An 18-year-old Malay gentleman was noted to have profound bilateral blurred vision for one month duration, associated with loss of weight, appetite, low grade fever and abdominal distension. Visual acuity on presentation was 6/60 on the right, counting finger on the left with no afferent pupillary defect. Anterior segments were unremarkable. Vitreous cells were occasional bilaterally. Fundus revealed multiple choroidal and sub-retinal Roth spots with areas of pre-retinal and intra-retinal haemorrhages, involving the macula in the left eye. Vessels were dilated and tortuous in all quadrants of the right eye. Many areas of capillary fall out at peripheral retina were demonstrated in fundus fluorescein angiogram. Further systemic and laboratory review confirmed the diagnosis of CML and chemotherapy was initiated. Both eye ischaemic retinopathy secondary to CML was confirmed and scatter pan retinal photocoagulation was performed bilaterally. Good improvement in vision noted during subsequent follow up to 6/24 on the right, 6/60 on the left. High levels of suspicion and accurate early recognition of fundus changes are vital in these types of cases to ensure the institution of prompt treatment.
A 21-year-old Chinese gentleman with no known medical illness, presented with a history of right painless blurring of vision with central scotoma of two weeks duration. He also had a history of multiple episodes of seizures prior to presentation. Visual acuity was 1/60 with unremarkable anterior segment findings and no relative afferent pupillary defect. Fundus examination of the right eye revealed dilated and tortuous retinal veins with multiple retinal capillary hemangiomas and sub retinal hard exudates at the macula with edema. A diagnosis of Von Hippel Lindau disease was made when a posterior fossa mass suggestive of hemangioblastoma with obstructive hydrocephalus was seen on computed tomography of the brain. Craniotomy with nodule excision was performed. The retinal capillary hemangiomas were treated with the combination of laser photocoagulation and intravitreal Ranibizumab injections. Visual acuity subsequently improved to 6/36.