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BRIEF REPORT
Year : 2000  |  Volume : 48  |  Issue : 3  |  Page : 231-3

Lid swelling and diplopia as presenting features of orbital sarcoid


Medical Research Foundation, 18 College Road, Chennai-600 006, India

Correspondence Address:
J Biswas
Medical Research Foundation, 18 College Road, Chennai-600 006
India
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Source of Support: None, Conflict of Interest: None


PMID: 11217257

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Keywords: Administration, Oral, Biopsy, Needle, Diagnosis, Differential, Diplopia, diagnosis, drug therapy, etiology, Edema, diagnosis, drug therapy, etiology, Eyelid Diseases,


How to cite this article:
Biswas J, Krishnakumar S, Raghavendran R, Mahesh L. Lid swelling and diplopia as presenting features of orbital sarcoid. Indian J Ophthalmol 2000;48:231

How to cite this URL:
Biswas J, Krishnakumar S, Raghavendran R, Mahesh L. Lid swelling and diplopia as presenting features of orbital sarcoid. Indian J Ophthalmol [serial online] 2000 [cited 2020 Apr 2];48:231. Available from: http://www.ijo.in/text.asp?2000/48/3/231/14868

Sarcoid is an idiopathic multisystem non-caseating granulomatous disease with protean clinical manifestations. In the eye, the common sites of involvement are the skin of eyelid, conjunctiva, uveal tract, retina, optic nerve and lacrimal gland.

Indian J Ophthalmol 2000; 48:231-33

The most common ocular manifestation in sarcoidosis is uveitis and the most common orbital manifestation is dacryoadenitis, which is frequently bilateral.[1] Sarcoid of the orbit can be with or without systemic disease. Involvement of the extraocular muscles in sarcoid is rare and a only few cases have been reported. We report a patient with sarcoid whose first symptoms and signs were due to involvement of the extraocular muscles (EOM).


  Case report Top


A 45-year-old male presented with diplopia in downward gaze, defective vision in the left eye and swelling of the left lower lid for the past 3 months. He complained of pain, redness, and irritation in both eyes. He was treated by ophthalmologist, but there was no improvement. He had no systemic complaints. On examination his visual acuity was 6/5, N6 in the right eye and 6/9+4, N6 in the left eye. Slitlamp examination of the right eye revealed no abnormality except for nuclear sclerosis and posterior subcapsular cataract. The lower lid of the left eye was swollen and a firm mass was felt on the orbital floor [Figure - 1]. The lid swelling was secondary to orbital mass. Slitlamp revealed nuclear sclerosis and posterior subcapsular cataract. The eye was otherwise normal. There was no relative afferent pupillary defect. The mass was immobile and tender. Computerized Tomography {CT) scan of the brain and orbit showed a diffuse mass lesion below the globe occupying the anterior half of the orbit [Figure - 2]. CT scan of thorax showed mediastinal and hilar lymph node enlargement. Ultrasonography of the right orbit showed gross thickening of superior rectus and increased echogenicity. Other extraocular muscles were pictured more easily than usual. Ultrasound scan of the left orbit showed thickening of the superior rectus, and low to moderate reflective echoes were seen within the superior rectus. There was no enlargement of the lacrimal gland. Diplopia charting showed horizontal separation o£ images by half an inch all over; there were right-over-left images, showing crossed diplopia. Maximum separation was seen on the left downward gaze. Colour vision was normal.

Laboratory investigations showed a normal haemogram, and erythrocyte sedimentation rate was 45-mm at first hour. Serum angiotensin converting enzyme (ACE) levels were 98 units (normal: 14-70 units). Serum lysozyme was 100 units. Intradermal purified protein derivative test for Mycobacterium tuberculosis was negative (3-mm induration). Rheumatoid factor, uric acid levels and thyroid hormone levels were normal. Fine needle aspiration biopsy (FNAB) of the lid mass showed scanty aspirate with acellular eosinophilic material with very sparse cells. As this was inconclusive, biopsy of the orbital mass was done and the histopathology showed a non-caseating granuloma consisting of epithelioid histiocytes, Langhan's giant cells and foreign body giant cells [Figure - 3]. There was minimal lymphocytic infiltrate around the granuloma. The stroma showed fibrosis. Special stains for acid fast bacilli and fungi were negative. Culture for acid-fast bacillus did not reveal any growth

An examination by an internist did not reveal skin nodules, lymphadenopathy or organomegaly. The patient was further reviewed by a pulmonologist who found no clinical respiratory abnormality though the chest X-ray showed hilar lymphadenopathy. The patient was started on tablet prednisolone 50 mg per day, tapered by 10 mg per week over a period of 11 weeks; and subsequently maintained on 10 mg of prednisolone on alternate days.

After 2 weeks, we noticed a marked reduction in lid swelling and the absence of diplopia. The patient was reviewed after a year. He had complaints of occasional diplopia in extremes of gaze. His best-corrected visual acuity was 6 / 6; N6. There was no lid swelling or congestion and both eyes were quiet. However diplopia charting revealed separation of images on laevo depression. The patient was put on prednisolone 10 mg on alternate days. Slitlamp examination revealed early posterior subcapsular cataract in both eyes. Ultrasound examination shows thickend extraocular muscle, particularly the superior rectus. Fundus examination revealed no abnormality. Diplopia charting showed evidence of crossed diplopia.


  Discussion Top


The unique feature of our case is the initial presentation of lid swelling, and diplopia which was secondary to orbital and extraocular muscle involvement resulting in the presenting symptoms and the signs of the patient, There are five previously reported cases of extraocular muscle involvement in sarcoid.[2-5] Four of them occurred in suspected or known sarcoid patients in which extraocular muscle involvement was entirely asymptomatic. In the fifth case, there was bilateral painful, external ophthalmoplegia and enlargement of all EOM on CT scan.

The initial clinical differential diagnosis of EOM enlargement and orbital mass in our patient included thyroid ophthalmopathy, pseudotumor, neoplasm, and sarcoid. In our patient, there were no external signs of thyroid disease and the thyroid function tests (TSH, T3, T4) levels were within normal limits. Ultrasound guided FNAB of the mass revealed only sparse cells. As it was not conclusive, biopsy of the orbital mass was done and the diagnosis established. FNAB in sarcoid has its limitations, as cytology may reveal a few giant cells, histiocytes and lymphocytes. Such a histopathologic picture can occur in tuberculosis, fungal infections or granulomatous variant of pseudotumour. However, FNAB helps to exclude a malignant tumour.

The chronic orbital inflammation due to sarcoid produces a desmoplastic response in the orbital tissues and the EOM leading to fixation of structures, which could account for diplopia.[6]

While reports of extraocular muscle involvement in sarcoid are rare, as many as 50% to 69% of patients with sarcoid have biopsy-proven skeletal muscle involvement. Musculoskeletal symptoms of weakness, muscle pain and arthritis occur in 17% to 69% of patients with sarcoid.[7] In our case, the lesions predominantly involved the anterior orbit. Subsequent investigations revealed radiological evidence of pulmonary sarcoid and elevated serum ACE levels. Patients with histopatho-logical features of sarcoid in the orbit should be investigated for systemic involvement. In the absence of systemic involvement one should consider the possibility of idiopathic granulomatous inflammation of the orbit. [8,9] The other granulomatous conditions in the orbit are Wegener's granulomatosis, tuberculosis and fungal granulomas. Wegener's granulomatosis is characterized by a necrotising vasculitis with granulomatous inflammation. Tuberculosis often shows caseation necrosis in the granuloma, and special stains for acid-fast bacilli are positive. Fungal granuloma is rare in immunocompetent patients. Aspergilloma can cause granulomatous inflammation in the orbit. Special stains and culture can detect the fungus.

Systemic corticosteroids are the mainstay of therapy in case of sarcoid. Oral prednisolone is given in initial dose of 1 mg/kg of body weight, and can be gradually tapered off, 10 mg per week after the inflammation is controlled. The patient may require 10 to 15 mg a day as a maintenance dose for several weeks to months. Cornbath and associates5 have reported a 15-year-old white boy of biopsy-proven sarcoidosis with extraocular muscle involvement that resolved completely on 60 mg of oral prednisolone; Subsequently on gradual tapering over 12 months to 20 mg every other day, the patient developed recurrence, and when the dosage was increased to 25 mg prednisolone on alternate days he showed response. The patient's ocular condition improved 5 months later with slow tapering of 15 mg of prednisolone every other day. In resistant cases, immunosuppressive therapy can be given.

Our patient responded promptly to corticosteroids with resolution of pain and improvement of ocular motility and decrease of diplopia. However when the corticosteroids were switched to alternate day therapy with tapered dosage, diplopia returned. This relapse at low doses is common in the corticosteroid treatment of sarcoid and frequently requires slow dose tapering and longer total duration of the corticosteroid treatment.


  Acknowledgement Top


We would like to acknowledge Dr. Raj B. Singh, Respiratory Physician, Apollo Hospitals, Chennai, for evaluating this case.



 
  References Top

1.
Jakobiec F, Bilyk JR, Font RL. Non-infectious orbital inflammations. In: Spencer WH, editor. Ophthalmic pathology - An Atlas and Textbook. W.B. Saunders; Philadelphia, 4th edition, 1996. Vol. 4, pp 2810-58.  Back to cited text no. 1
    
2.
Patrinely JR, Osborn AG, Anderson RL, Whiting AS. Computed tomographic features of non-thyroid extraocular muscle enlargement. Ophthalmology 1989;96:1038-47.  Back to cited text no. 2
[PUBMED]    
3.
Stannard K, Spalton DJ. Sarcoid with infiltration of the external ocular muscles. Br j Opthalmol 1985;69:562-66.  Back to cited text no. 3
[PUBMED]    
4.
Frank KW, Weiss H. Unusual clinical and histopathologic findings in ocular sarcoidosis. Br J Opthalmol 1983:67:8-16.  Back to cited text no. 4
    
5.
Cornblath WT, Elner V, Rolfe M. Extraocular muscle involvement in sarcoidosis. Ophthalmology 1993:100:501-5.  Back to cited text no. 5
    
6.
Rootman J. Pathophysiologic patterns of orbital disease. In: Diseases of the Orbit- A Multidisciplinary Approach. Philadelphia: J.B. Lippincott, 1988. pp 51-67.  Back to cited text no. 6
    
7.
Stjernberg N, Cajander S, Truedsson H, Uddenfeldt P. Muscle invovlement in sarcoidosis. Acta Med Scana 1981;209:213-16.  Back to cited text no. 7
[PUBMED]    
8.
Fujii H, Fujisada H, Kondo T, Takahasi T, Okada S. Orbital pseudotumour: Histopathological classification and treatment. Ophthalmologica 1985;190:230-42.  Back to cited text no. 8
    
9.
Mombaerts I, Schlingemann RO, Goldsehmeding R, Koorneef L, Idiopathic granulomatous inflammation of the orbit. Ophthalmology 1996;103:2135-41.  Back to cited text no. 9
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]


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