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 periorbital edema erythema erythema mild exotropia and proptosis. mild exotropia and proptosis.

First Presentation Imaging of the right orbit Imaging of the right orbit 2.7 x 1.8 x 3.3 cm soft tissue mass 2.7 x 1.8 x 3.3 cm soft tissue mass involving the sclera with deformation of the posterior globe. involving the sclera with deformation of the posterior globe. Pseudotumor orbitae (?) 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 goldnegative FTA-ABSNR RPRNR ACE21 C-ANCAnegative P-ANCAnegative X-ANCAnegative ANAnegative CXRwnl ESR25 CRP1.10 Chem-7wnl CBCDmild anemia Hgb/Hct12/36

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 dramatic reduction in size of the subretinal mass residual RPE changes residual RPE changes mild horizontal retinal striae in the superior macula. mild horizontal retinal striae in the superior macula. SD OCT: mild inner retinal distortion and subretinal fluid. SD OCT: mild inner retinal distortion and subretinal fluid. Imaging: substantially smaller scleral mass with less distortion of the posterior wall of the globe. 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