Table 1 Clinical features of 17 cases of orbital EAF
From: A rare lumpy inflammatory lesion of the orbit: a unique case report
Case | Age | Gender | Laterality | Position | Clinical features | Destruction | Serum IgG4 | Tissue IgG4 | ANCA | Treatment | Prognosis | Follow-up |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
Our case | 55 | Female | Right | Orbital mass | Eyelid swelling | None | Negative | Negative | Negative | Surgery+glucocorticoid | No recurrence | 2 months |
Heedari [5] | 69 | Male | Left | Medial orbital mass | Periorbital edema, nonaxial proptosis and lateral globe displacement, limited abduction | Medial orbital wall, inferior rectus, medial rectus muscles | Negative | Positive, 40–45% | Negative | Surgery+glucocorticoid | No recurrence | 4 months |
Mansfield [6] | 37 | Female | Left | Lateral orbital mass | Periorbital swelling | Scleral thickening | Negative | Positive, < 10% | Negative | Biopsy+ glucocorticoids+ rituximab+ methotrexate / azathioprine | Multiple recurrence | 18 months |
Okuyama [7] | 55 | Female | Bilateral | Upper eyelid conjunctivas | Conjunctival swelling | None | 179 mg/dl | Positive, 45% | – | Surgery | No recurrence | 6 months |
Legare [8] | 58 | Male | Left | Orbit and sinuses illustrated a solid mass | Eyelid swelling | Nasal soft tissue, medial periorbital soft tissue, bony nasal destruction, nasal septum and anterior nasal cavity mucosa | – | Positive | Negative | Surgery+glucocorticoid/ rituximab | Remission | 6 months |
Gorostis [9] | 61 | Male | Right | Ethmoido-orbital mass | Visual loss, pain, proptosis, eyelid oedema, headache, nasal obstruction | Ipsilateral ethmoidal air cells and optic nerve, the periorbital fat, osteolysis of the lamina papyracea, medial oculomotor muscles | > 135 mg/dl | Positive,> 40% | – | Surgery+glucocorticoid/dapsone, Surgery+glucocorticoid/ rituximab | Recurrence (5 years later), remission | 6 years |
Chen [10] | 32 | Female | Left | Upper eyelid lesion | Bleeding, itching, clear discharge, headaches | Bilateral preseptal soft tissue swelling, maxillary sinuses, nasal cavity | Negative | Positive，10% | Negative | Surgery+glucocorticoid/ rituximab | Remission | 6 months |
Faramarzi [11] | 35 | Male | Left | Medial canthal region mass, maxillary sinus mass | Progressive orbital swelling ，left epiphora, proptosis, anterolateral globe displacement, nasal obstruction | Lamina papyracea | – | – | – | Septoplasty, left uncinectomy, middle meatal antrostomy, and anterior ethmoidectomy | No recurrence | 12 months |
Radhakrishnan [12] | 38 | Female | Right | Inferior orbital mass | Limited abduction | Lateral rectus, inferior oblique muscles | – | – | Negative | Surgery+glucocorticoid | No recurrence | 3 months |
Takahashi [13] | 43 | Male | Right | Medial rectus muscle lesion | Visual loss, eyelid swelling | Medial rectus muscle and optic nerve with soft tissue swelling of the ethmoid sinus | Negative | Positive | Negative | Surgery+intravenous methylprednisolone and oral diaminophenyl sulfone，cyclophosphamide | No recurrence | 18 months |
Karligkiotis [14] | 46 | Male | Right | Orbital lesion | Proptosis, lateral globe displacement, right unilateral nasal obstruction, headache | Homolateral ethmoidal cells with oval configuration, right middle meatus, the ethmoid sinus | – | – | Negative | Surgery | Remission | 36 months |
Deshpande [15] | 63 | Male | Bilateral | Nasal and lacrimal glands, lung | Ocular tearing, nasal congestion, anosmia | Nose, middle turbinates | 1490 mg/dl | Positive,80% | Negative | Surgery+glucocorticoid | – | – |
Deshpande [15] | 31 | Female | – | Orbit, maxillary sinus, ethmoid, nasal | – | – | – | Negative | – | – | – | – |
Deshpande [15] | 54 | Female | Right | Lacrimal gland | – | – | – | Positive,97% | – | – | – | – |
Deshpande [15] | 55 | Male | – | Orbit | – | – | – | Positive, 68% | Negative | – | – | – |
Kiratli [16] | 30 | Female | Right | Inferomedial orbit | Ipsilateral epiphora | Anterior ethmoid cells and right middle turbinate | – | – | Negative | Fluorocortolon+ desloratadine+ Steroid | No recurrence | 32 months |
Azam [17] | 35 | Female | Right | Retrobulbar area of right orbit encasing the eyeball | Pain and right lower eyelid swelling | Subcutaneous fat | – | – | Negative | Surgery | No recurrence | 8 months |
Leibovitch [18] | 61 | Male | Right | Medial orbit | Periorbital edema and painless proptosis | Fat, medial orbital wall and middle ethmoidal cells | – | – | Negative | Oral steroids | No remission | 6 months |