Idiopathic Orbital Inflammatory Syndrome- A Case Series
Main Author: Aala Sabati
Abu Dhabi, United Arab Emirates
Sheikh Khalifa Medical City
Background(s): Idiopathic orbital inflammatory disease is an inflammatory eye disease causing non granulomatous changes on histopathology, frequently mistaken for pre-septal or orbital cellulitis. While it is commonly encountered among adult age group it is quite rare among pediatrics.
Method(s): To report a case series of 2 patients who presented with bilateral eye swelling.
Result(s): An 11-year-old previously healthy girl and a 12-year-old girl known to have type 1 diabetes mellitus were both evaluated for bilateral eye swelling. On examination, conjunctivitis and restriction of the eye movement were evident in both patients. Laboratory work up showed negative inflammatory markers in the first patient while they were elevated in the second patient, furthermore both patients had negative infectious work up including Tuberculosis, Syphilis and HIV and ACE levels. Both patients were treated initially with antibiotics. Moreover; MRI orbit was ordered illustrating bilateral myositis in the first patient and diffuse lacrimal gland enlargement in the second patient raising the suspicion of idiopathic orbital inflammatory disease, however further investigations weer required to establish a diagnosis for the second patient hence she underwent a biopsy of the lacrimal gland where the histopathology report showed severe active chronic inflammation associated with stromal fibrosis and no granulomas. Treatment with Corticosteroids was commenced for both patients resulting in significant clinical improvement and further confirming the diagnosis, however due to the ongoing inflammation the second patient was started on Methotrexate. Both patients have regular follow up with both Rheumatology and Ophthalmology services.
Conclusion(s): Idiopathic orbital inflammatory disease is a rare entity among pediatric age group. Yet, an early diagnosis through the joint care with the ophthalmology team and prompt treatment are vital to prevent complications such as retinal and extraocular muscle damage.