Orbital pseudotumor in children: the description of a pediatric case

Background: Idiopathic orbital inflammation also known as orbital pseudotumor, is a diagnosis of exclusion into a wide range of inflammatory lesions of the orbit including infection, systemic inflammatory conditions, foreign body, orbital myositis, Graves’ orbitopathy and neoplasms. Aim and Methods : To describe a case of orbital unilateral pseudotumor in a child presenting to our DepartmentResults: A 11-years-old girl of egyptian origin was admitted to our Department reporting eye, pain, headache, eyelid edema and periorbital swelling. Moreover she presented proptosis with occasional abdominal pain. She presented the first episodes 4 years before in Egypt with periorbital and palpebral oedema, hyperaemic skin of right eye; recurrent relapses occurred -every 3 or 4 months-with headache. She was treated with corticosteroids for 3 monthswith complete recovery. At our first examination the girl showed sideropenic microcitic anemia and high reactive count protein (RCP), and erythrocyte sedimentation rate (ESR). ASCA, ENA and anti beta2 GPI IgM were faint positive while others immunological parametres were within the normal range (ie :lymphocytes subpopulations, serum immunoglobulin). Brain and head magnetic resonance imaging showed thickening of the lateral rectus muscle and edema of infraorbital fat of the right eye, confirming the diagnosis of pseudotumor. She started treatment with Cyclosporine A at a dosage of 200 mg/day (4.5 mg/kg) and prednisone (1.5 mg/Kg/day) in agreement with of ophtalmologist. The patient showed a good clinical response to the therapy documented by the orbital ultrasound and ocular examination, with partial improvement of inflammation index. Conclusion Orbital pseudotumor is rare in pediatric age and differential diagnosis is necessary. Our case remains under review and other studies are necessary to characterize the systemic manifestations that could be linked to idiopathic orbital pseudotumor.