title: Factor V and VIII combined deficiency: clinical perioperative management for tonsillectomy in a child.
abstract: Combined factors V (FV) and VIII (FVIII) deficiency is a rarely seen hereditary coagulation disease. Experience of its management in surgery with a high-risk of bleeding is rare. The interest of this case report is to propose a strategy of perioperative management for such a deficit, but also to recall that a careful preoperative anesthetic evaluation with questioning and physical examination permits to detect unsuspected coagulation disorders and to schedule the preventive treatment. The protocol for the perioperative period consisted of the administration of desmopressin and fresh frozen plasma one hour before surgery. The administration of desmopressin was continued for 48hours. Fresh frozen plasma and tranexamic acid were administered during the first 9 postoperative days. A local bleeding occurred at 8 days (scab coming off) and required systematically a surgical hemostasis and an intensification of the therapeutic protocol. Recombinant plasmatic factor VIII was administered for 7 days together with a daily perfusion of fresh frozen plasma for a total treatment period of 14 days.
authors.affiliation: Service d'anesthésie réanimation 3, pôle d'anesthésie réanimation, CHU de Bordeaux, 33076 Bordeaux, France. Electronic address: romain.lanchon@gmail.com.
authors.forename: R
authors.initials: R
authors.lastname: Lanchon
authors.affiliation: Service d'anesthésie réanimation 3, pôle d'anesthésie réanimation, CHU de Bordeaux, 33076 Bordeaux, France.
authors.forename: F
authors.initials: F
authors.lastname: Robin
authors.affiliation: Service de réanimation pédiatrique, CHU de Bordeaux, 33076 Bordeaux, France.
authors.forename: O
authors.initials: O
authors.lastname: Brissaud
authors.affiliation: Service d'otolaryngologie, CHU de Bordeaux, 33076 Bordeaux, France.
authors.forename: M
authors.initials: M
authors.lastname: Marro
authors.affiliation: Service d'anesthésie réanimation 3, pôle d'anesthésie réanimation, CHU de Bordeaux, 33076 Bordeaux, France; EA4576, maladies rares : génétique et métabolique, université de Bordeaux, 33076 Bordeaux, France.
authors.forename: K
authors.initials: K
authors.lastname: Nouette-Gaulain
mesh_terms: D002675:Child, Preschool; D002944:Circumcision, Male; D005166:Factor V Deficiency / Q000150:complications / Q000628:therapy*; D006467:Hemophilia A / Q000150:complications / Q000628:therapy*; D006487:Hemostasis; D006801:Humans; D007430:Intraoperative Care; D008297:Male; D019990:Perioperative Care; D019106:Postoperative Hemorrhage / Q000628:therapy; D014068:Tonsillectomy / Q000379:methods*
keywords: Amygdalectomie; Anesthesiology; Anesthésie pédiatrique; Anomalies de la coagulation; Blood coagulation disorders; Complications postopératoires; Déficit en facteur V; Déficit en facteur VIII; Hemorrhage; Hémorragie; Otorhinolaryngologic surgical procedures; Perioperative period; Postoperative complications