A Case Report on the Surgical Extraction of a Third Molar in a Patient with Bernard–Soulier Syndrome, an Uncommon Disease

Abstract

We present a case of a patient with Bernard–Soulier syndrome (BSS) who had his impacted third mandibular molar surgically extracted. An uncommon congenital bleeding condition called BSS is inherited in an autosomal recessive fashion. The syndrome, which has an incidence of fewer than 1 in 1,000,000, is characterized by delayed bleeding time, thrombocytopenia, and abnormally large platelets. Even simple surgical procedures become difficult in these patients due to their increased propensity for bleeding. There are no established standards for the management of perioperative bleeding linked to BSS because the condition is uncommon. The regular management of these bleeding episodes involves the delivery of platelets matched to the patient’s human leukocyte antigen, which carries certain challenges. We explain the treatment plan used for a patient with BSS whose impacted third molar was successfully extracted surgically.

Keywords

• bernard–soulier syndrome
• bleeding disorder
• disimpaction
• epsilon aminocaproic acid