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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+
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
  1. aBrothers
  2. cGy Centigray, EOM Extraocular muscle motility, LP Light perception, OD Right eye, OS left eye, OU Both eyes, RAPD Relative afferent pupillary defect