Hemophilia presenting with intracranial hemorrhage. An approach to the infant with intracranial bleeding and coagulopathy
G. L. Bray and N. L. Luban
Division of Hematology-Oncology, Children's Hospital National Medical Center, Washington, DC 20010.
Intracranial hemorrhage (ICH) in the newborn or young infant is an uncommon
presenting manifestation of hemophilia. Its occurrence is almost always
preceded by mild-to-moderate head trauma, unlike adult hemophiliacs in whom
ICH occurs without prior head injury in 50% of cases. The bleeding event
may follow a minor complication of labor or delivery (eg, prolonged
second-stage labor or the use of forceps). Recent experience at our
institution, a major tertiary care children's hospital, indicates that the
diagnosis of hemophilia is often overlooked in a young infant presenting
with ICH, a history of perinatal or postnatal head injury, and a prolonged
activated partial thromboplastin time (PTT). Three young infants with
hemophilia (two moderate cases and one severe case) presented with head
trauma and were later found to have factor VIII deficiency. Despite
prolongation of the preoperative PTT in each case, hemophilia was not
considered before neurosurgery was performed. In one case, PTT prolongation
was ascribed to tissue thromboplastin-induced intravascular coagulation.
There was one death secondary to overwhelming intraventricular hemorrhage
and iatrogenic bacterial ventriculitis. Some specific diagnostic and
therapeutic recommendations are provided to assist the clinician in
evaluating a child with ICH and a prolonged PTT who is in need of immediate
neurosurgery.
