Clinical characteristics, histopathology, and treatment outcomes in adult and pediatric patients with nonspecific orbital inflammation

Abstract

PurposeTo evaluate the clinical characteristics, histopathology, and treatment outcomes in adult and pediatric patients with nonspecific orbital inflammation (NSOI).MethodsThis retrospective study evaluates 76 patients with NSOI. The patients were categorized in 9 groups according to the site of involvement and histopathology results. These groups included: anterior involvement, dacryoadenitis, myositis, perineural involvement, acute fat involvement, focal mass, orbital apex involvement, diffuse sclerosing form, and multiple tissue involvement. The course of the disease was categorized as acute, subacute, or chronic. The cases with symptom duration of less than 1 week were classified as acute, 1 week to 1 month as subacute, and more than 1 month as chronic.Results36 (47.4%) patients were males. The mean age was 41.68 ± 17.62 (6–75) years. The most common signs and symptoms were periorbital pain, periorbital edema, decreased ocular movements or diplopia, and conjunctival injection. The most common group was dacryoadenitis in 29 (38.1%) cases. The most common form of disease was the acute involvement (50% of patients). Most of the patients were treated by oral corticosteroids. Duration of follow-up was 7.17 ± 6.26 months. Recurrence occurred in 9 (11.8%) of patients during the follow-up period.ConclusionsThis study presents a new categorization in which multiple tissue involvements were separated. Some of the NSOI features differ between adults and children. In most patients, treatment especially with corticosteroids, resolves the clinical findings.

Orbital Myositis Mimicking Status Migrainosus

“…The most sensitive diagnostic tool to diagnose orbital myositis is orbital MRI. Typical findings of orbital myositis include signal enhancement and thickening in the involved extraocular muscles, including the myo-tendinous junction on fat-saturated T2-weighted images, as shown in this patient [6].…”

“…In addition to its rarity, pediatric orbital myositis is a challenging disease to diagnose because of its constitutional symptoms, bilaterality, and recurrence [4,5]. Because other ocular presentations such as proptosis, conjunctival injection, chemosis, periorbital area, and diplopia can be absent in pediatric patients, it is easy for physicians to misdiagnose orbital myositis as primary headache at the beginning of the disease course [6]. The differential diagnosis of orbital myositis includes orbital infections, thyroid-associated orbitopathy, lymphoma, systemic lupus erythematosus, myasthenia gravis, diabetes, immunoglobulin G4-related ophthalmic disease, Tolo-sa-Hunt syndrome, ophthalmoplegic migraine, optic neuritis, and carotid cavernous fistula [7][8][9].…”

Nonspecific orbital inflammation and thyroid eye disease, a rare comorbidity: report of two cases and review of literature

“…Described for the first time in 1905, NSOI refers to orbital inflammation without any specific local or systemic underlying pathologies. It is diagnosed if other similar conditions are ruled out including TED, orbital lymphoproliferative diseases, and other systemic inflammations involving the orbit [ 11 , 12 ].…”

“…This finding is not usually observed in NSOI. When extraocular muscles are involved, tendons are usually spared in TED but not in NSOI [ 2 , 11 , 12 ]. The presence of orbital masses are highly associated with NSOI or other orbital malignancies but not with TED [ 4 , 11 – 13 ].…”

“…Corticosteroids are the first-line treatment for the active phase of NSOI and TED [ 2 , 4 ]. Except for the sclerosing form, other types of NSOI dramatically respond to intravenous or oral steroids, and some researchers believe that the response rate to corticosteroids is a promising way to differentiate between TED and NSOI [ 11 , 12 ]. The molecular mechanism of action in corticosteroid therapy in these patients is not completely known yet.…”