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Jordan Spindle, Marco Pellegrini, Todd Shepler, Bita Esmaeli, Edward Wladis, Elena Piozzi, Carol Shields, Roman Shinder; Pediatric Idiopathic Orbital Inflammation. Invest. Ophthalmol. Vis. Sci. 2013;54(15):5666.
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Idiopathic orbital inflammation (IOI) typically presents in adults with acute orbital signs such as pain, proptosis, ocular injection, diplopia, periorbital edema, and chemosis. IOI in the pediatric population is rare with approximately 70 reported cases, and differs from IOI in adults in that affected children more often have bilateral disease, as well as constitutional symptoms. We report the clinical presentation, serology, radiography, histopathology, and treatment outcome of 24 children with IOI.
Records of 24 patients 18 years and younger with IOI were reviewed.
6 males and 18 females had a median age of 11 years (range 2-18, Table 1). 19 patients were found to have primary IOI, while 5 had recurrent disease with median age of diagnosis of 9.5 (range 5-16). 21 patients were found to have unilateral IOI (87%), while 3 patients had bilateral disease (13%). 13 (54%) had constitutional signs on presentation. Ocular findings included periorbital edema (75%), limited ductions with diplopia (32%), proptosis (29%), ptosis (29%), tenderness (21%), conjunctival injection (17%), dystopia (13%), decreased visual acuity (13%), and chemosis (8%). Radiographic findings included an orbital mass (54%), myositis (29%), dacryoadenitis (29%), extraorbital extension (13%) , optic nerve sheath enhancement (4%), and posterior scleritis (4%). Correctly diagnosed IOI by first health care provider occurred in 8 of the children (33%), with 16 (67%) misdiagnoses including orbital cellulitis (63%), preseptal cellulitis (13%), conjunctivitis (13%), chalazion (6%), allergic reaction (6%), malignancy (6%), and retinal detachment (6%).
IOI is a rare but important cause of acute orbital syndrome in the pediatric age range. We found 13% of children to have bilateral disease, and 54% to have associated constitutional signs and symptoms. The percentage of children presenting with bilateral disease in our series was less than the traditional quoted 33%. The percentage of patients presenting with constitutional symptoms in our series (54%) was consistent with past reports (53%). Clinicians should consider IOI in any child presenting with acute orbital signs, and confirm the diagnosis with serology, orbital radiography, and biopsy when warranted. A multidisciplinary team to include an ophthalmologist, pediatrician, radiologist, and rheumatologist should be recruited for prompt diagnosis and a tailored treatment plan for all children with IOI.
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