Intracranial and extracranial hemorrhages in newborns with hemophilia: A review of the literature

Purpose: Intracranial hemorrhage (ICH) and extracranial hemorrhage (ECH) in newborns with hemophilia were reviewed with respect to incidence, anatomic location, clinical presentation, and relationship to the mode of delivery and type of hemophilia. Materials and Methods: A MEDLINE search from 1964 to 1996 of all reports of neonatal hemophilia and head bleeds in children from birth to 1 month old was performed. ICH was defined as any bleed occurring within the cranial ca vity, and ECH was defined as hemorrhage occurring outside of the cranial ca vity, including subgaleal and cephalhematoma. The mode of delivery, type an d severity of hemophilia, and clinical presentation were also noted. Results: One hundred two newborns with hemophilia and cranial bleeds were d escribed in 33 publications. The cumulative incidence of ICH and ECH was 3. 58% in 5 studies that reported the total newborn population or that examine d birth records. The type of hemophilia was reported for 40 of 102 newborns and was hemophilia A in 87%, The mode of delivery was recorded in 46% (47 of 102) of the patients. Of these, 13% had cesarean delivery, and 87% were delivered vaginally (40% had spontaneous vaginal delivery, and 47% had vagi nal delivery with vacuum extraction or forceps). There were 109 episodes of ICH and ECH (65% were ICH, 35% were ECH, and 5.8% were a combination of bo th). Common clinical features of ICH and ECH included anemia, hypotension, shock, and lethargy. However, only patients with ICH were reported to have neurologic deficits (15%) and late neurologic sequelae (38%). Conclusion: In neonates with hemophilia and cranial bleeds, ICH occurs more often and is often associated with late neurologic deficits.