Diagnostic and therapeutic challenges of intracranial hemorrhage in neonates with congenital hemophilia: a case report and review

Abstract

Intracranial hemorrhage (ICH) in full-term neonates with hemophilia is uncommon. Retrospective studies estimate the incidence to be 3.4%-4.0%. However, ICH is the second most common initial hemorrhage for all infants with congenital hemophilia. Nearly half (41%) of the hemorrhages occur within the first week of life, and most hemorrhages are related to traumatic deliveries. But clinical signs of ICH in neonates are difficult to assess and often result in a delay in the diagnosis. The timely diagnosis of ICH is critical; however, the early and intensive factor replacement therapy that the patient needs is associated with the risk of inhibitor formation. We report a case of ICH in a neonate with severe hemophilia A, no family history, and inhibitor development after intensive therapy.

Keywords: Hemophilia; intracranial hemorrhage; neonatology.

Figure 1.

Possible clinical signs in neonates with intracranial hemorrhage (ICH). ^†Hypotension, tense fontanelle, pupillary/ocular changes, and apnea. *The most frequent first documented symptoms among children aged <2 years; often nonspecific.

Figure 2.

T1-weighted axial magnetic resonance image of the brain, indicating a right cerebellar subdural hematoma, bilateral superficial cerebellar parenchymal hemorrhage, and foramen magnum hemorrhage.