A 30 years old middle eastern female, presented to our facility with left eye floaters and blurring of vision for a few days. She didn't have history of any systemic illness. Her BCVA on presentation was 6/6 in the right eye and 6/9 in the left eye. The anterior segment showed variable size KPs at both corneas with + 1 and + 2 anterior chamber cells in both eyes. The posterior segment showed + 1 vitreous cells and significant vitreous condensations with mild peripheral vasculitis (Fig. 1a,1b). She was diagnosed as a case of bilateral intermediate uveitis and advised to start topical steroids. Fundus fluorescein angiography and uveitis workup were requested.
blood workup did not reveal any positive results. ACE, lysozyme, syphilis serology and QuantiFERON TB test were all negative.
Magnetic Resonance Imaging (MRI) showed multiple cerebral white matter spots which were rounded and oval small patchy areas of T2/FLAIR bright signal. Dawson finger appearance was noticed in some lesions. Spinal cord showed intramedullary T2 bright lesion opposite T6-T7 vertebrae with no post contrast enhancement.
On Cerebrospinal fluid (CSF) analysis, oligoclonal bands were noted and cell cytology of CSF showed small mature lymphocytes and Polymorphoneutrophils indicating mild cellular inflammation. NMO-IgG was negative.
Fundus fluorescein angiography (Fig. 1c,1d) showed bilateral diffuse peripheral capillaritis and non occlusive vasculitis involving secondary and tertiary veins with no retinal ischemia and no macular capillary leakage. Hyper fluorescence of the disc was noted in the late phase.
As the patient presented to us at the beggining of Covid-19 pandemic when there were no clear guidelines about the use of systemic steroids and immunosuppressives and she did not have obvious vision threatening pathology, she was observed with topical steroids only for 2 months then oral steroid was started at a dose of 60 mg after her floaters increased, she showed good response to oral steroids and was advised to start azathioprine 50 mg (as steroid sparing agent) to be escalated later to 2.5 mg/kg/day but her neurologist was planning to start interferon beta-1a and advised to stop azathioprine for fear of the synergistic myelosuppression effect of both medications, so azathioprine was stopped.
While she was tapering her oral steroids she presented with right eye sudden drop of vision and was found to have rhegmatogenous retinal detachment reaching the macula with superotemporal retinal dialysis in spite of the almost total improvement of her intraocular inflammation. She underwent pars plana vitrectomy and silicone oil tamponade then silicone oil removal after 5 weeks.
She was followed by her neurologist with a diagnosis of RRMS and started on Interferon beta-1a (44mcg). She had side effects with interferon after 3 months and her follow up MRI showed new demyelinating lesions indicating the non efficient response to interferon so it was stopped and she was planned to be shifted to Rituximab .
After 4 months she developed the same pattern of retinal dialysis in her left eye in spite of previous peripheral laser barricade and clinically inactive intermediate uveitis and underwent the same surgery followed by cataract surgery for both eyes for silicone oil induced cataract.
she was doing fine for 3 months after her surgeries and her oral steroid was tapered over this period and her vision was improved to 6/18 right eye and 6/9 in the left eye (as the detachment in the left eye was detected before involving the macula ).
She presented for her regular follow up and found to have bilateral mild peripapillary subretinal fluid both eyes (Fig. 2 ) so FFA was done and showed multiple pinpoint leakage at the posterior pole ( Fig. 3a, 3b ) after 5 days she developed bilateral multifocal exudative retinal detachment with minimal cells in the anterior chamber and vitreous cavity. Flaremetry readings were 35 right eye and 44 left eye and choroidal thickness was beyond the measurement of Enhanced depth imaging (EDI) OCT (Fig. 4a and 4b). She did not report the usual prodromal signs of VKH, except for mild tinnitus which could be related to MS. The patient was admitted for IV pulse steroid therapy and MRI was done and showed no deterioration of MS lesions and no evidence of meningeal or inner ear enhancement. ICG was performed during her admission and revealed hyperfluorescence of stromal choroidal vessels (Fig. 3c) and multiple hypofluorescent dark dots (Fig. 3d )
She received 5 doses of IV methylprednisolone, 1 gm daily and then shifted to oral steroids and referred to rheumatologist to start an immunosupressive treatment.
The exudative retinal detachment was resolved completely with the treatment and her vision was stabilized on 6/24 right eye and 6/9 left eye. Currently she is doing fine with no signs of active intraocular inflammation on Rituximab and azathioprine.