Autor(es): Martinho, L ; Câmara, L ; Batalha, S ; Rodrigues, J ; Carioca, F ; Marques, J
Data: 2022
Identificador Persistente: http://hdl.handle.net/10400.17/4035
Origem: Repositório do Centro Hospitalar de Lisboa Central, EPE
Assunto(s): CHLC ANS; Adolescent; Hemophilia B; Factor IX; Surgical Procedures, Operative
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 presente a case of a 14-year-old female, with mild hemofilia B (hemofilia 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 multidisciplinar 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.
