Consensus on Diagnostic Criteria of Idiopathic Orbital Inflammation Using a Modified Delphi Approach

Abstract

Importance: Current practice to diagnose idiopathic orbital inflammation (IOI) is inconsistent, leading to frequent misdiagnosis of other orbital entities, including cancer. By specifying criteria, diagnosis of orbital inflammation will be improved.

Objective: To define a set of criteria specific for the diagnosis of IOI.

Design, setting, and participants: A 3-round modified Delphi process with an expert panel was conducted from June 8, 2015, to January 25, 2016. Fifty-three orbital scientist experts, identified through membership in the Orbital Society, were invited to participate in on online survey and they scored, using 5-point Likert scales, items that are eligible as diagnostic criteria from the literature and from personal experience. The items were clustered around the anatomic subtypes of IOI: idiopathic dacryoadenitis and idiopathic orbital fat inflammation (2 nonmyositic IOIs), and idiopathic orbital myositis (myositic IOI). Items with dissensus were rescored in the second round, and all items with consensus (median, ≥4; interquartile range, ≤1) were ranked by importance in the third round.

Main outcomes and measures: Consensus on items to be included in the criteria.

Results: Of the 53 experts invited to participate, a multinational panel of 35 (66%) individuals with a mean (SD) years of experience of 31 (11) years were included. Consensus was achieved on 7 of 14 clinical and radiologic items and 5 of 7 pathologic items related to diagnosis of nonmyositic IOI, and 11 of 14 clinical and radiologic items and 1 of 5 pathologic items for myositic IOI. There was agreement among panelists to focus on surgical tissue biopsy results in the diagnosis of nonmyositic IOI and on a trial with systemic corticosteroids in myositic IOI. Panelists agreed that a maximum number of 30 IgG4-positive plasma cells per high-power field in the orbital tissue is compatible with the diagnosis of IOI.

Conclusions and relevance: An international panel of experts endorsed consensus diagnostic criteria of IOI. These criteria define a level of exclusion suggested for diagnosis and include tissue biopsy for lesions not confined to the extraocular muscles. This consensus is a step toward developing guidelines for the management of IOI, which needs to be followed by validation studies of the criteria.

Figure 1.. Flow Diagram of 3-Round Delphi Consensus Method

CDCIOI indicates consensus diagnostic criteria of idiopathic orbital inflammation.

Figure 2.. Development Process for the Expert Panel

Many panelists responded in all 3 Delphi rounds, and some in 2 rounds or in 1 round only.

Figure 3.. Idiopathic Dacryoadenitis

A, A man in his early 40s presented with a 1-month history of tender left upper eyelid swelling. B, Coronal magnetic resonance imaging (MRI). C, Axial MRI. The MRIs demonstrated an idiopathic orbital inflammation (IOI) pattern of a well-defined, enlarged lacrimal gland with homogeneous contrast enhancement. D, Surgical biopsy specimen of the lacrimal gland mass showed IOI features with lymphoplasmacytic infiltration focally organized into a lymphoid follicle with a germinal center. Fibrosis extends into the lacrimal gland with some preserved lacrimal gland (hematoxylin-eosin, original magnification x50).