What is included in perioperative management of patients with factor XIII (FXIII) deficiency undergoing routine surgical procedures?

Updated: Aug 01, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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All elective procedures require proper perioperative management. Note the following:

  • Patients with severe FXIII deficiency require FXIII replacement both preoperatively and postoperatively. Levels of as little as 3-5% may be sufficient to provide adequate hemostasis, and a single dose is sufficient to last several weeks unless excessive blood loss occurs. Serial factor levels must be performed to ensure adequacy of FXIII levels.

  • Procedures such as endoscopies, although considered routine for unaffected individuals, require preprocedural product replacement so that patients do not bleed during or following a needed biopsy. Postbiopsy replacement must continue until the biopsy site has healed.

  • Dental extractions or mucosal procedures can be handled using a single preprocedure dose of FXIII along with Amicar or AMCA. A standardized approach to dental extractions, as has been proposed for patients with hemophilia, may be used in patients with FXIII deficiency. Continuing antifibrinolytics on an outpatient basis for several days after a dental extraction is routine practice, with gradual tapering of dosage.

  • Avoidance of NSAIDs and other platelet-inhibiting drugs perioperatively is essential to minimize bleeding risk. Ice packs and pressure are always useful when feasible.

  • Application of fibrin glue as an ancillary measure is useful in helping control bleeding at surgical sites. Fibrin glue consists of a mixture of fibrinogen, thrombin, and FXIII used to cross-link freshly formed fibrin. Cryoprecipitate also has been used as a source of fibrinogen and FXIII, with the use of bovine thrombin to clot fibrinogen. Some preparations also incorporate antifibrinolytic agents to prevent clot lysis. In particular, fibrin glue has been useful in orthopedic surgery and with surgical procedures in patients with FXIII inhibitors. Bovine thrombin may elicit antibodies.

  • Bleeding from suture holes is a complication in a variety of invasive vascular procedures (surgery, radiologic procedure, coronary angiography). In an experimental porcine vascular graft model, fibrin sealant containing FXIII effectively reduced blood loss and reduced the time to achieve adequate hemostasis more than fibrin alone or thrombin-coated gelatin sponges. [118]

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