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Correspondence to Dr Amit Raj; [email protected]
Background
Eisenmenger syndrome (ES) is a common cause of cyanotic congenital heart disease. Individuals with ES often have a ventricular septal defect. This results in significant shunting of blood from left side of the heart to the right at birth. If not repaired in time, continued shunting of blood leads to pulmonary hypertension and shunt reversal. This causes arterial desaturation and central cyanosis. This leads to secondary erythrocytosis, iron deficiency and blood hyperviscosity. Thus, these patients are at risk of thrombosis. Our patient presented with central retinal artery occlusion (CRAO). He was a known case of ES and was on irregular treatment. Thus young patient presenting with CRAO should be evaluated for congenital cyanotic heart disease and ES. Timely diagnosis and management can prevent fatal complications like stroke and myocardial infarction.
Case presentation
A young boy in his 20s presented with sudden diminution of vision in right eye 2 days ago. He was a known case of ES secondary to a large congenital ventricular septal defect. Medical history was notable for prominent pulmonary hypertension and shortness of breath on exertion. There was no symptomatic coronary artery disease, giddiness or syncope. The patient was on tab sildenafil, aspirin and atorvastatin. Ophthalmic examination revealed corrected visual acuity of HM in right eye and 20/20 in left eye. Anterior segment examination was unremarkable. There was no evidence of iris neovascularisation. Intraocular pressure was normal. Funduscopy demonstrated diffuse retinal opacification and swelling at the posterior pole with a cherry red spot at the macula. There was sparing of the superonasal macula with a patent cilioretinal artery (figure 1). Intraretinal haemorrhages or cotton-wool spots were not noted. Optical Coherence Tomography (OCT) showed diffuse inner retinal thickening of the temporal macula (figure 2). Fluorescein angiography showed delayed arterial filling. These findings diagnosed him as CRAO with cilioretinal sparing. Physical examination revealed a harsh systolic murmur. Extremities showed digital erythema and severe clubbing (figure 3). Chest X-ray demonstrated cardiomegaly. Laboratory examination revealed haemoglobin—22 g/dL, haematocrit—60%, serum iron—20 µg/dL.
Differential diagnosis
CRAO can occur in young patients.1 2 The aetiology is multifactorial. The most common cause is hyperhomocysteinaemia.3 Elevated homocysteine and low methionine were found to be the risk factors for vascular occlusions in...