Evaluation of classification systems for nonspecific idiopathic orbital inflammation.

PURPOSE: To systematically analyze existing classification systems for idiopathic orbital inflammation (IOI) and propose and test a new best practice classification system. METHODS: A systematic literature search was conducted to find all studies that described and applied a classification system for IOI. Classification categories used in more than two studies were extracted, and criteria for these categories were defined using common descriptors. Using patient data, these newly defined classification systems were evaluated. Reliability was tested by inter- and intrarater agreement of two raters and distinction tested by evaluating clinical differences among classification categories. Feasibility, face validity, and content validity were qualitatively tested. RESULTS: The most frequently encountered IOI classification systems were based on onset (acute, chronic), histopathology (classic, granulomatous, sclerosing), or localization (diffuse, extraocular muscle, lacrimal gland, sclera, optic nerve). Systems based on histopathology and localization showed good reliability (κ values range 0.74-0.89), were easy to apply (feasibility), and described the biologic process (face validity). Because of static sampling, histopathology-based systems had moderate content validity and moderate distinction between classification categories. Being a static measure, localization had moderate content validity, but good distinction. It was found that content validity was improved by combining histopathology and localization into a two-dimensional classification system. CONCLUSIONS: This combined histopathology and localization-based classification system provides a repeatable, easy to use, plausible, and complete classification system that can be used to further advance the research of IOI.

Systemic immunoglobulin G4 (IgG4) disease and idiopathic orbital inflammation; removing 'idiopathic' from the nomenclature?

The discovery of systemic disease related to raised tissue and serum immunoglobulin G4 (IgG4) is changing diagnostic and therapeutic practice in many medical specialties. Orbital inflammation remains a diverse and heterogeneous group of disorders that can pose a diagnostic and therapeutic challenge, but with improved understanding and corresponding diagnostic advances the previously expansive group of idiopathies is reducing. The recent discovery that IgG4 has a causative role in a subtype of, what is currently termed, idiopathic orbital inflammation is encouraging. The term 'idiopathic' can now be removed from the nomenclature for another subtype of orbital inflammation. IgG4 disease should be especially considered in patients with a bilateral dacryoadenitis and systemic features (eg, lung and gastrointestinal involvement). However, reports are emerging suggesting that IgG4 may be responsible for more diverse disease subtypes. The relationship between IgG4-related disease and lymphoma remains unknown but vigilance is required.

Survey of 1264 patients with orbital tumors and simulating lesions: The 2002 Montgomery Lecture, part 1.

OBJECTIVE: To determine the incidence of specific orbital tumors based on patients referred to an ocular oncology center. DESIGN: Retrospective, observational case series. PARTICIPANTS: A total of 1264 consecutive patients referred to an ocular oncology service because of space-occupying orbital lesions. METHODS: A retrospective chart review was carried out for 1264 consecutive patients referred for a suspected orbital mass over a 30-year period. The lesions were grouped into general categories, as shown in "Results." The specific diagnosis in each case was based on clinical findings, computed tomography scan results, magnetic resonance imaging results, and histopathologic analysis results, when available. The number and percentage of benign and malignant tumors per age group also was determined. MAIN OUTCOME MEASURES: The incidence of orbital tumors and pseudotumors. RESULTS: Among 1264 consecutive patients, the number and percentage of lesions in each general category were as follows: cystic, 70 cases (6%); vasculogenic, 213 cases (17%); peripheral nerve lesions, 23 (2%); optic nerve and meningeal tumors, 105 (8%); fibrocytic lesions, 13 (1%); osseous and fibro-osseous tumors, 21 (2%); cartilaginous lesions, 1 (<1%); lipocytic and myxoid lesions, 64 (5%); myogenic tumors, 36 (3%); lacrimal gland lesions, 114 (9%); primary melanocytic lesions, 11 (<1%); metastatic tumors, 91 (7%); lymphoma and leukemia lesions, 130 (10%); secondary orbital tumors, 142 (11%); histiocytic lesions, 17 (1%); thyroid-related orbitopathy, 67 cases (5%); other inflammatory lesions, 133 cases (11%); and miscellaneous other lesions, 13 (1%). The most common diagnoses were: lymphoid tumor (139 cases;11%), idiopathic orbital inflammation (135 cases; 11%), cavernous hemangioma (77 cases; 6%), lymphangioma (54 cases; 4%), meningioma (53 cases; 4%), optic nerve glioma (48 cases; 4%), metastatic breast cancer (44 cases;4%), orbital extension of uveal melanoma (41 cases; 3%), capillary hemangioma (36 cases;3%), rhabdomyosarcoma (35 cases; 3%), dermolipoma (31 cases; 3%), herniated orbital fat (30 cases; 2%), dermoid cyst (26 cases; 2%), varix (26 cases; 2%), dacryops (19 cases; 2%), and other less common lesions. Of the 1264 lesions, 810 (64%) were benign and 454 (36%) were malignant. The percentage of malignant lesions was 20% in children (age range, 0-18 years), 27% in young adults and middle-aged patients (age range, 19-59 years), and 58% in older patents (age range, 60-92 years). Rhabdomyosarcoma was the most common malignancy in children, representing 3% of all orbital masses, and lymphoma was the most common malignancy in older patients, representing 10% of cases. CONCLUSIONS: A variety of tumors and pseudotumors can involve the orbit. In this series of 1264 lesions, 64% were benign and 36% were malignant. The percentage of malignant tumors increased with age, with malignancies being common in older patients because of the higher incidence of lymphoma and metastasis in the elderly.

Enfermedad inflamatoria idiopática de la órbita: (pseudotumor orbitario) / Idiopathic orbital inflammatory disease: (orbitary pseudotumor)

El propósito de este estudio fue la revisión de nuestra experiencia clínica con 24 casos observados de enfermedad inflamatoria idiopática de la órbita entre 1980 y 1996, el cuadro clínico de los pacientes fue evaluado retrospectivamente en la Unidad de Neuro-Oftalmología del Hospital Vargas de Caracas. Pacientes con promedio de edades desde 13 a 76 (media 42,66) años. Los tres signos principales más comunes fueron: Proptosis (74,99 por ciento), Dolor periorbitario (58,33 por ciento), Diplopía (54,16 por ciento). El diagnóstico fue realizado por clínica y estudios complementarios. Patrones imagenológicos fueron observados en 10 casos, siendo la enfermedad inflamatoria difusa la más común, segudo por el Síndrome Tolosa Hunt (3 casos). Dacrioadenitis (3 casos) y periescleritis (1 caso). La respuesta a tratamiento fue conocida para 18 de los 24 casos clasificados imagenológicamente. El tratamiento con altas dosis de corticosteroides fue bueno en 11 de 24 (45,83 por ciento) de los casos. Dos casos resistentes a corticosteroides fueron tratados con radioterapia. Un caso resistente respondió a ciclofosfamida. Tres casos adicionales tuvieron remisión espontánea. Sin embargo, 16 de 24 pacientes (66,67 por ciento) tuvieron una eventual buen resulatdo. En base a estos resultados recomendamos el uso de corticosteroides como tratamiento inicial en pacientes con EIIO

Orbital pseudotumor syndromes.

The disorder known as orbital pseudotumor and its related syndromes of myositis and dacryoadenitis continue to be a source of controversy in terms of histopathologic classification and approach to diagnosis and management. This review references recent papers regarding these issues.