Cataract formation in young individuals is uncommon and often prompts evaluation for underlying systemic or metabolic disorders. Haemophilia B, a rare X-linked recessive coagulation disorder caused by Factor IX deficiency, is primarily characterized by bleeding manifestations, with ocular involvement being infrequently reported. This case series describes three young male patients with known haemophilia B on long-term desmopressin therapy who presented with bilateral visual impairment and were found to have early-onset cataracts. The patients, aged 18, 30, and 44 years, demonstrated varying lenticular changes, including bilateral complicated cataracts and posterior subcapsular cataracts, with best-corrected visual acuity ranging from 3/60 to 6/60. Two patients exhibited iron deficiency anaemia and coagulation abnormalities, while one had normal activated partial thromboplastin time at presentation. Comprehensive ophthalmologic evaluation confirmed cataractous changes, and two patients successfully underwent phacoemulsification under perioperative Factor IX replacement with uneventful surgical outcomes and significant postoperative visual improvement. The third patient was advised to have surgery but was lost to follow-up. This series highlights the challenges of cataract management in haemophilia, emphasizing the importance of meticulous perioperative planning, multidisciplinary coordination, and adequate coagulation factor coverage to minimize intraocular bleeding risk. The potential contributory roles of iron deficiency anaemia and long-term desmopressin therapy in early cataractogenesis are also discussed. Although a direct causal relationship cannot be established, the occurrence of rapidly progressive bilateral cataracts in young haemophilia patients indicates that it is necessary to conduct routine ophthalmologic surveillance and heightened clinical awareness to prevent avoidable visual morbidity in this vulnerable population.
Shrivastava et al. (Thu,) studied this question.