Posterior Scleritis associated with Orbital Pseudotumor Nikolas London, MD Retina Consultants San Diego Ocular History 34-year-old man with 2 months of headache, progressive proptosis, pain, redness, and decreased vision in his right eye HPI: Pred Forte and scopolamine for NGAU x 4 weeks POHx: none PMH: Mitral valve prolapse Mental illness: self-described “not right in head” Jaw surgery 1994 ALL: mushrooms, mayonnaise, anabolic steroids SH: NC ROS: pan-negative First Presentation VA: bare CF OD, 20/25 OS Pupil: + RAPD OS by reverse IOP: 15 OU Hertel: 5mm proptosis OD SLE OD: 2+ conjunctival injection, 1+ AC and anterior vitreous cell First Presentation Funduscopy large amelanonic mass superior to the optic nerve head causing retinal folds and obscuration of the optic nerve head. First Presentation Fluoresceineangiography Early widefield angiogram of the right eye retinal distortion and folds. later frames: progressive stippled hyperfluorescence of the mass prominent leakage from the optic nerve head First Presentation Fluoresceineangiography Late frame widefield angiogram of the right eye leakage from the mass and optic nerve head inferior peripheral nonperfusion and adjacent vascular leakage. First Presentation US vertical axial B-scan ultrasound thickening of the posterior wall complex with sub-Tenon’s fluid (T-sign) shallow inferior retinal detachment First Presentation periorbital edema erythema mild exotropia and proptosis. First Presentation Imaging of the right orbit 2.7 x 1.8 x 3.3 cm soft tissue mass involving the sclera with deformation of the posterior globe. Pseudotumor orbitae (?) Diagnosis Posterior scleritis Associated to pseudotumor orbitae workup for infectious and inflammatory etiologies sent to Oculoplastics for evaluation to consider biopsy and rule out lymphoma. biopsy was refused because quite risky Laboratory Data Quantiferon gold FTA-ABS RPR ACE C-ANCA P-ANCA X-ANCA ANA CXR ESR CRP Chem-7 CBCD Hgb/Hct negative NR NR 21 negative negative negative negative wnl 25 1.10 wnl mild anemia 12/36 Treatment and Follow-Up after infectious etiologies were ruled out he was started on prednisone (60mg/day, 2 weeks) then reduction by 20 mg/week for 3 weeks, staying on 10 mg/day for several weeks rapid improvement of his symptoms and examination in between 1 week dramatic reduction in periorbital edema, erythema, propsosis, head tilt, and exotropia Follow-Up 1 week after begin of treatment dramatic reduction in size of the subretinal mass residual RPE changes mild horizontal retinal striae in the superior macula. SD OCT: mild inner retinal distortion and subretinal fluid. Imaging: substantially smaller scleral mass with less distortion of the posterior wall of the globe. Final Diagnosis Posterior scleritis associated with idiopathic orbital pseudotumor rapid resolution with oral corticosteroids Conclusion Posterior scleritis is a rare manifestation of orbital pseudotumor Other diagnoses, including tuberculosis, lymphoma, systemic lupus erythematosus, syphilis, and sarcoidosis should be considered