Perioperative management of a girl with hemophilia B undergoing dorsolumbar spine posterior instrumentation
DOI:
https://doi.org/10.25751/rspa.25544Keywords:
Hemophilia B, Major surgery, Factor IX, Case reportAbstract
Hemophilia B is a coagulation disorder characterized by a deficiency of clotting factor IX. Women are often heterozygous carriers of the disease, however if their clotting factor levels are less than 60%, they may have an increased bleeding tendency. This is even higher if levels are under 40%. We present a case of a 14-year-old female, with mild hemophilia B (hemophilia B carrier with factor IX level < 40%) who underwent a major surgery: a posterior spinal instrumentation from D6 to L1. The perioperative management was discussed, including the perioperative administration of blood products and coagulation adjuncts. This was coordinated by a multidisciplinary team (orthopedists, anesthesiologists, hematologists and nurses) to provide the best perioperative care and follow-up. Close collaboration and communication among/with the team members and the patient/family was vital throughout.
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Copyright (c) 2022 Liliana Martinho, Luisa Perry da Câmara, Sara Batalha, Joana Rodrigues, Filipa Carioca, Joana Marques
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