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A previously healthy 12 year old boy was referred to our unit in May 2000, with a history of persistent ocular pain and recurrent diplopia. The first disease manifestation had started three years before, when the patient suddenly presented with diplopia and painful periorbital and eyelid oedema. Limited abduction of the right eye was present. The treating ophthalmologist, after a thorough investigation that excluded brain tumours, orbital masses, and myasthenia gravis, prescribed naproxen (20 mg/kg/day) and systemic corticosteroid treatment (prednisone 1 mg/kg/day, tapered and withdrawn after 15 days); symptoms recovered completely in two weeks. A first magnetic resonance imaging (MRI) scan of the orbit had shown first degree exophthalmus of the right eye and oedema and thickening of the right rectus lateralis muscle (fig 1). Since then the boy had many episodes of ocular pain and diplopia, lasting from two to four weeks, affecting both eyes or alternatively the right and the left, at intervals of two to three months. No sequelae were detected after each relapse.
During the last relapse in October 1999, naproxen and high dose oral corticosteroid treatment (prednisone 2 mg/kg/day) were required to control the disease activity, which subsided over a period of two months. After a short period of wellbeing, the disease flared up again, and recurrence of orbital pain and diplopia was observed when steroids were reduced to 0.5 mg/kg/day. The boy was then admitted to our unit. He appeared well, with no constitutional symptoms. Ocular examination showed mild right exophthalmus and limited motion of both eyes.
Laboratory tests including muscle enzymes (alanine aminotransferase, aspartate aminotransferase, creatine kinase, lactate dehydrogenase, aldolase) values, complement levels, and thyroid function were all within the normal range. Serological tests were negative for viral and bacterial infections, and antibodies against Borrelia burgdorferi were absent. Autoantibodies (antinuclear antibodies, anti-dsDNA, anticardiolipin, antiextractable nuclear antigens, perinuclear antinutrophil cytoplasmic antibodies) were undetectable. Other markers that are considered measures of disease activity in juvenile inflammatory myopathies were evaluated: factor VIII related antigen levels were raised, while neopterin levels and the number of circulating B lymphocytes (CD19 positive cells) were normal. Electrocardiography and two dimensional echocardiography excluded a concurrent myocarditis. On the basis of clinical manifestations and immunological parameters systemic lupus erythematosus (SLE), scleroderma (Scl), Crohn's disease, and thyroiditis were excluded. Orbital MRI showed significant oedema and thickening of the left extrinsic and of the right medial rectus muscles. Electromyography showed increased insertional activity, fibrillations, and positive sharp waves. Ocular myositis was diagnosed. The oral prednisone dose was raised to 30 mg/day, and rapidly tapered after improvement of signs and symptoms. In November 2000, cyclosporin (3 mg/kg/day) was introduced; no relapse of the ocular findings has been seen so far, and prednisone has been progressively reduced to the current dose of 5 mg/day.
The group of idiopathic inflammatory myopathies encompasses a variety of common and uncommon syndromes. The uncommon variants of myositis include orbital myositis, a condition that is rare in adults and even rarer in children.1–4 Orbital muscle inflammation may be seen in association with other autoimmune diseases, such as SLE, Scl, giant cell myocarditis, and Crohn's disease. Primary conditions that it is important to distinguish from orbital myositis include thyroid eye disease; ocular myopathies, such as mitochondrial disorders and ocular dystrophies; and orbital pseudotumours. Cellulitis, neoplasms, arteriovenous malformations, and cavernous sinus thrombosis are also included in the differential diagnosis.
Orbital myositis implies orbital inflammation confined to one or more of the extraocular muscles. MRI shows muscle oedema of the affected muscle(s), and is useful for monitoring disease activity. Non-steroidal anti-inflammatory drugs are recommended as first line treatment, but systemic steroids are required in most cases. When steroids fail to control muscle inflammation, methotrexate and cyclosporin have been used with success.5 In our patient, cyclosporin was successful as a steroid sparing agent, because a rapid recurrence of symptoms had always occurred in the past when the corticosteroid dose was reduced, and at present, after six months of cyclosporin treatment, the boy is still asymptomatic and receiving a low dose of steroids.
Despite the rarity of this disorder, our case suggests that diplopia in a child requires rapid and extensive investigation that must include isolated ocular myositis in the differential diagnosis.