Sufiyan Shaikh_SCIENTIFIC POSTER 2015

We report a case of CNVM with RPE degenerative changes with sub RPE detachments in biopsy proven light chain nephropathy in a 33 year old female. A fundus change over a period of 3 years was monitored from the initial vis it. The patient underwent best corrected visual acuity (BCVA), fundus photography and ultrasound in the right eye during the initial visit of presentation. In the follow up visit after 3 years BCVA, fundus photography, optical coherence tomography, electroretinography, autofluorescence were performed in both the eyes. A 33 year old female who was a proven case of Light Chain Deposits Disease since 2009 presented to us with complaints of floaters and metamorphopia in the right eye. Patient had a h/o renal transplant for renal failure due to light chain disease which was diagnosed elsewhere. Patient was on low dose Prednisolone (5 mg) and immunosupressants (mycophenolate, tacrolimus and bortezomib) for the same and was taking clopidogrel tablets for the last 2 weeks after an A-V fistula was made for dialysis. On examination vision was BCVA was 6/6 and N6 OU. Anterior segment was unremarkable. Pupillary reactions were normal. Fundus examination showed pigmentary changes with choroidal elevation in the right eye and pigmentary changes in the left eye. Ultrasound was suggestive of sub retinal hemorrhage. A diagnosis of light chain retinopathy was made. Patient was also advised colour fundus photo, OCT and ERG for documentation but patient was lost for follow up. On systemic evaluation her urine protein electrophoretograph showed the presence of major fraction of albumin and minor fraction of all globulins (Albumin 78.6%, Alpha1 6.4%, Alpha2 4.0%, Beta 6.5%, Gamma 4.5%), Bone marrow trephine biopsy showed hypercellular cellular marrow. Renal biopsy showed mesangial nodular glomerulosclerosis, ischemic and global glomerulosclerosis, Immunofluorescence showed arteriosclerosis with multifocal tubular atrophy that was consistent with light chain nephropathy. The patient revisited the clinic in 2014 with complaint of night vision difficulty and difficulty in driving at night. Her ocular examination was same with fundus examination showed pigmentary changes with RPED in the right eye and pigmentary changes in the left eye with a small area of subretinal fibrosis, these pigmentary changes were hypofluorescent on autofluorescence imaging. Electroretinogram (ERG) recordings were done according to the international society for clinical electrophysiology of vision (ISCEV) guidelines. Full field ERG responses showed delayed and reduced scotopic and photopic responses in both the eyes. Both eye Optical Coherence tomography (OCT) was grossly normal except for a mild foveal thinning fovea (Foveal thickness right eye: 123 microns and left eye: 119 microns) and an bumpy RPE temporal to the fovea. Patient was advised a regular 6 month follow up. Patient however returned back the following month with history of sudden onset distortion of vision and seeing flashes of light in both the eyes more so in the left eye. On examination her visual acuity was reduced to 6/7.5 in the right eye and 6/9 in the left eye. Near vision was N6 in both eyes. Anterior segment was unremarkable. Fundus findings were similar to last visit. OCT both eyes was done to rule out any underlying macular pathology. OCT of the right eye was similar to previous visit (foveal thickness 123 microns). However left eye showed the presence of choroidal neovascularisation with trace subretinal fluid with increase in the foveal thickness (135 microns). Patient was given Injection lucentis for the same and is due for follow up.

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Sufiyan Shaikh