visual acuity 6/24 (with hypermetropic refractive amblyopia) within a week of surgical intervention. The hematological investigations were unremarkable including total neutrophil and lymphocyte counts and the fasting and postprandial sugars and Western blot for HIV were unremarkable. At no time was disc edema or papilledema noted during the entire hospital stay and follow-up. Histopathology of the mass demonstrated fungal elements which showed growth of fungal hyphae on culture.
| Discussion|| |
Optic neuritis encompasses two varied clinical presentations, namely papillitis and retrobulbar neuritis. While papilitis has specific clinical picture, retrobulbar neuritis is a diagnosis of exclusion and patients are treated empirically with systemic steroids regardless of underlying etiology ranging from demyelinating, inflammatory disease to compressive lesions. Diagnosis is based on characteristic rapid onset unilateral vision loss in young and middle-aged patients, with an RAPD. Neuroimaging is not resorted to in a resource poor country like ours and is often performed at a later date to rule out demyelinating disorders.
Sphenoid sinus lesions as a cause of retrobulbar neuritis has been infrequently documented in the past,  lately this entity is gaining wider recognition. ,,,, A review of optic neuritis cases with atypically severe headache picked up sphenoid sinus pathology ranging from a fungal infection, mucopurulent material, polyps, and necrotic tumor often requiring surgical interventions for visual recovery. The authors, Moorman et al., emphasized that patients presenting with isolated optic neuritis and atypical headache should undergo neuroimaging and an opaque sphenoid sinus should not be dismissed as coincidental but assumed to be pathological requiring urgent drainage.  This aspect is reiterated by a recent report from the Indian subcontinent of a similar patient of retrobulbar neuritis, painful ophthalmoplegia, and sphenoid sinus mucocele with documented improvement after surgical removal of the mucocele  and also other recent literature. ,,, The postulated reason for retrobulbar neuritis in such cases is mechanical compression of the optic nerve along with toxic neuritis. 
In this case, the severe, debilitating headache made us question our initial blanket diagnosis of retrobulbar neuritis and prompted a specific neuroimaging. Timely surgical intervention was both vision and life-saving. In the absence of specific neuroimaging, pure methyl prednisolone pulsed therapy (standard protocol for treating retrobulbar neuritis) if given to this case with underlying fungal lesion would have been devastating. This case highlights the need for a high index of clinical suspicion in cases of retrobulbar neuritis with atypical features especially severe retrobulbar pain.
| References|| |
Everberg G. Retrobulbar neuritis due to cyst of sphenoid sinus. Acta Otolaryngol 1955;45:492-7.
Vaphiades MS, Yunker JJ, Roberson GH, Meyer DR, Mills DM. Optic neuritis is nothing to sneeze at. Surv Ophthalmol 2007;52:106-10.
Arnavielle S, Vignal-Clermont C, Galatoire O, Jacomet PV, Klap P, Boissonnet H, et al.
Compressive optic neuropathy related to an anterior clinoid mucocele. J Fr Ophtalmol 2010;33:208.e1-6.
Levy J, Monos T, Puterman M. Bilateral consecutive blindness due to sphenoid sinus mucocele with unilateral partial recovery. Can J Ophthalmol 2005;40:506-8.
Chmielewski R, Paprocki A, Morawski K, Niemczyk K. Sphenoid sinus mucocele penetrating to the orbit, anterior and middle cranial fossae and parapharyngeal space: a case report. Otolaryngol Pol 2010;64:47-50.
Bahadir O, Arslan S, Arslan E, Türk A, Ural A. Sphenoid sinus mucocele presenting with unilateral visual loss: a case report. B-ENT 2011;7:65-8.
Moorman CM, Anslow P, Elston JS. Is sphenoid sinus opacity significant in patients with optic neuritis? Eye (Lond) 1999;13 (Pt 1):76-82.
Gupta AK, Menon V, Sharma P, Saxena R, Kumaran S. A sphenoid sinus mucocele simulating as retro bulbar optic neuritis. Indian J Ophthalmol 2012;60:216-8.
Rothstein J, Maisel RH, Berlinger NT, Wirtschafter JD. Relationship of optic neuritis to disease of the paranasal sinuses. Laryngoscope 1984;94:1501-8.