Restrictive medial rectus myositis in orbital sarcoidosis: A case report

Prerna Kataria; Neha K Sethi; Ashu Dihana; Ramneet Kaur Dhillon

doi:10.4103/IJO.IJO_3106_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 2 | Page : 510-512

Restrictive medial rectus myositis in orbital sarcoidosis: A case report

Prerna Kataria, Neha K Sethi, Ashu Dihana, Ramneet Kaur Dhillon
Ophthalmology and G.G.S Medical College and Hospital, Faridkot, Punjab, India

Date of Submission	26-Nov-2022
Date of Acceptance	10-Feb-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Neha K Sethi
G.G.S Medical College and Hospital, Faridkot, Punjab
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_3106_22

Abstract

Sarcoidosis is a systemic multiorgan granulomatous disease of unknown etiology in middle age group women. Orbital sarcoidosis is uncommon and the most common orbital manifestation is dacryoadenitis (70%). Extraocular muscle involvement is rare; if present, the superior rectus/levator complex and lateral rectus are most commonly involved. We report a 57-year-old male, a known case of biopsy-proven sarcoidosis, with medial rectus myopathy and lacrimal dacryoadenitis in the left eye, and angle closure with raised intraocular pressure (IOP) in both eyes. Imaging by computed tomography (CT) also confirmed the above findings. The forced duction test was positive confirming restrictive myopathy of the left medial rectus muscle. Orbital sarcoidosis has a highly diverse presentation; medial rectus involvement is a rare occurrence.

Keywords: Angle closure suspect, dacryoadenitis, forced duction test, medial rectus myopathy, orbital inflammation, orbital sarcoidosis, restrictive myositis

How to cite this article:
Kataria P, Sethi NK, Dihana A, Dhillon RK. Restrictive medial rectus myositis in orbital sarcoidosis: A case report. Indian J Ophthalmol Case Rep 2023;3:510-2

How to cite this URL:
Kataria P, Sethi NK, Dihana A, Dhillon RK. Restrictive medial rectus myositis in orbital sarcoidosis: A case report. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:510-2. Available from: https://www.ijoreports.in/text.asp?2023/3/2/510/375022

Sarcoidosis is an idiopathic multisystemic granulomatous disease. Ocular involvement occurs in 25–60% of patients with systemic sarcoidosis, anterior uveitis being the most common manifestation.^{[1],[2],[3],[4]} Ocular sarcoidosis can involve any part of the eye leading to conjunctival granuloma, episcleritis/scleritis, uveitis, optic neuropathy, and/or its adnexal tissues leading to lacrimal gland enlargement and orbital inflammation.^[5] Orbital sarcoidosis is uncommon and a few case series have been reported in the literature.^{[2],[3],[4],[6],[7],[8]} The most common orbital manifestation of sarcoidosis is dacryoadenitis.^[2] Although biopsy-proven systemic sarcoid myopathy occurs in over a half of patients, extraocular sarcoid myopathy with imaging evidence of extraocular muscle is rare.^[2],[3],[7] In such cases, the superior rectus/levator complex and lateral rectus are most commonly involved.^[3] Involvement of the medial rectus muscle is highly uncommon. Orbital sarcoidosis tends to occur in women in the sixth decade. In a case series by Vahdani et al. of 61 patients, 45 (74%) were female patients.^[6] We hereby report a 57-year-old male, a known case of biopsy-proven sarcoidosis, with imaging evidence of left medial rectus muscle involvement. In addition, the patient also had raised intraocular pressure (IOP) with angle closure in both eyes and no evidence of past or current uveitis.

Case Report

A 57 year old male, with biopsy proven sarcoidosis, presented with gradual progressive painless swelling of the upper and lower lids left eye since 2 years, and right eye since 6 months [Figure 1]. He had been diagnosed with sarcoidosis on orbital biopsy (showing granulomatous inflammation) done 2 years back for left-sided painless progressive proptosis. The patient also complained of progressive horizontal diplopia since 3 months; more pronounced in the right gaze on diplopia charting. There were soft non-tender multiple irregular nodules present in the lower lids of both eyes with dough-like consistency, not adherent to the overlying skin or underlying structures. His best-corrected visual acuity was 6/9 in both eyes. IOP by applanation tonometry was 26 and 28 mmHg in the right and left eyes, respectively. Anterior segment examination showed a shallow anterior chamber with Van Herick's grade 2 with NO1 (according to LOCS III classification) cataractous lens of both eyes. Gonioscopy showed 270° iridotrabecular contact suggestive of angle closure for which laser iridotomy of both eyes had been done. Dilated fundus examination with clear media showed a cup disc ratio of 0.3 with a sharp foveal reflex.

Figure 1: External photograph showing swollen lids (lower lids more than upper lids) of both eyes. Visual alignment is parallel in the primary gaze

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Extraocular movements were painless and full in the right eye; painless and restricted medially in the left eye [Figure 2]. A forced duction test was done which was positive confirming the restriction of the left medial rectus muscle.

Figure 2: (a) External photograph showing restriction of adduction of the left eye in right gaze. (b) External photograph showing normal left gaze in both eyes

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The patient was also a known hypertensive and recently diagnosed controlled diabetic. General physical examination was normal with oxygen saturation of 98% on room air. There was no enlargement of lymph nodes (periauricular or submandibular). Visual alignment was parallel in the primary gaze [Figure 1].

Complete blood counts showed increased eosinophil count, and the erythrocyte sedimentation rate was 35 mm in the first hour. Serum angiotensin-converting enzyme (ACE) levels were raised to 120 μg (normal: <40 μg/L). Serum and urine calcium levels were normal. High-resolution computed tomography (HRCT) chest showed multiple enlarged mediastinal and bilateral hilar lymph nodes and a small fibrotic patch in the right upper lobe with a few randomly scattered soft tissue density nodules in both lungs. Thyroid, liver, and renal function tests were normal.

Computed tomography (CT) orbit demonstrated ill-defined soft tissue thickening with surrounding fat stranding in the preseptal and periorbital region on both sides, and extension along the medial quadrant was noted on the left side; left medial rectus muscle was bulky suggesting myositis [Figure 3]a. A bulky left-sided lacrimal gland with indistinct fat planes was suggestive of lacrimal gland inflammation [Figure 3]b.

Figure 3: (a) CT scan (axial) showing ill-defined soft tissue density thickening with surrounding fat stranding in preseptal and periorbital region on both sides, and extension along the medial quadrant on the left side; bulky medial rectus muscle of left eye (black asterisk) is suggestive of medial rectus myositis. (b) CT scan (coronal) shows a bulky lacrimal gland (black asterisk) with indistinct fat planes suggestive of lacrimal gland enlargement on the left side. The figure also shows enhancement of medial orbital structures suggestive of orbital inflammation

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Thus, a diagnosis of left restrictive medial rectus myositis with lacrimal dacryoadenitis secondary to sarcoidosis was made. Oral corticosteroids were started and the patient responded well to the treatment.

Discussion

Involvement of the ocular adnexa in sarcoidosis is usually restricted to infiltration of the skin, conjunctiva, lacrimal glands, and uveal tissue. Lacrimal glands are involved in 70% of patients with orbital sarcoidosis.^[3] Our patient likewise had unilateral dacryoadenitis as seen on a CT scan. In contrast, manifestations of sarcoidosis of retrobulbar space are uncommon, myositis being the most unusual.^{[2],[3],[6],[8]} The superior rectus/levator complex and lateral rectus are most commonly involved—possibly due to spill-over inflammation from granulomatous dacryoadenitis.^[3] Involvement of the medial rectus is very uncommon. In the case series by Vahdani et al.,^[3] only 4 out of 20 patients of sarcoidosis with extraocular muscle involvement had medial rectus myopathy. Our case had extensive involvement of the medial rectus of the left eye; a forced duction test also suggested restrictive myopathy.

Clinically, orbital sarcoid may be confused with Graves' orbitopathy, particularly in this case given the painless nature of swelling and raised IOP. However, it can be excluded by the absence of any other features of Graves' orbitopathy, such as lid retraction, and normal thyroid function. The cause of raised IOP cannot be clearly comprehended - may be steroid induced, secondary to restrictive myopathy, or due to angle closure.

Conclusion

To conclude, sarcoidosis of the orbit has a very heterogenous presentation, besides the disease itself being a diagnostic challenge. The unique features of our case were male sex; unilateral medial rectus myositis with dacryoadenitis; myopathy and dacryoadenitis evident on orbital imaging; gonioscopic angle closure; and positive biopsy findings.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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