Idiopathic orbital inflammatory disease

Abstract

The purpose of this study was to review our clinical experience with 132 idiopathic orbital inflammation cases seen between 1971 and 1994. Clinical charts of the patients were evaluated retrospectively. Patient age ranged from 5 to 80 (mean 46.5) years. The follow-up period was between 6 to 172 (mean 42.8) months. Proptosis (82%), motility restriction (54%), visual acuity loss (38%) were the three more common presenting signs. The diagnosis was made by open biopsy in 96 cases and clinically in 36 cases. Radiologic patterns of involvement were available for 84 cases. Diffuse inflammatory disease (40 cases) was the most frequent radiologic pattern followed by myositis (21 cases) and dacryoadenitis (14 cases). Focal encapsulated mass (5 cases), Tolosa-Hunt syndrome (2 cases) perineuritis (1 case) and periscleritis (1 case) were the other types of orbital pseudotumors. The response to treatment was known for 60 of the 84 radiologically classified cases. High-dose oral corticosteroid treatment was successful in 35 out of 60 (58.3%) cases and radiotherapy in 9 out of 14 (64.3%) cases resistant to corticosteroids. One resistant case responded to cyclophosphamide and 2 cases with focal mass lesions were treated with orbitotomy. Four additional cases had spontaneous remission. Overall, 51 out of 64 patients (79.7%) had an eventual good outcome. In conclusion, we found open biopsy to be a safe and reliable procedure in orbital pseudotumors with the exclusion of acute phase cases. The success rate of treatment for this group of orbital disorders is high with the exception of certain disease patterns.

Orbital myositis: Diagnosis and management

“…Other signs and symptoms may include chemosis, visual loss, eyelid hyperemia and ptosis, optic neuropathy, retinal folds or detachment, and uveitis. OID can occur at any age, but is more common in the fourth to fi fth decades of life and seems to have no gender predilection [5,23]. In patients younger than 20 years, there is often a higher frequency of ptosis and bilaterality [28].…”

“…The most commonly reported clinical fi ndings are variable depending on the patient series and include proptosis (32%-82%), periorbital edema (42%-75%), pain (17%-69%), ocular motility restriction (46%-54%), and conjunctival congestion (33%-48%) [5,8,23,27,28]. The presence of a palpable mass and increased orbital pressure are variably identifi ed in 36% to 65% and 2% to 56% of patients, respectively [8,23,28]. Other signs and symptoms may include chemosis, visual loss, eyelid hyperemia and ptosis, optic neuropathy, retinal folds or detachment, and uveitis.…”

“…Idiopathic OID can range from a localized infl ammatory disease primarily involving the muscles (myositis), lacrimal system (dacryoadenitis), optic nerve (perineuritis), or sclera (periscleritis), to a diffuse disease affecting multiple structures of the anterior, posterior, or entire orbit [4,24,25]. Although bilateral cases may occur, most cases of idiopathic OID are unilateral [5,23,26]. Symptoms usually develop over the course of days (acute) to weeks (subacute), but may also occur insidiously over a period of months (chronic).…”

“…Idiopathic orbital myositis is in the spectrum of OID, a noninfectious infl ammatory process of the orbit without evidence of a local or systemic cause [4,5,22,23]. Following Grave's orbitopathy and lymphoproliferative disorders, OID is among the most common (up to 6.3%) of all orbital diseases [5].…”

Orbital Myositis

“…Cyclosporine (Sanchez-Roman, et al 1993), cyclophosphamide (Gunalp et al, 1996), and methotrexate (Kubota & Kano, 2007) have been use with variable results.…”