Extranodal Rosai-Dorfman Disease of the Orbit: Clinical Features of 8 Cases.

PURPOSE: To report on the presentation, radiography, histology, and treatment of 8 cases of extranodal Rosai-Dorfman disease involving the orbit. METHODS: Multicenter retrospective case series. RESULTS: Five males and 3 females had a median age of 10 years (range 2-78 years). Presenting signs and symptoms included proptosis, periorbital pain, palpable mass, blepharoptosis, decreased vision, diplopia, impaired extraocular motility, and afferent pupillary defect. Four patients had bilateral orbital disease, while 4 had unilateral disease. Six cases were extraconal, 1 was intraconal, and 1 was both intra- and extra-conal. Four cases had only extranodal disease without lymphadenopathy (3 of which had localized orbital disease). Diagnosis was confirmed by exam, orbital, and/or systemic radiography, and biopsy in all cases. Treatment strategies included excision or debulking, systemic corticosteroids, chemotherapy, radiotherapy, observation or a combination thereof. At last follow up, 4 patients were disease free, while 4 had residual improved disease. CONCLUSIONS: Rosai-Dorfman disease of the orbit is a rare clinical entity. Purely extranodal disease is rare, with isolated orbital disease being exceedingly rare. This study is unique in that 4 of 8 patients had strictly isolated extranodal disease of the orbit. A large majority of the cases had disease in the extraconal space, contrasting with previous reports. In addition, lacrimal gland disease, particularly bilateral involvement, was prominent in the current study. Although there is no consensus on treatment, surgical excision should be attempted if plausible in symptomatic patients especially if the orbit represents a localized site of disease.

Atypical idiopathic orbital inflammation in a young girl.

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.

Theories of upper eyelid blepharoplasty.

This article provides an overview of upper eyelid anatomy and racial variations, functional indications in upper lid blepharoplasty, and various surgical techniques.

Noninvasive techniques in periorbital rejuvenation.

The combination of noninvasive treatments in the periorbital area can be used to achieve dramatic and long-lasting results. New technologies and current therapies may supplement or even delay traditional surgical procedures.

Combination therapies in oculofacial rejuvenation.

The oculo-facial surgeon's quest for non-invasive procedures is a response to increasing patient demands for enhanced results without any downtime. Technology has obliged us with multiple injectable and light therapeutic modalities that tighten skin, relax and fill in wrinkles, and improve skin dyspigmentation and texture. When these techniques are combined, the results are superior to the use of individual therapies alone. The foundation for combination therapies are: botulinum toxin, filling agents, chemical peels, intense pulsed light, non-ablative and ablative lasers.