Table 1 Demographics and clinical features/intervention of 8 patients (11 eyes) with ophthalmic RDD
From: Ophthalmic Rosai–Dorfman disease: a multi-centre comprehensive study
Case no. | Age (years) | Gender | Laterality | Visual acuity | Symptoms/signs | Duration of complaints (years) | Relevant Systemic involvement | Orbital localization | Intervention |
|---|---|---|---|---|---|---|---|---|---|
1a | 25–29.9 | Male | Bilateral | OD 20/30 OS 20/200 | Decreased vision OU, proptosis OU, RAPD OS | 8 | Bilateral paranasal sinuses infiltration | Intraconal & extraconal in both orbits, secondary optic neuropathy in both eyes | Debulking of masses in both orbits & oral prednisolone, chemotherapy |
2a | Male | Bilateral | OD LP OS 20/100 | Decreased vision in OD more than OS, left lower eyelid swelling, proptosis OU, RAPD OD | OD 1 year OS 6 years | Isolated brain and facial tumors, bilateral paranasal sinuses infiltration | Intraconal & extraconal in both orbits, right intracanalicular optic nerve compression | Debulking of masse in the left orbit & oral prednisolone, chemotherapy | |
3 | 30–34.9 | Male | Left | 20/25 | Left lower eyelid swelling, proptosis | 1.5 | Bilateral submandibular glands enlargement | Diffuse involvement of extraconal and intraconal spaces | Orbital debulking, submandibular gland removal, oral prednisone |
4 | Female | Left | 20/25 | Left upper and lower eyelid swelling, dystopia, proptosis, inferonasal orbital mass | 3 | Bilateral maxillary and ethmoidal lesions | Inferior, extraconal | Oral prednisone+ Radiotherapy 4000 cGy | |
5 | 40’s | Male | Left | 20/40 | Elevated temporal scleral lesion manifesting as nodular scleritis | 0.16 | Thickening of the skin of the auricle and subcutaneous nodules of the arm | – | Subconjunctival and oral steroids |
6 | 70–74.9 | Female | Bilateral | 20/100 OU (Bilateral cataract) | Right upper eyelid swelling, enlargement of right lacrimal gland causing dystopia | 1 | Intracranial/dural lesions | Right supero-temporal and left inferotemporal, extraconal. Enlargement V2 and V3 branches | Oral prednisone |
7 | Female | Right | 20/50 | Right lower eyelid swelling, proptosis, mild limitation of EOM | 6 | Bilateral maxillary sinus infiltration and brain extension | Inferior, extraconal | Radiotherapy & oral prednisolone | |
8 | Female | Left | 20/30 | Left upper eyelid swelling, dystopia, ptosis | 0.5 | Bilateral submandibular glands enlargement | Supero-temporal, extraconal | Oral prednisone |