Management of hydrocephalus in infants with severe hemophilia A: report of 2 cases

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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.

ABBREVIATIONS DWM = Dandy-Walker malformation; EHL = extended half-life; EVD = external ventricular drain; FVIII = factor VIII; HA = hemophilia A; ICH = intracranial hemorrhage; ICP = intracranial pressure; rFVIII = recombinant FVIII; SDH = subdural hematoma; SHL = standard half-life; VP = ventriculoperitoneal.

Article Information

Correspondence Annie I. Drapeau: Nationwide Children’s Hospital, The Ohio State University College of Medicine, Columbus, OH. annie.drapeau@nationwidechildrens.org.

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.

© AANS, except where prohibited by US copyright law.

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Figures

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    Axial T2-weighted (left) and sagittal FIESTA (right) MR images obtained at presentation, showing intraventricular hemorrhage, moderate ventricular enlargement, and an incidental suprasellar cyst.

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    Limited T2-weighted MR images obtained at 6 months postoperatively, showing an incidentally found Chiari malformation type 1 with cerebellar tonsillar descent to 9.8 mm below the foramen magnum.

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    Preoperative sagittal T2-weighted MR image showing hypoplasia of the cerebellar hemispheres and vermis, a retrocerebellar cyst communicating with the fourth ventricle, and an enlarged posterior fossa. Also observed were hypoplasia of the brainstem and mild ventriculomegaly of the lateral and third ventricles.

  • View in gallery

    Coronal CT scans obtained preoperatively (left) and on postoperative day 1 (right). Note the bilateral subdural bleeds of 5 and 4 mm at the vertex.

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