Invasive procedures in the oral cavity of individuals with Bernard–Soulier syndrome: An integrative review

Abstract

Bernard-Soulier syndrome (BSS) is an inherited bleeding disorder characterized by macroplatelets and thrombocytopenia, prolonged bleeding time, and a prevalence of less than 1 in 1,000,000. In view of the recognition of the risk of bleeding and the management of daily surgical practice in these patients, adequate strategies are necessary to provide the safest care. This article aims to perform an integrative review of the literature on the management of invasive procedures in the oral cavity of individuals with BSS. Method: The PubMed/Medline and LILACS databases were searched using Boolean operators related to BSS, bleeding disorders, and oral care. Results: As a result, only five articles with the main theme were included: one letter to the editor and four case reports, described chronologically as to date of publication, classification of the article, and medical/odontological measures taken. Conclusion:We conclude with this review the need for adequate knowledge of surgeons regarding coagulation disorders and the need to discuss and plan procedures with the hematology team, as well as the importance of the notion of management of possible complications resulting from invasive treatments in the oral cavity of patients with BSS.

Manejo cirúrgico em paciente com síndrome de Bernard-Soulier: relato de caso

“…Due to the rarity of the disorder, there are no well-defined protocols for the treatment 9,10 . However, antifibrinolytic therapies, platelet transfusions and hormone therapy have been used for prophylaxis in patients with BSS, the latter in women 8 .…”