A 14-Year-Old Boy With Painful Periorbital Edema

Alexis R. Pascoe, BS; Amit R. Bhatt, MD; Kimberly G. Yen, MD; Eric J. Kim, MD; Madhuri Chilakapati, MD


July 10, 2019


Idiopathic orbital inflammation is a benign inflammatory-mediated condition of the orbit without a known systemic or local etiology.[5] The condition is typically seen in middle-aged adults and affects women more than men.[5] Despite being a rare condition in the younger population, one study[1] found that 11.5% of patients with idiopathic orbital inflammation were aged 20 years and younger, whereas another found that 6.6% of their study population included patients also aged 20 years and younger, with equal frequency of occurrence in the first and second decades.[2] Although believed to be an idiopathic condition, it has been associated with a history of otitis media, sinusitis, or upper respiratory infection,[2] and as an immune-mediated process, such systemic immunologic disorders as Crohn disease, ankylosing spondylitis, myasthenia gravis, rheumatoid arthritis, and systemic lupus erythematous have also been associated.[6]

Ptosis and proptosis are seen more commonly, with ptosis being the most common sign.[1] Idiopathic orbital inflammation in the pediatric population will more likely be bilateral with an association of a recent history of trauma.[2] On examination, intraocular findings such as iritis, eosinophilia, uveitis, and papilledema are more likely to be seen.[1,2,3] Patients may or may not complain of decreased visual acuity.[2,4] Swelling will be worse in the morning and will decrease throughout the day.[2] Earlier in the course of the disease, restrictions in motility are present, with abduction and elevation being the most severely disturbed.[2] There can be lacrimal involvement, as in this case, as well as infiltration into other extraocular structures such as the orbital fat and extraocular muscles.[3] The presence of certain symptoms allows for a localization of the inflammation. If visual acuity is impaired, one can deduce that there is involvement of the optic nerve or the posterior sclera, whereas if there is pain with movement, inflammation in the extraocular muscles is likely present.[6]

Patients might also present with headache, fever, vomiting, pharyngitis, anorexia, abdominal pain, and lethargy.[2,4]

Imaging assists with determining whether a biopsy is necessary, and also with assessing for malignancy and orbital cellulitis.[3] Biopsy of the lesion is used to confirm the diagnosis and to rule out possible granulomatous inflammation or vasculitis.[1] Histologically, it depicts eosinophils, plasma cells, and lymphocytes merged into a pleomorphic cellular infiltrate. There will be varying levels of reactive fibrosis present in the sample. If the sample has sparse inflammation compared with the presence of fibrosis, idiopathic orbital inflammation is of the sclerosing subtype.[6]

Systemic corticosteroids are considered the mainstay treatment.[3] Long-term systemic corticosteroid therapy, starting with dexamethasone, is effective.[1]

Of note, a new category of disease has gained recognition in the last few years: immunoglobulin G4 (IgG4)-related orbital inflammation (IgG4-ROI). IgG4-ROI is similar to idiopathic orbital inflammation, in that the mechanism of disease relies on fibroinflammatory lesions. The key distinction is that IgG4-ROI lesions consists of IgG4+ plasma cells. The treatment of IgG4-ROI is also corticosteroid therapy.[7] Because of the similarities, being aware of this condition is beneficial in establishing the most accurate diagnosis.

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