Vitreous haemorrhage revealing haemophilia C

Abstract

Aims/Purpose: To report a rare case of vitreous haemorrhage revealing factor XI deficiency in 83‐year‐old man.Results: A 83‐year‐old man presented with 6 months history of painless visual loss in the right eye (OD). There was no prior ophthalmic history, trauma or unusual bleeding tendency. Ophthalmic examination showed a best corrected visual acuity limited to hand motion in OD and 20/40 in the left eye (OS). Slit examination revealed the presence of bilateral grade 3 nuclear cataract. Fundus examination was unremarkable in OS but could not be visualized in OD. B scan ultrasonography revealed vitreous haemorrhage without retinal detachment. Etiological assessment revealed prolonged activated partial thromboplastin time. Further investigations revealed the presence of a hereditary coagulopathy, namely, haemophilia C or factor XI deficiency (factor XI level < 1%). The patient underwent successful cataract extraction and pars plana vitrectomy with silicone oil tamponade without prophylactic treatment. Peri‐operative fundus examination revealed superior branch retinal vein occlusion. Partial visual recovery was noted. No bleeding events were observed during follow‐up.Conclusions: Factor XI deficiency is a rare disorder. Haemorrhagic symptoms are variable since there is no correlation between haemorrhagic risk and factor level. In severely affected patients, cataract extraction and vitrectomy can be performed safely without prophylactic treatment.