Title : Managing ocular haemorrhage in haemophilia: Key challenges and considerations
Abstract:
Background: Haemophilia is an inherited coagulation disorder characterised by deficiency or dysfunction of clotting factors, predisposing affected individuals to prolonged bleeding following trauma or surgery, as well as spontaneous haemorrhages. Ocular involvement is rare but potentially devastating, with a high risk of irreversible visual loss if not promptly recognised and managed. Effective outcomes rely on early diagnosis and coordinated multidisciplinary care between ophthalmology and haematology services.
Case Presentation: A 59-year-old man presented with a 3-day history of sudden, painless blurring of vision and floaters in the left eye. His medical history was significant for haemophilia A, hypertension, and Charcot–Marie–Tooth disease, with a background of easy bruising. Ocular examination revealed hand-movement vision in the left eye with a grade 1 relative afferent pupillary defect. Fundus examination demonstrated a large submacular haemorrhage. Coagulation studies showed a prolonged activated partial thromboplastin time with normal prothrombin time and international normalised ratio, alongside markedly reduced factor VIII levels. Urgent haematology co-management was initiated. However, owing to limited local availability of clotting factor replacement, the patient required transfer to a tertiary centre. Delay in factor VIII optimisation resulted in disease progression, culminating in no perception of light vision complicated by vitreous and suprachoroidal haemorrhage.
Conclusion: This case highlights the critical impact of delayed access to clotting factor replacement in haemophilia-related ocular haemorrhage. Timely and adequate factor VIII replacement is essential to achieve haemostasis and prevent irreversible visual loss. In resource-limited settings, restricted availability of coagulation factor concentrates remains a major barrier to effective care, leading to preventable morbidity. Strengthening supply chains, expanding government funding, and incorporating rare bleeding disorder therapies into national essential medicines lists are imperative. Addressing these gaps is crucial not only for preserving vision but also for improving overall outcomes and quality of life in patients with haemophilia.
