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.
Correspondence Annie I. Drapeau: Nationwide Children’s Hospital, The Ohio State University College of Medicine, Columbus, OH. email@example.com.
INCLUDE WHEN CITING Published online November 2, 2018; DOI: 10.3171/2018.8.PEDS18409.
Disclosures Dr. Dunn reports grants, personal fees, and nonfinancial support from Bayer, CSL Behring, Pfizer, Shire, and Hema Biologics; grants and personal fees from Bioverativ; personal fees and nonfinancial support from Medscape; grants from Alnylam and Octapharma; and personal fees and nonfinancial support from Novo Nordisk, during the conduct of the study; and other from Little Seed Inc.
AnderssonNGAuerswaldGBarnesCCarcaoMDunnALFijnvandraatK: Intracranial haemorrhage in children and adolescents with severe haemophilia A or B—the impact of prophylactic treatment. Br J Haematol179:298–3072017
AuerswaldGBidlingmaierCKurnikK: Early prophylaxis/FVIII tolerization regimen that avoids immunological danger signals is still effective in minimizing FVIII inhibitor developments in previously untreated patients—long-term follow-up and continuing experience. Haemophilia18:e18–e202012
BladenMMainEKhairKHubertNKoutoumanouELiesnerR: The incidence, risk and functional outcomes of intracranial haemorrhage in children with inherited bleeding disorders at one haemophilia center. Haemophilia22:556–5632016
FlanneryAMDuhaimeACTamberMSKempJ: Pediatric hydrocephalus: systematic literature review and evidence-based guidelines. Part 3: Endoscopic computer-assisted electromagnetic navigation and ultrasonography as technical adjuvants for shunt placement. J Neurosurg Pediatr14 (Suppl 1):24–292014
KulkarniRPresleyRJLusherJMShapiroADGillJCManco-JohnsonM: Complications of haemophilia in babies (first two years of life): a report from the Centers for Disease Control and Prevention Universal Data Collection System. Haemophilia23:207–2142017
MahlanguJNRagniMGuptaNRangarajanSKlamrothROldenburgJ: Long-acting recombinant factor VIII Fc fusion protein (rFVIIIFc) for perioperative haemostatic management in severe haemophilia A. Thromb Haemost116:1–82016
NelsonMDJrMaederMAUsnerDMitchellWGFenstermacherMJWilsonDA: Prevalence and incidence of intracranial haemorrhage in a population of children with haemophilia. The Hemophilia Growth and Development Study. Haemophilia5:306–3121999
Sasaki-AdamsDElbabaaSKJewellsVCarterLCampbellJWRitterAM: The Dandy-Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes. J Neurosurg Pediatr2:194–1992008
StieltjesNCalvezTDemiguelVTorchetMFBriquelMEFressinaudE: Intracranial haemorrhages in French haemophilia patients (1991–2001): clinical presentation, management and prognosis factors for death. Haemophilia11:452–4582005
WarfBCCampbellJWRiddleE: Initial experience with combined endoscopic third ventriculostomy and choroid plexus cauterization for post-hemorrhagic hydrocephalus of prematurity: the importance of prepontine cistern status and the predictive value of FIESTA MRI imaging. Childs Nerv Syst27:1063–10712011