What next and what to expect?
A 17-year-old female presented with painless progressive loss of vision in both eyes (OU) with multiple episodes of syncope and postural giddiness for 1 month. On ocular examination, best-corrected visual acuity (BCVA) was 1/60 in the right eye (OD) and finger counting close to the face in the left eye (OS); intraocular pressure was within normal limits in both the eyes. Slit-lamp examination revealed corkscrew conjunctival vessels [Fig. 1a), anterior chamber (AC) cells 1(+), and iris neovascularization. Pupils were mid-dilated and sluggishly reacting to light. Fundus examination revealed hyperemia and neovascularization of the disc, dilated retinal veins with a beaded appearance, patches of retinal whitening over the macula and peripapillary area, and arterio-venous (AV) anastomosis [Fig. 1b and 1c].
What is the Next Step?
- Systemic examination
- Computed tomography (CT)-angiography
- Fundus fluorescein angiography and optical coherence tomography
- All of the above
On systemic examination, the radial artery pulse could not be felt, and the carotid pulsations were felt very feebly on both sides. The pulse rate measured in the right popliteal artery was 80/min. Blood pressure (BP) was not recordable in the upper limbs. BP recorded in the right lower leg was 150/90 mm Hg.
CT angiography revealed severe luminal narrowing involving the left common carotid artery and left subclavian artery at the ostial level, and juxta-ostial narrowing of the right brachiocephalic trunk with multiple enhancing collateral channels noted in the neck [Fig. 1d-f].
Proliferative retinopathy in both eyes was treated with pan-retinal photocoagulation (PRP) and one intravitreal injection of bevacizumab (Avastin) in OS. She received oral prednisolone for the systemic condition. Subsequently, she underwent phacoemulsification with foldable intraocular lens implantation in OU. She had a BCVA of 20/120 in OD and 20/400 in OS at the last follow-up visit.
Diagnosis: Takayasu arteritis (TA) with Takayasu retinopathy
Correct Answer: B
TA is a chronic inflammatory disease affecting the large and medium-sized vessels, predominantly in the aorta and its main branches leading to the arterial wall thickening, fibrosis, stenosis, and thrombus formation resulting in cerebral and ocular ischemia. Ophthalmic features in TA result from either due to the obliteration of arteries causing ischemic ocular changes (hypotensive retinopathy) or due to renal artery stenosis causing uncontrolled hypertension (hypertensive retinopathy). Manifestations due to retinal hypoxia are microaneurysms, AV anastomosis, retinal vein occlusion, proliferative retinopathy, cataract, neovascular glaucoma, ocular ischemic syndrome, ischemic optic neuropathy, optic atrophy, retinal detachment, etc.
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Conflicts of interest
There are no conflicts of interest.
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