Case Report Perioperative Management of a Child with von Willebrand Disease Undergoing Surgical Repair of Craniosynostosis: Looking at Unusual Targets Isabelle Maquoi, MD* Vincent Bonhomme, MD, PhD* Jacques Daniel Born, MD, PhD† Marie-Franc ¸oise Dresse, MD, PhD‡§ Elisabeth Ronge-Collard, MD§ Jean-Marc Minon, MD, PhD§ Pol Hans, MD, PhD* We report the successful management of a craniosynostosis repair in a child with severe Type I von Willebrand disease diagnosed during the preoperative assess- ment and treated by coagulation factor VIII and ristocetin cofactor. Collaboration among the anesthesiologist, the neurosurgeon, the clinical pathologist, and the pediatric hematologist is important for successful management. (Anesth Analg 2009;109:720 –4) The perioperative management of patients undergo- ing surgical repair of craniosynostosis is a challenge for anesthesiologists because of the risk of extensive bleeding. 1,2 We report a pediatric patient with von Willebrand disease (vWD) admitted for surgical cor- rection of craniosynostosis. CASE DESCRIPTION Permission was obtained from the parents to report our observations. A 10-mo, 9-kg male child was admitted to the neurosurgical department for remodeling of sagittal cranio- synostosis. At 1 mo, he had undergone an uneventful repair of an inguinal hernia. He had also been anesthetized 1 wk before admission for a computed tomography scan with three-dimensional reconstruction of his malformation. Ex- cept for the dolichocephalic aspect of the skull, the physical examination was normal. During the preoperative visit, the child’s father mentioned a history of vWD in his own family although he himself had no coagulation disorder. The mother reported that the child had had recurrent knee hematomas since he began to walk. The anesthesiologist ordered a coagulation and hemostasis profile, which showed the following results: a platelet count at 353  10 3 mm -3 , a closure time of Collagen/adenosine diphosphate PFA-100 test (Siemens, see Appendix for details) at 289 s (normal range [NR]: 71–111 s), a closure time of Collagen/Epinephrine PFA-100 test at 223 s (NR: 74 –116 s), an activated partial thromboplastin time (aPTT) at 37 s (NR: 28 – 43 s), a prothrombin time (PT) at 13.4 s with an interna- tional normalized ratio at 1.0 (NR: 1.0 –1.2), a coagulation factor VIII (FVIII) at 53% (NR: 50%–150%), a von Willebrand factor antigen (vWF:Ag) at 17% (NR: 60%–150%), a ristocetin cofactor activity (vWF:RCo) at 9% (NR: 70%–132%), and an A positive blood type. As laboratory results were consistent with a severe Type I vWD, we confirmed the diagnosis by ordering a plasma vWF multimers assay. Neurosurgery was delayed to develop a perioperative care strategy with the pediatric hematologist and the clinical pathologist. Based on our plan, the patient received 250 IU of FVIII and 550 IU of vWF:RCo (Hemate P) IV 1 h before induction of anesthesia. The same dose was repeated twice daily for the first 48 h after surgery and then modified to keep the FVIII plasma level in the 80%–100% range during the first postoperative week. Premedication consisted of intrarectal midazolam (0.4 mg/kg) and atropine (0.125 mg). In the operating room, two venous catheters, an arterial catheter, and standard monitoring were placed. Anesthesia was induced IV with a bolus of 0.2 g/kg sufentanil and 5 mg/kg thiopental. Tracheal intubation was facilitated by rocuronium (0.5 mg/kg). Maintenance of anesthesia was achieved with sevoflurane (2.5%–3% end-tidal) vaporized in an oxygen/nitrous oxide mixture (Fio 2 : 0.5). The patient received 10 mg/kg tranexamic acid IV at the beginning of surgery. Baseline fluid requirement was ensured by continuous infusion of a crystalloid (Plasmalyte-A) at a 4 mL  kg -1  h -1 rate. The surgical intervention was uneventful and lasted 2 1 /2 h. At the beginning of surgery, the hematocrit value was 27.7%. The estimated blood loss was isovolumically re- placed by a colloid (third generation hydroxyethyl starch, From the *University Department of Anesthesia and ICM, †Department of Neurosurgery, ‡University Department of Pediat- rics, §Liege Hemophilia Treatment Center, and Department of Laboratory Medicine, Hemostasis and Thrombosis Unit, CHR Cita- delle, Liege, Belgium. Accepted for publication April 15, 2009. Supported by Department of Anesthesia and ICM of Liege University Hospital, Department of Neurosurgery of CHR Cita- delle, and Department of Laboratory Medicine of CHR Citadelle. Reprints will not be available from the author. Address correspondence to Pol Hans, MD, PhD, University Department of Anesthesia and ICM, Bd du 12eme de Ligne, 1, 4000 Liege, Belgium. Address e-mail to pol.hans@chu.ulg.ac.be. Copyright © 2009 International Anesthesia Research Society DOI: 10.1213/ane.0b013e3181aedbf9 Vol. 109, No. 3, September 2009 720