Heterochromia Iridum with Sudden Vision Loss in an Elderly Man - A Trick to Treat.

Dear Editor,

Acquired heterochromia iridum usually occurs in Fuchs heterochromic iridocyclitis, trauma with long-standing hyphema, ciliary body and iris tumors, iatrogenic due to certain medications, syndrome associations in Parry–Romberg syndrome, iridocorneal endothelial syndrome, and Horner's syndrome.[1] The association of heterochromia iridum in eyes with spontaneous suprachoroidal hemorrhage has been poorly described in the literature. We describe the case of an 84-year-old male who presented with sudden severe visual loss and abnormal discoloration in one eye.

An 84-year-old male, a known hypertensive and carotid arterial disease on oral anticoagulants, presented with sudden, painful severe vision loss and abnormal discoloration in the right eye for 4-day duration. He had no history of recent trauma, Valsalva or intraocular surgery, glaucoma, diabetic retinopathy, and age-related macular degeneration in any eye. On examination, visual acuity was no light perception, intraocular pressure (IOP) was 48 mmHg, iris showed heterochromia iridum, the pupil was mid-dilated not reacting to light, and the posterior segment was suggestive of dense vitreous hemorrhage [Figure 1]. The left eye was pseudophakic with normal anterior and posterior segments. Ultrasonography B-scan of the right eye showed hyperechoic suprachoroidal fluid accumulation with overlying choroidal detachment [Figure 2a]. The differential diagnoses which were considered in this case scenario were as follows: choroidal melanoma, exudation from distant metastases, malignant hypertension, ocular ischemic syndrome, and caroticocavernous fistula. Contrast-enhanced computed tomography scan of the brain and orbits were not suggestive of cerebral metastases or caroticocavernous fistula. Coagulation profile showed raised prothrombin time (68 s) and deranged international normalized ratio (INR) (7.0), and carotid artery Doppler scan was suggestive of 90% stenosis.

Figure 1

External photograph showing heterochromia iridum at presentation

Figure 2

Right eye ultrasonography B-scan showing (a) hemorrhagic choroidal detachment suggestive of kissing choroidals at presentation, (b) resolution of choroidal detachment and clearing of vitreous hemorrhage after 2 weeks

With a provisional diagnosis of spontaneous suprachoroidal hemorrhage, we decided to withhold his anticoagulant therapy and start conservative treatment with watchful observation. Surgical drainage was deferred due to deranged INR and anticipated expulsive bleeding on sclerotomy, moreover to avoid chances of rebleeding. At 2-week follow-up, he had responded well to topical medications. His pain was relieved, IOP was controlled, the choroidal detachment had settled with slow clearing of vitreous hemorrhage, but there was no improvement in vision [Figure 2b]. The heterochromia resolved at 6-month follow-up while the eye became prephthisical but asymptomatic. There had been neither recurrence of bleeding nor any similar episode in the fellow eye during this period.

Heterochromia iridum, i.e., abnormal discoloration of iris, can be a subtle sign but a harbinger of many grave ocular conditions in the elderly, like suprachoroidal hemorrhage which can be rarely spontaneous without any preexisting ocular pathology. It has been described in patients with or without preexisting retinal pathologies such as high myopia, proliferative diabetic retinopathy, and age-related macular degeneration.[2] Systemic risk factors include carotid artery disease, chronic renal failure, diabetes mellitus, systemic hypertension, use of oral anticoagulants, and thrombolytic.[3] The successful management of the suprachoroidal hemorrhage often leads to the resolution of heterochromia. The presentation of suprachoroidal hemorrhage as heterochromia iridum has not been reported before, and this case reveals the importance of such a finding in elderly patients who are on anticoagulation therapy.

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