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      Idiopathic Orbital Myositis in a 9-Year-Old Girl: A Case Report

      letter
      1 , 1 , *
      Iranian Journal of Pediatrics
      Kowsar
      Myositis, Inflammatory, Orbit

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          Abstract

          Dear Editor, Non-specific orbital inflamation (NSOI) syndrome is a non-granulomatous, non-infectious and non-neoplastic orbital disease with unknown etiology. Despite being rare in children, NSOI accounts for up to 6 - 17% of all pediatric orbital inflammatory diseases and several conditions such as cellulitis, rhabdomyosarcoma and leukemia are considered in the differential diagnosis (1). The most frequent subtype of NSOI is orbital myositis (29%), followed by dacryoadenitis with myositis (8%), orbital apex inflammation (9%) and others (22%) (2). It clinically presents as a mass in the eyelids, restricted ocular motility, eyelid edema and increased orbital pressure. While in adults it manifests itself more often as proptosis, it more frequently presents in children as ptosis (3). Its exact cause is unknown, however, immunologic mechanisms have been addressed (4). While a definitive diagnosis of the disease is established by means of the orbital tissue biopsies, in most cases the disease can be diagnosed by clinical and radiological examinations (5). We present a case with unusual presentation of IOIP admitted to our clinic with sudden onset of diplopia and reduction of outward gaze in the left eye. A 9-year-old girl presented to the pediatric clinic with a nonspecific headache, eye pain and restriction of left outward gaze and diplopia. Despite systemic antibiotic therapy following a preliminary diagnosis of afebrile cellulitis, the progression of complaints were noted and our clinic was consulted. Visual acuity was 20/20 in both eyes, biomicroscopic and fundus examination was normal. The exophthalmometry readings of the right and left eye were 15 mm and 18 mm, respectively. Left ocular eye movement was limited in outward gaze (Figure 1). Worth 4 dot test result was determined to be diplopia in far and near evaluation. Laboratory studies were normal. On the orbital MRI, taken SE T1-weighted images following gadopentetate dimeglumine (Gd-DTPA) on the axial and coronal plane, left medial rectus muscle was normal but increased signal intensity on T2W sequences was noted, and the presence of marked enhancement of contrast material following intravenous administration of contrast material (IVCM) (Figure 2). The imaging of the medial rectus muscle is typical of myositis and this typical image is used for differentiating NSOI from thyroid ophthalmopathy. The systemic steroid treatment (oral prednisone 1 mg/kg tapered slowly for one month) was initiated with a preliminary diagnosis of NSOI. Diplopia and ocular motility restriction were not present a week after control examination. MRI showed regression of the isolated myositis in the medial rectus. The definitive diagnosis of orbital subacute myosistis was established. Figure 1. Limitation of Left Ocular Eye Movement Figure 2. A, T1; B, T2 Childhood NSOI differs from NSOI in adult age. Whilst the disease seems to be one-sided in adult age, it is bilateral in childhood and recurrence rate is more common. In addition, in childhood NSOI, papilledema and iritis can be encountered (4, 5). Orbital myositis is a non-specific, localized inflammatory orbital process that may involve extraocular muscles. While adult and childhood NSOI cases have marked symptoms, including local orbital fındings related to extraocular muscles, lacrimal gland, anterior orbit, orbital apex and diffuse inflammation, patients with orbital myositis may have only ocular motility restrictions and diplopia. In addition, these cases may present without the clinical picture proptosis and ptosis (3, 6). A variety of afebrile patients diagnosed with orbital cellulites go through a process of misdiagnosis not responding to antibiotic therapy (7). Moreover, some of these cases may be considered as the Graves ophthalmopathy; however, differential diagnosis is established with CT. No involvement of the tendons of the muscles occur in Graves patients, however, both muscle and tendon involvement is observed in orbital myositis cases. The acute onset of pain is more characteristic of orbital myositis. While in Graves patients the most common affected muscle is inferior rectus, in isolated orbital myositis patients the most affected muscle is medial rectus. It is important to confirm differential diagnosis with rhabdomyosarcoma as well. In rabdomyosarcoma, there exists an orbital space-occupying extremely fast growing mass, with bone erosion. Definitive diagnosis is determined by biopsy or pathological examination following excision of total mass. The inflammation associated with other orbital processes may also improve with systemic steroid administration. But with a detailed history, physical examination, directed laboratory, and radiologic studies (MR and CT), and tissue for diagnostic studies and response to anibiotic therapy may help to come to correct diagnosis. Furthermore, patients with orbital myositis respond to systemic steroid therapy more dramatically. In conclusion, childhood orbital myositis, being usually a rarely considered diagnosis, is likely to be overlooked in childhood. Orbital myositis, a subgroup of childhood NSOI, should be included in the differential diagnosis in pediatric patients without clinically marked proptosis, ptosis, pain, fever and chemosis but with the sudden onset of ocular motility restrictions and diplopia. Thus, unnecessary use of antibiotics, unwarranted biopsies and delayed diagnoses could be avoided.

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          Most cited references7

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          Idiopathic orbital inflammation: distribution, clinical features, and treatment outcome.

          To evaluate the distribution and clinical as well as treatment outcome characteristics of idiopathic orbital inflammation with the aim of delineating a more systematic approach to diagnosis and treatment. A 10-year retrospective review of patients with idiopathic orbital inflammation treated at one institution. Ninety eyes in 65 patients (22 men and 43 women) were studied. Diagnoses were isolated dacryoadenitis (n = 21), isolated myositis (n = 19), concurrent dacryoadenitis and myositis (n = 5), orbital apex syndrome (n = 6), and idiopathic inflammation involving the preseptal region, supraorbital region, sclera, Tenon capsule, orbital fat, or optic nerve (n = 14). The mean age at presentation was 45 years. Pain and periorbital swelling were the most common clinical features and were observed in 45 (69%) and 49 (75%) patients, respectively. Seventeen patients (26%) had bilateral involvement. Biopsy was performed in 19 patients (29%) with atypical presentations or who failed to respond to the initial therapy. Patients were treated with steroids alone (n = 45), steroids and subsequent radiation therapy (n = 8), steroids and nonsteroidal anti-inflammatory agents (n = 6), nonsteroidal anti-inflammatory agents alone in mild cases (n = 2), and, rarely, radiation therapy without steroids (n = 1) or surgical debulking alone (n = 1). Of 65 patients, 41 (63%) represented treatment successes, with complete symptom relief at the time of the last follow-up, and 24 (37%) represented treatment failures, with partial or no relief of symptoms. Treatment failures were often characterized by recurrence of inflammation after a period of quiescence (58%) and unremitting, recalcitrant inflammation (38%); 1 patient ultimately required an exenteration. Systemic steroid with a slow taper has been the established first-line treatment for idiopathic orbital inflammation, but refractory cases accounted for a significant portion of treatment failures in our study, reflecting the need for a more systematic approach to the study of this multifaceted disease and for therapeutic alternatives to systemic steroids.
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            Inflammatory disorders of the orbit in childhood: a case series.

            To describe a series of cases of orbital inflammatory disorders in children. Retrospective case series. The medical records of pediatric patients diagnosed with orbital inflammation between September 1, 2002, and December 31, 2008, at Texas Children's Hospital were reviewed. Data collected included age at presentation, final diagnosis, treatment, workup and evaluation, need for biopsy and biopsy results, and involvement of lacrimal gland and muscles. Twelve cases were identified. Six cases were males and 6 were females with age at presentation ranging from 1.3 to 16.2 years (mean, 11.9 years). The most common presentation was lacrimal gland enlargement, which was bilateral in 3 cases. Other common presenting signs were proptosis, extraocular motility limitation, and pain on eye movement. Half of our patients had systemic complaints at presentation, the most common of which was fever. Four patients were diagnosed as having a systemic cause and 2 of these patients had systemic symptoms. Idiopathic orbital inflammatory conditions in children are uncommon, but can be associated with systemic conditions. Patients typically have lacrimal gland involvement, pain with eye movement, proptosis, and motility deficits at presentation. Bilateral cases may have a higher incidence of systemic disease. Copyright © 2010 Elsevier Inc. All rights reserved.
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              Idiopathic orbital inflammatory pseudotumor in Chinese children.

              To study the clinical features of idiopathic orbital inflammatory pseudotumor (IOIP) in Chinese children. Pediatric patients with IOIP seen between Jan. 1, 1978 and Dec. 31, 1999 in the Eye Hospital, Zhongshan Ophthalmic Center, Sun Yat-sen University were evaluated retrospectively. Their clinical features were compared with those of adult cases of IOIP. Of the 209 cases, 24 (11.5%) were equal to or less than 20 years old; 14 were female and 10 were male. There were 11 in the right eye, 9 in the left eye and 4 in both eyes. Palpable mass (58%), ocular motility restriction (46%), swollen eyelid (42%), proptosis (42%) and high orbital pressure (42%) were the five most common presenting signs in children with IOIP. Ptosis occurred more often in pediatric IOIP cases (38%) than in adult IOIP cases (9%) (P < 0.0003). However, compared with adult IOIP cases, pediatric patients showed less proptosis (69% vs. 42%, P = 0.0074). According to radiological and surgical findings, a local mass within the orbit was the most frequent subtype (50%), followed by dacryoadenitis (29%), myositis (8%), perineuritis (4%), eyelid pseudotumor (4%), and diffuse orbital inflammation (4%). The frequency of clinical subtypes in children was similar to that in adults. After systemic corticosteroids, surgical management and local radiotherapy the full recovery response rate was 29% and the total effective rate was 92%. Pediatric IOIP accounted for 11.5% of all IOIP patients. Pediatric cases had more sign of ptosis and less sign of proptosis than in adult IOIP. The full recovery response rate was low in children with IOIP.
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                Author and article information

                Journal
                Iran J Pediatr
                Iran J Pediatr
                10.5812/ijp
                Kowsar
                Iranian Journal of Pediatrics
                Kowsar
                2008-2142
                2008-2150
                27 June 2015
                June 2015
                : 25
                : 3
                : e371
                Affiliations
                [1 ]Istanbul Kartal Dr. Lutfi Kirdar Training and Research Hospital, Eye Clinic, Istanbul, Turkey
                Author notes
                [* ]Corresponding author: Isil Kutluturk, Istanbul Kartal Dr. Lutfi Kirdar Training and Research Hospital, Eye Clinic, Istanbul, Turkey. Tel: +90-5058611984, Fax: +90-2625266664, E-mail: slkutluturk@ 123456gmail.com
                Article
                10.5812/ijp.25(3)2015.371
                4505988
                76cd8dda-a465-4d19-8562-8fc32e947bf8
                Copyright © 2015, Growth & Development Research Center.

                This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

                History
                : 13 June 2014
                : 04 January 2015
                Categories
                Letter

                Pediatrics
                myositis,inflammatory,orbit
                Pediatrics
                myositis, inflammatory, orbit

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