| Literature DB >> 33318556 |
Min Joung Lee1,2, Bronwyn E Hamilton3, David Pettersson3, Kimberly Ogle4, Jennifer Murdock4, Roger A Dailey4, John D Ng4, Eric A Steele4, Rohan Verma4, Stephen R Planck4, Tammy M Martin4, Dongseok Choi4,5,6,7, James T Rosenbaum4,8,7.
Abstract
Radiologic orbital imaging provides important information in the diagnosis and management of orbital inflammation. However, the diagnostic value of orbital imaging is not well elucidated. This study aimed to investigate the diagnostic accuracy of orbital imaging to diagnose orbital inflammatory diseases and its ability to detect active inflammation. We collected 75 scans of 52 patients (49 computed tomography (CT) scans; 26 magnetic resonance (MR) imaging scans). Clinical diagnoses included thyroid eye disease (TED) (41 scans, 31 patients), non-specific orbital inflammation (NSOI) (22 scans, 14 patients), sarcoidosis (4 scans, 3 patients), IgG4-related ophthalmic disease (IgG4-ROD) (5 scans, 3 patients), and granulomatosis with polyangiitis (GPA) (3 scans, 1 patient). Two experienced neuroradiologists interpreted the scans, offered a most likely diagnosis, and assessed the activity of inflammation, blinded to clinical findings. The accuracy rate of radiological diagnosis compared to each clinical diagnosis was evaluated. Sensitivity and specificity in detecting active inflammation were analyzed for TED and NSOI. The accuracy rate of radiologic diagnosis was 80.0% for IgG4-ROD, 77.3% for NSOI, and 73.2% for TED. Orbital imaging could not diagnose sarcoidosis. Orbital CT had a sensitivity of 50.0% and a specificity of 75.0% to predict active TED using clinical assessment as the gold standard. The sensitivity/specificity of orbital MR was 83.3/16.7% for the detection of active NSOI. In conclusion, orbital imaging is accurate for the diagnosis of IgG4, NSOI, and TED. Further studies with a large number of cases are needed to confirm this finding, especially with regard to uncommon diseases. Orbital CT showed moderate sensitivity and good specificity for identifying active TED.Entities:
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Year: 2020 PMID: 33318556 PMCID: PMC7736889 DOI: 10.1038/s41598-020-78830-0
Source DB: PubMed Journal: Sci Rep ISSN: 2045-2322 Impact factor: 4.379
Demographics of patients and number of imaging studies.
| Clinical diagnosis | TED | NSOI | Sarcoidosis | IgG4-ROD | GPA |
|---|---|---|---|---|---|
| Number of patients | 31 | 14 | 3 | 3 | 1 |
| Number of patients with orbital biopsy results | 4 | 12 | 1 | 3 | 1 |
Age at first imaging, mean (SD), years | 56.5 (10.6) | 48.1 (17.4) | 56.0 (10.2) | 43.7 (15.5) | 30 |
| Gender (M:F) | 12:19 | 2:12 | 2:1 | 2:1 | 1:0 |
| Number of scans | 41 | 22 | 4 | 5 | 3 |
TED thyroid eye disease, NSOI non-specific orbital inflammation, IgG4-ROD IgG4-related ophthalmic disease, GPA Granulomatosis with polyangiitis.
Figure 1Two consecutive magnetic resonance (MR) scans of 1 patient with thyroid eye disease. (A) FLAIR MR image at initial visit shows enlarged superior rectus in the right orbit with slightly increased signal (white arrow). (B) After 8 months, fat suppressed T1-WI contrast enhanced MR of the same patient demonstrates mildly asymmetric enlargement of all extraocular muscles in both orbits with intense associated enhancement. The radiologic diagnosis for these 2 scans was non-specific orbital inflammation.
Figure 2Representative images of non-specific orbital inflammation (NSOI). (A) Coronal, contrast-enhanced computed tomography (CT) image showed inferonasal diffuse infiltrative mass involving inferior rectus and medial rectus muscles (white arrows). The clinical and radiological diagnosis was NSOI. (B) Fusiform mild enlargement of both lacrimal glands shows isodensity and minimal enhancement on contrast-enhanced CT (white arrows). Preseptal soft tissue swelling is also present. The clinical diagnosis was lacrimal NSOI, compatible with the radiologic diagnosis.
Figure 3Representative images of granulomatosis with polyangiitis (GPA) and IgG4-related ophthalmic disease (IgG4-ROD). (A) Coronal unenhanced computed tomography scan shows diffuse, homogeneous, retrobulbar mass affecting extraconal and intraconal space of the right orbit (black arrow). Note nasal septal destruction and the contiguous sinus involvement with orbital wall and orbital floor destruction, characteristic of GPA (white arrow). The radiologic diagnosis was GPA, compatible with the clinical diagnosis. (B) Asymmetric, bilateral lacrimal gland enlargement is seen on coronal, fat suppressed T1-weighted contrast-enhanced magnetic resonance image. Right infraorbital nerve enlargement (white arrow) is reported as a more specific finding of IgG4-ROD, and the radiologic diagnosis was IgG4-ROD.
Accuracy rate of radiologic diagnosis for various orbital inflammatory diseases.
| Clinical diagnosis | Accuracy rate of radiologic diagnosis (true:false) | Erroneous diagnoses details |
|---|---|---|
| TED n = 41 | 73.2 (30:11) | NSOI: 6, IgG4-ROD: 4, Normal:1 |
| NSOI n = 22 | 77.3 (17:5) | TED:1, IgG4-ROD:1, Sarcoid:1, Normal:1, Infection:1 |
| Sarcoidosis n = 4 | 0 (0:4) | TED:1, NSOI:1, meningioma:2 |
| IgG4-ROD n = 5 | 80 (4:1) | Sarcoid:1 |
| GPA n = 3 | 100 (5:0) | – |
TED thyroid eye disease, NSOI non-specific orbital inflammation, IgG4-ROD IgG4-related ophthalmic disease, GPA Granulomatosis with polyangiitis.
Sensitivity and specificity of the imaging study for assessment of inflammation activity in TED and NSOI.
| TED (n = 41) | Sensitivity (95% CI) | Specificity (95% CI) | |||
|---|---|---|---|---|---|
| Clinically active | Clinically inactive | ||||
| CT (n = 34) | Active | 9 | 4 | 50.0 | 75.0 |
| Inactive | 9 | 12 | (26.7–73.2) | (47.4–91.7) | |
| MR (n = 7) | Active | 7 | 0 | 100 | – |
| Inactive | 0 | 0 | (56.1–100) | ||
TED thyroid eye disease, NSOI non-specific orbital inflammation.
Measurement items for the analysis of orbital diseases on imaging.
| Measurements | |
|---|---|
| Extraocular muscles | Inferior rectus muscle enlargement (> 6.5 mm) |
| Medial rectus muscle enlargement (> 5.1 mm) | |
| Lateral rectus muscle enlargement (> 4.1 mm) | |
| Superior rectus muscle complex enlargement (> 5.2 mm) | |
| Superior oblique muscle enlargement (> 3.2 mm) | |
| Fatty infiltration of extraocular muscles | |
| Lacrimal gland | Lacrimal gland enlargement |
| Orbital fat | Excess orbital fat |
| Orbital fat stranding | |
| Orbital apex and optic nerve | Orbital apex fat effacement grading 0 none; 1 (1–25%); 2 (25–50%); 3 (> 50%) |
| Retrobulbar optic nerve sheath complex (> 7.1 mm) | |
| Waist portion of optic nerve sheath complex (> 5.4 mm) | |
| Proptosis | CT-based exophthalmometry using lateral to medial orbital rims-corneal surface in the axial plane |
| Superior ophthalmic vein | Superior ophthalmic vein enlargement (> 2 mm axial or > 3 mm coronal) |
| Trigeminal nerve | Infraorbital nerve enlargement (> 2.0 mm) |
| Any trigeminal nerve involvement; comment | |
| Sinus | Ipsilateral/adjacent involved sinus disease 0 = none; 1 = trace; 2 = trace to 25%, 3 = 25–50%; 4 = > 50% |
| Signal characteristics | Enhancement pattern |
| Signal intensity on T2-weighted images on magnetic resonance images |