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Year : 2008  |  Volume : 1  |  Issue : 1  |  Page : 28-31 Table of Contents   

Optic nerve head sarcoid granuloma treated with intravenous methyl prednisolone

Medical Research Foundation, Sankara Nethralaya, Chennai, India

Correspondence Address:
Jyotirmay Biswas
Medical and Vision Research Foundation, Sanakara Nethralaya, 18, College Road, Chennai-600 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-620X.43318

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A 49-year-old housewife presented with progressively worsening vision in the right eye over a period of two months. Ocular examination revealed a mass arising from the optic nerve head associated with disc edema, flame shaped hemorrhages and mild vitritis. After thorough investigation and establishment of a diagnosis of optic nerve head sarcoidosis, the patient was initiated on intravenous methyl prednisolone.

Keywords: Optic nerve, granuloma, sarcoidosis, steroids

How to cite this article:
Jafferji SS, Biswas J. Optic nerve head sarcoid granuloma treated with intravenous methyl prednisolone. Oman J Ophthalmol 2008;1:28-31

How to cite this URL:
Jafferji SS, Biswas J. Optic nerve head sarcoid granuloma treated with intravenous methyl prednisolone. Oman J Ophthalmol [serial online] 2008 [cited 2022 Aug 8];1:28-31. Available from: https://www.ojoonline.org/text.asp?2008/1/1/28/43318

   Introduction Top

There are several causes for optic nerve head granulomas; sarcoidosis is a rare cause. The diagnosis of sarcoid granuloma is a challenge considering the nonavailability of a histopathology specimen. However, with increasing knowledge of the subject and newer diagnostic modalities and new classifications, [1] the sensitivity of making a correct diagnosis has increased over the past few years. We, therefore, choose to present a rare case of optic nerve head granuloma due to sarcoidosis treated successfully with intravenous methyl prednisolone.

   Case Report Top

A 49-yr-old housewife was referred with complaints of floaters and progressive poor vision in the right eye worsening over two months. She did not have any constitutional symptoms. She denied history of trauma or surgery. There was no history indicative of tuberculosis. She did not have any joint pains or ulcerations anywhere in the body. There was no previous history of diabetes or hypertension

Systemic examination was essentially normal. Ophthalmic examination showed a best corrected visual acuity of 6/24 in the right eye and 6/6 in the left eye. Intra ocular pressures by applanation tonometry were 14 and 16 mmHg in the right and left eyes, respectively. Anterior segment examination was essentially normal with the right eye having vitreous cells of one plus yellow-white in color. Fundoscopy in the right eye showed disc hyperemia with mild swelling and a mass abutting the optic nerve head on the macular side associated with a streak of hard exudates and optic nerve head vascular tortuosity with fresh flame shaped hemorrhages [Figure 1]. Pars plana exudates were also present. The left eye fundus examination was within normal limits.

Investigations done included a complete blood count with differential count, erythrocyte sedimentation rate using the Westergen method, kidney and liver function tests and a fasting blood sugar, all of which were within normal limits. Mantoux test and chest X-ray were done to rule out tuberculosis and were found to be normal. The inorganic serum phosphorous and calcium were within normal limits.

The serum angiotensin converting enzyme level was elevated at 56.7 IU/L (8 - 52), and the serum lysozyme was borderline elevated at 459 units (0 - 500). Computed Tomography (CT) scan of the orbit and brain did not show any space occupying lesion or features of raised intracranial pressure, whereas the ocular ultrasound showed a mass abutting the optic nerve head measuring approximately one mm in size [Figure 2].

The patient was started on pulsed intravenous methyl prednisolone at a dose of one gram daily for three days, which was followed with oral high dose prednisolone at 1.5 mg/Kg/ day and tapered at rate of 10 mg weekly, along with oral calcium supplement and ranitidine. Oral methotrexate 15 mg weekly was also initiated along with folic acid five mg daily [Table 1].

At one week review, the granuloma already showed features of regression. The patient was advised to do complete blood counts fortnightly and monthly liver function tests.

While on follow up, at the five month interval, the patient was noted to have developed satellites at the edge of the granuloma. The dosage of methotrexate was stepped up and oral steroids were reintroduced. Improvement was seen and the steroids along with the methotrexate were gradually retapered. At the two year follow up visit complete regression of the granuloma was seen along with peripapillary scarring [Figure 3] and [Figure 4].

   Discussion Top

A clinical diagnosis of optic nerve head granuloma was made as per the new edition of the guidelines for the diagnosis of ocular sarcoidosis. [1]

Biochemical and serological investigations done strongly contributed towards sarcoidosis; hence the indication for initiating therapy. It was not possible to get a biopsy in this patient since she did not manifest any other systemic findings. Moreover, her investigations were within normal limits. The serum calcium and phosphorous limits were borderline along with the serum lysozyme possibly due to the early detection of the disease and the patient having only optic nerve head involvement.

Since sarcoidosis is a condition which is treated with systemic steroids, we decided to initiate intravenous methyl prednisolone on the basis of a vision threatening lesion. The patient responded well to therapy with regression of the granuloma and improvement in both vision and symptoms.

To date, there are only ten reported cases in the literature with a diagnosis of optic nerve head granuloma of which seven cases were accompanied by histopathological diagnosis [Table 2]. [2],[3],[4],[5],[6],[7],[8] However, there are no cases which have been treated with intravenous methyl prednisolone with a positive response to therapy and a follow up of two years.

We present a rare case of optic nerve head granuloma which responded primarily to intravenous methyl prednisolone, and was subsequently maintained on oral steroids and immunosuppressive therapy.

   References Top

1.Asukata Y, Ishihara M, Hasumi Y, Nakamura S, Hayashi K, Ohno S, et al. Guidelines for the diagnosis of ocular sarcoidosis. Ocul Immunol Inflamm 2008;16:77-81.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Gass JD, Olson CL. Sarcoidosis with optic nerve and retinal involvement Arch of Ophthalmol 1976;94:945-50.  Back to cited text no. 2    
3.Lustgarten JS, Mindel JS, Yablonski ME, Friedman AH. An unusual presentation of isolated optic nerve sarcoidosis. J Clin Neuroophthalmol 1983;3:13-8.  Back to cited text no. 3  [PUBMED]  
4.Jordan DR, Anderson RL, Nerad JA, Patrinely JR, Scrafford DB. Optic nerve involvement as the initial manifestation of sarcoidosis. Can J Ophthalmol 1988;23:232-7.  Back to cited text no. 4  [PUBMED]  
5.Castagna I, Salmeri G, FamÓ F, Trombetta CJ, Ricci R, Tracl˛ CD. Optic nerve granuloma as first sign of systemic sarcoidosis. Ophthalmologica 1994;208:230-2.  Back to cited text no. 5    
6.Beck AD, Newman NJ, Grossniklaus HE, Galetta SL, Kramer TR. Optic nerve enlargement and chronic visual loss. Surv Ophthalmol 1994;38:555-66.  Back to cited text no. 6  [PUBMED]  
7.El Zarka A, Hille K, Ruprecht KW. Granulomatous process of the optic papilla as an initial sign of systemic sarcoidosis. Klin Monatsbl Augenheilkd 1995;206:134-5.  Back to cited text no. 7  [PUBMED]  
8.Brindeau C, Glacet-Bernard A, Roualdes B, Abd Alsamad I, Oubraham H, Meyrignac C, et al. Sarcoid optic neuropathy. J Fr Ophtalmol 1999;22:1072-5.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1], [Table 2]

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