Clinical Spectrum of Intracranial Bleeds in Patients with Bleeding Disorders- Experience from a Tertiary Center in Karnataka.

ABSTRACT

The most frequent emergency event in haemophilia and other bleeding disorders is intracranial haemorrhage (ICH), most of which is caused by trivial trauma, affecting around 3–10%. We studied the spectrum of presentation of ICH at our institute.METHODSThis was retrospective analysis of all patients with diagnosed or suspected bleeding diathesis and ICH presenting at the Clinical Haematology Department, BMCRI. They underwent complete coagulation workup, inhibitor screening, plain CT- brain and other relevant investigations.RESULTS3.26% of patients with bleeding disorders presented with ICH of over 2 years. MF ratio was 131. 78.6% had Haemophilia A, 7.1% each had Haemophilia B, Factor XIII deficiency and Hypofibrinogenemia. 13 (92.9%) had severe Haemophilia A or B and 1 (7.1%) had mild Haemophilia B. Their ages ranged from 2 - 69 years. 50% were adults with the oldest being 69 years. Out of 7 children with Intracranial bleeds, 5 (71.4%) were <5 years. 2 (18.2%) with Haemophilia A had inhibitors. None were hypertensive. There was recent history of trauma in 9 (64.3%). All (100%) had severe headache, 21.4% had vomiting and 28.6% had seizures at presentation. Subdural haemorrhage was seen in 9 (64.3%), subarachnoid haemorrhage in 1 (7.1%), epidural bleed in 1 (7.1%), and intraparenchymal bleed in 3 (21.4%). All patients received appropriate factor replacement and supportive treatment. 9 (64.3%) received factor replacement only, 5 (35.7%) with SDH required additional burr hole evacuation. 2 (14.3%) patients died and 12 (85.7%) responded to treatment. None had sequelae in this series.CONCLUSIONS3.26% of our cohort presented with intracranial bleed over a period of two years. Majority with ICH were Haemophilia A and had severe deficiency with history of trauma. Headache is the commonest presentation and astute and keen clinical suspicion is paramount for instituting early treatment. SDH was most common in our series and majority responded to conservative management with factors.