{
  "abstract": "Background and Aims Inherited Factor XI deficiency, also known as Rosenthal syndrome or Hemophilia C, is a rare disease caused by variants in the FXI gene, resulting in impairment of the intrinsic pathway of blood clotting and increased bleeding during surgery. Although severe deficiency is rare, it has significant anaesthetic implications.Methods The authors describe the perioperative management of a 80-year-old female patient with severe hereditary Factor XI deficiency proposed for elective total knee arthroplasty. Considering significant hemorrhagic dyscrasia during a previous orthopedic surgery and the patient‘s hematological status, a multidisciplinary team discussion was conducted, with administration of fresh frozen plasma the day before the procedure. On the day of surgery, intravenous general anaesthesia combined with ultrasound-guided continuous adductor canal block was performed, with bolus administration of ropivacaine 0,75%. Multimodal analgesia incorporated intravenous acetaminophen and metamizole. Hemodynamic monitoring included the placement of an arterial line, along with three wide-bore peripheral intravenous catheters. Additionally, tranexamic acid and fresh frozen plasma were administered during surgery guided by thromboelastogram.Results The procedure was uneventful with an estimated blood loss of 200 ml, as well as the postoperative recovery, with no hemorrhagic dyscrasia being reported. The patient was successfully discharged ten days after the surgery, with consistently low visual analog scale scores and with no rescue analgesia other than acetaminophen, metamizole, and perineural ropivacaine 0,2%.Abstract P144 Figure 1Ultrasound-guided continuous adductor canal blockAbstract P144 Figure 2Continuous adductor canal blockConclusions Perioperative multidisciplinary management of patients with severe Factor XI deficiency is essential for optimizing the patient‘s hematological status, choosing anaesthetic techniques with a lower risk of complications, and ensuring clinical monitoring in the postoperative period.",
  "authors": [
    {
      "affiliations": [
        "Anestesiologia, Unidade Local de Saúde de Santa Maria, Lisbon, Portugal"
      ],
      "name": "Leonor Ladeira Rodrigues"
    },
    {
      "affiliations": [
        "Anestesiologia, Unidade Local de Saúde de Santa Maria, Lisbon, Portugal"
      ],
      "name": "Alice Nunes Carvalho"
    },
    {
      "affiliations": [
        "Anestesiologia, Unidade Local de Saúde de Santa Maria, Lisbon, Portugal"
      ],
      "name": "Joana Beisl Ramos"
    }
  ],
  "title": "P144 Anaesthesia management of a patient with inherited factor XI deficiency: a case report",
  "uid": "1f0d70da-6206-5d49-97b2-695050e6cbf0"
}