A 40 years old Indian woman presented with bilateral acute loss of vision since 10 days. She had history of acute redness and pain in both eyes in the past and was treated as acute anterior uveitis. She had narrow angles in both eyes and bilateral yag peripheral iridectomy was done in the past. She had history of infrequent headaches and reduced hearing in both ears. She neither gave any history of similar ocular complaints in the past nor any treatment for eye problem. She had unremarkable systemic illness and no past history of ocular trauma and ocular surgery. Her visual acuity in the right eye was counting finger, N<6/60 and in the left eye was 1/60, N<6/60. Intra ocular pressure (IOP) was 14 mm Hg in both the eyes. Anterior segment examination showed few old pigments on corneal endothelium and AC cells 1+ and vitreous cells 1+ in both the eyes. Fundus examination showed hyperemic discs with multiple pockets of serous neurosensory detachments in the both the eyes (Fig.1a). Preliminary diagnosis of Vogt-Koyanagi-Harada disease(VKH) was made.
On fundus fluorescein angiography (FFA), there were hypofluorescent dots in early frames followed by multiple areas of pin point leakages and subretinal fluid accumulation in late phases in both the eyes. The disc showed leakage in both the eyes. There was no evidence of capillary nonperfusion in the peripheral retina (Fig.1 b,c,d,e). On optical coherence tomography(OCT)(Cirrus HD-OCT), there was evidence of serous neurosensory detachment with intervening septae and corrugated retinal pigment epithelium in both the eyes (Fig 2 a,b). The sub foveal choroidal thickness was 367μm in right eye and 387μm in left eye.
The patient received 3 doses of intravenous methyl prednisolone (IVMP) 1 gram for 3 consecutive day followed by tapering dose of oral steroids@ 1mg/kg b.w. After 3 days of IVMP, the patient was assessed and as the disc edema subsided, the right eye showed presence of neovascularization of disc (NVD). She was then subjected to an optical coherence tomography angiography (OCTA)(Zeiss Angioplex) which showed an abnormal large vessel rising above the plane of retina, best observed in vitreous plane of OCTA (Fig. 3d). The patient showed marked visual gain and the serous detachments completely resolved in 2 weeks. She was also put on oral azathioprine 50mg twice a day after receiving 2 weeks of oral steroids after baseline liver function tests examination and oral steroids were slowly tapered and stopped after 18 weeks. On her last follow-up, she was receiving azathioprine 50 mg once a day and her visual acuity was 6/9, N8 in right eye and 6/12, N8 in the left eye. The NVD had completely regressed, and documented on OCTA.
A 52 year old Indian man presented with history of blackout in front of left eye on changing posture since 4 months and seeing floaters in the left eye since 10 days. He was a known case of diabetes and hypertension since 4 months and 7 years respectively and was on treatment for the same. He was a diagnosed case of non-alcoholic cirrhosis on treatment with a gastroenterologist. His best corrected visual acuity in both the eyes was 6/6, N6. IOP was 15 mmHg in both the eyes. Anterior segment examination in both the eyes were within the normal limits. Gonioscopy was performed which showed open angles with no evidence of neovascularization. Fundoscopic evaluation in the right eye was normal and left eye showed gross disc neovascularisation with surrounding serous retinal detachments, hypopigmentation around and inferior to optic nerve head with streak of preretinal haemorrhage and some vitreous haemorrhage (Fig. 4b). There was no evidence of any intraretinal haemorrhages, microaneurysms or diabetic and/or hypertensive retinopathy changes. The retinal vessels were of normal calibre and no venous beadings could be seen.
On FFA, the right eye was normal in all phases but the left eye showed areas of early choroidal hypofluorescence in prearterial and arterial phases. There was a mesh of arborizing vessels seen over and around the optic disc which showed hyperfluorescence due to dye leakage from the fragile new vessels. Multiple pin point leaks could be seen scattered around the disc which showed increased leakage and some subretinal pooling in late frames of angiogram (Fig. 4 c,d,e,f). Areas of blocked fluorescence corresponding to preretinal heme were seen inferior to ONH. The retinal vessels did not show any signs of leakage or staining and there were no areas of capillary non-perfusions. No e/o leaking microaneurysms.
OCT through the optic disc showed presence of subretinal hyporeflective space with increase retinal nerve fibre layer suggestive of disc edema and surrounding neurosensory detachment in the left eye (Fig. 5a). OCTA of left eye through the disc showed presence of interwoven thin vascular complex above and around the disc in an arborizing pattern in the vitreous frame (Fig. 6c). OCT and OCTA images of the right eye were normal. The sub foveal choroidal thickness was 373μm in left eye.
He was advised baseline uveitis investigations. His Haemoglobin was 13.9 g/dl, total leucocyte count was 5200 cells/cumm, differential leucocyte count showed neutrophils (60), lymphocytes (36), eosinophils (04), monocytes (0), basophils (0). His contrast enhanced CT scan(CECT) few sub-centimetric bilateral mediastinal lymph nodes with calcified lymph nodes in left hilum. His ESR was 28 mm in first hour and Mantoux test (5Tuberculin Units(TU), 48 hours) was 22mm x 24mm. Serum ACE levels were normal. Fasting blood sugar levels was 127 mg/dl and post prandial was 156 mg/dl and BP was recorded as 136/76 mm Hg.
He was diagnosed as left eye chronic VKH disease with ONH neovascularization. He was advised intravenous methyl prednisolone (IVMP) 1 gm x 3 days for 3 consecutive days after physician clearance followed by slow tapering dose of oral steroids @ 1mg/kg/bw under strict blood sugar monitoring. He received 1 dose of Bevacizumab (1.25mg/0.05ml) and dexamethasone (400 microgram/0.1 ml). We planned to put him on oral Azathioprine for his low grade inflammation but he did not get physician clearance in view of deranged liver enzymes and pre-existing non alcoholic cirrhosis pattern on ultrasonography, so oral steroids were slowly tapered and continued for 24 months. His NVD in left eye regressed completely with treatment was documented on OCTA (Fig. 6d).