Intracranial hemorrhage in neonates with unrecognized hemophilia a: A persisting problem

Hemophilia is a rare disorder, and an uncommon cause of intracranial hemorr hage in neonates. We present 2 patients with hemophilia A, who presented wi th massive subdural hemorrhages on day 5 and day 4 postpartum, Both were ta ken urgently to surgery without a diagnosis of hemophilia being established . Neither patient had a family history of hemophilia, and both were born fo llowing difficult deliveries. The activated partial thromboplastin time (AP TT) was normal in patient No. 1 (subsequent factor VIII level 10%). In pati ent No. 2, the APTT was slightly prolonged, but initially interpreted as be ing within the normal range for age (subsequent factor VII level of <1%), P atient 1 rebled, required a second operation, and had a poor outcome. Patie nt 2 was given prophylactic fresh frozen plasma, and made a good recovery. Factor VIII assay should be performed in all term male babies presenting wi th intracranial hemorrhage. in urgent circumstances, prophylactic clotting therapy should be administered during surgery to prevent postoperative blee ding in an undiagnosed hemophiliac. Copyright (C) 2001 S. Karger AG, Basel.