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

Abstract

Hemophilia is a rare disorder, and an uncommon cause of intracranial hemorrhage in neonates. We present 2 patients with hemophilia A, who presented with massive subdural hemorrhages on day 5 and day 4 postpartum. Both were taken urgently to surgery without a diagnosis of hemophilia being established. Neither patient had a family history of hemophilia, and both were born following difficult deliveries. The activated partial thromboplastin time (APTT) was normal in patient No. 1 (subsequent factor VIII level 10%). In patient No. 2, the APTT was slightly prolonged, but initially interpreted as being within the normal range for age (subsequent factor VII level of < 1%). Patient 1 rebled, required a second operation, and had a poor outcome. Patient 2 was given prophylactic fresh frozen plasma, and made a good recovery. Factor VIII assay should be performed in all term male babies presenting with intracranial hemorrhage. In urgent circumstances, prophylactic clotting therapy should be administered during surgery to prevent postoperative bleeding in an undiagnosed hemophiliac.