Purpose
To describe a series of cases of orbital inflammatory disorders in children.
Design
Retrospective case series.
Methods
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.
Results
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.
Conclusions
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.
Orbital inflammatory disorders are a significant cause of severe visual loss and oculomotor dysfunction. Inflammatory syndromes of the orbit can be idiopathic, such as in idiopathic orbital inflammatory disease, orbital myositis, and dacryoadenitis. Specific systemic diseases, including sarcoidosis, Graves disease, Wegener granulomatosis, and histiocytosis, also should be considered in patients with signs and symptoms of orbital inflammation at presentation. Idiopathic orbital inflammation is the third most common cause of unilateral exophthalmos in adults, after Graves disease and lymphoproliferative diseases. Studies have reported this condition to be the cause of 4.7% to 17.6% of all orbital disorders and to be responsible for 5% to 20% of all orbital biopsies. An association has been found between idiopathic orbital inflammation and various infectious processes, such as upper respiratory tract infections and flu-like viral illness, although the exact nature of these associations remains unclear.
The current literature states that only 6% to 17% of the orbital inflammatory disorders reported occur in pediatric patients, with most overall cases described being in middle-aged adults. There are relatively few studies describing orbital inflammatory disorders and their association with systemic diseases in pediatric patients. The purpose of our study was to increase our understanding of the signs, symptoms, and association with systemic disease of orbital inflammatory conditions in the pediatric population.
Methods
A computer search was performed to identify all pediatric patients from 0 to 18 years of age with an orbital inflammatory disorder who had been seen by a pediatric ophthalmologist at our institution between September 1, 2002, and December 31, 2008. We searched for patients who were seen for orbital inflammatory disease, orbital myositis, proptosis, dacryoadenitis, eyelid inflammation, posterior scleritis, and orbital inflammation. Cases of orbital and preseptal cellulitis, tumors of the orbit, dacryocystitis, and histiocytosis were excluded. The charts were reviewed for age at presentation, final diagnosis, treatment, workup and evaluation, need for biopsy and biopsy results, and involvement of lacrimal gland and muscles. Criteria for orbital biopsy were refractory or rebound inflammation when treated with corticosteroids or if a fluid pocket was seen on imaging that would raise concern for an abscess.
Results
Electronic medical record review revealed 12 patients with orbital inflammatory disorders that had been seen between September 1, 2002, and December 31, 2008. There were 6 males and 6 females, with age at presentation ranging from 1.3 to 16.2 years (mean, 11.9 years; standard deviation, 4.2 years).
The presenting orbital symptoms were enlargement of lacrimal gland (66.7%), proptosis (50%), pain on eye movement (50%), eye movement abnormality (50%), ptosis (25%), diplopia (25%), water eyes (16.7%), chemosis (16.7%), swollen eyelids (16.7%), and blurry vision (8.3%). The most common presentation, lacrimal gland enlargement, was bilateral in 3 cases.
Half of our patients had systemic symptoms at presentation, the most common of which was fever, present in 33% of the patients. Other presenting systemic symptoms were upper respiratory infection (16.7%), weight loss (16.7%), cough (8.3%), migratory joint pain (8.3%), myalgias (8.3%), headaches (8.3%), dry skin (8.3%), and emesis (8.3%). A comprehensive list of systemic symptoms corresponding to cause is presented in the Table . Four patients were diagnosed as having a systemic cause and 2 of these patients had associated systemic symptoms at presentation. Ten patients were treated with systemic corticosteroids, and all but 1 (Case 12) responded.
| Age (yrs) | Case No. | Diagnosis | Fever | URI | Weight Loss | Cough | Migratory Joint Pain | Myalgia | Headache | Dry Skin | Emesis | Bilateral | Systemic Diagnosis |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1.3 | 1 | Myositis/Brown syndrome | — | ||||||||||
| 6.3 | 2 | Dacryoadenitis | x | — | |||||||||
| 11.1 | 3 | Dacryoadenitis | x | x | — | ||||||||
| 11.3 | 4 | Dacryoadenitis | x | x | x | x | Sarcoidosis | ||||||
| 11.9 | 5 | Dacryoadenitis | — | ||||||||||
| 12.8 | 6 | Dacryoadenitis | — | ||||||||||
| 13.9 | 7 | Myositis | x | x | Group A Streptococcus | ||||||||
| 14.1 | 8 | Dacryoadenitis | x | Chlamydia (presumed) | |||||||||
| 14.2 | 9 | Orbital pseudotumor | x | — | |||||||||
| 14.3 | 10 | Dacryoadenitis | x | Sarcoidosis | |||||||||
| 15.1 | 11 | Dacryoadenitis | — | ||||||||||
| 16.2 | 12 | Orbital pseudotumor | x | x | x | x | x | — |
The patients diagnosed with dacryoadenitis (n = 6) who did not have sarcoidosis as a cause all had lacrimal gland enlargement at presentation, whereas some had extraocular limitation (n = 2), pain on eye movement (n = 2), proptosis (n = 3), watery eyes (n = 1), or a combination thereof at presentation. Of these 6 patients, 3 were treated with oral corticosteroids alone (Cases 2, 6, and 11), 1 initially was treated with clindamycin because of concern of orbital cellulitis by the emergency room physician who first saw the patient (Case 5), 1 was treated with both antibiotics and corticosteroids (Case 3), and 1 was treated with doxycycline alone for a presumed Chlamydia infection based on clinical presentation and personal history, not proven with cultures or polymerase chain reaction (Case 8). The 4 patients who received corticosteroids had full resolution of their dacryoadenitis. The outcomes are unknown for the patient who received clindamycin and the patient treated with doxycycline alone because they did not return for follow-up after being seen in the eye clinic.
Of the patients ultimately diagnosed with sarcoidosis (n = 2), one was a 14 year-old boy with primarily renal involvement and no systemic symptoms (Case 10); the other was an 11-year-old girl with pulmonary disease (lung biopsy was diagnostic) with cough, fever, and weight loss at presentation (Case 4). Both cases had bilateral dacryoadenitis at presentation.
Two patients were diagnosed with orbital myositis. One was believed to be secondary to an upper respiratory infection resulting from group A Streptococcus (Case 7), and the other had idiopathic Brown syndrome at presentation (Case 1).
Diffuse orbital inflammation was the diagnosis in 2 patients. Of these, one was treated with oral corticosteroids and methotrexate but did not respond. She was lost to follow-up after she returned to Trinidad (Case 12). The other patient had complete resolution after treatment with oral corticosteroids alone (Case 3).
Four patients underwent a biopsy of the involved muscle, the orbit, or the lacrimal gland. Biopsy demonstrated lymphocytic infiltrate in all cases. One patient required kidney biopsy to confirm the diagnosis of sarcoidosis (Case 10), and one had received a previous lung biopsy (Case 4). All patients underwent orbital imaging except for Case 5, who had unilateral dacryoadenitis with no pain or other orbital signs.
Disease recurrence was documented in Cases 1, 7, and 10 diagnosed respectively with orbital inflammatory disease with associated Brown syndrome and ptosis, myositis secondary to group A Streptococcus , and sarcoidosis.
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