The authors report on the clinical course of two infants with severe hemophilia A (HA) and concomitant progressive hydrocephalus that required management with a ventriculoperitoneal shunt. The first child, with known HA, presented with a spontaneous intracranial hemorrhage and acquired hydrocephalus. He underwent cerebrospinal fluid diversion with a temporary external ventricular drain, followed by placement of a ventriculoperitoneal shunt. The second child had hydrocephalus secondary to a Dandy-Walker malformation and was diagnosed with severe HA during preoperative evaluation. He underwent placement of a ventriculoperitoneal shunt after progression of the hydrocephalus. The authors also review the treatment of hydrocephalus in patients with HA and describe the perioperative protocols used in their two cases. Treatment of hydrocephalus in infants with HA requires unique perioperative management to avoid complications.
Keywords: DWM = Dandy-Walker malformation; Dandy-Walker; EHL = extended half-life; EVD = external ventricular drain; FVIII = factor VIII; HA = hemophilia A; ICH = intracranial hemorrhage; ICP = intracranial pressure; SDH = subdural hematoma; SHL = standard half-life; VP = ventriculoperitoneal; hemophilia; hydrocephalus; intracranial hemorrhage; rFVIII = recombinant FVIII; ventriculoperitoneal shunt.