Atypical Idiopathic Orbital Inflammation in a Young Girl

Abstract

A 7-year-old girl presented with chronic right periocular pain worst in abduction, edema, and proptosis with radiographic evidence of right medial rectus myositis. After a negative workup, she was diagnosed and treated for idiopathic orbital inflammation (IOI) with a brisk response to oral steroids. She returned 8 months later with left periocular pain worst in abduction, proptosis, and radiographic evidence of left lateral rectus myositis. She was treated for recurrent and alternating IOI with intravenous (IV) glucocorticoids with resolution. One year later, she recurred with similar left orbital signs and symptoms and was found to have a left inferior oblique myositis on MRI. She was treated with oral steroids and methotrexate with clinical resolution. Ten months later, she presented with left periocular pain worst on adduction and was found to have left medial rectus myositis on MRI. She was treated with IV steroids, IV immunoglobulin, and rituximab with resolution of all symptoms. She is currently maintained on methotrexate and oral glucocorticoids. The atypical pediatric case of IOI over a 4-year period is described in this study.

A Case of Idiopathic Orbital Inflammation in a Seven-Year-Old Child

“…Compared to adults, pediatric IOI is more commonly associated with bilateral involvement and constitutional signs [1,8]. Only one-third of cases in the pediatric age group have bilateral presentation [7][8][9]. Constitutional signs occur in about 50% of cases [1,8].…”

“…Children most commonly present with a palpable mass, pain, proptosis, eyelid edema, restricted extraocular movements, and increased intraocular pressure (IOP) [ 1 ]. Affected children are at risk of recurrence and may have bilateral involvement and constitutional signs, such as headache, fever, lethargy, vomiting, and abdominal pain [ 1 , 5 - 8 ].…”

“…IOI is a diagnosis of exclusion [ 8 ]. It is crucial to rule out secondary causes of orbital inflammation and proptosis in children, such as orbital cellulitis, rhabdomyosarcoma, leaking dermoid cyst, thyroid-associated orbitopathy (TAO), leukemia, lymphoma, and histiocytosis X [ 1 , 2 , 4 , 6 , 8 , 9 ].…”

“…Children usually respond well to corticosteroid therapy [ 4 ]. Biopsy is only reserved for refractory or atypical conditions [ 2 , 4 , 8 ].…”

Decreased CD3-CD16+CD56+ natural killer cell counts in children with orbital myositis: a clue to disease activity

“…3 The differential diagnosis of paediatric OM also includes rhabdomyosarcoma, thyroid eye disease, leukaemia, sarcoidosis, vasculitis, tuberculosis, histiocytosis, ruptured dermoid cyst, arteriovenous malformations and paranasal sinus mucocele. [7][8][9][10][11][12][13][14][15] OM has traditionally been a diagnosis of exclusion based on clinical and radiographic data. 7 8 The utility of biopsy in children with OM disease is unclear.…”