A 10-year-old girl with juvenile idiopathic arthritis (JIA) was followed in the ophthalmology clinic for 2 years with a diagnosis of episcleritis after recurrent episodes of a unilateral painful eye with left lateral scleral injection. She denied having diplopia, photophobia, headache, or visual loss. Examination revealed a normal fundus and no signs of uveitis. She was treated repeatedly with topical steroids, and each attack settled within a few days. She then presented with a proptosed, red, painful left eye associated with diplopia. Examination revealed temporal scleral injection, proptosis of 7 mm with no signs of uveitis, and a normal fundus. After conservative treatment, her symptoms resolved by day 3. Full blood count, vasculitic screen, and serum ACE were normal. The MRI scan revealed a serpiginous soft tissue structure medial to the left lateral rectus muscle (Figure). A diagnosis of left orbital varices was made. Episcleritis and scleritis are exceptionally rare in definite cases of JIA. Their appearance should initiate a reexamination of the original diagnosis to one more likely to be associated with episcleritis. Arthritis associated with systemic vasculitis, sarcoidosis, or inflammatory bowel disease1Read R.W. Weiss A.H. Sherry D.D. Episcleritis in childhood.Ophthalmology. 1999; 106: 2377-2379Abstract Full Text Full Text PDF PubMed Scopus (24) Google Scholar may resemble polyarticular JIA with extraarticular features developing several years after the onset of arthritis. These conditions may present with episcleritis or scleritis. Orbital varices usually are seen in children as a dark blue swelling in the superomedial part of the orbit, orbital hemorrhage, proptosis,2Wright J.E. Sullivan T.J. Garner A. Wulc A.E. Moseley I.F. Orbital venous anomalies.Ophthalmology. 1997; 104: 905-913Abstract Full Text PDF PubMed Scopus (134) Google Scholar or intermittent exophthalmos that can be brought on by bending or by the Valsalva maneuver.3Yazici B. Yazici Z. Gelisken O. An unusual case: bilateral orbital varices.Acta Ophthalmol Scand. 1999; 77: 453-455Crossref PubMed Scopus (13) Google Scholar Our patient appeared to have intermittent dilation of the episcleral varix in the absence of noticeable proptosis or exacerbation on the Valsalva maneuver for a few years before symptomatic proptosis occurred. Delays in diagnosis were also compounded by the rare coincidence of this orbital tumor with polyarticular JIA. The difficulties of imaging of low-flow lesions are also exemplified by this case, in which the acute CT scan showed an indistinct soft tissue mass. Despite initial MRI findings, MRI 3 months after acute proptosis did not show varices on venography. Color Doppler ultrasound and orbital venography, however, have been useful.3Yazici B. Yazici Z. Gelisken O. An unusual case: bilateral orbital varices.Acta Ophthalmol Scand. 1999; 77: 453-455Crossref PubMed Scopus (13) Google Scholar Conservative treatment is the rule, except when complications have occurred,4Sales M.J. Frise P. Roux F.X. Caumon C. Orbital varices: apropos of a case.Bull Soc Ophtalmol Fr. 1989; 89: 1287-1291PubMed Google Scholar as surgery can be complicated and hazardous. It is important to consider the more unusual and occasionally more serious differential diagnoses of painful red eyes in children with systemic disease. The atypical presentation in our case may have delayed diagnosis. On clinical suspicion of a retrobulbar mass, ophthalmologic review and imaging should be obtained immediately.
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