Review of Inherited Coagulation Disorders

Abstract

Most invasive dental procedures elicit some degree of bleeding which ultimately leads to clotting and eventual hemostasis. However, patients with inherited coagulation disorders may exhibit prolonged or, in some cases, excessive bleeding requiring multiple perioperative interventions. Von Willebrand disease is the most common inherited coagulopathy and often manifests via easy bruising, epistaxis, or prolonged bleeding. Hemophilia A (factor VII) and B (factor IX) are factor deficiencies that are clinically indistinguishable and managed according to severity and the required dental treatment. Other coagulopathies are rare (ie, inheritance is autosomal recessive) and may only become evident in homozygotes or compound heterozygotes. Current lab values and medical consultation with the patient's hematologist are imperative prior to rendering invasive dental treatment. There are a myriad of sedation and general anesthesia considerations, including risks for epistaxis with nasal instrumentation and bruising with improper patient positioning. Preoperative treatment with desmopressin or factor replacement may be required and generally should facilitate normal hemostasis. Additional therapies should be considered to help ensure adequate postoperative hemostasis, including pressure dressings, resorbable clotting materials, laser therapy, and oral rinses.

Keywords: Anesthesia considerations; Clotting cascade; Dental considerations; Factor deficiencies; Hemophilia; Von Willebrand disease.

Figure.

Coagulation Cascade The classic coagulation cascade model of hemostasis consisting of the intrinsic, extrinsic, and common pathways.