Haemophilia , a genetic bleeding disorder where blood doesn’t clot properly, is usually hereditary and can be passed down from parent to child. The condition is caused by a lack of, or improper functioning of certain clotting factors. This can lead to uncontrolled bleeding after injuries, or even without a clear trigger.
Why It Matters
One of the patients in the study was a 45-year-old man with moderate haemophilia and no other illness. For ocular surgery, clotting factor levels are recommended at 70% to 80%, with a minimum of 50% in emergencies. Traumatic hyphaema or pooling of blood inside the front chamber of the eye due to an injury is quite common in a child.
The headache was eventually attributed to an intraorbital haemorrhage, by which point the eye was lost. Usually when this gap is discussed, the focus is on the orthopaedic impact on patients – repeated joint bleeds, arthropathy, a young man with the knees of a seventy-year-old. While this is an important aspect of the condition, it leads to lesser conversations on the other effects of haemophilia on the body – the effect on the eyes and vision. This is irreversible when it happens, and can cost patients with undiagnosed haemophilia their sight. The onset can be sudden. He came with a sudden one-sided headache. There was no eye pain and no injury that would have made him consult an ophthalmologist. A common scenario is a patient with undiagnosed haemophilia being taken into an operating theatre in an emergency. The usual pre-operative panel that includes a complete blood count, a chest radiograph and a urinalysis, does not detect a clotting factor deficiency. In such a case, surgery may result in bleeding in the eye after the procedure, with resultant complications. Two more factors make the risk harder to tackle. About a third of people born with haemophilia have no family history at all, so an unremarkable family tree is not reassurance. And mild haemophilia frequently goes undetected until adulthood, surfacing only after a dental extraction, an accident or surgery. The clinical baselines are well established. Blood in the anterior chamber or vitreous is treated as an emergency requiring immediate treatment. None of this is achievable if the diagnosis is unknown, and is difficult if the clotting factors are not readily available.
How It Works
A 2025 review in the Journal of the Association of Physicians of India put prevalence of diagnosed and registered haemophilia cases in India at 0.9 per 1,00,000. However, statistical modelling puts the true disease burden much higher, at around 1,36,000. India has the world’s second-largest haemophilia burden.
Pre-operative Considerations for Bleeding Disorders
To enhance patient safety prior to surgical procedures, it is crucial for pre-operative checklists to include a specific inquiry regarding bleeding history. This should cover prolonged bleeding following tooth extractions, incidents of easy bruising, and heavy menstrual periods. Additionally, when ocular bleeding appears disproportionate to the trauma sustained, an Activated Partial Thromboplastin Time (APTT) test and factor assay are recommended to assess potential underlying bleeding disorders. Furthermore, establishing referral pathways between district eye units and the nearest haemophilia treatment centres is essential for timely management of patients with bleeding concerns.
