Endodontic management of a patient with Bernard-Soulier syndrome

Bernard-Soulier syndrome is a rare inherited disorder with giant platelets, thrombocytopenia and a prolonged bleeding time. These abnormalities are caused by genetic defects of the glycoprotein Ib/IX/V complex that constitutes the von Willebrand factor receptor on the platelet surface. We are documenting a 30-year-old female patient reported with a chief complaint of swelling in relation to right maxillary canine for 5 days. The primary treatment remains platelet transfusion. Root canal treatment was performed following platelet rich plasma transfusion. As the tooth was necrotic and had a periapical pathology post-operative bleeding was absent. The root canal treatment was uneventful and the patient was asymptomatic. Optimum oral hygiene practices were emphasized to avoid dental diseases.